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.








Sunday, December 18, 2011

Answer of Dermatopathology Case 111


Darier's Disease (Keratosis Follicularis)

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Abstract

Darier disease: case report with oral manifestations.Med Oral Patol Oral Cir Bucal. 2006 Aug 1;11(5):E404-6.
Darier disease, also known as keratosis follicularis or dyskeratosis follicularis, is a rare autosomal dominant genodermatosis. It is clinically manifested by hyperkeratotic papules primarily affecting seborrheic areas on the head, neck and thorax, with less frequent involvement of the oral mucosa. When oral manifestations are present, they primarily affect the palatal and alveolar mucosa, are usually asymptomatic, and are discovered in routine dental examination.
Histologically, the lesions present suprabasal clefts in the epithelium with acantholytic and dyskeratotic cells represented by corps ronds and corps grains. This paper reports a case of an adult male patient presenting clinical signs of Darier disease in the palatal mucosa and skin on the neck and upper limbs. Intraoral biopsy of the affected area, analysis of family history and evaluation by a multidisciplinary team led to the diagnosis of Darier disease.
Dental professionals and pathologists should be aware of these lesions to allow correct diagnosis and proper management of this disease.

Comedonal, cornifying and hypertrophic Darier's disease in the same patient: a Darier combination.J Dermatol. 2006 Jul;33(7):477-80.
Our 42-year-old patient had comedonal Darier's disease (DD) on the face, comedonal cornifying DD on the upper back, and hypertrophic DD on both legs.
Biopsies taken from face, upper back and medial sides of the legs were found to be compatible with these clinical subtypes. The comedonal type was in the classical place, but the hypertrophic and cornifying types were not in the usual sites.
In addition to the classic histopathology of DD, we noted multiple, warty dyskeratoma-like structures in the comedonal type, marked compact hyperkeratosis in the cornifying type, and marked papillomatosis in the hypertrophic type.

Dermatopathology Case 111

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Case 111


A 16 year old boy with yellowish brown,warty papules on the forehead. Small pits are noted on the palms and soles of the feet. Red and white bands are noted in the nails along with V-shaped nick at the free margin of the nail.


Saturday, December 17, 2011

Answer of Dermatopathology Case 110

Dermatofibroma with Monster Cells

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Abstracts:

Giant dermatofibroma with monster cells.Am J Dermatopathol. 2002 Feb;24(1):36-8.
We report a case of a 64-year-old woman with a giant dermatofibroma on her back with the unusual histologic feature of monster cells. The firm, exophytic, 3-cm nodule had purple and yellow components with surface telangiectasia. Histologic examination demonstrated characteristic findings of a dermatofibroma, including rete ridge flattening and bridging; a stroma containing scattered, large, round, eosinophilic collagen bundles; and a polymorphous dermal infiltrate of spindle and xanthomatous cells with scattered siderophages. Some xanthomatous cells demonstrated features consistent with monster cells, including huge bizarre nuclei and one or more nucleoli. Immunohistochemical staining for factor XIIIa was positive. A diagnosis of giant dermatofibroma with monster cells (DFMC) was made. Giant dermatofibromas are rare, with monster cells being an uncommon finding in dermatofibroma. To our knowledge, this is the first report of DFMC.

Case of dermatofibroma with monster cells: a review and an immunohistochemical study.Am J Dermatopathol. 1997 Jun;19(3):312-5.
We report a dermatofibroma with monster cells. The patient was a 79-year-old woman who had a dark-brown nodule of her left leg for approximately 3 years. The lesion was composed of spindle-shaped fibroblastic cells, histiocytic cells, and multinucleated giant cells. Most of the histiocytic cells had foamy cytoplasm with numerous hemosiderin deposits. In addition to these cells, bizarre multinucleated cells with markedly hyperchromatic nuclei and xanthomatous cells with very large nuclei (monster cells) were also noted. No mitotic figures of the cellular components were present. This lesion has been shown to be completely benign despite the presence of pleomorphic or bizarre cells. From a clinical standpoint, recognition of a benign lesion of this type is very important since an incorrect histologic interpretation could result in inappropriate treatment.

