Erosive pustular dermatosis of the scalp: a multicentre study.

BACKGROUND: Erosive pustular dermatosis of the scalp (EPDS) is characterized by crusted erosions or superficial ulcerations that lead to scarring alopecia. OBJECTIVES AND METHODS: We performed a multicentre retrospective clinical study including 56 patients (29 females and 27 males, mean age 62.7) with a confirmed EPDS in order to describe epidemiology, clinical findings and therapeutic choices of this disease. RESULTS: Mechanical/chemical trauma was reported in 28.6%, a previous infection in 10.7%, a previous cryotherapy in 5.4% androgenetic alopecia in 48.2% and severe actinic damage in 25%. Trichoscopy showed absence of follicular ostia, tufted and broken hair, crusts, serous exudate, dilated vessels, pustules and hyperkeratosis. Histopathology revealed three different features, depending on the disease duration. The most prescribed therapy was topical steroids (62.5%), followed by the combination of topical steroids and topical tacrolimus (8.9%), systemic steroids (7.1%) and topical tacrolimus (5.4%). A reduction of inflammatory signs was observed in 28 patients (50%) treated with topical steroids and in all three patients treated with topical tacrolimus. CONCLUSION: The relatively high number of patients collected allowed us to identify a better diagnostic approach, using trichoscopy and a more effective therapeutic strategy, with high-potency steroids or tacrolimus, which should be considered as first-line treatment.

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.

[Multiple glomangioma]. / Glomangioma múltiple.

A case of multiple glomus tumour is described in a 33 year old man. The clinical and histopathological features are discussed, with the review of the literature. We discuss the types of glomus tumour and its differential features.

Necrobiotic xanthogranuloma with cardiac involvement.

A patient with necrobiotic xanthogranuloma (NXG) and paraproteinaemia, who was followed-up for several years, and treated with low-dose chlorambucil, died as a result of a respiratory illness. The significant findings at autopsy were a xanthogranuloma of the spleen and giant cell myocarditis. The myocardial lesions were composed of macrophages, giant cells and lymphocytes. This finding is important because four of five known autopsied patients with NXG have had giant cell myocardial disease, and an effort at antemortem diagnosis should be made.

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.

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.

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.

Comparison of the pathology of fascia in eosinophilic myalgia syndrome patients and idiopathic eosinophilic fasciitis.

The L-tryptophan eosinophilic myalgia syndrome (EMS) clinically has some similarities with idiopathic eosinophilic fasciitis (EF). In order to study the pathology of both syndromes, we analyzed 21 biopsies of patients with EMS and 8 with idiopathic EF. In both diseases there is dermal and fascial mucin and dermal edema, but this was more common in EMS. EMS is also characterized by dilated lymphatics, dermal and septal sclerosis and macrophage-rich inflammation. Neural inflammation was seen in 4 of the cases with EMS and in none with idiopathic EF. In both syndromes, there are many histopathological similarities. The differences may be due to sampling and to sample size. The nerve lesions of EMS may result from the nature of lymphocyte-macrophage inflammation, or the effect of the eosinophil neurotoxin and may not be a primary event.

Malignant proliferative angioendotheliomatosis or angiotropic lymphoma associated with a soft-tissue lymphoma.

We report a 63-year-old man with violaceous nummular patches on the trunk. Histopathologic studies were consistent with a diagnosis of malignant angioendotheliomatosis or angiotropic lymphoma. Immunohistochemical study of skin was positive for UCHL-1 antigen and leukocyte common antigen and negative for L-26, Ulex europaeus lectin I, vimentin, cytokeratin, and epithelial membrane antigen. Ultrastructural study ruled out an endothelial origin of the neoplastic cells. These data confirmed the diagnosis of malignant proliferative angioendotheliomatosis. Five years before, a soft tissue lymphoma had been excised. This is an unusual case of malignant angioendotheliomatosis for the following two reasons: (1) a previous association with a soft tissue lymphoma and (2) the rarely described T immunophenotype of neoplastic lymphoid cells.

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.

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.

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.