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.

Saturday, January 30, 2010

Answer of Dermatopathology Case 37

Dermatitis Herpetiformis

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Dermatitis herpetiformis. An update of the pathogenesis.Hautarzt. 2009 Aug;60(8):627-30, 632.

The multifactorial pathogenesis of dermatitis herpetiformis is reviewed in light of current experimental data. Genetic background, gluten consumption, and abnormal immune and autoimmune reactions are the most important pathogenetic factors, but other agents also participate in the disease development. The predisposing and inducing factors are summarized, while the pathophysiological steps leading to the development of skin symptoms are detailed.

Dermatitis herpetiformis. A clinical chameleon.Hautarzt. 2006 Nov;57(11):1021-8; quiz 1029.

Celiac disease is a genetically determined bowel disease also influenced by exogenous factors in which exposure to grain components triggers a chronic immune response with intestinal symptoms. Dermatitis herpetiformis represents the cutaneous manifestation of celiac disease. While intense pruritus is the characteristic symptom, clinical signs can be highly variable, ranging from grouped papulovesicles with excoriations or eczema-like lesions to minimal variants of discrete erythema and digital purpura. Diagnosis depends on direct fluorescence studies of perilesional skin displaying granular IgA deposits in dermal papillae. Suspecting and then searching for dermatitis herpetiformis is often clinically challenging, as the disease is a true chameleon with many clinical faces. Dapsone therapy alleviates the cutaneous symptoms and signs, but does not prevent the systemic complications of celiac disease; thus, strict adherence to a gluten-free diet is strongly advisable.

Autoantibodies against epidermal transglutaminase are a sensitive diagnostic marker in patients with dermatitis herpetiformis on a normal or gluten-free diet. J Am Acad Dermatol. 2009 Jul;61(1):39-43. Epub 2009 Apr 2.

BACKGROUND: Dermatitis herpetiformis (DH) is a cutaneous manifestation of gluten-sensitive enteropathy (celiac disease). Patients with DH demonstrate circulating IgA antibodies against epidermal transglutaminase (eTG) and tissue transglutaminase (tTG). It has been suggested that eTG is the autoantigen of DH. OBJECTIVE: The purpose of this study was to characterize the autoimmune response to eTG and tTG in patients with DH on a normal or gluten-free diet (GFD). METHODS: Sera from 52 patients with DH were studied for the presence of IgA antibodies to eTG and tTG by enzyme-linked immunosorbant assay. In 38 patients, serum was obtained before initiation of a GFD, whereas 14 patients had been on a GFD for at least 2 years. RESULTS: Autoantibodies against eTG were detected in 36 of 38 patients (95%) and those against tTG in 30 of 38 patients (79%) with DH on a normal diet. Of 14 patients on a long-term GFD, 7 patients were free of DH lesions and did not require dapsone treatment. None of these patients showed circulating antibodies against eTG or tTG. The remaining 7 patients on a GFD were not able to stop taking dapsone. All these patients demonstrated anti-eTG antibodies, whereas only 3 of them showed additional reactivity against tTG. LIMITATION: Autoantibody levels against eTG and tTG before and after introduction of a GFD were not examined in the same patients. CONCLUSION: Our data suggest that antibodies to eTG are the most sensitive serologic marker in treated and untreated patients with DH and confirm the central role of eTG in the pathogenesis of this disease.

Dermatopathology Case 37


Case 37

A 35 year old man with small intensely itchy blisters and papules on both the elbows. The patient has a history of celiac disease.

Thursday, January 21, 2010

Answer of Dermatopathology Case 36

Angiolymphoid Hyperplasia with Eosinophilia (Epithelioid Hemangioma)

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Epithelioid hemangioma (angiolymphoid hyperplasia with eosinophilia) of theorbit: a case report. J Med Case Reports. 2007 Jun 25;1:30.
ABSTRACT: BACKGROUND: Angiolymphoid hyperplasia with eosinophilia (ALHE) and Kimura's Disease (KD) share many clinical and histopathological features. Although they were once considered different stages of the same disease, they arenow known to represent separate entities. Recently, ALHE is being called epithelioid hemangioma (EH), a term that better describes the possible neoplastic nature of the entity. CASE PRESENTATION: An eighteen year-old Asian female presented with a three-month history of fluctuating swelling and ptosis of the left upper eyelid. Computed tomography disclosed a distinct homogeneous lesion in the left superior orbit, molding to the globe and other orbital structures. At histopathological evaluation the lesion was composed of numerous blood vessels lined by plump endothelial cells with oval nuclei protruding into the lumen. Surrounding the vessels, there was a chronic inflammatory infiltrate with a large proportion of eosinophils. Based on clinical and histopathological findings, the diagnosis of EH was made. CONCLUSION: Although exams like blood count, urinalysis and whole body scans can assist in the differential diagnosis, EH can be diagnosed and differentiated from KD on histopathological grounds. The presenceof vascular hyperplasia with plump endothelial cells protruding into the lumen is the most important discriminator in establishing the diagnosis of EH. Such distinction is crucial for the patient because EH is not associated with any ofthe systemic manifestations present in KD.