Dermatofibroma with monster cells.Am J Dermatopathol. 1987 Oct;9(5):380-7.
Nineteen cases of dermatofibroma associated with monster cells are reported. The term "monster" (an animal with a strange or terrifying shape, one unusually large for its kind) implies a strikingly atypical cell with an extremely large nucleus. Except for monster cells, these 19 lesions had all of the typical histopathological findings of dermatofibroma. The clinical diagnosis for 16 of these lesions was dermatofibroma (or histiocytoma). Three lesions were submitted without any clinical diagnosis. Eighteen of 19 lesions occurred on the extremities. One was on the back. Monster cells are seen in the early, histiocytic stage of dermatofibroma when lipophages and/or siderophages are usually present in large numbers. Only rarely were mitotic figures seen in dermatofibromas with monster cells, and they were neither numerous nor atypical. It is important for histopathologists to distinguish dermatofibroma with monster cells from cutaneous malignant fibrous histiocytoma and radiation sarcoma. The criteria for differentiation concern primarily the architectural pattern of the lesion rather than its cytological features.

Dermatopathology Case 110

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Case 110


A 55 year old female with a brownish firm nodule on the back. The nodule is 2.5 cm in diameter.

Diagnosis

Saturday, December 3, 2011

Answer of Dermatopathology Case 109



HMB-45 : Positive





Dermatopathology Case 109

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Case 109


A 72 year old male with a skin lesion on the forehead.


Wednesday, November 30, 2011

Answer of Dermatopathology Case 108


Median Raphe Cyst

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Abstract:
Median raphe cyst of the penis.Dermatol Online J. 2005 Dec 1;11(3):37.
Cysts of the median raphe are uncommon. We describe a 43-year-old man with an asymptomatic nodule on the glans penis. Excision of the lesion was performed followed by histological and immunohistochemical studies. Histopathological examination revealed a solitary unilocular cystic cavity in the corium, lined by a pseudostratified columnar epithelium, among which mucinous cells where identified. Hints of decapitation secretion occurred at the apex of the luminal layer. An immunohistochemical study using a standard avidin-biotin peroxidase method disclosed CK7 and CK13 reactivity in the columnar cells. Epithelial membrane antigen (EMA) and carcinoembryonic (CEA) antigen immunoreactivity occurred at the apical border of the luminal cells. No staining was obtained with anti-CK20, human milk fat globulin 1 (HMFG1) and anti- S100 protein. The study supports its histogenetic relationship with the urothelium.

Dermatopathology Case 108

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Case 108
A 38 year old male with a nodule on the glans penis.

Tuesday, November 29, 2011

Answer of Dermatopathology Case 107


Naevoid Melanoma

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In general, common naevi are diagnosable at low power.
It is important to exclude the possibility of naevoid melanoma.
This is a rare variant that mimics benign naevi and is difficult to recognise.
The correct diagnosis is frequently made only after the patient has developed a metastasis.
At low power, naevoid melanoma may have a nodular architecture, and exhibit other features of a common banal naevus, including circumscription and at least relative symmetry.
Naevoid melanoma lacks the prominent junctional activity and pagetoid spread usually associated with superficial spreading melanoma.
Common intradermal naevus “matures” (there is an overall decrease in nest size and cellular and nuclear size with depth).
On low power examination, naevoid melanoma may appear to mature with depth, yet closer inspection reveals that the cells at the base of the lesions are similar in size to those of the superficial dermal component.
Only at higher power the distinguishing characteristics of naevoid melanoma can be appreciated.
These features include :
1) A monotonous population of small round cells with prominent nucleoli.
2) From a few to numerous mitoses any where in the lesion.
3) Other features that may be present include individual cell necrosis and atypical mitoses.

Dermatopathology Case 107

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Case 107

A 45 year old female with a pigmented lesion on the left side of the chest.
Diagnosis

Monday, November 28, 2011

Answer of Dermatopathology Case 106


Desmoplastic Spitz Naevus

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Abstract:

