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An unusual case of hirsutism
  1. P Maheux1,
  2. L-G Ste-Marie2,
  3. R Matte2
  1. 1Division of Endocrinology and Metabolism, Université de Sherbrooke, Canada
  2. 2Division of Endocrinology, Hôpital Saint Luc du Centre hospitalier de l’Université de Montréal, Canada
  1. Correspondence to:
 Dr Pierre Maheux
 Division of Endocrinology and Metabolism, Université de Sherbrooke, 3001–12th Avenue North, Sherbrooke, Québec, Canada J1H 5N4;

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A 25 year old HIV positive woman presented with a progressive history of hirsutism (fig 1A) unresponsive to oral contraceptives and cyproterone acetate. During the summer, bullous lesions appeared on the dorsal area of both hands (fig 1B). Laboratory studies disclosed the following values: plasma free testosterone 14 pmol/l (normal 2.4–12.5), DHEA-S 14 mmol/l (normal 1.1–9.2), alanine aminotransferase 65 UI/l (normal <20), aspartate aminotransferase 90 UI/l (normal <20), and alkaline phosphatase 116 UI/l (normal 28–36). A 24 hour urine collection showed raised concentrations of uroporphyrin (4908 μg, normal <25), heptacarboxyporphyrin (2030 μg, normal <7), hexacarboxyporphyrin (113 μg, normal <6), and pentacarboxyporphyrin (264 μg, normal <7). The coproporphyrin level was 136 μg (normal <110). Porphyria cutanea tarda is usually sporadic and caused by a diminution in the activity of liver uroporphyrinogen decarboxylase. The manifestations include a bullous dermatosis on sun-exposed areas, poikilodermatous scarred areas dotted with milia, hyperpigmentation, facial hypertrichosis, and often biochemical evidence of liver disease.1,2 Alcohol and oestrogens are known to be important precipitating agents. Few cases of porphyria cutanea tarda have been associated with HIV infection.3–5

Figure 1

 (A) Patient showing hirsutism on face and (B) bullous lesion on hand (published with permission).