Sneddon-Wilkinson disease treated with etanercept: report of two cases.

Sneddon-Wilkinson disease (SWD), also known as subcorneal pustular dermatosis, is a rare, chronic eruption that is often difficult to treat, particularly in patients who do not respond to or cannot tolerate dapsone. Few case reports exist of patients with SWD treated with antitumour necrosis factor-alpha therapy. We report two patients with SWD refractory to numerous treatments, who responded to etanercept (in combination with low-dose acitretin in one case).

Pleomorphic T-cell infiltrate associated with molluscum contagiosum.

The authors observed a pleomorphic lymphocytic infiltrate composed of CD8 cytotoxic/suppressor T-cells in two pediatric cases associated with molluscum contagiosum. T-cell clonality was not detected. In both cases, the lesions resolved after the biopsy was performed. The patients were otherwise healthy, and no evidence of lymphoproliferative process was detected on follow-up. The authors believe the pleomorphic lymphoid infiltrate is inflammatory and reactive in nature. The close apposition of lymphocytes to molluscum bodies and cytoid bodies with high expression of CD30 and the proliferating marker Ki67 is suggestive of a cytotoxic cell-mediate blastic reaction against poxvirus antigens.

Tubulopapillary hidradenoma-like tumor of the mandible: clinicopathologic and immunohistochemical features.

Tubulopapillary hidradenoma is a benign sweat gland tumor that appears as a well-defined, superficially located dermal nodule. It combines ductal as well as apocrine and eccrine glandular differentiation. Microscopically, the tumor is composed of tubular structures that characteristically show intraluminal non-villous papillary projections and a peripheral myoepithelial cell layer. A tumor that is histologically and immunohistochemically identical to tubulopapillary hidradenoma occurred in the mandible of a 73-year-old man and resulted in considerable diagnostic difficulty. The neoplasm developed in a mandibular cyst and recurred 5 years after initial enucleation. This is the first report of a central (intraosseous) sweat gland adenoma of the mandible. The differential diagnosis and possible histogenesis are discussed.

Cutaneous granular cell angiosarcoma.

The majority of cutaneous angiosarcomas display typical architectural features of irregular anastomosing vascular channels in the dermis and subcutis. Nuclei are usually hyperchromatic and pleomorphic but the volume of cytoplasm of the neoplastic cells is often small. Diagnosis can be made readily on an adequate biopsy. We recently experienced difficulty diagnosing an angiosarcoma composed predominantly of cells with abundant granular cytoplasm. We were able to compare the present case with sections obtained from the only other reported example. The architectural expression of an anastomosing vascular pattern in areas of tumor, combined with the positive staining for Factor VIII-related antigen (FVIIIRAg) and Ulex europaeus agglutinin-1 (UEA1) enabled us to make a diagnosis of angiosarcoma. The tumor failed to stain for the other endothelial markers (CD31 and CD34) which were positive in the original case. A marker for lysosomes (CD68) stained the granules in both cases. The granular cell variant of cutaneous angiosarcoma is very rare. Diagnosis is possible by recognizing the typical anastomosing neoplastic vascular channels at the periphery of the lesion, and by use of a combination of lectin (UEA1) and immunohistochemical (FVIIIRAg, CD34 and CD31) endothelial markers.

Large-cell acanthoma of the skin. A study by image analysis cytometry and immunohistochemistry.

