TREATMENT OF PICCARDI-LASSUEUR-GRAHAM LITTLE SYNDROME WITH CYCLOSPORINE-A

Piccardi-Lassueur-Graham Little syndrome (PLGLS) is characterized by c icatricial scalp alopecia associated with keratotic lesions of glabrou s skin and non-cicatricial alopecia of the axilla and pubis. The etiop athogenesis of this syndrome is still unknown. Many therapeutic agents have been proposed for PLGLS. As cyclosporin A (CyA) has been success ful in treating other inflammatory disease, it was used in 2 out of 6 cases of PLGLS recently observed in our Institute. Clinical, biochemic al and histopathological studies were carried out on 6 patients affect ed by PLGLS. In 2 patients CyA 5 mg/kg/day per os for 4 weeks, followe d by 3 mg/kg/day for a further 20 weeks, was given. Three patients wer e not treated because of hypertension or hepatic disease and, 1 patien t because of the long duration of the disease. In both cases the treat ment proved to be effective within 6 months. No adverse reactions were observed. CyA has been shown to be a useful and safe drug to control some of the skin signs of PLGLS. This treatment is particularly useful in the first stages of the disease, when perifollicular, lymphohistio cytic infiltrates are more evident and dermal alteration has not becom e definitive. Proof of the efficacy of CyA in PLGLS can only be shown from the results of controlled trials, but clinicians should consider its use in the treatment of the syndrome.