Angiolymphoid hyperplasia with eosinophilia (epithelioid hemangioma) of the lung: a clinicopathologic and immunohistochemical study of two cases. Am J Clin Pathol. 2005 May;123(5):762-5.
Two cases of primary angiolymphoid hyperplasia with eosinophilia (epithelioidhemangioma) (ALHE/EH) of the lung are described. Both patients are white, a 60-year-old man and a 27-year-old woman. One patient had a long-standing history of asthma, and the other had symptomatology related to the pulmonary mass. Wedge resections were performed in both cases, and both lesions shared similar histopathologic changes, mainly the presence of a tumor mass with a marked presence of eosinophils in the background, lymphoid hyperplasia, and marked proliferations of small-caliber vessels. Immunohistochemical studies using leukocyte common antigen, L-26, and UCHL-1 nicely stained the lymphoid component of the lesion, and CD31 clearly outlined the vascular component of the process. Clinical follow-up demonstrated that the woman died of status asthmaticus, andthe man was alive and well 1 year after surgical resection of the lesion. Both cases highlight the ubiquitous distribution of ALHE/EH and underscore theimportance of keeping these lesions in the differential diagnosis of vascular and lymphoid lesions of the lung.

Dermatopathology Case 36

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Case 36

A 32 year old Asian man presenting with a dome-shaped, crusted nodule (3 cm in diameter) on the scalp.

Answer of Dermatopathology Case 35

Chromomycosis (Chromoblastomycosis)

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Difficult mycoses of the skin: advances in the epidemiology and management of eumycetoma, phaeohyphomycosis and chromoblastomycosis.Curr Opin Infect Dis. 2009 Dec;22(6):559-63.
PURPOSE OF REVIEW: Eumycetoma, phaeohyphomycosis and chromoblastomycosis are subcutaneous mycoses having in common the fact that they are acquired as a result of penetrating trauma to the skin and may be caused by a large variety of fungi. This article will review recent data regarding the epidemiology and treatment of these infections. RECENT FINDINGS: Recent epidemiologic observations in these mycoses include an increased incidence of phaeohyphomycosis in immunosuppressed patients, the association of polymorphisms in genes involved in innate immunity, the occurrence of eumycetoma caused by Madurella mycetomatis and the nosocomial acquisition of phaeohyphomycosis. The management of these infections continues to be challenging. An approach that involves early diagnosis, the use of systemic antifungal agents and local therapies, including surgical removal of lesions, is the basis of the treatment of these diseases. SUMMARY: It is crucial that the epidemiology and clinical presentation of these infections are understood if weare to improve their outcomes.

Chromoblastomycosis: an overview of clinical manifestations, diagnosis and treatment.Med Mycol. 2009 Feb;47(1):3-15. Epub 2008 Dec 9.
Chromoblastomycosis is one of the most frequent infections caused by melanized fungi. It is a subcutaneous fungal infection, usually an occupational related disease, mainly affecting individuals in tropical and temperate regions. Although several species are etiologic agents, Fonsecaea pedrosoi and Cladophialophora carrionii are prevalent in the endemic areas. Chromoblastomycosis lesions are polymorphic and must be differentiated from those associated with many clinical conditions. Diagnosis is confirmed by the observation of muriform cells in tissue and the isolation and the identification of the causal agent in culture. Chromoblastomycosis still is a therapeutic challenge for clinicians due to the recalcitrant nature of the disease, especially in the severe clinical forms.There are three treatment modalities, i.e., physical treatment, chemotherapy and combination therapy but their success is related to the causative agent, the clinical form and severity of the chromoblastomycosis lesions. There is no treatment of choice for this neglected mycosis, but rather several treatment options. Most of the patients can be treated with itraconazole, terbinafine or a combination of both. It is also important to evaluate the patient's individual tolerance of the drugs and whether the antifungal will be provided for free or purchased, since antifungal therapy must be maintained in long-term regimens. In general, treatment should be guided according to clinical, mycological and histopathological criteria.

Dermatopathology Case 35

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Case 35

A 42 year male with a verrucous, warty plaque in the distal part of the left extremity.

