Guan-Yu Chen¹, Hamm-Ming Sheu², WenChieh Chen³

¹Department of Dermatology, Armed Forces Taichung General Hospital, Taichung, Taiwan, ²Department of Dermatology, College of Medicine, National Cheng Kung University, Tainan, Taiwan, ³Department of Dermatology, Chang Gung University, Kaohsiung Chang Gung Memorial Hospital, Kaohsiung, Taiwan

Objectives: Skin changes, including acne, alopecia, hirsutism, seborrhea and acanthosis nigricans, usually herald the clinical manifestation of hyperandrogenism. Causes associated with hyperandrogenism can be categorized as medication-related or endogenous non-tumor or tumor related androgen overproduction. Although the majority of female patients with acne or androgenetic alopecia possess no endocrine disorder, hyperandrogenism with hyperandrogenaemia should be considered in those with severe acne of sudden onset or conspicuous male-pattern baldness with hairline recession. There are, however, individual differences between acne severity and the circulating androgen levels. Many women with female pattern hair loss have no other clinical or biochemical evidence of androgen excess.

Methods: We describe two young women with primary amenorrhea displaying prominent hyperandrogenaemia but subtle cutaneous manifestations.

Results: The first one presented with vertical alopecia (at grade I of Ludwig’s classification), had elevated level of serum dehydroepiandrosterone sulfate (> 800 mg/dl) and was suspected to be a case of late-onset, non-classical adrenal hyperplasia. The second case with mild acne had a soaring serum level of total testosterone above 9,000 ng/dl, which was derived from an androgen-secreting adrenal adenoma over-expressing steroidogenic acute regulatory protein, P450 side-chain cleavage enzyme and aromatase.

Conclusions: Our cases give further evidence to the notion that peripheral end-organ response also plays an important role in the pathogenesis of androgen-dependent dermatoses. The possibility of adrenal tumor should be explored in patients with escalated circulating testosterone.