DERMATOPATHOLOGY CASES: Self-Assessment Cases: Editor - Dr Sampurna Roy MD

Digital Images of interesting cases that will include the full spectrum of Dermatopathology, presented in the form of quiz.

The answer of the cases include related links and recent abstracts of articles.








Wednesday, December 30, 2009

Answer of Dermatopathology Case 28



Molluscum Contagiosum

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Abstract:

Mollusca contagiosa. From paediatric dermatology to sexually transmitted infection.Hautarzt. 2009 Jun;60(6):472-6.
Molluscum contagiosum (MC) is a common cutaneous infection caused by the molluscipox virus (MCV) and can affect both children and adults. Molluscum contagiosum is relatively frequent in children aged 1-5 years old and can be localized almost anywhere on the body, but in adults it is regarded as a sexually transmitted infection (STI). MCV can be transmitted directly from person to person or by autoinoculation. MC in adults characteristically involves the genital area but extragenital appearance can be more typically seen in patients with immunosuppressive conditions, especially in HIV/AIDS. The onset of MC inHIV-positive individuals can be regarded as a part of the immune reconstitution inflammatory syndrome (IRIS). MC probably affects both sexes equally in children, whereas it seems that in adults the incidence is more prevalent in males. Therapy is controversial but may be considerably beneficial in preventing transmission or autoinoculation. At present there is no aetiological treatment of MC and most treatment options are mechanical sometimes causing discomfort or are not sufficiently evidence-based. Attention should be given to the extragenital site of involvement in adults and HIV testing should be recommended. Both children and adults with MC should be educated to avoid scratching and skin contact with others to prevent transmission and autoinoculation. Adult patients with MC shouldbe carefully screened for other STIs and appropriately counseled.

Molluscum contagiosum and dental caries: A pertinent combination. J Indian Soc Pedod Prev Dent. 2009 Oct-Dec;27(4):197-201.
Background: In recent decades, there has been a tremendous surge of interest inissues related to child health. The present study was carried out to reveal theDMFT/deft status of children in the age group of 1-12 years, residing inorphanages from Pune, India. Aims and Objectives: To explore the DMFT/deft status in various orphanages from Pune (India). To find out differences, if any, betweenthe DMFT/deft status in orphanages from Pune, India, and the general population of children below 12 years of age in Maharashtra state, India as well as all over India. Results: DMFT/deft of the orphanages was found to be 2.58 in the age group1-5 years and 2.5 in the age group 6-12 years with caries prevalence in 70.27% of the children. An incidental finding of Molluscum contagiosum was observed in asignificant percentage of children. The total percentage of children afflicted by Molluscum contagiosum was found to be 24.32%. Conclusion: The higher incidence of Molluscum contagiosum in children residing in orphanages as compared to thenormal population of the same age group was reflected in the higher incidence of DMFT/deft in orphanages as compared to the normal population.

Molluscum contagiosum: Descriptive study. An Pediatr (Barc). 2009 Oct 30.
INTRODUCTION: Molluscum contagiosum is a cutaneous viral infection that often requires assistance. The aim of our study is to review the cases admitted in our clinic, evaluate the epidemiological features and the treatment prescribed. PATIENTS AND METHODS: We conducted a descriptive survey of the patients diagnosed of molluscum contagiosum in our clinic (Complexo Hospitalario Arquitecto Marcide-Novoa Santos, Ferrol, Spain) between June 16th 2008 and January 15th 2009. The following dates were recorded in all cases: age, sex, personal history of atopy, swimming pool attendance, number of lesions and treatment prescribed.RESULTS: 140 cases of molluscum contagiosum were included in the study. Average age was 10.7 years. 51.43% of patients had a personal history of atopy and 72.1% used to attendance swimming-pool. Average number of lesions was 13.3, with a higher number of them in males, atopic and swimming-pool attendants. Curettage was the treatment performed in 86.4% of cases. CONCLUSIONS: Atopic dermatitis and swimming-pool attendance were associated in our study with a higher frequency andnumber of molluscum contagiosum. Although different therapeutic options must be evaluated depending on the patient and clinical skills, curettage is the most frequent treatment performed by dermatologists.

Molluscum contagiosum: immunomorphological aspects of keratinocytes markers of differentiation and adhesion. J Cutan Pathol. 2009 Dec;36(12):1279-85.
BACKGROUND: Molluscum contagiosum (MC) is a Molluscipox virus infection of keratinocytes with hyperplasia and intracytoplasmic inclusions - the molluscumbodies (MBs). Few papers address cytokeratins (K) profile in MC, mainly focusing terminal keratinization process. METHODS: Forty-one MC lesions were subjected to immunohistochemical technique to verify K1, K10, K14, K16, involucrin, filaggrin,E-cadherin and p63 expression. MC immunolabeling pattern was compared to adjacent normal appearing epidermis (ANAE). RESULTS: In MC and ANAE, K1/K10 were expressedin suprabasal layers, K14 was expressed in basal and suprabasal layers and K16 was expressed through all spinous layer. Involucrin and filaggrin were observed in granular, spinous and in basal layer of ANAE and MC. E-cadherin was present upto the first layers of MC while ANAE exhibited E-cadherin labeling at basal andspinous layers. Basal and spinous layers keratinocytes nuclei, in both MC and ANAE, express p63. CONCLUSION: Infection by Molluscipox virus alters keratinocyte differentiation status. The presence of K14 and p63 in spinous layer, as well as early expression of involucrin and filaggrin, associated to a hyperproliferative state disclosed by K16 expression, may be a result of disruption in keratinocytes maturation process. The changes observed at ANAE may represent early events in keratinization disturbance.

Dermatopathology Case 28

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Case 28

A 17 year old boy with a pearly white papule on the forehead.

Tuesday, December 29, 2009

Answer of Dermatopathology Case 27


Sebaceoma

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Abstract:

Sebaceous lesions and their associated syndromes: part I. J Am Acad Dermatol. 2009 Oct;61(4):549-60; quiz 561-2.
Sebaceous neoplasms have long been a source of confusion to dermatologists and pathologists alike. Disagreements regarding nomenclature, classification, and management have been longstanding. Sebaceous lesions represent a broad spectrum of interesting entities that range from hamartomas, hyperplasias, and benign tumors to highly malignant neoplasms. This article discusses the clinical and pathologic features of sebaceous hyperplasia, nevus sebaceous of Jadassohn, sebaceous adenoma, seboacanthoma, sebaceous epithelioma, sebaceoma, mantleoma, basal cell carcinoma with sebaceous differentiation, sebomatricoma(sebomatrixoma), and sebaceous carcinoma. Controversies regarding these lesionswill be explored, and any relationship with Muir-Torre syndrome will be discussed.

Rippled-pattern sebaceoma: a clinicopathological study. Am J Dermatopathol. 2009 Jun;31(4):364-6.
We summarized the clinicopathological data of rippled-pattern sebaceoma diagnosed at Sapporo Institute for Dermatopathology and compared it with those of sebaceoma without rippled pattern. Eighty cases of sebaceoma, comprising 37 male and 43 female patients with a mean age at resection of 62.9 +/- 17.0 years, were reviewed. The lesions were located most frequently on the face (45.0%).Twenty-one (26.3%) of 80 cases of sebaceoma exhibited a rippled pattern. Rippled-pattern sebaceoma arose predominantly in males and most frequently on the scalp, whereas sebaceoma without rippled pattern occurred more frequently infemales and on the face. Histopathologically, sebaceoma without rippled pattern frequently associated with other neoplastic lesions including sebaceous nevus, seborrheic keratosis, and trichoblastoma on the same lesion; however, there were no associated lesions in rippled-pattern sebaceoma.