Wednesday, January 20, 2010

Answer of Dermatopathology Case 34

Bacillary Angiomatosis

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Bartonellosis. Clin Dermatol. 2009 May-Jun;27(3):271-80.
Bartonella spp are fastidious bacteria that occur in the blood of man and mammals; they are usually vector borne but can also be transmitted by animal scratches and bites. The bartonelloses of medical importance comprise Carrión's disease, trench fever, cat-scratch disease, bacillary angiomatosis, and peliosis hepatis. Carrión's disease, known as Oroya fever in the acute phase and verruga peruana (Peruvian wart) in its chronic form, has curious manifestations that,until recently, have been restricted in their geographic distribution to dwellers of the high, dry Andean valleys, but new sites of disease are emerging. Trench fever is associated with louse-borne disease and homelessness. Cat-scratch disease, bacillary angiomatosis, and peliosis hepatis are increasingly being recognized as causes of human disease, especially in susceptible population groups such as HIV-infected persons. The Bartonella spp are considered emerging human pathogens. The clinical manifestations, differential diagnosis, laboratory diagnosis, and treatment of these conditions are discussed.

Bacillary angiomatosis.J Dtsch Dermatol Ges. 2009 Sep;7(9):767-69.
An infection with Bartonella henselae transmitted from domestic cats to humans by scratching normally leads to cat-scratch disease. When the human host has severe immunosuppression or HIV infection, the potentially life-threatening disease bacillary angiomatosis can develop. A 79-year-old man presented with livid-erythematous, angioma-like skin lesions. We considered a cutaneous infiltrate from his known chronic lymphocytic leukemia, Merkel cell carcinoma, cutaneous metastases of internal tumors, cutaneous sarcoidosis, mycobacterial infection and even atypical herpes simplex infection. The correct diagnosis was proven histologically and by PCR. Because of increasing numbers of immunosuppressed and HIV-positive patients, as well as an infection rate of 13% for B. henselae in domestic cats in Germany, one must be alert to the presence of bacillary angiomatosis.

Bacillary angiomatosis: microbiology, histopathology, clinical presentation, diagnosis and management.Bol Asoc Med P R. 1996 Apr-Jun;88(4-6):46-51.
Bacillary angiomatosis is known to be caused by a rickettsial organism; Rochalimaea henselae. This causative agent has been compared with different microorganisms and clinical conditions that appear in similar settings but thathave been clearly differentiated from them; i.e. Cat-scratch disease (Afipiafelis), Bartonella bacilliformis, other Rochalimaea sp., Kaposi's sarcoma, Lobular capillary hemangioma, Angiosarcoma, and Epithelioid hemangioma. Clinically the bacillary angiomatosis (BA) skin lesions vary from a single lesionto thousands. The cutaneous lesion appears as a bright-red round papule, subcutaneous nodule, or as a cellulitic plaque. When the lesion is biopsied it tends to blanch-out, bleed, and cause pain. The patient might present with signs and symptoms of chills, headaches, fever, malaise, and anorexia with or without weight loss. The extracutaneous lesions found in BA tend to be from multiple organs affecting from the oral lesions to anal mucosal lesions to widespread visceral lesions. The sites of preferences for BA lesion manifestation tend to be the liver, spleen , lymph nodes, and bone. To diagnose bacillary angiomatosis the physician should prepare a differential diagnosis based primarily on its histopathological and clinical characteristics. To confirm the results from the stain, electron microscopy can identify the bacillus and pin-point the diagnosis of bacillary angiomatosis. The lesions presented by BA respond well to therapy with erythromycin 500mg four times daily for a duration of 2 weeks to 2 months. In case of intolerance to erythromycin the second line of drug that successfully treats the BA bacillus is doxycycline. If relapses of the BA lesion recur, then a prolonged antibiotic therapy is necessary and in AIDS patients the duration maybe extended as life-long suppressive therapy.

Dermatopathology Case 34



Case 34

A 36 year old HIV- positive woman presenting with multiple red papules all over her body.

Monday, January 18, 2010

Answer of Dermatopathology Case 33

Glomeruloid Hemangioma

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POEMS syndrome with an isolated lytic lesion and glomeruloid hemangiomas: report of one case. Rev Med Chil. 2009 May;137(5):680-4. Epub 2009 Jul 17.
POEMS syndrome (polyneuropathy, organomegaly, endocrine abnormality, M-protein, plasma cell dyscrasia, and skin lesions) is a rare atypical plasma cell dyscrasia with characteristic para neoplastic manifestations. Glomeruloid hemangioma is a typical skin change pathogenetically related with elevated levels of Vascular Endothelial Growth Factor (VEGF). We report a 69 year-old woman that presented cachexia associated with diabetes, hypothyroidism and severe sensitive motor polyneuropathy. Her skin changes included hyper pigmentation, acrocyanosis and glomeruloid hemangioma. The subsequent study revealed a monoclonal gammopathy
lambda type; a unique lytic vertebral lesion and a clonal plasma cell proliferation. Treatment with prednisone 0.5 mg/kg and melphalan 0,25 mg/kg in cycles of 4 days every 4 weeks was started, but the patient was lost from follow up.

