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 AfricanAmerican 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 3 α -androstenediol glucuronide (3 α -AG) took longer compared to other measurable androgens; accompanied by anhidrosis. The association of remitting anhidrosis with normalized levels of 3 α -AG suggests it might be a possible mechanism for anhidrosis. High 3 α -AG levels might implicate the increased peripheral conversion of weak pro-androgens with different biochemical structure. We recommend obtaining 3 α -AG beside other androgens in virilized patients with atypical dermatological symptoms in the face of hyperandrogenism.

Type 1 interferon signature and cytotoxic T lymphocyte activation targeted against sweat ducts in inflammatory acquired idiopathic generalized anhidrosis

“…The contribution of androgenic hormones to the aetiology of AIGA is supported by a case report in which a 15‐year‐old girl patient developed AIGA concomitant with an androgen‐producing tumour in the adrenal gland. She gradually recovered from AIGA after tumour removal and normalization of serum androgen level 40 . The cause of the predominant incidence of AIGA among young men remains unknown; however, GCDFP15 may play a role 10,27 .…”

“…She gradually recovered from AIGA after tumour removal and normalization of serum androgen level. 40 The cause of the predominant incidence of AIGA among young men remains unknown; however, GCDFP15 may play a role. 10,27 GCDFP15 is produced by dark cells in an androgen-dependent manner.…”

Acquired Generalized Anhidrosis: Review of the Literature and Report of a Case with Lymphocytic Hidradenitis and Sialadenitis Successfully Treated with Cyclosporine

“…Among cases of anhidrosis, AGA is rare [13,15,22,23,24,25,26,27,44,47,48,49,50,51,52,53,54,55,56,57,58,59,60,61,62,63,64,65] (table 3). A total of 37 previous cases of AGA with detailed clinical features were identified in the English language literature as far as we could determine.…”