A case of sebaceoma with extensive apocrine differentiation.Am J Dermatopathol. 2008 Aug;30(4):408-11.
Apocrine differentiation is a rare event in sebaceoma, and only 3 cases have been reported. We report a case of sebaceoma with extensive apocrine differentiationon the scalp in a 73-year-old Japanese woman. The resected tumor was located entirely within the dermis and subcutis as a well-circumscribed, lobulated, solid, and partially cystic mass, measuring 35 mm at the largest diameter. Histopathologically, it was composed of uniform basaloid cells with clusters of sebocytes, squamous islands of ductal structures, and apocrine cells with apparent decapitation secretion. Nuclear atypia of all types of cells was inconspicuous, and mitotic figures were infrequent. We considered the lesion tobe a sebaceoma with apocrine differentiation.

Sebaceoma of the external ear canal: an unusual location. Case report and review of the literature. J Cutan Pathol. 2008 Oct;35(10):963-6.
Sebaceous neoplasms of the external ear canal are extremely rare. Only two cases of sebaceous neoplasms have been reported in the English literature, a sebaceous carcinoma and a sebaceous adenoma. We report a case of sebaceoma of the external ear canal. To the best of our knowledge, sebaceoma of the auditory canal has not been reported previously. We highlight the differential diagnosis, particularly sebaceous carcinoma and basal cell carcinoma with sebaceous differentiation. Awareness of the possible occurrence of sebaceoma in the auditory canal mayprevent the diagnostic pitfall of misidentifying this tumor.

Dermatopathology Case 27

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Case 27

A 40 year old male with a solitary nodule (2 cm in diameter) on the left side of the face.

Answer of Dermatopathology Case 26


Glomangioma

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Abstract:

Sclerotic Glomus Tumor. Am J Dermatopathol. 2009 Oct 30.
We report an unusual histopathological variant of a glomus tumor that arose in a peculiar topographic site, a sclerotic glomus tumor. Unlike conventional glomus tumors or glomangiomas that have a loose fibrous stroma with variable hyaline and myxoid changes, the case reported herein had a diffuse, hyalinized, sclerotic stroma. A further difference was that the majority of glomus tumors and glomangiomas occur in the subungual area, trunk, or extremities, whereas the present tumor occurred on the ear. Due to the peculiar histological features and location, other tumors were considered in the differential diagnosis to include Merkel cell carcinoma, primitive neuroectodermal tumor, and small cell melanoma. This article illustrates a unique variant of a glomus tumor, which to our knowledge has not been previously described.

Localized multiple glomangiomas on the foot. J Dermatol. 2009 Nov;36(11):604-7.
We report a 15-year-old Japanese male with multiple, soft, blue and painlessnodules on the left foot. The lesions had developed when the boy was 3 years old, and had enlarged gradually thereafter. None of his family members had any similar eruptions. All the lesions were resected under local anesthesia. Histological examination revealed cystically dilated spaces lined by endothelial cells and a few outer layers of glomus cells in the dermis and fat tissue. Immunohistochemistry demonstrated that the tumor cells were positive for alpha-smooth muscle actin. We diagnosed this case as localized multiple glomangioma. Multiple glomus tumors are much less common than solitary ones, and localized multiple glomus tumors are extremely rare. Because the clinical differential diagnosis of multiple glomangiomas includes common venous malformation, particularly blue rubber bleb nevus syndrome, histopathological studies should be performed.

Unusual skin tumors: Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans. Surg Clin North Am. 2009 Jun;89(3):727-38.
This article discusses the epidemiology, etiology, presentation, pathology, evaluation and staging, and treatment of unusual skin tumors, such as Merkel cell carcinoma, eccrine carcinoma, glomus tumors, and dermatofibrosarcoma protuberans.

Symplastic glomus tumor - a rare but distinct benign histological variant withanalogy to other 'ancient' benign skin neoplasms. J Cutan Pathol. 2009 Oct;36(10):1099-102. Epub 2009 Jul 7.
A 78-year-old woman presented with a nail deformity of the index finger of theleft hand associated with paroxysmal pain upon cold exposure. Histologically, a well-circumscribed tumor of 3 mm diameter was found in the dermis. The neoplastic cells in some areas were of pronouncedly variable size and cytomorphology, mostly epithelioid in shape, with eosinophilic cytoplasm and indistinctly defined cell borders. Pronounced nuclear pleomorphism and atypia were striking features, but no mitotic figures were noted. Multinuclear cells were present as were numerous small-to-medium vessels throughout the tumor. The tumor stroma showed myxoidareas. Immunohistochemistry showed cytoplasmic and membranous expression ofsmooth muscle actin and vimentin. The histological features and immunoprofile were consistent with the diagnosis of symplastic glomus tumor, a rare histological variant, which has been defined as a glomus tumor exhibiting marked nuclear atypia, in the absence of any other criteria for malignancy. The biological behavior of the tumor is benign. It is essential to differentiate this entity from malignant glomus tumor, which has metastatic potential. Even prominent cellular atypia and nuclear pleomorphism in a glomus tumor as in our case is not a marker of malignancy in the absence of additional criteria.

Dermatopathology Case 26

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Case 26

A 45 year old female with a painful, bluish nodule on the left upper arm.

Monday, December 28, 2009

Answer of Dermatopathology Case 25


Clear Cell Acanthoma

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Abstract:

Comparing in vivo reflectance confocal microscopy, dermoscopy, and histology ofclear-cell acanthoma. Dermatol Surg. 2009 Jun;35(6):952-9.

BACKGROUND: Clear cell acanthoma (CCA) is a rare, benign neoplasm of unknown etiology, whose dermoscopic and histological features have been previously described. Usually, CCA can be diagnosed by clinical and dermoscopic examination. In some cases, diagnosis remains uncertain, and histological examination is needed. The aim of this paper was to describe the features of reflectance confocal microscopy (RCM) in diagnosing CCA, compare them with findings on dermoscopy and histology, and evaluate their possible usefulness in CCA evaluation. PATIENTS AND METHODS: Five lesions diagnosed clinically as CCA were imaged using dermoscopy and RCM. All lesions were surgically excised to confirm the diagnosis and compare the morphological attributes under light microscopy with in vivo imaging. RESULTS: RCM showed well-circumscribed lesions, often edged by a hyperkeratotic collarette with parakeratosis ; inflammatory cells in thespinous layer ; large keratinocytes ; acanthosis with papillomatosis ; epidermal disarray ; and dilated capillaries forming glomeruloid shapes in the upper dermis. CONCLUSIONS: In this small study, RCM was able to identify most of the established diagnostic histological features of CCA. RCM appears to be a useful tool for in vivo diagnosis of CCA and may help avoid unnecessary biopsies.

Clear-cell acanthoma versus acanthosis: a psoriasiform reaction pattern lacking tricholemmal differentiation. Am J Dermatopathol. 2007 Aug;29(4):378-84.