Glomeruloid haemangioma is not always associated with POEMS syndrome.Clin Exp Dermatol. 2009 Oct;34(7):800-3. Epub 2008 Dec 9.
Glomeruloid haemangioma is considered a specific marker of POEMS (polyneuropathy, organomegaly, endocrinopathy, M-protein and skin changes) syndrome and it is usually but not always associated with multicentric Castleman's disease. We report a 78-year-old man who presented with a single, red-violet soft nodule with superficial telangiectases on the scalp. Histopathologically, the lesion consisted of lobules of coiled aggregated capillaries that involved the lumina of dilated vascular structures, mimicking renal glomeruli.
A collagenous stroma separated the capillary lobules, and eosinophilic, periodic-acid-Schiff positive globules of varying sizes and shapes were seen within the cytoplasm of endothelial cells. Immunohistochemical studies with antibodies against IgA and IgG, and against the kappa and lambda light chains of immunoglobulins showed immunoreactivity within the eosinophilic globules. Results of complete blood count, liver, renal and thyroid function tests, fasting blood sugar measurement, serum levels of oestradiol, testosterone, prolactin and cortisol, serum protein electrophoresis, immunoelectrophoresis and immunofixation yielded normal or negative results. No Bence-Jones proteinuria was found in a sample from a 24-h urine collection. To our knowledge, only two cases of glomeruloid haemangioma have been previously reported in patients without POEMS syndrome. We describe the third case of glomeruloid haemangioma in a patient without features of POEMS syndrome.

Eosinophilic globules in 3 cases of glomeruloid hemangioma of the head and neck: a characteristic offering more evidence for thanatosomes with or without POEMS. Am J Dermatopathol. 2008 Dec;30(6):539-44.
Glomeruloid hemangiomas (GHs) are glomeruli-like capillary tufts lined by endothelial cells that contain periodic acid-Schiff (PAS) positive eosinophilic globules (EGs). These hemangiomas are characteristic cutaneous manifestation of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M-protein, and Skin changes). Hemangiomas histologically identical to GHs but not associated with POEMS have recently been designated as papillary hemangiomas. In this report, we present solitary head and neck GHs in 3 patients, 2 without POEMS, with attention to the characteristic EGs. We performed immunostains for hemoglobin A, kappa and lambda light chains, factor VIII-related antigen, CD31 and CD34, PAS stain after diastase digestion (PASD), and electron microscopic examinations on routinely fixed tissues containing EGs. Eosinophilic globules stained uniformly positive for PASD but only peripherally positive for hemoglobin and light chains on surfaces, with interiors negative for antigens. Factor VIII-related antigen and CD31 and CD34 confirmed cells containing EGs to be endothelial. Electron microscopic examination suggested that EGs are enlarged secondary lysosomes (thanatosomes). These features fail to support red blood cells or immunoglobulins as EG constituents. Glomeruloid hemangiomas may be vascular proliferations stimulated by endothelial cells' protein phagocytosis but not by phagocytosis of either hemoglobin -containing red blood cells or
immunoglobulins. The vascular lesions in POEMS syndrome appear identical to papillary hemangioma in cases without the other syndromic manifestations.

Dermatopathology Case 33


Case 33

A female 55 year old with a history of Poem's syndrome. Multiple violaceous papules (5 mm in diameter) are present on the left upper limb.

Answer of Dermatopathology Case 32

Sebaceous Hyperplasia

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Sebaceous gland hyperplasia of the foreskin.Clin Exp Dermatol. 2009 Apr;34(3):372-4. Epub 2008 Dec 9.
Two men, aged in their 20s, presented with multiple, soft, rounded papules on the prepuce. The lesions were centrally umbilicated, resembling molluscum contagiosum, but clearly distinct from Tyson's glands. Surface microscopy showed well-defined, milky-white, bag-shaped structures, which under histological examination were found to be sebaceous glands with various features of hyperplasia. A lymphocytic T-cell infiltrate, closely associated with progressive degeneration and destruction of the sebocytes, was visible around the glands. In the differential diagnosis of penile papular lesions, this unusual clinicalnpresentation supported by dermatoscopy is consistent with preputial sebaceous gland hyperplasia. As both patients had a prominent T-cell infiltration, it ispossible that under inflammatory stimulation, sebaceous glands undergo hypertrophy and gradual central involution.