Although large-cell acanthoma is a well-known clinicopathological entity, its biologic spectrum and nature are still subject to debate. We studied seven cases of large-cell acanthoma by image analysis cytometry for DNA content and by immunohistochemistry, using antibodies to proliferating cell nuclear antigen (PCNA)/cyclin. The data were compared with individual cases of seborrheic keratosis (SK), actinic keratosis (AK), and Bowen's disease (BD). The DNA distribution of large-cell acanthoma was variable. There were varying peaks at the DNA index values of 1 and 2 (diploid and tetraploid values), but all cases contained a significant aneuploid population between DNA index of 1 and 2. The mean DNA index was 1.44 (1.27-1.77); 1-20% of the cells exceeded 2, and 0-2% exceeded 3. The DNA index for lesions in the other differential diagnostic groups studied was as follows: SK, 1.0; AK, 1.4; BD, 1.8. The percentage of cells with positive nuclear staining for PCNA/cyclin was < 20% in all cases of large-cell acanthoma. The discrepancy between the high number of aneuploid and tetraploid cells observed on the DNA distribution curve and the lack of evidence for significant proliferation based on immunohistochemical stains suggest that these cells are resting cells with abnormal DNA clone. Although these results provide additional information about the biologic nature of large-cell acanthoma, they do not resolve the controversial nosologic status of lesions in this histologic group.

Adenolipoma of the skin: a report of nine cases.

BACKGROUND: We have identified nine cases of a variant of superficial lipoma that are characterized by the presence of eccrine sweat glands. OBJECTIVE: The purpose of this study was to elucidate the clinical and histopathologic features of this distinctive lesion. METHODS: Nine cases of lipoma that contain eccrine glands were retrieved from our pathology files from 1989 through 1992. Their clinical and histopathologic features were studied and compared with those of conventional lipoma. RESULTS: The clinical features of these lesions did not deviate appreciably from those of the usual lipomas. However, their microscopic appearances differed by the presence of displaced and distorted eccrine glands. One lesion had the added features of a myxolipoma. CONCLUSION: Adenolipoma of the skin is a distinct lesion that can occur in the dermis or subcutaneous tissue.

Fine-needle aspiration biopsy: dermatofibrosarcoma protuberans.

Dermatofibrosarcoma protuberans (DFSP) represents a spectrum of mesenchymal spindle cell tumors that typically involve both dermis and subcutis. Presented herein are six cases of DFSP, four of which were initially diagnosed by FNAB. The cytologic features useful in the identification of this lesion on Papanicolaou- and Diff-Quik-stained smears are discussed. Chief among these are the storiform stromal fragments, presence of entrapped adipose tissue and the recognition of fibrohistiocytic spindle cells. The potential pitfalls and the differential diagnostic possibilities of spindle-cell lesions, particularly those of fibrohistiocytic origin are discussed.

Fibroepithelioma-like changes associated with anogenital epidermotropic mucinous carcinoma. Fibroepitheliomatous Paget phenomenon.

We describe two patients with crusted perineal plaques that were biopsied and diagnosed as Paget's disease. Resection specimens of each contained a dermal mucinous carcinoma with extensive epidermotropism and coexistent epidermal basaloid proliferations closely resembling fibroepithelioma (Pinkus). The presence of the Paget phenomenon was supported by histochemical, immunohistochemical, and ultrastructural evidence. No other primary neoplasms were found in either patient. Followup at 2 1/2 and 3 1/2 years, respectively, has been negative. We conclude that either the fibroepitheliomatous changes may be induced by or may represent a collison (unlikely) with the epidermotropic mucinous carcinoma. It is proposed that the concept fibroepitheliomatous Paget phenomenon be used to stand for the histologic changes common to our cases as well as those previously reported.

Syringomatous squamous tumors of the breast.

Four cases are reported of syringomatous squamous tumors of the breast occurring in women aged 37 to 70 years. The lesions were characterized histologically by relatively well-circumscribed tumor-like nodules composed of a proliferation of teardrop or comma-shaped islands of squamous epithelium. The squamous epithelial islands contained central lumens lined by eosinophilic cuticles and were surrounded by a densely cellular fibrous matrix, thus closely resembling the growth pattern of dermal eccrine syringomas. The lesions appeared to arise de novo from breast parenchyma without evidence of transitions with the surrounding normal or hyperplastic mammary epithelium and were not associated with the overlying skin or nipple epidermis. In all cases, the surrounding breast tissue showed fibrocystic and benign proliferative changes, and in one case, the lesion was found in the vicinity of a large cyst surrounded by microcalcifications. All patients were treated by local surgical excision and have shown no evidence of recurrence over a follow-up period of 1 to 6 years. The histologic differential diagnosis and the possible pathogenesis of these lesions are discussed.

