• T Jansen, D Regele, C G Schirren, B Trautner, and G Plewig.
• Dermatologische Klinik und Poliklinik der Ludwig-Maximilians-Universität München.
• Hautarzt. 1998 Dec 1; 49 (12): 932-5.

AbstractA 24-year-old woman with lymph vessel dysplasia had experienced a progressive edema of her legs since her second year of life and progressive facial edema for the past year. She also had telangiectasias and papules on the background of a diffuse erythema as well as marked seborrhea on her face. Histopathological examination of a representative facial lesion revealed a granulomatous dermatitis with periadnexal distribution mainly consisting of lymphocytes and histiocytes. In addition, there was a moderate fibrosis of the dermis with numerous mast cells. By duplex ultrasound, a diagnosis of a massive edema of the legs without evidence for chronic venous insufficiency was made. The clinical and histopathological findings were consistent with solid persistent erythema and edema of the face associated with rosacea and lymph vessel dysplasia. The chronic course, absence of serological abnormalities and nonspecific histopathological features as well as resistance to therapy are the most important diagnostic criteria of this disease also known as Morbihan's disease.

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