Clear-cell acanthoma (CCA) has been reported to be a benign epidermal neoplasm; however, several authors have suggested alternative differentiation as well asother nosologic categories, including a reactive dermatosis. Fourteen CCAs, ten tricholemmomas, and seven cases of psoriasis were reviewed with conventional microscopy, periodic acid-Schiff stains, and immunohistochemical stains. Twelve of fourteen (86%) CCAs were associated with underlying or adjacent conditions.The CCAs stained immunohistochemically in a pattern similar to normal epidermis and psoriasis. Tricholemmomas stained in a distinctly different pattern with MNF116 and NGFR/p75. These cases demonstrate CCA in settings that reflect chronic inflammation, primarily scars and stasis dermatitis, and with an immunophenotype that parallels psoriasis. These findings support the contention that CCA does not show outer follicular sheath (tricholemmal) differentiation. Furthermore, these cases lend additional support to the contention that CCA represents a psoriasiform reaction pattern, which, in appropriately taken biopsies, usuallyhas a demonstrable associated condition . Nonetheless, the precise nosology of this phenomenon has yet to be elucidated completely.

Dermatopathology Case 25

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Case 25

A 34 year old male with a slow growing reddish-brown papule on the left lower leg.

Answer of Dermatopathology Case 24



Poroma


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Abstract:


Poromas and porokeratosis in a patient treated for solid-organ and haematological malignancies. Clin Exp Dermatol. 2009 Dec 2.
We describe a patient with previous solid-organ (testicular, oesophageal) and haematological (acute myeloid leukaemia) malignancies, in whom chronic cutaneous graft-versus-host disease was complicated by poromas and porokeratosis. Chemotherapy, total body irradiation, longstanding immunosuppression and ultraviolet radiation may all have played a part in the pathogenesis of the skin tumours.

Poroma of the hip and buttock.Dermatol Online J. 2009 May 15;15(5):10.
Poroma is a benign adnexal skin tumor seen in middle aged individuals with no sex predilection. The acral sites are the most commonly affected regions. Hip or buttock as a location of origin has rarely been reported. We report two cases of poroma, one located on the hip of a 75-year-old man and the other on the buttock of a 60-year-old man.

New concepts on the histogenesis of eccrine neoplasia from keratin expression in the normal eccrine gland, syringoma and poroma.Br J Dermatol. 2008 Sep;159(3):633-45. Epub 2008 Jul 19.
BACKGROUND: Peripheral and luminal layers of eccrine sweat gland ducts are self-renewing structures. Proliferation is restricted to the lowermost luminal layer, but randomly scattered in the peripheral layer. Each layer exhibits differential expression of keratins K5/K14 and K6/K16. Keratin K1 occurs only in peripheral cells and the novel keratin K77 is specific for luminal cells. OBJECTIVES: To investigate the expression of luminal (K77), peripheral (K1) and further discriminatory keratins in two eccrine sweat gland tumours: syringoma,thought to show differentiation towards luminal cells of intraepidermal sweat ducts and eccrine poroma, considered to arise from poroid cells, i.e. peripheral duct cells; and keratinocytes of the lower acrosyringium/sweat duct ridge differentiating towards cells of intradermal/intraepidermal duct segments. METHODS: Paraffin-embedded sections were examined by immunohistochemistry using several keratin, smooth muscle actin and Ki-67 antibodies. RESULTS: We confirmed the ductal nature of syringomas. Despite drastic morphological alterations inboth layers, their keratin patterns remained almost undisturbed compared with normal ducts. In eccrine poroma epidermal keratins K5/K14 were ubiquitously expressed in all poroid cells. Cell islands deviating morphologically from poroid cells contained epidermal keratins K1/K10. K77 expression was limited to luminal cells of intact duct structures within the tumours. CONCLUSIONS: Syringomas are benign tumours of luminal cells of the lower most intraglandular sweat duct. Poroid precursor cells of poromas do not comprise peripheral duct cells nor do poromas differentiate towards peripheral or luminal duct cells. Instead, poroid cells consist only of keratinocytes of the lower most acrosyringium and the sweat duct ridge and poromas tend to differentiate towards the cells of the upper acrosyringium.

A single lesion showing features of pigmented eccrine poroma and poroid hidradenoma. J Cutan Pathol. 2008 Sep;35(9):861-5. Epub 2008 Apr 17.
Poroid hidradenoma (PH) is a variant of poroma. This entity was defined by Abenoza and Ackerman in 1990. This neoplasm shows architectural characteristics of hidradenoma (tumor cells confined entirely within the dermis in both solid and cystic components) and cytologic characteristics of poroid neoplasm (poroid and cuticular cells, the latter showing ductal differentiation). We herein document a case of single poroid lesion with the features of both eccrine poroma and PH. The patient was a 55-year-old woman with a pigmented nodular lesion on her upper back for 7 years. The histopathologic features of the lesion were consistent with those of eccrine poroma and PH. Unlike most eccrine poromas, this case was pigmented, clinically and microscopically.

Dermatopathology Case 24

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Case 24

A 62 year old male with a greyish brown nodule on the left sole. The nodule is 1.5 cm in diameter. Patient has a history of oesophageal carcinoma.

Wednesday, December 23, 2009

Answer of Dermatopathology Case 23



Pilomatrixoma (Pilomatricoma ; Calcifying epithelioma of Malherbe)


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Abstract:

Fine needle aspiration cytology of pilomatrixoma and differential diagnoses.Acta Cytol. 2009 Nov-Dec;53(6):683-8.
OBJECTIVE: To review the cytologic features of pilomatrixoma to allow a definitive diagnosis at the time of aspiration and correct patient management. STUDY DESIGN: Three patients each presented with a mass lesion and on fine needle aspiration were diagnosed as either having pilomatrixoma or a benign skin adnexal tumor. The diagnosis was confirmed on histology. A retrospective analysis of the cytology was performed. RESULT: The most consistent and predominant features for diagnosis were basaloid cells, anucleate squamous cells and calcification in an inflammatory background, often with giant cells. Only focal evidence of true shadow or "ghost" cells was seen in one case on the Diff-Quik smear. Therefore, lack of shadow cells does not preclude correct diagnosis in the presence of theother relevant cytologic features. If all the major components of pilomatrixoma are present in an aspirate, the diagnosis should not be problematic. However, if one component predominates, this may lead to an erroneous diagnosis of otherbenign and malignant lesions. CONCLUSION: Knowledge of the cytologic features of pilomatrixoma will allow correct patient diagnosis and management.

Pilomatrixoma of the forearm: a case report.Iowa Orthop J. 2009;29:121-3. Pilomatrixoma is a benign neoplasm derived from hair follicle matrix cells. Involvement of the upper extremities is relatively uncommon and can be mistaken for malignancy. We present the case of a 52-year-old woman with a pilomatrixoma of the forearm, and we review the literature regarding pilomatrixomas in the upper extremity.

Recurrent pilomatricoma on the left eyebrow: case report. Arq Bras Oftalmol. 2009 May-Jun;72(3):380-3.
Pilomatricoma is a benign neoplasm originated in the pilosebaceous follicle which appears as an intradermal nodule during childhood, in most cases. It is generally covered with normal skin, reaching a diameter of 1.5 cm on average, and it often shows no recurrence after surgical excision. The authors describe a case in whicha 26-year-old patient presented a tumoral lesion on the upper left superciliary area was submitted to total excision, and diagnosed as pilomatricoma by the histological study. According to the patient, this lesion had a progressive and slow growth of about two years. Eight months after the first surgical intervention, there was a new tumor growth in the region, this time quicker and worsen, with ulceration on the small skin area that covered the lesion, thus leading to malignity suspicion. After the conduction of a new excision within a security margin, the histological test confirmed that it was a pilomatricoma lesion and the piece displayed no sign of malignity. The patient has been followed up for one year, showing no signs of recurrence so far.

