[Alopecia areata and diffuse hypotrichosis associated with Ullrich-Turner syndrome. Presentation of 4 patients]. / Alopecia areata und diffuse Hypotrichose assoziiert mit Ullrich-Turner-Syndrom. Vorstellung von 4 Patientinnen.

Gonosomal aberrations such as Turner's syndrome are frequently associated with autoimmune diseases or with serological markers for autoimmune diseases. An autoimmune origin has been suggested for alopecia areata. The simultaneous occurrence of alopecia areata and Turner's syndrome has not formerly been reported. We present here four female patients with Turner's syndrome, who also developed typical alopecia areata. In three of these cases alopecia areata first appeared during adolescence, whilst the fourth patient showed an early manifestation at the age of 10. Two of the patients had alopecia areata of the ophiasis type, while the condition was localized in one and diffuse in the fourth. In addition, two of the patients had diffuse hypotrichosis of the scalp and reduced sweat gland activity, with no other signs of ectodermal dysplasia. The third patient had not develop body hair after her childhood, except for some sparse thin hair in the axilla and in the genital area. The coincidence of alopecia areata and Turner's syndrome may indicate some genetic relationship between the two entities.

Elephantiasis nostras verrucosa: beneficial effect of oral etretinate therapy.

Elephantiasis nostras verrucosa is characterized by chronic secondary, non-filarial lymphoedema due to recurrent lymphangitis, dermal fibrosis, and epidermal changes consisting of hyperkeratotic, verrucous and papillomatous lesions. Histologically, there is pseudoepitheliomatous hyperplasia. Therapeutic efforts should aim to reduce lymph stasis, which will also lead to improvement of the cutaneous changes. In this study, rapid disappearance of the hyperkeratotic and verrucous lesions, remarkable flattening of the papillomatous nodules and improvement of lymphoedema occurred in three obese patients treated with etretinate in an initial dose of 0.6-0.75 mg/kg/day for 4-6 weeks. Monitoring of plasma concentrations of etretinate, acitretin and 13-cis-acitretin by HPLC revealed sufficient short-time absorption (4 h) and bioavailability of the drug (30 days; two out of three patients). Long-term maintenance therapy in one patient produced a remarkable improvement in the lymphoedema; another patient relapsed after discontinuation of the etretinate and responded again after this was reintroduced. In the third patient treatment was withdrawn because of an increase in triglycerides, but improvement persisted 6 months later. The clinical side-effects of oral retinoid therapy were moderate and well tolerated.