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MULTIFOCAL FIBROSCLEROSIS: CUTANEOUS ASSOCIATIONS
Author(s) -
Fischer G. O.,
Launey W. E.
Publication year - 1986
Publication title -
australasian journal of dermatology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.67
H-Index - 53
eISSN - 1440-0960
pISSN - 0004-8380
DOI - 10.1111/j.1440-0960.1986.tb00279.x
Subject(s) - medicine , etiology , vasculitis , pathology , fibrosis , disease
S ummary There is a group of disorders characterized by simultaneous and progressive fibrosis of several discrete regions of the body. This disease complex, “multifocal fibrosclerosis”, most commonly involves the retroperitoneal and mediastinal compartments and may be accompanied by cutaneous lesions. In most cases the aetiology is unknown but there is good evidence, supported by the patient we describe, for a vasculitic origin in a significant proportion of patients. We report a case of multifocal fibrosclerosis associated with cutaneous lesions. These included livedo reticularis and recurrent, painful nodules and pustules on the legs and feet, which progressed to chronic ulceration. Histologically there was evidence of an underlying necrotising vasculitis. A review of the cutaneous changes that have been reported in association with multifocal fibrosclerosis allows speculation regarding the aetiology of the disease process.