Pilomatricoma can differentiate not only towards hair matrix and hair cortex, but also follicular infundibulum, outer root sheath and hair bulge. Exp Dermatol. 2009 Aug;18(8):734-7. Epub 2009 Mar 16.
Pilomatricoma is believed to differentiate towards the hair matrix and hair cortex. To elucidate the origin of differentiation in pilomatricoma, we studied the expression of epithelial keratin (K) and filaggrin (filament aggregating protein) in pilomatricoma. An immunohistochemical study has been made of 53 casesof pilomatricoma using 10 monospecific anti-keratin antibodies and anti-filaggrin antibody. Basophilic cells, transitional cells and shadow cells did not reactwith epithelial keratins and filaggrin antibodies as well as hair matrix and haircortex. Instead, infundibular-type epithelium was positive for K1, K10 andfilaggrin. Epithelium showing trichilemmal keratinization was positive for K14 and K16. The hair bulge-like structure was positive for K19. The differentiation of pilomatricoma is diversified, and is heterogeneous in epithelial keratin and filaggrin expression. Our results for keratin and filaggrin expression suggested that pilomatricoma can differentiate not only towards hair matrix and haircortex, but also follicular infundibulum, outer root sheath and hair bulge.

Pilomatrixoma of the ocular adnexae: clinical and histologic analysis (13 cases). Klin Monbl Augenheilkd. 2009 May;226(5) :404-8. Epub 2009 Apr 27.
BACKGROUND: Pilomatrixoma (calcifying epithelioma of Malherbe) is a rare benign tumour originating from the matrix of the hair root. In this study, we evaluated the clinical and histopathological characteristics of all the respective tumour specimens submitted to our laboratory since 1997. PATIENTS/MATERIALS AND METHODS:We reviewed the clinical and histologic findings of 13 pilomatrixomas localised to the ocular adnexae. Paraffin sections were stained with H&E and PAS and, inaddition, immunohistochemistry was performed using markers for macrophages (CD68), for proliferative activity (Ki67), and for proliferation/apoptosis(CAS/CSE1L). RESULTS: All 13 pilomatrixomas were of reddish-livid colour andlocalised on the upper eyelid and/or near the eyebrow. The patients ages ranged from 4 to 84 years without preference for either gender. In 3 cases the correct diagnosis was made intraoperatively, while no pilomatrixoma was diagnosed priorto surgery. The most frequent false diagnoses were retention cyst (n = 3),atheroma (n = 2), and abscess (n = 2). There was no tumour recurrence in any of the cases within the time period presented. All 13 pilomatrixomas demonstrated the characteristic histology with areas of basophilic islands, shadow cells, calcification and a chronic granulomatous inflammation with foreign body giant cells in varying amounts. Immunhistochemical staining with anti-Ki67 andanti-CAS/CSE1L indicated a regular cellular maturation of the basophilic cells. No correlation was found between any of the histological or immunohistochemical markers and clinical parameters such as size, age and gender of the patient and duration of tumour presence prior to surgery. CONCLUSIONS: This case series represents one of the largest published so far, and underscores that pilomatrixoma can appear at any age. While it is usually not recognised clinically, it should be considered in the differential diagnosis especially for tumours of the upper eyelid. Our immunohistochemical findings support the basically benign nature of a pilomatrixoma but also point to the chronic inflammatory stimulus that can lead to complications after incomplete removal.

Dermatopathology Case 23

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Case 23

A 5 year old boy with a solitary firm nodule on the cheek.

Tuesday, December 22, 2009

Answer of Dermatopathology Case 22


Fibroepithelioma of Pinkus

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Abstract:

Variations in clinical presentation of basal cell carcinoma. Acta Clin Croat. 2008 Mar;47(1):25-30.

Basal cell carcinoma (basalioma, BCC) is the most common skin cancer and the mostcommon human malignancy in general, with a continuously increasing incidence. In most cases, BCC develops on chronically sun-exposed skin in elderly people, most commonly in the head and neck region. Besides chronic UV radiation, other risk factors for the development of BCC include sun bed use, family history of skin cancer, skin type 1 and 2, a tendency to freckle in childhood, immunosuppression, previous radiotherapy, and chronic exposure to certain toxic substances such as inorganic arsenic. There are numerous variations in clinical presentation of BCC, such as nodular BCC, ulcerating BCC, pigmented BCC, sclerosing BCC, superficialBCC, and fibroepithelioma of Pinkus. Each varies in terms of clinicalpresentation, histopathology and aggressive behavior. Treatment modalities for BCC include surgical excision, cryosurgery, curettage, electrodessication, radiotherapy, photodynamic therapy, topical cytostatics, and immunomodulators. If left untreated or inadequately treated, BCC may become invasive and locally destructive, although it very rarely metastasizes. Due to the extremely high incidence of BCC, medical professionals should be familiar with its manifold clinical presentations.

Fibroepithelioma of pinkus in a 9-year-old boy: a case report. Cases J. 2008 Jun 27;1(1):21.

ABSTRACT: Fibroepithelioma of Pinkus (FEP) is a rare indolent variety of basalcell carcinoma that is typically polypoid and located on the trunk of adult malesaged 40-60 years. Basal cell carcinoma (including FEP) is very rare in the pediatric population. We are reporting such a case occurring in a 9-year-old boy.

Fibroepithelioma of Pinkus with pleomorphic epithelial giant cells. Dermatol Online J. 2008 Dec 15;14(12):13.

Fibroepithelioma of Pinkus is a rare, indolent variant of basal cell carcinoma(BCC). The presence of pleomorphic giant cells in such a tumor is extremely rare and to our knowledge, only one such case has been previously reported in theliterature. We report another case occurring as a pedunculated, gluteal lesion in an 82-year-old man. The nodule was lightly pigmented, polypoid, and measured 1.0 cm in greatest dimension. Immunohistochemical staining confirmed that the giant cells were of epithelial origin and that the proliferative rate of these cells was low.

Expression of androgen receptor by fibroepithelioma of Pinkus: evidence supporting classification as a basal cell carcinoma variant? Am J Dermatopathol. 2007 Feb;29(1):7-12.

The classification of fibroepithelioma of Pinkus as basal cell carcinoma or trichoblastoma remains controversial. Immunohistochemical stains for androgenreceptor may be useful in differentiating basal cell carcinoma fromtrichoepithelioma or trichoblastoma. We studied androgen receptor expression in13 fibroepitheliomas of Pinkus, 11 basal cell carcinomas, 12 trichoepitheliomas, and 3 trichoblastomas. Androgen receptor expression was present in 77% (10/13) of fibroepitheliomas of Pinkus, 73% (8/11) of basal cell carcinomas, 17% (2/12) of trichoepitheliomas, and 0% (0/3) of trichoblastomas. Androgen receptor expressionwas significantly higher in fibroepitheliomas of Pinkus compared withtrichoepitheliomas and trichoblastomas (P = .0007), but not basal cell carcinoma (P = 1.00). Tumor-associated Merkel cells, a feature of benign follicular tumors,was identified by cytokeratin 20 stains. Merkel cells were identified in 85%(11/13) of fibroepitheliomas of Pinkus, 27% (3/11) of basal cell carcinoma cases, and 73% (11/15) of benign follicular tumors. Cytokeratin 20 expression was significantly higher in fibroepithelioma of Pinkus and benign follicular tumorscompared with basal cell carcinomas (P = 0.0111 and P = 0.025, respectively). No significant difference was found between fibroepitheliomas of Pinkus and trichoepitheliomas and trichoblastomas (P = 1.00). Similar to basal cellcarcinomas, fibroepitheliomas of Pinkus express androgen receptors, potentially supporting classification as a basal cell carcinoma. Conversely, fibroepitheliomaof Pinkus demonstrates retention of Merkel cells, a feature of benign follicular tumors. Immunophenotypic evidence for the classification of fibroepithelioma of Pinkus remains inconclusive. In small, partial biopsy specimens, coexpression of androgen receptor and cytokeratin 20 may aid in the diagnosis of fibroepithelioma of Pinkus.

