SKIN MANIFESTATIONS OF CUSHING-DISEASE IN CHILDREN AND ADOLESCENTS BEFORE AND AFTER THE RESOLUTION OF HYPERCORTISOLEMIA

Gushing disease (GD) is a common cause of endogenous hypercortisolism in childhood. Its skin manifestations include striae, facial acne, hir sutism, acanthosis nigricans, fungal infections, hyperpigmentation and easy bruisability. We followed 36 children and adolescents with GD (1 4 boys and 22 girls), to define the natural history of skin disease in endogenous hypercortisolism. Physical examination and 24 hour urinary free cortisol (UFC) and 17-hydroxycorticosteroid (17-OHS) excretion v alues were obtained preoperatively and quarterly for 18 months. Preope ratively our patients exhibited purple subcutaneous striae (77.7%), st eroid-induced acne (58.3%), hirsutism (63.7% of the 22 girls), acantho sis nigricans (27.7%), ecchymoses (27.7%), hyperpigmentation (16.6%), and fungal infections (11.1%). The levels of UFG and 17-OHS preoperati vely were 351.84 +/- 243.85 mu g/m(2)/day (mean +/- SD) and 17.92 +/- 7.86 mg/g creatinine/day, respectively. No correlation was found betwe en these levels and the severity of the lesions. All patients were cur ed. Symptoms decreased dramatically within the 3 postoperative months and progressively disappeared within the first year of the follow-up p eriod with the exception of light-colored striae; they were present in 5.6% of the patients at 18 months postoperatively. No acanthosis nigr icans or hyperpigmentation were observed at 3 months postoperatively. Hirsutism was not present at 9 months postoperatively. We conclude tha t in children with GD the skin is affected at multiple sites; however, the severity of the manifestations does not correlate with the bioche mical indices of the disease. With the exception of striae, cutaneous effects of endogenous hypercortisolism completely heal within the firs t year after surgical cure of the disease.