Comparative clinicopathological study of intraoral sebaceous hyperplasia and sebaceous adenoma.Oral Surg Oral Med Oral Pathol Oral Radiol Endod. 2009 Jan;107(1):100-4.
OBJECTIVE: The objective of this study was to compare the clinicopathological features of oral sebaceous hyperplasia and sebaceous adenoma. STUDY DESIGN:Clinical data, microscopical characteristics, and ki-67 immunoexpression were comparatively analyzed on 2 intraoral sebaceous adenomas, 6 intraoral sebaceous hyperplasias, and 21 normal intraoral sebaceous glands. RESULTS: Clinically, sebaceous glands presented as multiple separated papules, sebaceous hyperplasias as a single enlarged papule, and sebaceous adenoma as a well-defined nodule.Microscopically, sebaceous adenoma presented an increased number of lobules, smaller lobules, and a greater number of germinative/squamous cells. Sebaceous hyperplasia also had an increased number of lobules and fewer number of germinative/squamous cells, as compared to normal oral sebaceous glands. Ki-67 expression was seen only in germinative cells and counts were higher in sebaceousadenomas followed by hyperplasias and normal glands. CONCLUSIONS: Sebaceous hyperplasias and adenomas showed different clinical, microscopic, andproliferative characteristics, suggesting the usefulness of the studied criteria on diagnosis of these uncommon oral lesions.

Dermatopathology Case 32

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Case 32

A 45 year old female with a solitary yellowish, umbilicated papule 2.5mm in diameter. The lesion is located on the left side of the face.


Friday, January 15, 2010

Answer of Dermatopathology Case 31


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Reticulohistiocytoma (solitary epithelioid histiocytoma): a clinicopathologic and immunohistochemical study of 44 cases. Am J Surg Pathol. 2006 Apr;30(4):521-8.
Reticulohistiocytoma and multicentric reticulohistiocytosis are designations for uncommon, incompletely characterized histiocytic proliferations of the skin or soft tissues. In this study, we analyzed a uniform group of44 lesions composed of epithelioid histiocytes, comprising a subset of lesions originally designated as reticulohistiocytoma, and propose designating them as "solitary epithelioid histiocytoma" (SEH), in line with the recently published classification proposal for histiocytic disorders. There were 26 males and 18 females with a median ageof 35 years (range, 2.5-74 years). All patients had a superficial, circumscribed, mildly elevated, solitary lesion (size range, 1.5-11 mm; median, 4 mm), located in the trunk wall (n = 16), lower extremity (n = 12), head and neck (n = 8,including 2 in the oral cavity), upper extremity (n = 6), penis (n = 1), and an unspecified site (n = 1). Histologically, the lesions typically involved upperand mid-dermis and were not ulcerated. They were composed of large epithelioid histiocytes with a varying number of lymphocytes and neutrophils. The histiocytes had abundant, typically densely eosinophilic, cytoplasm and mostly mild, if any, nuclear atypia. Multinucleated forms with randomly oriented nuclei were alsopresent. The histiocytes had low mitotic activity (range, 0-4 mitoses per 10 wide HPFs; median, 1 mitosis per 10 HPFs). The lesions contained varying numbers of CD3-positive T cells, whereas B lymphocytes, plasma cells, eosinophils, and mast cells were scant, if present at all. Immunohistochemically, the epithelioidhistiocytes were positive for CD163, CD68, lysozyme (variably), and vimentin.They often had focal nuclear immunoreactivity for microphthalmia transcription factor, and they sometimes had focal reactivity for Factor XIIIa and S-100protein. Membrane positivity for CD31, CD43, and CD45 was variable. The epithelioid histiocytes were consistently negative for CD3, CD20, CD30, HMB45,and keratins. All 12 patients with follow-up information had an uneventful clinical course with no recurrences (median, 13 years). SEH is a benign, probablyreactive, histiocytic proliferation of unknown etiology. It needs to be distinguished from Rosai-Dorfman disease, juvenile xanthogranuloma, a variety of granulomatous conditions, and some malignant neoplasms, including histiocyticsarcoma, melanoma, and epithelioid sarcoma.