Fibroepithelioma of pinkus is a fenestrated trichoblastoma. Am J Dermatopathol. 2005 Apr;27(2):149-54.

Pinkus described "pre-malignant fibroepithelioma" as a proliferation that gave rise to many tiny basal cell carcinomas within each lesion. Later authors have generally considered it to be an unusual variant of basal cell carcinoma (BCC).The delineation of trichoblastoma as the general term for the benign counterpart of BCC raises the possibility that the fibroepithelioma of Pinkus (FEP) would be better classified under that rubric. To address this subject, we examined the records of 114 patients with FEP for body site, age and sex distribution, and sections from 75 lesions. All FEP examined show a blunt interface with the underlying dermis (where one could be seen), differentiation toward follicular bulbs and papillae, and large areas of cellular stroma. FEP has a slight female preponderance in contrast to BCC, which is more common in males. Unlike the common types of BCC, FEP has an overwhelming predilection for the trunk and extremities, and only 5% of tumors are set in a dermis with significant amounts of solar elastosis. Next, FEP, BCC, and FEP with BCC-like areas were stained with MIB-1 (to assess proliferation), p53 (an oncogene product), and CK20 (a Merkel cell marker) antisera. FEP shows a low level of staining for p53 and MIB-1, in contrast to conventional BCCs that over-express these markers. FEP also shows retention of Merkel cells, a characteristic of benign neoplasms with follicular germinative differentiation but not in general of BCC. The BCC-like areas in some FEP tumors reflect these staining tendencies with less striking differences. Given the contrast between FEP and BCC with respect to site of occurrence, relationship to sun damage, histopathologic features, and immunohistochemical studies, it appears that FEP more closely resembles trichoblastoma than BCC.

Dermatopathology Case 22

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Case 22

A 54 year old male presents with a solitary, pedunculated on the left upper extremity. The lesion is reddish brown in colour and 1.5 cm in diameter.

Answer of Dermatopathology Case 21


Bowen's Disease

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Abstract:

Bowen's disease of the nipple in a young man with AIDS: a case report.Clin Breast Cancer. 2009 Feb;9(1):53-5.
Bowen's disease, or squamous cell carcinoma in situ (SCCIS) of the skin, is a malignant neoplasm restricted to the epidermis, without evidence of dermal invasion. It usually develops in sun-exposed area of skin, but other sites can also be affected. Bowen's disease of the nipple is extremely rare and has thus far been reported only in women. We present the case of Bowen's disease of the nipple in an HIV-positive male patient who presented with a scaly lesion on nipple for one year. He also had past genital infection with human papillomavirus, but he was found to be negative for high-risk subtypes. Biopsy of the lesion revealed SCCIS of the nipple areola complex, with extension into the underlying lactiferous ducts of the breast. There was no evidence of invasive carcinoma. The patient was treated with a simple mastectomy with sentinel lymphnode biopsy. With the advent of highly active antiretroviral treatment, chronicnon-HIV related conditions have become more important, male breast cancer beingone of them. To the best of our knowledge, this is the first reported case in the worldwide literature of Bowen's disease of the nipple in a young immunocompromised male patient. More aggressive therapy in HIV-positive malepatients presenting with precancerous and cancerous breast lesions is recommended.

Expression of p53 and p16 in actinic keratosis, bowenoid actinic keratosis and Bowen's disease.J Eur Acad Dermatol Venereol. 2009 Jun 8.
Introduction Bowen's disease (BD) and bowenoid actinic keratosis (bAK) have traditionally been differentiated according to the presence or absence of dysplasia in the follicular epithelium. p16 has been suggested to be a useful tool to make the differential diagnosis between BD and AK and as a marker of bad prognosis. Materials Five biopsies of BD, five of AK and five of bAK where stained for p53 and p16. Results All lesions showed positive immunostaining of p53, affecting to the lower two thirds of the epidermis in BD and bAK, and only the basal layer in non-bAK. All the BD and bAK cases were positive for p16, showing a similar immunostaining pattern, whereas no staining was observed in non-bAK. Discussion and conclusion These findings suggest a common pathogenicmechanism for BD and bAK. bAK might have worse prognosis than AK. p16 might not be useful as a tool for differential diagnosis between AK and BD because bAK and BD show an extremely similar immunohistochemical pattern. Conflicts of interest None declared.

Immunohistology is valuable in distinguishing between Paget's disease, Bowen's disease and superficial spreading malignant melanoma.Histopathology. 1990 Jun;16(6):583-8.
Thirty-nine lesions, originally diagnosed as Paget's disease (19), superficial spreading melanoma (13) or Bowen's disease (7), were reviewed. Supplementary immunohistochemistry on routine processed tissue, using antibodies to cytokeratins (PKK1 and M717) and to S-100 protein, was carried out. Two of the lesions, originally classified as Bowen's disease and superficial spreading type malignant melanoma respectively, were reclassified as Paget's disease based on the immunohistochemical findings. Both lesions contained pagetoid cells positive with PKK1. The results indicate that immunohistochemistry may sometimes be valuable in establishing the correct diagnosis in such lesions.

Dermatopathology Case 21

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Case 21

A 65 year old male with a solitary, asyptomatic, slowly growing, erythematous, well-demarcated scaly patch on the left side of the neck, 4 cm in diameter.

Monday, December 21, 2009

Dermatopathology Case Index: Case 1 to Case 20


Dermatopathology Case Index :


Case 1 = Cutaneous Leiomyosarcoma

Case 2 = Sebaceous adenoma in a patient with Muir-Torre syndrome

Case 3 = Large Cell Acanthoma

Case 4 = Eccrine Porocarcinoma

Case 5 = Colloid Milium

Case 6 = Primary Cutaneous Apocrine Carcinoma

Case 7 = Primary Cutaneous Adenoid Cystic Carcinoma

Case 8 = Cutaneous Lymphadenoma

Case 9 = Eccrine Spiradenoma

Case 10 = Primary Cutaneous Actinomycosis

Case 11 = Aggressive Digital Papillary Adenocarcinoma

Case 12 = Trichoepithelioma

Case 13 = Trichofolliculoma

Case 14 = Warty Dyskeratoma

Case 15 = Cylindroma

Case 16 = Trichilemmoma

Case 17 = Fordyce's Spot (Ectopic sebaceous glands)

Case 18 = Endometriosis

Case 19 = Spiradenocarcinoma (Malignant Eccrine Spiradenoma)

Case 20 = Dermatophyte Infection

Answer of Dermatopathology Case 20


Dermatophyte Infection


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Abstracts:

Dermatophyte infections.Am Fam Physician. 2003 Jan 1;67(1):101-8.

