Giant cell granulomatous pulmonary and myocardial lesions in necrobiotic xanthogranuloma with paraproteinemia.

OBJECTIVE: To report three cases of pulmonary or myocardial disease (or both) and necrobiotic xanthogranuloma. MATERIAL AND METHODS: Giant cell granulomas of the lung and myocardium were demonstrated in three patients who had pulmonary and myocardial lesions of necrobiotic xanthogranuloma in conjunction with skin lesions, leukopenia, paraproteinemia, and complement deficiencies. The patients were two men who were 47 and 64 years of age and a 39-year-old woman. RESULTS: Biopsies of skin and visceral lesions showed asteroid and cytoplasmic inclusions. B-cell lymphoid nodules were found. In one of the male patients, a major clonal T-cell receptor gene rearrangement was detected in the peripheral blood. Prednisone was ineffective in two of the patients. The other patient experienced regression of skin lesions and diminishment of a chest nodule after receiving alkylating agent therapy. CONCLUSION: Establishing the correct diagnosis is important, and apparently it is possible to establish the nature of the myocardial and pulmonary lesions with use of appropriate scans and by biopsy. Successful treatment of necrobiotic xanthogranuloma skin lesions with corticosteroids or alkylating agents (or both) implies that evolution of serious disease that compromises the heart and lungs could be controlled.

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.

Adult lipophagic atrophic panniculitis.

We report two patients with chronic, recurrent, nodular panniculitis lesions that later developed areas of lipoatrophy. Histologically, there was a consistent lobular lipophagic replacement of fat cells with lipophagic giant cells. The clinical appearance was that of tender, erythematous, superficial or subcutaneous, symmetrical nodules and plaques of 1-2 weeks' duration. The lesions could occur with episodes of fever. One patient had hepatomegaly and the other had an increased sedimentation rate and leucocytosis. The histology and the clinical pattern of the panniculitus syndrome resembled those of lipophagic lipoatrophy of childhood. This is a panniculitis of unknown cause in which the principal inflammatory cell response in the subcutaneous tissue is the macrophage.

Cutaneous mucormycosis.

We report two cases of primary cutaneous mucormycosis in immunocompromised patients. Although their clinical presentation showed nonspecific necrotic ulcers, the histopathologic features were diagnostic. In both cases, broad, branching, nonseptate hyphae were found in the dermis and subcutaneous tissue. In one case the hyphae could be observed in the epidermis. To our knowledge, this is the only reported case of primary cutaneous mucormycosis with hyphae detectable in the epidermis.

Histologic findings of Hailey-Hailey disease in a patient with bullous pemphigoid.

The association of bullous pemphigoid with other immune-mediated diseases has been described; however, coexistence with other bullous dermatoses is unusual. We describe a patient with longstanding localized bullous pemphigoid and the recent finding of a Hailey-Hailey-like histologic pattern in a biopsy specimen obtained at a site of active pemphigoid involvement, as confirmed by immunofluorescence studies. Although this case suggests the possibility of coexistence of two bullous diseases, the Hailey-Hailey-like pattern more likely represents an incidental microscopic finding or a solitary acantholytic lesion.

Eruptive histiocytoma.

A case of adult cutaneous eruptive histiocytoma is reported. The lesions showed self-healing capacity in a 9-year period. Electron microscopic studies revealed comma-shaped bodies and multiple melanosomes in the cytoplasm of some cells. Normolipemic xanthelasma was present, and the patient had polyclonal gammopathy and hypocomplementemia. Resolution of the lesions produced pigmented atrophic macules. Generalized eruptive histiocytoma and benign cephalic histiocytosis could be forms of the same non-X histiocytosis.

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.