Biphasic sarcomatoid basal cell carcinoma (carcinosarcoma): four cases with immunohistochemistry and review of the literature.

BACKGROUND: Biphasic sarcomatoid carcinoma (BSC), or carcinosarcoma, is an uncommon biphasic neoplasm that has been reported in diverse anatomical sites. The tumor is composed of a malignant epithelial component intimately associated with a malignant mesenchymal component, which may be homologous or heterologous. Twenty-three cases of primary cutaneous BSC have been reported in the English literature. In only eight of these cases was basal cell carcinoma the epithelial component. METHODS: We report a further four cases of primary cutaneous biphasic basal cell carcinoma, and include the clinical, histological and immunohistochemical features. RESULTS: The four cases showed basal cell carcinoma associated with a pleomorphic sarcomatous stroma. In addition, myofibroblastic differentiation and foci of osteoid were present in one case, and leiomyosarcomatous areas in another. The epithelial components were positive for several epithelial markers. The mesenchymal components were positive for vimentin and CD99, and negative for epithelial markers. p53 was positive with equal intensity in both epithelial and mesenchymal components. A significantly worse outcome was observed in patients with tumors measuring 40 mm or more at excision. CONCLUSIONS: The sarcomatous component of the tumor is best regarded as a metaplastic transformation of the carcinomatous component. These tumors are potentially aggressive if incompletely excised, and complete resection is recommended.

Neutrophilic fixed drug eruption.

A 49-year-old man presented with a reproducible, localized amoxycillin-clavulanic acid-induced eruption. The histopathology from lesional skin revealed a neutrophilic dermatosis. These histological findings have not been reported in previous fixed drug eruptions. A brief review is undertaken comparing fixed drug eruption and the group of neutrophilic dermatoses with our case presentation. We propose a new entity of neutrophilic fixed drug eruption.

Treatment of lichen planus.

Lichen planus is an inflammatory pruritic dermatosis in which T lymphocytes attack the basal epidermis, producing characteristic clinical and histological lesions. Mild cases can be treated successfully with rest, topical corticosteroids with or without wet dressings, or occlusion. Severe generalised cases may require systemic corticosteroids. Chronic, aggressive lesions may need additional therapy ranging from psoralen plus ultraviolet A (PUVA) to retinoids or cyclosporin. Complications ranging from infectious, bullous disease or ulceration require special treatment considerations.

Cutaneous and subcutaneous leiomyosarcoma: a clinicopathological review of 14 cases with reference to antidesmin staining and nuclear DNA patterns studied by flow cytometry.

Among 14 patients (11 female and three male) with cutaneous and subcutaneous leiomyosarcomas, metastatic spread occurred in six of the 13 patients with adequate follow-up. The tumours were classified with regard to tumour grade, number of mitotic figures per 10 high-power fields, reactivity to antidesmin antibodies, and DNA pattern as assessed by flow cytometry. Antidesmin antibody staining was present in only six cases and seemed to be less common in the higher-grade tumours. The DNA pattern was aneuploid in five cases and non-classifiable in one case; all of these cases had metastatic spread. None of the eight remaining diploid cases had metastatic spread. DNA content was a strong predictor of metastatic potential.

The Auckland skin clinic tinea pedis and erythrasma study.

Three hundred consecutive patients attending the Auckland Hospital dermatology clinic were examined clinically and microbiologically for evidence of interdigital or plantar mycological or bacterial infection. One hundred and nine patients were shown by laboratory investigation to have an infection. Of these, only 89 displayed clinical signs of infection. Erythrasma, a desquamative condition caused by Corynebacterium minutissimum, was the commonest infection in this study. The commonest dermatophyte isolated was Trichophyton mentagrophytes var interdigitale. There was a significant rise in the incidence of both types of infection with age.

The heterogeneity of Jessner's lymphocytic infiltration of the skin. Immunohistochemical studies suggesting one form of perivascular lymphocytoma.

Seventeen patients (six women and 11 men) with Jessner's lymphocytic infiltration of the skin were studied. Nineteen biopsy specimens were analyzed with the following monoclonal antibodies: UCHL1 (T cells), 4KB5, L26 (B cells), LN1 (germinal center B cells), and BerH2 (Ki-1+ cells). Routine direct immunofluorescence was performed on 15 specimens. In 10 of 19 specimens both B and T cells were demonstrated; the B cells were in close proximity to small blood vessels. In 50% of these cases the B cell component expressed LN1 positivity. We use the term perivascular lymphocytoma to describe this pattern. Nine specimens showed T cells only. No Ki-1+ cells were detected. Routine immunofluorescence studies were performed on 15 specimens. In six of seven specimens in which there was a mixed cell population of B and T cells, results were completely negative. In contrast, six of the remaining eight specimens with a predominantly T cell population revealed weak, patchy, granular, positive labeling for immunocomponents, particularly IgM and C3 at the basement membrane. These findings suggest that Jessner's lymphocytic infiltration is a heterogeneous disorder with at least two separate immunophenotypes. The finding of perivascular follicular center differentiation in more than half the specimens suggests a possible relationship in these cases to other benign lymphoid hyperplasias (pseudolymphomas).