Dermatophytes are fungi that require keratin for growth. These fungi can cause superficial infections of the skin, hair, and nails. Dermatophytes are spread by direct contact from other people (anthropophilic organisms), animals (zoophilicorganisms), and soil (geophilic organisms), as well as indirectly from fomites. Dermatophyte infections can be readily diagnosed based on the history, physical examination, and potassium hydroxide (KOH) microscopy. Diagnosis occasionally requires Wood's lamp examination and fungal culture or histologic examination.Topical therapy is used for most dermatophyte infections. Cure rates are higher and treatment courses are shorter with topical fungicidal allylamines than with fungistatic azoles. Oral therapy is preferred for tinea capitis, tinea barbae, and onychomycosis. Orally administered griseofulvin remains the standardtreatment for tinea capitis. Topical treatment of onychomycosis with ciclopiroxnail lacquer has a low cure rate. For onychomycosis, "pulse" oral therapy withthe newer imidazoles (itraconazole or fluconazole) or allylamines (terbinafine) is considerably less expensive than continuous treatment but has a somewhat lower mycologic cure rate. The diagnosis of onychomycosis should be confirmed by KOH microscopy, culture, or histologic examination before therapy is initiated, because of the expense, duration, and potential adverse effects of treatment.

Microscopically differentiating dermatophytes from sock fibers.J Am Acad Dermatol. 2009 Dec;61(6):1024-7.

BACKGROUND: Dermatophytes are responsible for a number of superficial fungal infections that affect millions worldwide. During microscopic observation apotassium hydroxide (KOH) fungal smear, various filamentous materials such as common textile fibers from socks can obfuscate proper discernment of dermatophytes. OBJECTIVE: To differentiate dermatophytes from 9 common sock fibers. METHODS: Nine different textile fiber samples were microscopically analyzed by using a KOH direct smear test; their defining structural features were compared and contrasted with those of dermatophytes. RESULTS: Although thereare several similarities, sock fibers tend to have a non-septate, uniformstructure which differentiates them from dermatophytes. Sock fibers are also significantly larger than dermatophytes and can be viewed better at lower magnifications. LIMITATIONS: There is a lack of sock samples with 100% textile fiber composition. Also, fibers were examined in a clean setting, without the detritus that normally accompanies dermatophytes in a clinical setting.CONCLUSION: While textile fibers may be present in KOH preparations, their general appearance typically differs from that of dermatophytes; an observer who is familiar with these distinctions will be able to differentiate between the two.

Dermatophytosis: a summary of dermatomycosis as a proposal for future revisionof the guidelines.Nippon Ishinkin Gakkai Zasshi. 2009;50(4):199-205.

In preparing guidelines for dermatomycosis (tinea, trichophytia,dermatophytosis), we have primarily summarized the disease types and treatments as described in 4 textbooks used in Japan and abroad. We present our classification draft based on these following descriptions. In Japan, any dermatophytosis other than favus or tinea imbricata is considered to be tinea,while outside Japan, favus and tinea imbricata are also classified as tinea.Tinea capitis is classified together with trichophytia superficialis capillitiiand kerion celsi, in a group that tends to include asymptomatic carriers. Most textbooks generally classify trichophytia profunda of the glabrous skin andgranuloma trichophyticum as subtypes of tinea corporis. Tinea faciei can easilybe misdiagnosed, but in many cases can be distinguished from tinea corporis byits specific clinical picture. Tinea unguium is regarded as one type of onychomycosis. We present a summary of dermatomycosis treatment as a proposal for future revision of the guidelines. One of the problems in the treatment of tinea capitis is that the safety of itraconazole (ITZ) and terbinafine hydrochloride(TBF) in children has not been established. Severity criteria for concomitant useof oral medications in the treatment of tinea pedis remains to should be established. Although many clinical studies concerning tinea unguium have beenpublished, 3 of the 4 textbooks we consulted clearly stated that most of thosestudies were conducted by pharmaceutical companies. Further studies on the etiology and disease severity of tinea unguium are needed.

Dermatopathology Case 20

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Case 20

24 year old male. Skin biopsy for diagnosis.

Sunday, December 20, 2009

Answer of Dermatopathology Case 19



Spiradenocarcinoma (Malignant Eccrine Spiradenoma)

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Abstract:

Spiradenocarcinoma arising from a spiradenocylindroma: unusual case withlymphoepithelioma-like areas. J Cutan Med Surg. 2009 Jul-Aug;13(4):215-20.
BACKGROUND: Hybrid skin adnexal tumors are common, and spiradenocylindroma is well described. OBJECTIVE: However, malignant transformation in this setting isinfrequent, especially resemblance to lymphoepithelioma-like carcinoma of skin,which is not associated with Epstein-Barr virus. METHODS: A 65-year-old female presented with ataxia and a skin nodule composed of a hybrid adnexal tumor(spiradenoma and cylindroma) that transitioned into an undifferentiated carcinoma with attendant lymphocytes and plasma cells. There was widespread disseminationof the undifferentiated component to regional neck lymph nodes. RESULTS: The undifferentiated component resembled a lymphoepithelioma-like carcinoma and showed focal evidence of tubular and squamous differentiation. The tumor was Epstein-Barr encoded RNAs (EBER) negative by in situ hybridization. No evidence of neuroendocrine differentiation was seen in the tumor, despite the patient having symptoms of paraneoplastic ataxia that improved after surgery. CONCLUSION:This case highlights the transition of a benign hybrid tumor(spiradenocylindroma) into a spiradenocarcinoma that resembled lymphoepithelioma-like carcinoma of skin. It also highlights two unusual features: widespread lymph node dissemination and presentation with paraneoplastic syndrome-associated ataxia.

Spiradenocylindrocarcinoma. Report of a case with a low-grade component of spiradenocarcinoma and an immunohistochemical study. Am J Dermatopathol. 2009 Aug;31(6):594-8.
Spiradenocylindrocarcinoma is a very rare malignant cutaneous neoplasm of the folliculosebaceous-apocrine unit. We report a case of this hybrid tumor in a 42-year-old woman. The tumor consisted of 2 circumscribed nodules with areas of cylindrocarcinoma and low-grade spiradenocarcinoma. In the overlapping areas, both spiradenomatous and cylindromatous features were observed. Expansion of the tumor beyond the fibrous pseudocapsule into the adjacent tissue was present. Furthermore, tumor cells were demonstrating mild to moderate pleomorphism and an increased mitotic index. p53 and ki-67 were among the positive immunohistochemical markers. A relatively small number of tumor cells expressed estrogen receptors. The aim of this study was to investigate the nature of thisrare tumor of the skin appendages.

