摘要:

Rosai-Dorfman disease (RDD) is a rare but distinctive clinicopathologic entity of unknown etiology affecting lymph nodes as well as extranodal sites. Although cutaneous involvement in RDD is common, purely cutaneous disease is rare and not well documented. We report 22 patients with cutaneous and superficial subcutaneous RDD. The lesions presented as papules and nodules, often with discoloration (9/22) and frequent multifocality (13/22), without predilection for a specific site of the body. Age distribution was wide and ranged from 15 to 68 years, with a median of 43.5 years. Of the 17 patients for whom information on racial background was available, 7 were Asian, 8 were white, and 2 were black, with a marked female predominance (2:1). The lesions resolved in 6 of 13 patients for whom follow-up data were available, regardless of the treatment given. Lesions persisted or recurred in 7 patients. Histologically, the lesions are invariably characterized by a proliferation of polygonal S100-positive histiocytes showing emperipolesis and a mixed inflammatory infiltrate. This study characterizes the histologic spectrum of cutaneous RDD in regard to variation in the numbers of typical S100-positive histiocytes and emperipolesis, variation in the quality and quantity of the inflammatory response, and the degree of stromal fibrosis, which resulted in a strikingly storiform growth pattern in six lesions and a lobulated pattern in two lesions. Whereas the clinical as well as histologic appearance of the cutaneous and subcutaneous lesions in the purely extranodal forms of RDD is indistinguishable from that of systemic RDD, this study emphasizes that purely cutaneous RDD is a distinct clinical entity in regard to its epidemiology and remains localized to the skin even with long-term follow-up. Patients with purely cutaneous RDD are of an older age at onset of disease (median = 43.5 years), with a reversed male/female ratio. There are no significant systemic extracutaneous or serologic manifestations. Whereas systemic RDD is commonly seen in blacks and only rarely reported in Orientals, the majority of the patients in this series with purely cutaneous RDD are Asians and whites.

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Tattoo reactions are histologically diverse. In general, dermal changes predominate, although epidermal changes such as acanthosis or spongiosis can also be seen. The chronic inflammatory cell infiltrate can be nodular, lichenoid, or granulomatous. Occasionally, the dermal infiltrate may be so dense as to suggest a diagnosis of cutaneous lymphoma. We report an unusual tattoo reaction that mimicked morphea histologically.

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

A 53-year-old man reported a pigmented lesion on his forearm that he had first become aware of approximately 30 years previously. More recently, the lesion had become symptomatic and was excised because of concern of melanoma; however, during tissue processing, an embedded metallic object was found. Histologic examination of the tissue surrounding the site of the metallic object confirmed an inner zone of iron deposition with a distinctive histologic appearance indistinguishable from rust, associated with a foreign body reaction. Surrounding this was an outer zone of siderophages with the more usual histologic appearance. Chemical analysis of the foreign body confirmed its ferruginous nature. On subsequent questioning, the patient informed us that he had worked with heavy equipment in a mine at the time that he had first noticed the lesion.

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Plexiform fibrohistiocytic tumor (PFT) is a rare but distinctive soft tissue tumor of children and young adults characterized by a mixture of histiocyte-, myofibroblast-, and osteoclast-like giant cells arranged in a plexiform pattern. We report the clinicopathologic and immunohistochemical features of an apparently unique case of PFT without multinucleated giant cells presenting in a 3-year-old child. Light microscopy revealed a subcutaneous tumor composed of a plexiform proliferation of histiocyte- and myofibroblast-like cells. Multinucleated osteoclast-like giant cells, the third classic cellular component of this mesenchymal neoplasm, were not observed. The differential diagnosis is also discussed. Emphasis is placed on the importance of the recognition of PFT, because it may exhibit an aggressive behavior.

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

We reviewed the pathologic findings on a family with multiple hereditary trichoepitheliomas. Although the majority of the lesions were trichoepitheliomas, basal cell carcinomas, spiradenomas, and spiradenomas with cylindromatous foci (spiradenocylindroma) were present, representing a spectrum of lesions exhibiting folliculosebaceous (trichoepithelioma, basal cell carcinoma) and apocrine (spiradenoma, spiradenocylindroma) differentiation. Multiple familial trichoepitheliomas may be a syndrome whereby tumors develop from undifferentiated germinative cells of the folliculosebaceous-apocrine unit. Published findings regarding the genetics of this syndrome and solitary trichoepitheliomas are reviewed; although the molecular basis for the tumors has yet to be determined, current data suggest that a tumor suppressor gene may be involved.