Desmoplastic Spitz nevus: a histopathological review and comparison with desmoplastic melanoma. Ann Dermatol Venereol. 2009 Oct;136(10):689-95.
INTRODUCTION: Desmoplastic Spitz nevus is a rare variant of spitzoid tumours characterized by dermal proliferation of large epitheliod and/or fusiform melanocytes within a desmoplastic stroma, comprising thick, eosinophilic collagen bundles. It occurs most frequently in young adults and children, and exhibits a predilection for the limbs. Histologically, this entity may be mistaken for cutaneous fibrous tumours or desmoplastic melanoma. To establish useful histological criteria for differential diagnosis of desmoplastic Spitz nevus, we conducted a retrospective study comparing data concerning the clinical, demographic and histological characteristics of desmoplastic Spitz nevus and desmoplastic melanoma.
PATIENTS AND METHODS: We reviewed the slides for 28 cases of desmoplastic Spitz nevus and for 13 cases of desmoplastic melanoma obtained from the files of Pinkus Dermatopathology Laboratory (Monroe, MI, USA) during the period 1993-2005.
RESULTS: Desmoplastic Spitz nevus was more common in women (sex ratio M/F=0.4) and younger patients (mean age: 28.2 years) whereas the mean age of patients with desmoplastic melanoma was significantly higher (mean age: 75 years). From a histopathological point of view, symmetry, circumscription, melanocytic maturation and involvement of adnexal structure were significantly more frequent in desmoplastic Spitz nevi. The presence of melanocytic junctional nests associated with discohesive cells, variations in size and shape of the nests, lentiginous melanocytic proliferation, actinic elastosis, pagetoid spread, dermal mitosis, perineural involvement and brisk inflammatory infiltrate were significantly more frequent in desmoplastic melanoma. No significant difference was found concerning epidermal hyperplasia, presence of Kamino bodies or moderate inflammatory infiltrate.
CONCLUSION: The combination of architectural and cytological features was useful in differentiating desmoplastic Spitz nevus from desmoplastic melanoma. A complete excision biopsy is mandatory in such tumors as the architectural criteria are necessary for a correct diagnosis.

Dermatopathology Case 106

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Case 106

A 28 year old female with a reddish brown nodule on the left leg.
Diagnosis

Sunday, November 27, 2011

Answer of Dermatopathology Case 105

Immunostaining, staining reveals that the epithelial cells are positive for cytokeratin , estrogen receptor (ER) and progesterone receptor (PR).


PR (Progesterone Receptor):Positive


ER (Estrogen Receptor): Positive


Cytokeratin: Positive




Abstract:

Cutaneous ciliated cyst of the right lower leg. Pathol Int. 1999 Apr;49(4):354-7.
A 23-year-old Japanese woman with a cutaneous ciliated cyst on her right lower leg is reported. A subcutaneous cyst, measuring 2.5 cm in diameter with papillary projections into the lumen, was lined with ciliated cuboidal to columnar epithelia with partial stratification, histologically. These lining cells did not produce mucin. Immunohistochemically, the ciliated lining cells of the cyst were diffusely positive to epithelial membrane antigen and cytokeratin. In addition, positive immunoreaction with anti-desmin monoclonal antibody was observed in the body of the cilia. Less than 10% of the epithelial cells revealed positive immunoreaction to S-100 protein and estrogen receptor.

Dermatopathology Case 105

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Case 105

A 38 year old female with a cystic lesion on the lower part of the left leg. The lesion is 2.5cm in diameter.


Diagnosis

Saturday, November 26, 2011

Answer of Dermatopathology Case 104

Allergic Granulomatosis (Churg-Strauss Syndrome)

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The three major microscopic features of Allergic Granulomatosis are:
1) Necrotizing vasculitis
2) Tissue infiltration by eosinophils
3) Extravascular granulomas
It should be noted that the 3 features mentioned above do not always coexist.

In Case No 104 there is small-vessel necrotizing vasculitis together with extensive tissue infiltration by eosinophils. No granulomas were present in the multiple sections taken. Hence in this case detailed clinical history played a very important role to reach the final diagnosis.

Dermatopathology Case 104

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Case 104

A 42 year old female with history of asthma and allergic rhinitis. There is marked peripheral blood eosinophilia. Erythemous macules are present on the extremities. Biopsy from the skin lesion.

