Icthyosiform Eruption and Swollen Articulations in a Girl

Abstract

We report the case of a 2½-year-old girl who was diagnosed with atopic dermatitis at 5 months of age. She received treatment with hydrocortisone cream 1% and prednicarbate cream without improvement. At the age of 17 months, she developed swollen painful joints and photophobia. Joint involvement included the wrists, knees, ankles, and elbows. Her past medical history was otherwise negative, with a normal pregnancy and delivery. The child's mother was a hepatitis B carrier.The patient was initially assessed by the rheumatology department because of her polyarthritis and difficulty in ambulating because of pain. On physical examination, she presented with boggy articulations, tenosynovitis, and dactylitis of multiple joints, including the knees, wrists, interphalangeals, and shoulders.She presented to our dermatology clinic with a generalized icthyosiform erythrodermic eruption with infiltrated erythematous papules on her extremities, face, abdomen, and palms (Figure 1). Ophthalmology examination showed granulomatous uveitis, and treatment was started with atropine and prednisone eye drops 3 times a day.Laboratory investigations showed a C-reactive protein level of 28 mg/L, a white cell count of 16 000 10 9 /L, a hemoglobin level of 102 mg/L, and platelets at 662 000 10 9 /L. Immunoglobulins, C3, and C4 were normal. Electrolytes, including calcium, liver, and renal function tests, were normal. Antinuclear antibodies and rheumatoid factor were negative. Angiotensinconverting enzyme (ACE) levels were elevated at 87 (normal, 5-83 U/L). Serology for hepatitis B and tuberculin skin tests were negative.Radiological investigations revealed a normal chest film, but hand X rays showed soft tissue swelling in the wrists and in multiple interphalangeal joints. A skin biopsy showed parakeratosis, with epitheloid infiltrates, histiocytes, and mononuclear inflammatory cells forming noncaseating granulomas. A few small granulomas were present in the subcutaneous adipose tissue. No vasculitis was seen, and the cultures were negative (Figure 2).The clinical findings and histopathology were suggestive of sarcoidosis, and the patient started treatment