Reticulohistiocytoma involving the skin, subcutaneous tissue and a regional lymph node.Pathol Int.1996 Jul;46(7):531-7.
A 30-year-old female complained of a surface-eroded solitary nodule on the right thigh.
Histologically, the dermal lesion consisted of uniform-sized polygonal cells with eosinophilic, 'ground glass' cytoplasm. Mitoses were infrequent. Under the histopathologic diagnosis of amelanotic melanoma, wide resection of the skin and dissection of the inguinal lymph nodes were performed. The subcutaneous tissue and a lymph node showed nodular proliferation of histiocytoid cells, in association with hemosiderin-laden multinucleated giant cells. The mononuclear cells were immunoreactive for factor XIIIa, while the multinucleated cells were positive for CD68, lysozyme and HLA-DR. In the lymph node tissue, a considerable number of mononuclear cells positive for CD68 were noted. CD34, alpha-smooth muscle actin, desmin and HMB45 were negative. Ultrastructurally, the mononuclear cells were rich in 100 nm vesicles and 180-350 nm lysosome-like granules. Interdigitation of the plasma membranes was seen in the multinucleated cells. The patient did not complain of joint symptoms, and has been disease-free for 5 years. The histologic and immunohistochemical features are consistent with so-called 'reticulohistiocytoma', though the site of histiocytic growth was unusual.

Reticulohistiocytoma and multicentric reticulohistiocytosis. Histopathologic and immunophenotypic distinct entities. Am J Dermatopathol. 1994 Dec;16(6):577-84. Am J Dermatopathol. 1995 Dec;17(6):625-6.
The clinicopathological and immunohistochemical features of four patients with systemic multicentric reticulohistiocytosis (MR) were compared with five cases of solitary and one case of multiple reticulohistiocytoma (RH), which were confined to the skin only. The MR cases mostly affected the limbs of older women, while RH affected young male adults without preference to site. Characteristically, both entities consisted of oncocytic mononuclear histiocytes (with granular eosinophilic cytoplasm similar to oncocytic thyroid cells) and multinucleated histiocytes with a ground-glass appearance, which appeared to be much larger (> 200 microns) and bizarre in cases of RH compared with cases of MR (50-100 microns). In RH a variable number of vacuolated, spindle-shaped, and xanthomatized mononuclear histiocytes were also present. Immunohistochemical profiles showed positivity of mononuclear histiocytes with HHF35, factor XIIIa, and LN3 (HLA-DR), with a variable number of multinucleated histiocytes in RH showing binding with peanut agglutinin. In mono- and multinucleated histiocytes in both entities macrophage markers KP1 (CD68), KiM1P, HAM56, lysozyme, and alpha 1-antitrypsin were positive. However, macrophage markers MAC387 (L1 antigen) and Leu-M1 (CD15) were negative. Vimentin was universally positive in both conditions, with all other markers (S100, desmin, smooth muscle-specific actin, and QBEnd 10 [CD34]) negative. This study shows that histology supplemented by immunocytochemistry delineates MR from RH and immunohistochemical profiles indicate a cell lineage relationship between RH and adult xanthogranuloma.

Solitary reticulohistiocytoma in pregnancy: immunohistochemical and ultrastructural study of a case with unusual immunophenotype.J Cutan Pathol. 1995 Apr;22(2):177-81.
A case of a solitary reticulohistiocytoma during pregnancy is reported. The tumor arose on the right thigh of a 31-year-old woman during the 2nd month of gestation. Physical examination 6 months later revealed a 9 x 6 mm yellowish white papule. A biopsy showed a dermal proliferation of spindle to polygonal cells embedded in fibrous tissue with admixed neutrophils, eosinophils, and lymphocytes. Immunohistochemistry was positive for S-100, factor XIIIa, HLA-DR, KP-1, vimentin, and neuron-specific enolase. Ultrastructurally, the cells showed abundant lipid, lysozomes, and endoplasmic reticulum. No Birbeck granules could be identified. The results suggest an aberrant immunophenotype intermediate between indeterminate cells and dermal dendritic cells. The relationship of these lineages to reticulohistiocytoma and other dermal proliferative disorders is reviewed.

Multicentric reticulohistiocytosis presenting with destructive polyarthritis , laryngopharyngeal dysfunction, and a huge reticulohistiocytoma. J Clin Rheumatol.2006 Oct;12(5):252-4.
Multicentric reticulohistiocytosis (MRH) is a rare multisystemic disease presenting with skin lesions and erosive polyarthritis and is often associated with malignancy. We describe a 60-year-old woman with diffuse papulonodular skin eruptions and progressive osteolytic bone damage over the bilateral hands, humeral head, and acromioclavicular joints within 2 years. Moreover, dysphagia and a hoarse voice occurred in this patient and an unusual huge mass reticulohistiocytoma--developed over the left upper back. Tissue biopsy of the skin lesions, laryngeal nodules, and this large mass showed infiltration of numerous CD68(+) histiocytes and multinucleated giant cells with abundant eosinophilic ground-glass cytoplasm. Combination therapy with steroids and methotrexate improved her cutaneous, joint, and laryngopharyngeal symptoms. The large reticulohistiocytoma resolved with methylprednisolone pulse therapy. This polyarthritis, which can be confused with rheumatoid arthritis, can be diagnosed by careful immunohistochemical examination of biopsies. To prevent the irreversible disease process, early and aggressive therapy is necessary.