Epidermotropic neuroendocrine carcinoma. Immunohistochemical differentiation from simulators, including malignant melanoma.

Epidermotropic neuroendocrine carcinoma (NEC) is rare. Based on such a case in an 88-year-old woman with a facial NEC showing epidermotropism with a pagetoid growth pattern, we asked whether several similar tumors involving the epidermis could be easily differentiated by immunohistochemical methods. We constructed a panel of control cases (2 each) for NEC, clear cell Bowen's disease (CCBD), Paget's disease (PD), superficial basal cell carcinoma (SBCC), cutaneous T-cell lymphoma (CTTL), and superficial spreading malignant melanoma (SSMM) to compare with our patient. A panel of antibodies including epithelial membrane antigen (EMA), neuron specific enolase (NSE), AE1/3 cytokeratin (CK), carcinoembryonic antigen (CEA), leukocyte common antigen (LCA), S-100, and HMB-45 were applied. Cutaneous NEC controls and our patient's tumor were strongly positive for EMA and NSE and had paranuclear dot-like cytoplasmic positivity for CK. CCBD was moderate to strong for CK. PD was strong for CEA. SBCC was essentially negative for all. CTLL was strong for LCA. SSMM was strong for S-100 and HMB-45. Controls were either negative or weak for the antibodies not mentioned. We conclude that this antibody panel can reliably differentiate these epidermotropic or juxtaepidermal tumors in diagnostic dermatopathology and should be applied to lesions requiring separation beyond H & E capabilities, especially with superficial shave biopsies showing small cell "Pagetoid" growth patterns.

Human papillomavirus--associated vulvar verrucous carcinoma in a 20-year-old with an intact hymen. A case report.

A 20-year-old woman with vulvar verrucous carcinoma, the youngest such patient reported to date, had human papillomavirus DNA 6/11 identified with an in situ hybridization technique. In addition to her youth, the patient was unusual in that she had an intact hymen. Although she denied having engaged in sexual activity, venereal transmission of the virus may have occurred without vaginal penetration.

Deep juvenile xanthogranuloma. Subcutaneous and intramuscular forms.

Juvenile xanthogranuloma occurring in soft tissue is rare and has received little attention. This report describes cases of deep juvenile xanthogranuloma occurring in the soft tissues of three children. Each tumor was a solitary lesion that arose, respectively, in the superficial skeletal muscles of an 8-month-old girl, the subcutis of the scalp of a 3-month-old boy, and the subcutis of the forehead of a 10-year-old girl. Two lesions were grossly firm, tan-yellow, and homogeneous. Histologically, the subcutaneous lesions were relatively circumscribed; the third lesion infiltrated muscle and contained widely separated skeletal muscle fibers. All lesions showed sheets of uniform amphophilic or acidophilic cells with occasional eosinophils and rare Touton giant cells. In two cases and in cutaneous controls, positive immunoperoxidase stains (HAM-56, HHF-35, and vimentin) supported macrophagic-myofibroblastic differentiation. S-100 protein, MAC-387, and factor XIIIa were negative. Electron microscopy in one case also supported macrophagic-myofibroblastic differentiation. Langerhans granules were absent. Follow-up of 7, 6, and 5 years indicated no recurrences. The differential diagnosis includes deep fibrous histiocytoma and cellular subcutaneous neural tumors.

The rule of halves. A method of controlling the uniform "cutting-in" of skin biopsies.

This article describes a method of uniform gross-room sectioning of skin biopsy specimens, especially excisional biopsy specimens, to insure even thickness and maximum control of the tissue before paraffinization. This is accomplished by sequentially halving portions of the specimen after each cut as opposed to serially cutting the skin from one end to the other.