A study of factor XIIIa and MAC 387 immunolabeling in normal and pathological skin.

Immunolabeling with two new antibodies, Factor XIIIa and MAC 387, has been studied in routinely processed biopsy specimens of normal skin, subcutaneous tissues, lymph nodes, and a variety of pathological conditions. These presumptive cell markers of the monocyte-macrophage lineage appear to label totally different and possibly mutually exclusive subsets of cells. In normal skin, Factor XIIIa labeled fixed dermal connective tissue cells, emphasizing their dendritic morphological appearance. Factor XIIIa expression in fibroproliferative conditions and spindle cell tumors varied: in particular, scars, keloids, and dermatofibrosarcomas do not label, whereas histiocytomas, fibrous papule of the nose, and atypical fibroxanthomas were well labeled. In inflammatory conditions, increased numbers of Factor XIIIa cells were found, but most macrophages and epithelioid and multinucleate cells did not label. In contrast, normal dermal connective tissue cells did not label with MAC 387, nor did the constituent cells of various fibroproliferative disorders. In inflammatory conditions, variable numbers of MAC 387-positive cells were observed, corresponding to histiocytes and macrophages, but labeling of epithelioid cells and multinucleate foreign body giant cells was variable. Histiocytosis X cells do not express either label. The theoretical and practical implications of these results are explored.

Reticulohistiocytoma cutis--review of 15 cases and an association with systemic vasculitis in two cases.

We reviewed 15 cases of reticulohistiocytoma cutis (RHC). Three cases were of solitary lesions. Four cases were associated with a destructive arthritis but no other systemic features. One additional patient had a destructive arthritis and lesions of reticulohistiocytoma in synovium, larynx, and mucosa. Three patients had associated xanthelasma. Two cases were associated with internal malignancy (metastatic malignant melanoma, adenocarcinoma of the bowel). Two cases were sequentially associated with systemic vasculitis (Wegener's granulomatosis, periarteritis nodosa). This occurrence of systemic vasculitis in RHC patients is unique. RHC may have variable cutaneous and systemic manifestations and associations with malignancy and immunoinflammatory diseases, including vasculitis.

Subcutaneous xanthogranulomatosis: an inflammatory non-X histiocytic syndrome (subcutaneous xanthomatosis).

The histopathologic features in four cases of subcutaneous xanthogranuloma were reviewed. All patients were elderly men and no associated diseases or hyperlipidemia were present. The microscopic picture was uniform in seven biopsy specimens: foam cells and masses circumscribed by Touton cells replacing the adipose tissue. Most cells, including foam cells, showed a positive reaction for the macrophage marker MAC387. Diffuse, sparse T cells and focal perivascular B cell nodules were identified. Electron microscopic examination showed histiocytes and lysosome-rich cells with myelin bodies. All patients are alive and well. We believe that we have identified a form of adult subcutaneous xanthogranuloma syndrome that can be initially confusing and lead to overdiagnosis and overtreatment.

Lichenoid dermatitis: a clinicopathologic and immunopathologic review of sixty-two cases.

We reviewed 62 cases (26 men and 36 women, median age 55 years) of previously diagnosed lichenoid dermatitis and correlated the histologic and immunodermatologic findings with the clinical diagnosis and course. The clinical diagnostic groups were contact dermatitis (6 cases), drug eruption (14), lupus erythematosus (6), lichen planus (3), cutaneous T cell lymphoma (3), chronic dermatitis or neurodermatitis (19), and miscellaneous dermatologic disorders (11). With the use of individual histologic features, a correlation with specific clinical conditions was possible, and the clinical groups of dermatitis, drug eruption, lupus erythematosus or lichen planus, and T cell infiltration could be selected histologically. Direct immunofluorescence studies were of most help in the diagnosis of lupus erythematosus and atypical lichen planus, but these studies should be performed in all cases involving lichenoid inflammation. Although previously the nonspecific term lichenoid dermatitis was used to classify the histologic features in these cases, we found that by careful correlation of histologic, immunodermatologic, and clinical features, we were often able to provide a specific diagnosis.