Dermatopathology Case 31


Case 31

A 38 year old female with a superficial, circumscribed, mildly elevated, solitary lesion. The lesion is 5 mm in diameter and located on the forehead.


Tuesday, January 5, 2010

Answer of Dermatopathology Case 30


Visit: Xanthelasma;

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Xanthelasma and juvenile xanthogranuloma in a 7-year-old boy. Ann Dermatol Venereol. 2009 Oct;136(10):723-6. Epub 2009 May 15.

BACKGROUND: Palpebrum xanthelasma is the most common type of xanthoma seen inadults but it is extremely rare in children. We report an original case of bilateral xanthelasma palpebrarum associated with juvenile xanthogranuloma (JXG) in a 7-year-old child. Only two cases of xanthelasma in children have been described to date. The association of xanthelasma and JXG has never been described. PATIENTS AND METHODS: A 7-year-old boy presented xanthelasmas on both eyelids. At the same time, pinkish JXG papules appeared on the child's trunk. The boy had been diagnosed at the age of 10 months with myelogenous leukaemia, which was in remission. He also had a familial history of hypercholesterolaemia. The skin lesions were removed and microscopic examination confirmed the diagnosis of xanthelasmas and JXG. DISCUSSION: This patient's presentation is unusual inseveral respects: the presence of xanthelasma in a child, appearance of JXG at an advanced age, and the association of these two diseases in a child with a past history of leukaemia. The occurrence of these skin lesions did not appear to be linked to the history of malignant blood disease in this patient.

Unknown: bilateral symmetrical papules on the eyelid.Dermatol Online J. 2009 May 15;15(5):13.

Eyelid lesions frequently are a diagnostic challenge. We report a case of a 46-year-old woman with a 5-year history of yellowish symmetric progressivelygrowing papules on the eyelids, resembling xanthelasma. A skin biopsy wasperformed that revealed the rare variant of clear cell syringoma. The lesionswere treated with CO2 laser and surgical excision; there was no evidence of recurrence after 6 months of follow-up.

Xanthelasma palpebrarum and its relation to atherosclerotic risk factors and lipoprotein. Int J Dermatol. 2008 Aug;47(8):785-9.

OBJECTIVES: To investigate the association between xanthelasma, atherosclerotic risk factors, and lipoprotein (Lp) (a), and to determine whether xanthelasma may be a cutaneous marker for atherosclerosis. METHODS: One hundred consecutive patients with xanthelasma and 100 age- and sex-matched patients without xanthelasma, seen during the same time period (controls), were included in this study. The prevalence of cardiac risk factors, the rates of atherosclerotic disease, Framingham risk scores, and Lp (a) levels were compared between the patient groups. RESULTS: Hyperlipidemia was found to be significantly more common in patients with xanthelasma (P = 0.001) ; however, the rate of clinically overt cardiovascular disease and future cardiovascular risk, assessed by the Framingham risk score, were similar between the groups. No significant difference was observed in serum Lp (a) levels between the groups. CONCLUSIONS: In patients with xanthelasma, no increase was observed in the rate or risk of cardiovascular disease. Moreover, no relationship was found between Lp (a) levels and xanthelasma.

Xanthelasma and lipoma in Leonardo da Vinci's Mona Lisa.Isr Med Assoc J. 2004 Aug;6(8):505-6.

The painting Mona Lisa in the Louvre, Paris, by Leonardo da Vinci (1503-1506), shows skin alterations at the inner end of the left upper eyelid similar to xanthelasma, and a swelling of the dorsum of the right hand suggestive of asubcutaneous lipoma. These findings in a 25-30 year old woman, who died at the age of 37, may be indicative of essential hyperlipidemia, a strong risk factor for ischemic heart disease in middle age. As far as is known, this portrait of Mona Lisa painted in 1506 is the first evidence that xanthelasma and lipoma were prevalent in the sixteenth century, long before the first description by Addison and Gall in 1851.

Dermatopathology Case 30


Case 30

A 40 year old female with a yellow, soft plaque on the upper eyelid.

