Benign symmetric lipomatosis Launois-Bensaude successfully treated with extensive plastic surgery.

Benign symmetric lipomatosis is a rare condition predominantly seen in male patients. The etiology is unknown, and therapy is difficult, although liposuction has had effect. We describe a 38-year-old male patient who had suffered from severe adipositas since the age of 7. Due to an ulceration in the groin, he later needed intensive care, and in order to normalize his condition, extensive surgical treatment became necessary.

[Benign symmetric lipomatosis--Launois-Besaude syndrome. A case report]. / Benign symmetrisk lipomatose--Launois-Bensaudes syndrom. En kasuistikk.

We describe the case of a 38-year-old male with a benign symmetrical lipomatosis Launois-Bensaude. He has suffered from severe obesity since the age of seven. At the age of 30, hypogonadism was diagnosed and treated. He developed a Pickwick-syndrome, with hypokapnia and secondary polycytemia. Ulcerations developed on the lower parts of his legs, which were treated conservatively for many years at our dermatological clinic. Because of intertriginous dermatitis in the right groin, he treated himself with external corticosteroids containing dequalonium. Ulceration and fatty necrosis followed, necessitating extensive plastic surgical intervention, with removal of approximately 30 kg of skin and adipose tissue.

Mutilating palmoplantar keratoderma successfully treated with etretinate.

Three patients, a mother and her two sons, with mutilating palmoplantar keratoderma (Vohwinkel's syndrome) were successfully treated with oral etretinate (Tigason). All of them had keratotic constriction furrows of one or more digits (pseudo-ainhum) with threatening spontaneous amputation. Oral treatment with etretinate brought about disappearance of the pseudo-ainhums and normalization of the digital blood circulation. The hyperkeratotic skin became thinned and pliable with a dose-dependent trend towards redness and atrophy. Continued long term etretinate medication has made it possible for these patients to fulfill their social and occupational activities. All 3 patients had acoustic impairment of high-pitched tone perception.

Mixed bullous disease with labile erythrocyte sedimentation rate.

A woman, aged 66, fulfilled all the usual criteria of dermatitis herpetiformis. Subsequently, she developed circulating IgA and IgM basement membrane zone antibodies, a labile erythrocyte sedimentation rate (ESR), and the clinical picture changed to one of bullous pemphigoid. Her labile ESR was obviously caused by a factor related to the erythrocytes. Direct Coombs test was positive.