Morphologic diversity of malignant neoplasms arising in preexisting spiradenoma, cylindroma, and spiradenocylindroma based on the study of 24 cases, sporadic oroccurring in the setting of Brooke-Spiegler syndrome. Am J Surg Pathol. 2009 May;33(5):705-19.
The authors present a series of 24 malignant neoplasms arising in preexistingbenign spiradenoma (20), cylindroma (2), and spiradenocylindroma (2). Nineteen patients (12 females, 7 males; age range, 41 to 92 y) had a solitary neoplasm (size range, 2.2 to 17.5 cm; median 4 cm), whereas the remaining 5 (4 females, 1 male; age range, 66 to 72 y) manifested clinical features of Brooke-Spiegler syndrome (BSS), an autosomal dominantly inherited disease characterized by widespread, small, benign neoplasms on which background larger malignant lesions appeared. Microscopically, all cases showed the residuum of a preexisting benign neoplasm. The malignant components of the lesions were variable and could be classified into 4 main patterns, occurring alone or in combination: 1) salivary gland type basal cell adenocarcinoma-like pattern, low-grade (BCAC-LG); 2) salivary gland type basal cell adenocarcinoma-like pattern, high-grade (BCAC-HG); 3) invasive adenocarcinoma, not otherwise specified (IAC-NOS); and 4) sarcomatoid(metaplastic) carcinoma. In 1 case of IAC-NOS, an in situ adenocarcinoma was also found, presumed to have evolved from an adjacent adenomatous and atypical adenomatous component. Cases harboring a sarcomatoid carcinoma featured amalignant epithelial component composed of varying combinations of BCAC-HG,BCAC-LG, IAC-NOS, or squamous cell carcinoma, whereas the sarcomatoid component appeared as either a pleomorphic or spindle-cell sarcoma. Additionally, in 2 cases there were foci of heterologous chondrosarcomatous differentiation and in 1case there was rhabomyosarcomatous differentiation. Of the 21 patients with available follow-up (range, 3 mo-15 y; average 4.8 y; median 3.5 y), 10 were without evidence of disease, 1 was alive with metastatic disease, 1 was alive with BSS, 3 developed local recurrences, 4 had died of disease, and 2 were dead of other causes. The histologic pattern of the malignant neoplasm correlated to some extent with the clinical course. BCAC-LG neoplasms showed a less aggressive course, with local recurrences but no distant metastases, whereas the BCAC-HG neoplasms typically followed a highly aggressive course resulting in the death 3 of 6 patients with BCAC-HG. Patients with sarcomatoid carcinoma had a relatively good survival. Molecular genetic investigations revealed no mutations in the CYLD gene in the 4 sporadic cases investigated. One patient with BSS revealed a novel missense germline mutation in exon 14 (c. 1961T>A, p. V654E), whereas a living descendant of another deceased patient demonstrated a recurrent nonsense germline mutation in exon 20 (c. 2806C>T, p. R936X). Given the morphologic diversity and complexity of the neoplasms in question, we propose using a more specific terminology with the precise description of the neoplasm components, rather than generic and less informative terms such as "spiradenocarcinoma" or "carcinoma ex cylindroma."

Dermatopathology Case 19

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Case 19

A 65 year old woman with a long standing nodule on the scalp.
The asymptomatic nodule was present for last 20 years.
Since last two months the nodule became tender and rapidly enlarged in size.
The lesion is now 7 cm in diameter.

Answer of Dermatopathology Case 18


Endometriosis

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Note: Cutaneous endometriosis indicates that scar tissue is particularly susceptible to endometrial implantation. Abdominal wall endometriosis is usually associated with incisional abdominal scars and occurs most commonly following a Caesarean section. It has been suggested that the umbilicus acts as a physiologic scar with a predisposition for developing endometriosis. Umbilical endometriosis has been well described in the literature, occurring either spontaneously or following laparoscopic procedures in which a trocar was placed through the umbilicus.

Abstract:

Cutaneous endometriosis.Singapore Med J. 2008 Sep;49(9):704-9.
INTRODUCTION: Cutaneous or subcutaneous endometriosis is a rare entity that is anoften overlooked because of chronic abdominal pain. METHODS: We reviewed the ten cases of cutaneous endometriosis that presented to this hospital over a seven-year period. RESULTS: The mean age of patients at presentation was 36.3 years. All our patients presented with cyclical abdominal pain. There was a considerable delay in the diagnosis and offer of treatment, the mean length of time between onset of symptoms to surgery being 31.7 months (range 1-62 months). The onset was spontaneous in 40 percent of our patients and the rest had scar endometriosis. The patients with spontaneous onset of disease had a more severe pelvic disease compared to those with scar endometriosis. Complete surgical excision was curative but left a large defect requiring polypropylene mesh repair in two patients. CONCLUSION: Cutaneous endometriosis should be suspected in any female presenting with cyclic or non-cyclic pain emanating from a mass in the vicinity of a previous surgical scar, the umbilicus or in the inguinal region. Surgical excision of the cutaneous endometriotic implants can be easily performed and is curative.

Umbilical endometriosis: A radical excision with laparoscopic assistance. Int J Surg. 2009 Nov 24.
BACKGROUND: Umbilical endometriosis represents the most common site of cutaneous endometriosis. Although its treatment is typically surgical, in literature the approach used is variable and extends from diathermocoagulation to omphalectomy. Such superficial treatments for umbilical endometriosis can predispose the patient to a relapse of the disease. We here present seven cases of umbilical endometriosis treated with radical surgery with a laparoscopically-assisted approach, with a complete and long-term disease-free follow-up. CASES: Seven cases of umbilical endometriosis, four of which relapsing from a prior superficial treatment, were treated radically with a laparoscopically-assistedapproach, with a long-term disease-free follow-up. CONCLUSION: Although a medical treatment can be considered, the treatment of choice in these patients should be that of excisional surgery so as to avoid lesion relapse and the risk of oncogenic transformation. Despite umbilical endometriosis is a rare finding, this relatively small case series treated by laparoscopically-assisted omphalectomy shows a complete resolution of the lesion and symptoms along with good aesthetic results at a long-term follow-up.

Spontaneous endometriosis in an umbilical skin lesion. Acta Dermatovenerol Alp Panonica Adriat. 2009 Sep;18(3):126-30.
Cutaneous endometriosis of the umbilicus is an unusual condition with unclear pathogenetic mechanisms that might be mistaken for a malignant condition. A 46-year-old woman presented with a cutaneous black mass in the umbilicus. The lesion was removed surgically and histological analyses revealed that it consisted of endometrial tissue. There was no recurrence at 18-month follow-up. Endometriosis of the umbilicus is a rare condition and the pathogenesis is not completely elucidated. According to one theory, intraperitoneal endometrial tissue is translocated during endoscopic surgery or other surgical procedures that involve the umbilicus. However, in this case there was no history ofabdominal wall surgery. We conclude that endometriosis is important to considerin cases of unclear skin lesions of the umbilicus, even in cases with no previous abdominal surgery. Moreover, umbilical endometriosis of the skin can have different appearances that resemble malignant tumors, and radical surgery with histology is therefore indicated.

Endometriosis risk in relation to naevi, freckles and skin sensitivity to sunexposure: the French E3N cohort.Int J Epidemiol. 2009 Aug;38(4):1143-53. Epub 2009 Apr 7.
BACKGROUND: Endometriosis is an important women's health issue, however its aetiology remains unknown. An association between endometriosis and cutaneous melanoma was described, possibly explained through common genetic features. To further investigate this association, we assessed the link between phenotypic traits predisposing to melanoma and the risk of endometriosis. METHODS: Using a case-control design, we analysed data from 97,215 women of the Etude Epidémiologique auprès de femmes de l'Education Nationale study, a cohort of98,995 French women insured by a national health scheme mostly covering teachers, and aged 40-65 years at inclusion in 1990. Risk estimates were computed using unconditional logistic regression models. RESULTS: After adjustment for potential confounding factors, there was a positive dose-effect relationship between riskof endometriosis (reported as surgically ascertained, n = 4241) and skin sensitivity to sun exposure [moderate: odds ratio (OR) 1.09, 95% confidence interval (CI) 0.99-1.19; high: OR 1.22; 95% CI 1.10-1.36; compared with none;Ptrend < ptrend =" 0.005). CONCLUSION: This study is, to our knowledge, the first to report a positivedose-effect relationship between the risk of endometriosis and skin sensitivity to sun exposure, and number of naevi and freckles. These data suggest that endometriosis and melanoma may share some genetic features.