Diagnosis

Thursday, November 24, 2011

Answer of Dermatopathology Case 103

Infantile Hemangioma

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Abstract:

Comparative study on pathology of noninvoluting congenital hemangioma and infantile hemangioma.Zhonghua Zheng Xing Wai Ke Za Zhi. 2011 May;27(3):178-81

OBJECTIVE: To distinguish noninvoluting congenital hemangioma (NICH) and infantile hemangioma (IH) by comparing the pathological structure and marker antigen expression.
METHODS: From Jan. 2005 to Aug. 2010, 39 paraffin-embedded samples, including 13 cases of NICH, 13 cases of proliferating IH and 13 cases of involuting IH, were collected from operation. Hematoxylin-eosin staining was used to observe the pathological structure. Immunohistochemical analysis was also performed to investigate the expression of Glut-1.
RESULTS: The lobules of capillaries were well-defined in NICH. The lobules were surrounded by abundant fibrous tissue. The capillaries were often large and integrity in NICH. There were few mitosis and apoptosis in endothelial cells and stromal cells in NICH. While in IH, the pathologic findings were totally different. Immunochemistry revealed that the Glut-1 was expressed in endothelial cells of IH, but not in NICH.
CONCLUSIONS: NICH has a steady histologic structure and low proliferation, while the endothelial cells in proliferative IH has a high proliferation. Glut-1 can be used as the reliable marker antigen for differential diagnosis of NICH and proliferative infantile hemangiomas.

Dermatopathology Case 103

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Case 103

A 9 month old child with a reddish nodule on the forehead.


Wednesday, November 23, 2011

Answer of Dermatopathology Case 102

Acrodermatitis Chronica Atrophicans

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There is superficial and deep chronic inflammatory cellular infiltrate in the dermis.
It is moderately heavy and composed mainly of lymphocytes together with histiocytes and plasma cells.
There is telangiectasia and the cellular infiltrate is prominent around the blood vessels.
Inflammatory cells are also present around the adnexae.
Thickened collagen bundles are haphazardly present in the upper half of the dermis.
There is some atrophy of the dermis and of the subcutis. The epidermis also shows variable atrophy with loss of rete pegs.

Dermatopathology Case 102

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Case 102


A 68 year old male with ill-defined erythema on the extensor surface of the lower end of the right leg extending to the dorsum of the foot.There is associated wrinkling of the skin.


Monday, November 21, 2011

Dermatopathology Case Index: Case 81 to Case 100


Dermatopathology Quiz - Case Index

Case 81 = Langerhans Cell Histiocytosis

Case 82 = Rheumatoid Nodule

Case 83 = Gouty Tophus

Case 84 = Polymorphous Light Eruption

Case 85 = Graft-Versus-Host Disease

Case 86 = Pilar Sheath Acanthoma

Case 87 = Angiokeratoma

Case 88 = Neurothekeoma (Nerve Sheath Myxoma)

Case 89 = Digital Mucous Cyst

Case 90 = Lichen Simplex Chronicus

Case 91 = Proliferating Trichilemmal Cyst (Pilar Tumour)

Case 92 = Subcorneal Pustular Dermatosis (Sneddon-Wilkinson disease)

Case 93 = Urticaria Pigmentosa

Case 94 = Hypertrophic Lichen Planus

Case 95 = Papillary Eccrine Adenoma

Case 96 = Primary Cutaneous Anaplastic Large Cell Lymphoma

Case 97 = Mycosis Fungoides-associated Follicular Mucinosis

Case 98 = Epithelioid Hemangioendothelioma

Case 99 = Kaposiform Hemangioendothelioma

Case 100 = Retiform Hemangioendothelioma

Answer of Dermatopathology Case 101

Microvenular Hemangioma


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Abstract:

Microvenular hemangioma. Ann Dermatol Venereol. 2004 May;131(5):475-7.
INTRODUCTION:
Microvenular hemangioma belongs to the group of acquired vascular tumors. It is important to differentiate such lesions because of their prognostic and nosologic consequences. We report a case of microvenular hemangioma.
CASE REPORT:
A 31 year-old man presented with a 3 cm erythematous and asymptomatic nodule of the abdomen, which had grown for 2 months. Histopathology showed the irregular dermal proliferation of small vessels, composed of capillaries and venules, without atypia. No relapse was noted 6 months after complete exeresis.
DISCUSSION:
Microvenular hemangioma is a recently described vascular tumor. The first three cases were reported in 1989, with the denomination of "microcapillar hemangioma". Twenty-one further cases have been reported since 1991. We discuss the typical clinical and histological characteristics of this lesion and present criteria permitting the differential diagnosis with other vascular neoplasms. Dermatologists should be aware of this lesion, notably for the differential diagnosis with early onset Kaposi's disease.

Dermatopathology Case 101

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Case 101

A solitary erythematous nodule on the right forearm of a 45 year old woman.

Diagnosis