The natural course of Sneddon syndrome: clinical and magnetic resonance imaging findings in a prospective six year observation study.

Sneddon syndrome (SS) is increasingly recognised as a cause of ischaemic stroke in young adults. As the natural course of SS is not well defined, the authors performed a prospective six year clinical and neuroradiological follow up study. Thirteen patients with definite diagnosis of SS (livedo racemosa, characteristic skin biopsy, and history of stroke) entered a follow up programme that consisted of clinical examinations, two magnetic resonance imaging (MRI) investigations, and a comprehensive laboratory follow up protocol. The most frequent clinical findings during follow up had been headache (62%) and vertigo (54%). Seven patients (54%) suffered from transient ischaemic attacks, however, completed stroke has not been obtained during follow up. Progression of white matter lesions detected in MRI were present in 10 of 13 patients. Laboratory follow up protocol revealed transient antiphospholipid antibodies in two subjects. This prospective six year follow up study suggests a low incidence of territorial stroke but outlines progressive leucencephalopathy in patients with SS.

Dermal spindle cell lipoma: plexiform and nodular variants.

We report on eleven cases of a distinctive dermal spindle cell lipoma characterized by a mixture of mature adipocytes and spindle-shaped cells in a fibromucinous background. Six lesions showed a moderately well circumscribed plexiform pattern, five a well demarcated nodular pattern with compression of surrounding connective tissue and prominent stromal fibrosis. Clinically, the plexiform type mostly occurred in the thigh-groin-buttock area and the nodular type in the head-neck or acral location. While plexiform lesions were predominantly seen in middle-aged females, nodular types occurred in young adults of either sex. No recurrence was seen in five patients with follow-up. The tumour cells were vimentin positive and a thin cytomplasmic rim of S-100 protein positivity was seen in mature adipocytes. Ultrastructural studies revealed lipoblastic differentiation of spindle-shaped cells with lipid droplets and basal lamina formation. Dermal spindle cell lipomas seem to be the dermal counterpart of the most subcutaneously located spindle cell lipoma.

Effects of adequate versus inadequate treatment of cutaneous manifestations of Lyme borreliosis on the incidence of late complications and late serologic status.

Eighty-two patients who were treated at the Department of Dermatology, Innsbruck, Austria, from 1980 to 1987 for cutaneous manifestations of Lyme disease were subjected to a clinical follow-up investigation aimed at detecting dermatologic, neurologic, and internal late complications of borreliosis. Only 54 of these patients had received adequate antibiotic treatment according to current standards. Also, their sera were investigated for the presence of immunoglobulin G (IgG) and IgM Borrelia burgdorferi antibodies by an indirect immunofluorescence assay, three different enzyme-linked immunosorbent assays, and immunoblotting. As a control, the sera of 126 healthy blood donors were investigated with the same assays. Results showed no unambiguous clinical late complications of Lyme borreliosis, even in inadequately treated or untreated patients. Seropositivity varied considerably according to the assay used; the indirect immunofluorescence assay yielded the highest scores. The proportion of seropositive results (immunofluorescence assay) was 59% in patients with erythema chronicum migrans, 69% in those with lymphocytoma cutis, and 100% in those with acrodermatitis chronica atrophicans (overall 63%); in contrast, only 31% of the blood donor control group were found to be seropositive. Seropositivity did not correlate with adequacy of treatment, interval between onset of symptoms and treatment, time span since treatment, age of patients, and presence of antinuclear antibodies. Immunoblot pattern showed high incidence of antibodies against the 29/31-kD (outer surface proteins OspA and OspB) and 55/58-kD antigens in general and against the 41-kD protein (flagellin) in patients with acrodermatitis chronica atrophicans only.