Virilizing para-adrenocortical adenoma associated with idiopathic-acquired generalized anhidrosis in an adolescent girl

Pinar Gumus, Mark Luquette, Marie Louise Haymon, Evans Valerie, Jaime Morales, Alfonso Vargas

Research output: Contribution to journalArticlepeer-review

1 Scopus citations

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

Original languageEnglish (US)
Pages (from-to)233-235
Number of pages3
JournalJournal of Pediatric Endocrinology and Metabolism
Volume24
Issue number3-4
DOIs
StatePublished - Apr 1 2011

Keywords

  • 3α-androstenediol glucuronide
  • adolescence
  • adrenal adenoma
  • anhidrosis
  • virilization.

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