RESUMEN
An 18 years olds girl, presented with complaints of continued low grade fever, jet black skin rash and photosensitivity, generalized hyperpigmentation, marked anorexia with extreme lethargy, dull aching abdominal pain with frequent loose stools. Painless oral ulcers, moderate loss of hair, remarkable weight loss, with joint pain, morning stiffing, colour changes of fingers on exposure to cold with digital ulceration. She was amenorrhoic during the period of illness. She was extremely ill with moderate anaemia, raised temperature, postural hypotension, pigmented butterfly rashes on the face and discoid lesions all over the body. Marked pigmentation on the palmer creases, base of the nails, knuckles of the hands, buccal mucosa, gums, tongue, aerola of breasts and valvae, with oral ulcers were present. There were symmetrical polyarthritis with restriction of movement. Laboratory investigations showed an elevated ESR, low haemoglobin, normal platelets, total and differential count of WBC, prothrombin time and APTT were normal, with positive ANA and anti-ds DNA antibody, normal IgG anticardiolipin antibody and a positive direct Coombs' test, with moderate proteinuria. X-ray chest and KUB regions were normal. Abdominal CT scan showed bilateral adrenal cortical atrophy. Tuberculin test was negative. There was hyponatraemia and relative hyperkalaemia. Low serum cortisol level at 8:00 am and positive short synecthine test. Treatment started with prednisolone 1mg/kg in three divided doses which resulted in dramatic improvement, then prednisolone was gradually tappered to 7.5 mg daily. She was in remission during four years follow-up