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August 1978

Two Brothers With Ichthyosis Congenita With Thymic Involution

Author Affiliations

From the Department of Dermatology, Kurashiki Central Hospital, Kurashiki, Japan.

Arch Dermatol. 1978;114(8):1225. doi:10.1001/archderm.1978.01640200077021

We report the cases of two brothers with fatal ichthyosis congenita in which thymic involution was demonstrated during postmorten examination.

Report of Cases 

Case 1.—  An infant boy was born at full-term, 2,780 g, on Feb 3, 1971. The mother, aged 24, was primipara. The newborn infant had ectropion of the upper eyelids, eclabium of the lips, and flat ears. Hard, collodion-like membranes covered the whole body with transverse fissures. There was no consanguinity relation in either parent. The total serum protein level was 5.1 g/dl. Levels of IgG, IgA, and IgM were within normal limits. The patient was nursed in an incubator, and was given betamethasone, 0.5 mg/day, and ampicillin trihydrate, 100 mg/day, orally for first seven days. At 13 days, recurrent attacks of spiking temperature ranging up to 40 °C were seen. Chest roentgenogram showed acute pneumonia on the right side. In spite of intensive antibiotic and supportive