Monday, January 4, 2010

Answer of Dermatopathology Case 29

Pilomatrix Carcinoma

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Pilomatrix carcinoma presenting as an extra axial mass: clinicopathological features. Diagn Pathol. 2008 Nov 29;3:47.
ABSTRACT: Pilomatrix carcinoma is the rare malignant counterpart of pilomatrixoma, a skin adnexal tumour originating from hair matrix cells. Pilomatrix carcinoma can arise as a solitary lesion de novo, or through transformation of a pilomatrixoma. Pilomatrixoma was first described erroneously as being of sebaceous gland origin but was later discovered to be derived from hair matrix cells. They are rare, slow growing tumours of the skin found in thelower dermis and subcutaneous fat and are predominantly found in the neck and the scalp. While known to be locally aggressive, no malignant form was thought toexist until it was described relatively recently. Since then, approximately ninety cases of pilomatrix carcinoma have been reported.We report the case of a 41 year old mentally retarded male who had a longstanding lesion in the left neck for approximately fifteen years previously diagnosed as a pilomatrixoma. He presented with severe headache, falls and visual disturbance and a biopsy showed pilomatrix carcinoma of the occipital region which, on computed tomography ( CT ) invaded the occipital bone, the cerebellum and the left temporal lobe. At hisinitial presentation he had a craniotomy and subtotal excision of the lesion but received no adjuvant therapy. After an early intracranial recurrence he had further debulking and adjuvant external beam radiotherapy. He has had no further intracranial recurrence after three and a half years of follow-up. Here wepresent the pathological features of this uncommon tumour.

Pilomatrix carcinoma with visceral metastases: case report and review of the literature. J Plast Reconstr Aesthet Surg. 2009 Dec;62(12):e574-7.
Pilomatrix carcinoma, the malignant equivalent of pilomatrixoma, is rare among skin cancers. In the literature, there have been 80 cases of pilomatrix carcinoma reported, and among them nine were with metastases. The clinical presentation of this case is suggestive for the biology and of the usual history of this neoplasm. The patient was a 53-year-old male who had been treated 2 years earlier for a pilomatrix carcinoma located in the posterior part of the neck. The clinical presentation had been characterised by sudden paraplegia caused by vertebral collapse at T4 due to bone metastases. The patient underwent a firstsurgery for vertebral stabilisation and medullary decompression; then, he had a second operation for the resection of the local relapse of the tumour. Literature review and analysis of this case show that the pilomatrix carcinoma should be regarded as a highly locally aggressive tumour, with a high rate of local recurrence as well as metastases.

An unusual composite pilomatrix carcinoma with intralesional
melanocytes: differential diagnosis, immunohistochemical evaluation, and review of the literature. Am J Dermatopathol. 2008 Apr;30(2):174-7.
We report a case of an extremely rare histologic combination of pilomatrix orpilomatrical carcinoma with admixed melanocytes within the same tumor mass. Pilomatrix carcinoma is a neoplasm of low-grade malignancy that is characterized by a tendency for recurrence but low risk of metastasis. A 77-year-old male presented with a nodule on the bridge of the nose that was excised. Histologically, it was typified by asymmetry and poor circumscription, the presence of several variably sized and shaped basaloid aggregations, and surface ulceration. The tumors were composed of pleomorphic basaloid cells with prominent nucleoli and frequent mitoses (some of which were atypical) accompanied by central areas with keratotic material, shadow cells, and foci of necrosis. In addition, intermingled with the pilomatrix carcinoma, several easily identified pigmented cells with dendritic processes were present singly and as smallaggregates. There was no atypia associated with the melanocytic component.Immunohistochemistry revealed the CK14 to be positive mainly within thekeratinizing and the squamous epithelial elements of the tumor. The melanocytic component was strongly immunoreactive for S100, melanoma cocktail (HMB45 andMelan-A), and microphthalmia transcription factor. Pilomatrix carcinoma with melanocytes should be distinguished from the conventional pilomatrixoma with pigmentation, melanocytic matricoma, melanoma, and pigmented basal cell carcinomawith matrical differentiation. Clinicians and pathologists should be aware of the occurrence of pilomatrix carcinoma with melanocytes because of its potential for diagnosis as melanoma. This peculiar lesion recapitulates the intimate relationship existing between matrical epithelium and melanocytes in the embryonal hair follicle or in the anagen stage of the hair cycle. It is possible that sun damage played a role in stimulating migration of melanocytes among matrical cells in this case.

Dermatopathology Case 29


Case 29

A 56 year old male with a skin lump, 5 cm in diameter, on the posterior part of the neck.