By Gary M. White, MD

The autosomal recessive congenital ichthyoses (ARCI) are nonsyndromic disorders characterized by a variable degree of generalized desquamation and erythema. Most patients present at birth as a colloidion baby. Mutations in TGM1 underlie many ARCIs. Bathing suit ichthyosis (BSI) is a minor variant of ARCI characterized by skin manifestations mainly limited to the trunk.


Patients are typically born with a collodion membrane. The rash improves, but an ichthyosis persists on the trunk, neck and scalp. There is no distribution along Blaschko lines.


JAMA Derm 2017;153;537.


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