[Churg-Strauss syndrome: a disabling disease]. / Síndrome de churg-strauss: uma doença incapacitante.

Churg-Strauss syndrome (CSS) is an infrequent vasculitis that affects small to medium-sized vessels. We describe a 51 year-old-female admitted to our inpatient unit with bullae on her right foot and forearm with pain, paresthesias and impotence of the foot. There was rapid clinical deterioration with lost of gait and peripheral eosinophilia. Histopathology showed many extravascular eosinophils. Bone marrow had an increased number of eosinophils and their precursors with no neoplastic cells infiltration. Electromyogram revealed mononeuritis multiplex with bilateral sciatic and right femoral nerve involvement. She fulfilled the eligibility criteria of American College of Rheumatology (ACR) and Chapell Hill Conference Consensus (CHCC) of CSS so corticosteroids and cyclophosphamide and rehabilitation program were begun with good clinical and laboratorial response. This report illustrates the importance of identifying atypical cutaneous features of CSS for the early diagnosis of this rare condition and the role of a multidisciplinary team in this multissystemic disease.

Generalized skin drug eruption to natalizumab in a patient with multiple sclerosis.

We report a generalized skin eruption in a young man being treated with natalizumab, a new drug used in patients with multiple sclerosis.

Cutaneous clear cell myomelanocytic tumour: a new member of the growing family of perivascular epithelioid cell tumours (PEComas). Clinicopathological and immunohistochemical analysis of seven cases.

AIMS: To analyse seven cases of cutaneous myomelanocytic tumour histologically and immunohistochemically. Perivascular epithelioid cell tumours (so-called PEComas) are rare and recently delineated neoplasms occurring in the lung, kidney, pancreas, uterus, falciform ligament, vulva, heart, prostate and soft tissues. PEComas are characterized by a perivascular location of neoplastic cells showing a broad spectrum of epithelioid and spindled cells with clear, and granular pale eosinophilic cytoplasm, and a variable expression of melanocytic and muscle markers, whereas S100 protein and cytokeratins are usually absent. METHODS AND RESULTS: We report seven cases of cutaneous myomelanocytic tumour arising on the lower (six cases) and upper (one case) extremities of female adults (age range 30-66 years). In all cases an ill-defined dermal lesion with extension into subcutaneous tissue was noted. The neoplasms contained numerous blood vessels with a lace-like pattern and slightly thickened vessel walls, and were composed of perivascular epithelioid cells containing clear or focally granular pale eosinophilic cytoplasm and round vesicular nuclei with small, sometimes slightly enlarged nucleoli. Increased proliferative activity and tumour necrosis were not seen. Immunohistochemically, tumour cells stained positively for HMB-45, microphthalmia transcription factor, and NKIC3 in all cases, whereas perivascular expression of alpha-smooth muscle actin and focal positivity for desmin were noted in one case each only. Two out of four cases tested stained focally positive for calponin. No expression of S100 protein and pancytokeratin was present. Despite incomplete/marginal excision in three cases none of the neoplasms has recurred locally so far. CONCLUSIONS: With the presented series of cutaneous myomelanocytic tumours the clinicopathological spectrum of PEComas is expanded.