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Tumor of the follicular infundibulum (TFI) is a rare epithelial tumor, and its histogenesis is still somewhat debatable. The diagnosis of TFI cannot currently be made before biopsy, and it is most often misdiagnosed as basal cell carcinoma (BCC). A 78-year-old woman presented to the dermatology clinic with a slightly erythematous nodule surrounded by fine telangiectasia on her right temple. Histopathologic examination of the lesion revealed an epithelial tumor with a plate-like growth pattern as is typical of TFI. In the same specimen, an unusual trichilemmal tumor with multiple epidermal connections was observed adjacent to the foci of TFI. The lobular tumor was composed of a large mass of keratinocytes with pale cytoplasm arranged as sheets and interconnecting ribbons. Several cells with hyperchromatic nuclei and small foci of compact cornification were also noted within the bulk of the tumor. The histopathologic features of the unusual tumor were reminiscent of BCC with trichilemmal differentiation or desmoplastic trichilemmoma.

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Syringoid eccrine carcinoma is an extremely rare cutaneous malignant tumor, thought to be derived from eccrine sweat apparatus. We report a case of syringoid eccrine carcinoma occurring on the scalp of a 66-year-old woman and analyzed its cytokeratin expression immunohistochemically to clarify its histogenesis. The tumor consisted mainly of numerous small cords and nests extending from the reticular dermis to the subcutaneous tissue, which formed luminal or tubular structures mimicking the nests of syringoma. Immunohistochemical analysis revealed that most tumor cells expressed simple epithelial cytokeratins (CKs 7, 8, 18, 19) suggesting their sweat secretory differentiation, and that a small number of tumor cells showed an expression of stratified epithelial cytokeratins (CKs 5, 14) suggesting their ductal differentiation. We believe that the syringoid eccrine carcinoma of our case may differentiate mainly toward the sweat secretory cells rather than toward the dermal ductal cells.

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Primary cutaneous posttransplantation B-cell lymphoproliferative disorder is rare. The few previously reported patients were all treated with surgery, radiotherapy, or lowering of immunosuppression. We describe a 65-year-old woman presenting with an intermammary skin ulcer 21 years after renal transplantation, proving on biopsy to be an Epstein Barr virus (EBV)–related posttransplantation B-cell lymphoproliferative disorder. A few weeks later,without further clinical intervention,the skin ulcer showed complete clinical regression. Hematologic staging evaluation showed no evidence of extracutaneous involvement. Despite continuation of immunosuppression, the patient stayed free of disease until 18 months after initial diagnosis, when she developed a progressive hemiparesis and died of acute myocardial infarction. At autopsy, a recurrent B-cell posttransplantation lymphoproliferative disorder in the left side of the thalamus region (measuring 1 × 0.8 cm) was established. The long interval between the primary cutaneous lesion and the localized brain recurrence supports primary skin posttransplantation lymphoproliferative disorder, especially because the patient was not treated for her posttransplantation lymphoproliferative disorder. Review of the literature on primary cutaneous posttransplantation B-cell lymphoproliferative disorder and this case gives the impression that cutaneous posttransplantation B-cell lymphoproliferative disorders of B-cell lineage behave in a more benign manner than identical lesions arising extracutaneously. Because of the rare occurrence of posttransplantation B-cell lymphoproliferative disorder primarily involving the skin, extracutaneous origin should be excluded. If B-cell lineage can be established, EBV is present, alterations in oncogenes or tumor suppressor genes associated with malignant lymphoma are absent, and bcl-6 gene mutation associated with progression is absent, initially aggressive treatment might be avoided. However, long-term clinical follow-up with prolonged maintenance therapy (reduction of immunosuppression or antiviral therapy) for prevention of recurrent posttransplantation lymphoproliferative disorder seems indicated, as is demonstrated by the case reported in the current study.

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID

摘要:

Follicular mycosis fungoides (FMF) is a rare cutaneous T cell lymphoma characterized by an atypical lymphoid infiltrate spreading within and around hair follicles without epidermotropism or follicular mucin deposits. Its occasional presentation with minimal epidermal involvement and/or follicular mucinosis suggests the need for uniform histologic criteria. We describe a new case of FMF associated with follicular mucinosis and discuss its morphologic spectrum of presentation.

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID

ISSN:0193-1091    

出版商:OVID    

年代:2002

数据来源: OVID