LEYDIG-CELL HYPOFUNCTION RESULTING IN MALE PSEUDO-HERMAPHRODITISM

  • 1 January 1982
    • journal article
    • research article
    • Vol. 37  (5) , 675-679
Abstract
An 11-yr-old patient with male pseudohermaphroditism presented with essentially normal-appearing female external genitalia. When examined, inguinal gonads, redundant foreskin and some posterior labial fusion were found. Evaluation revealed basal testosterone (T) levels ranging from 65-107 ng/dl with slightly elevated serum gonadotropin levels (luteinizing hormone [LH]: 76 ng/ml, and FSH: 568 ng/ml). Neither T nor its precursors increased with human chorionic gonadotropin (hCG) stimulation. Progesterone (P), 17-hydroxyprogesterone (17-OHP) and cortisol (F) responses to ACTH were normal. Androgen binding and 5.alpha.-reductase activity in cultured genital skin fibroblasts were normal. These data, plus the microscopic finding of a markedly reduced number of Leydig cells, strongly suggest that the male pseudohermaphroditism in this patient was due to inadequate Leydig cell function unrelated to LH receptors.

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