title: Virilizing para-adrenocortical adenoma associated with idiopathic-acquired generalized anhidrosis in an adolescent girl.
abstract: Adrenocortical tumors are rare in childhood and adolescence. Virilization, alone or in combination with signs of overproduction of other adrenal hormones, is the most common clinical presentation. Here we report an unusual case of an African-American female adolescent presenting with idiopathic acquired generalized anhidrosis, dysregulation of body temperature, absence of adult body odor and dry skin in the face of a virilizing para-adrenocortical adenoma. Virilization signs regressed soon after removal of the tumor, but normalization of the 3alpha-androstenediol glucuronide (3alpha-AG) took longer compared to other measurable androgens; accompanied by anhidrosis. The association of remitting anhidrosis with normalized levels of 3alpha-AG suggests it might be a possible mechanism for anhidrosis. High 3alpha-AG levels might implicate the increased peripheral conversion of weak pro-androgens with different biochemical structure. We recommend obtaining 3alpha-AG beside other androgens in virilized patients with atypical dermatological symptoms in the face of hyperandrogenism.
authors.affiliation: Division of Pediatric Endocrinology, Louisiana State University Health Sciences Center and Children's Hospital, New Orleans, LA, USA.
authors.forename: Pinar
authors.initials: P
authors.lastname: Gumus
authors.forename: Mark
authors.initials: M
authors.lastname: Luquette
authors.forename: Marie Louise
authors.initials: ML
authors.lastname: Haymon
authors.forename: Evans
authors.initials: E
authors.lastname: Valerie
authors.forename: Jaime
authors.initials: J
authors.lastname: Morales
authors.forename: Alfonso
authors.initials: A
authors.lastname: Vargas
mesh_terms: D000293:Adolescent; D000315:Adrenalectomy; D018246:Adrenocortical Adenoma / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D015113:Androstane-3,17-diol / Q000031:analogs & derivatives / Q000097:blood; D001833:Body Temperature Regulation; D005260:Female; D006801:Humans; D007007:Hypohidrosis / Q000097:blood / Q000150:complications* / Q000175:diagnosis; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D014770:Virilism / Q000097:blood / Q000150:complications* / Q000175:diagnosis
chemical_list: D015113:Androstane-3,17-diol; C028895:androstane-3,17-diol glucuronide