Primary umbilical endometriosis: a rare variant of extragenital endometriosis. Pathologica. 2008 Dec;100(6):473-5.
Endometriosis is defined as the presence of extra-uterine endometrial tissue. The prevalence rate of umbilical endometriosis ranges from 0.5 to 1.0% of all patients with extragenital endometriosis. In this report, we present a case of primary umbilical endometriosis to highlight the challenges encountered during diagnosis. A nulliparous 39-year-old woman presented to our department with a 2-year history of a tender, painful and non-reducible, firm umbilical mass thatenlarged slowly reaching 2 cm in diameter. She had never been pregnant nor had any abdominal surgery. There was no sequential bleeding. Ultrasound of the umbilical region showed a well defined, oval shaped anechoic area. Histological examination on a cutaneous biopsy concluded umbilical endometriosis. The patient was subsequently referred to a gynecologist and underwent surgery with en bloc excision of the lesion. Generally, umbilical endometriosis presents as a roundish tumuor that can either partly or completely occupy the umbilical scar with intermittent bleeding. Characteristically, the mass increases with the menstrual cycle, becoming more evident and usually harder and is associated with cyclic pain. Its pathogenesis remains uncertain. Clinical diagnosis is difficult, and umbilical endometriosis can be easily confused with other conditions such asbenign and malignant tumours. Ultrasound examination is useful, and surgical excision is the treatment of choice. Several cases of malignant transformation have also been described.

Morphological variations of scar-related and spontaneous endometriosis of the skin and superficial soft tissue: a study of 71 cases with emphasis on atypical features and types of müllerian differentiations. J Am Acad Dermatol. 2007 Jul;57(1):134-46.
BACKGROUND: Seventy-one cases of scar-related and spontaneous endometriosis ofthe skin and superficial soft tissue were studied, with a focus on atypical features and types of müllerian differentiation. All patients were women, whoseages ranged from 22 to 65 years (median, 32 years). METHODS: Histological, immunohistochemical, and electronmicroscopic studies were performed. Clinical information was ascertained via a questionnaire solicited by the referring physicians. RESULTS: All types of metaplastic changes of müllerian epithelium were found, including tubal (61%), oxyphilic (15%), hobnail (10%), mucinous (4%),and papillary syncytial (3%) metaplasia. Atypical features included reactive atypia (23%) and atypical mitoses in glandular epithelium (6%). Stromal changes included smooth muscle metaplasia (31%), decidualization (<1%), stromal endometriosis (<1%), and elastosis (<1%). Other features recognized included lipoblast-like cells (15%), some with intranuclear inclusions; atypical/degenerative myocytes (10%) ; spiral arteries (4%); and perineurial invasion (<1%). CD56 staining identified large granular lymphocytes in 15 of 20 studied specimens. Ultrastructurally, these cells showed cytoplasmic granules, some with a delimiting membrane. LIMITATIONS: This study utilizes tissue specimens that mainly were received as consultations; therefore some inherent selection bias exists. Specimens were randomly selected for CD56 immunostaining, leading also to potential sampling error. CONCLUSIONS: All types of müllerian metaplasia can be encountered in cutaneous endometriosis. In addition, so-called atypical features described in endometriosis affecting other anatomic sites may be seen in the skin. Some features may represent a diagnostic pitfall.

Dermatopathology Case 18

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Case 18

A 28 year old woman with a history of Caesarian section 2 years ago. There was a low transverse incision scar. A bluish- gray tender mass developed on the scar. The lesion is 3 cm in diameter.

Saturday, December 19, 2009

Answer of Dermatopathology Case 17



Fordyce's Spot (Ectopic sebaceous glands)

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Abstract:

Treatment of Fordyce spots with CO2 laser.Dermatol Surg. 2003 Aug;29(8):869-71.

BACKGROUND: Fordyce spots are heterotopic sebaceous glands that can be located at the lips' vermilion or the oral mucosa. Although this is considered a rathercommon disorder, a treatment for this condition that sometimes affects patientsfrom only a cosmetic viewpoint has not yet been described. OBJECTIVE: To evaluateCO2 superpulsed laser treatment in two subjects with Fordyce spots. METHODS: Two patients with papules and yellowish plaques at the upper lip corresponding to Fordyce spots were treated with coherent Ambulase CO2 superpulsed laser (CoherentMedical, Palo Alto, CA) ; after informed consent was obtained, two to three passeswere performed in one session using 2 and 4 W and a spot size of 2 mm. RESULTS:Complete re-epithelization was observed 2 weeks later with no residual Fordyce papules in the treated area and no side effects. CONCLUSION: Our findings suggestthat CO2 superpulsed laser can be considered a safe and effective treatment forpatients with Fordyce spots, offering excellent cosmetic results.

Dermatopathology Case 17

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Case 17

A 58 year old male with clusters of yellowish spots (2 mm in diameter) on the upper lip.
Biopsy of the yellowish spot.

Monday, December 14, 2009

Answer of Dermatopathology Case 16


Trichilemmoma

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Abstract:

Atypical clinical appearance and localization of trichilemmoma. a case report. Pathologica. 2009 Jun;101(3):133-4.
Trichilemmoma is a benign cutaneous tumor that shows characteristics of differentiation similar to the outer hair sheath. We report the case of a woman presenting with a nodular tender mass of the back that was diagnosed as anisolated trichilemmoma. Several lines of evidence suggest that trichilemmoma should be considered in the differential diagnosis of any indistinct facialpapule. This report documents a non-facial example of trichilemmoma. Atypical clinical appearance and localization of this neoplasm in our patient suggest thatonly histological findings are specific of this tumor.

Detection of Human Papillomavirus infection in trichilemmomas and verrucae using in situ hybridization. J Cutan Pathol. 2009
Background: It is hypothesized that trichilemmomas are 'burned out' verrucae. By performing in situ hybridization using HPV type-specific probes, we explored this concept. Methods: Verrucae vulgaris and plantaris were positive controls, andinverted follicular keratoses (IFKs) were negative controls. Additionally, all lesions were tested for HPV genital types (low and high risk). Results: We analyzed 9 trichilemmomas, 20 verrucae vulgaris, 8 verrucae plana, 3 verrucae plantaris and 6 IFKs. All trichilemmomas were negative for HPV types 1, 2 andgenital types. Conversely, 9/20 verrucae vulgaris, 2 verrucae plantaris, and 1verruca plana were positive for HPV type 2. Among HPV-2 positive lesions, 2/2verrucae plantaris and 1 verruca vulgaris (chin) were strongly positive forgenital-type HPV. One verruca plana (shin) was positive for genital-type HPVonly. All 6 IFKs were HPV negative. Conclusions: Using HPV type 1 and 2-specific probes and mixed genital-type probes, we were unable to detect HPV intrichilemmomas. This suggests that HPV-1, HPV-2 and low and high riskgenital-type HPVs are not involved in the histogenesis of trichilemmoma. We also showed that genital HPV types could be present in non-genital verrucae.

Trichilemmoma: an immunohistochemical study of cytokeratins. Br J Dermatol. 2003 Jul;149(1):99-104.
BACKGROUND: The histogenesis of trichilemmoma remains unclear. OBJECTIVES: To clarify the histogenesis of trichilemmoma by evaluating its cytokeratin (CK) expression. METHODS: In three cases of trichilemmoma, CK expression was studied immunohistochemically using seven antikeratin antibodies against CK1, 10, 14-17 and 19, respectively. RESULTS: CK1 and CK10 were present in keratinizing ductal epithelium. CK14 was present in the whole layer. CK15 was present in suprabasal layers in two cases. CK16 was present in the suprabasal layer, but was absent in keratinizing ductal epithelium. CK17 was present in suprabasal layers and the sebaceous duct-like structure. CK19 was totally absent. CONCLUSIONS: These results showed that trichilemmoma may differentiate mainly towards twodirections: infundibular keratinization and proliferation of the outer root sheath with undifferentiated and pluripotent characteristics.

Dermatopathology Case 16

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Case 16

A solitary, dome-shaped flesh coloured papule on the face of a 50 year old female.