Blue nevus and nevus of Ota associated with dural melanoma.

A 41-year-old woman with a complex congenital nevus that possessed features of both a blue nevus and a nevus of Ota is described. She was found to have melanotic hyperpigmentation of the underlying subcutaneous tissue and dura mater. Two separate melanomas developed in the tissues underlying her nevus (one in the subcutaneous tissue and another intracranially, arising from the dura).

Necrotizing fasciitis: a clinical, microbiologic, and histopathologic study of 14 patients.

We studied the clinical, microbiologic, and histopathologic findings from 14 patients with necrotizing fasciitis; also tissue obtained at autopsy was available from six patients. In all cases, material cultured was positive for multiple organisms, including Clostridia and fungi; organisms were identified by histochemical staining of tissue sections in 12 of 14 cases. The histologic pattern comprised edema, necrosis, and inflammation of skin, subcutaneous fat, and fascial tissue. Hyalin necrosis of sweat glands was observed in five patients. Thrombosis of vessels at all levels was a prominent feature, suggesting that study of coagulation factors may be important. Diagnosis may be confirmed by the histologic picture; however, microbiologic material is essential to guide therapy.

Unilesional mycosis fungoides: clinical, microscopic and immunophenotypic features.

Seven cases are reviewed in which the histologic and immunohistologic features were those of mycosis fungoides, although the patients presented with solitary lesions which did not recur after local therapy (excision in six, radiotherapy in one), with follow-up of 10 months to 18 years. Immunophenotypic staining of paraffin-embedded tissue revealed a predominant T-lymphocyte proliferation in all cases, None of the four cases studied for BER-H2(Ki-1) were positive, in contrast to the "activated" types of spontaneously regressing lymphoid lesion, lymphomatoid papulosis and "Ki-1" lymphoma. Electron microscopy in one case demonstrated the highly convoluted nuclei of the T cells within the infiltrate. These seven cases represent examples of unilesional mycosis fungoides in which the disease has not recurred after therapy, despite features indistinguishable from those of typical generalised mycosis fungoides-type of cutaneous T-cell lymphoma.

The current treatment of scleroderma.

The treatment of scleroderma is determined by the stage of the disease, associated organ involvement, or the presence of features overlapping those of other connective tissue disease. Raynaud's phenomenon is responsive to vasoactive medication, but recently heat and plasma exchange have been shown to be more effective, reducing the need for systemic medication. In stages II and III of the disease, administration of non-toxic penicillamine in low doses for 2 to 4 years is the preferred treatment. Plasma exchange may offer some hope in the early stages. The treatment of the renal crisis of scleroderma with angiotensin-converting enzyme inhibitors has reduced mortality from this complication. These drugs are currently the preferred treatment for the hypertension of renal scleroderma. The symptomatic treatment of the pulmonary, gastrointestinal, and soft tissue complications of scleroderma is also discussed.

Unilesional mycosis fungoides: a distinct entity.

Four cases of solitary lesions showing the histologic features of mycosis fungoides are described. Three patients had an erythematous scaling plaque, whereas the fourth had a solitary nodule. The lesion was located on the arm in two patients, on the foot in one patient, and on the back in one patient. No preceding factors were noted, and no lymphadenopathy or other significant illness was found on examination. Histologic examination demonstrated features of a cutaneous T cell lymphoma with epidermotropism. Monoclonal markers on frozen and paraffin sections revealed a predominant T cell infiltrate. Three patients were treated with local excision. The remaining patient was treated with radiotherapy. The lesions have not recurred, and no new lesions have developed after individual follow-up of 7 months, 7 1/2 months, 13 years, and 18 years. These cases appear to be unique reports of solitary mycosis fungoides behaving in a biologically benign manner. The relationship to Woringer-Kolopp disease and lymphomatoid papulosis is discussed.

Clear-cell, basal cell carcinoma: histopathological, histochemical, and electron microscopic findings.

Another histological variant of basal cell carcinoma (BCC) is described. This clear-cell variant appeared as a recurrent lesion on the back of an elderly man. Histological examination revealed that part of the tumor was composed of clear cells with faintly eosinophilic cytoplasm. Degenerative changes were present, along with calcium deposition. Mucin was present within the stroma and within the degenerative areas of the tumor. Staining with anti-S-100, anti-keratin, and anti-carcinoembryonic antigen antibodies was negative. Electron microscopy demonstrated epithelium-derived cells with marked phagolysosomal accumulation within the cytoplasm. We conclude that this clear-cell variant is due to degenerative change within a BCC.