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Congenital Ichthyosiform Erythroderma. Presented by Dr. John P. Scully, Reading, Pa.
R. H., a white woman aged 30 years, soon after birth acquired a bullous eruption on an erythrodermic background. The bullous lesions had lessened by the end of her first year. She has had persistent dryness and scaling of the skin during her lifetime. During the past summer recurrent pruritic erythematous lesions of the lower extremities were secondarily infected at times.
The blood count, sedimentation rate, fasting blood sugar, results of urinalysis and blood urea nitrogen were normal. A hemolytic Staphylococcus aureus, coagulase-positive, was cultured from the leg.
The biopsy report was as follows: "The epidermis was moderately and densely hyperkeratotic. The rest of the epidermis was acanthotic and highly edematous; Spongiosis was conspicuous at some places. The significant lesion was a thin vesicle or bleb which lay at the level of the stratum granulosum and extended barely to
Gilman RL, Twining HE. PHILADELPHIA DERMATOLOGICAL SOCIETY. AMA Arch Derm Syphilol. 1950;62(5):762–770. doi:10.1001/archderm.1950.01530180151041
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