Clinico-pathological analysis of the cutaneous lesions of a patient with type I pseudohypoaldosteronism.

The autosomal recessive form of type I pseudohypoaldosteronism (PHA-I) is an unusual disorder characterized by aldosterone resistance at the target organs, which leads to an excessive loss of sodium chloride through urine, sweat and saliva, among other secretions. Such a high concentration of salt in the sweat during the depletive crises directly causes inflammation and damage in the eccrine structures, with cutaneous lesions similar to those appearing in miliaria rubra. We report an autosomal recessive PHA-I in a 4-year-old girl, with cutaneous lesions mimicking miliaria rubra, that improved after treatment with astringent solutions and avoidance of profuse sweating.