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January 1995

Recurrent Urticarial Skin Eruption Since Infancy

Arch Dermatol. 1995;131(1):83-84. doi:10.1001/archderm.1995.01690130085016

REPORT OF A CASE  A 17-year-old boy presented with a history of an intermittent widespread urticarial eruption (Figure 1), fever, and generalized malaise beginning at age 4 months. Individual attacks were acute; lasted about 24 hours; were accompanied by transient chills, rigors, arthralgia, aching, and limb pain; and typically recurred at 6- to 7-day intervals. At 6 months, he experienced persistent leukocytosis (11 to 13×109/L) and an elevated erythrocyte sedimentation rate (100 to 130 mm/h) without any apparent cause. At age 10 years, perceptive deafness was noted, which increased slowly over many years.Physical examination showed short stature (152 cm), weight deficiency (37.7 kg), and facies characterized by maxillary hypoplasia, open bite, thin lips, and a hooked nose. Hippocratic fingers, clubbing of nails (Figure 2), and bilateral pes cavus were also noted. His knees appeared deformed with limited range of motion. Pubertal development was normal, but mild mental

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