Seudohipoaldosteronismo tipo I múltiple: presentación neonatal y seguimiento

Pseudohypoaldosteronism type 1 is a rare autosomal recessive disorder with salt‐wasting, hyperkalemia, metabolic acidosis, and multiorgan aldosterone unresponsiveness, which results in an excessive loss of sodium chloride through urine, sweat and saliva, among other secretions. Inflammation of and around the damaged eccrine glands has been attributed to the deleterious effects of excessive eccrine gland salt exposure. Such a high concentration of salt in the sweat causes cutaneous lesions similar to those appearing in miliaria rubra.

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Newborn with pseudohypoaldosteronism and miliaria rubra

Akçakuş

Köklü

Poyrazoğlu

et al. 2006

Int J Dermatology

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

Martin¹,

Calduch²,

Monteagudo³

et al. 2005

Acad Dermatol Venereol

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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.

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Miliaria rubra and thrombocytosis in pseudohypoaldosteronism: Case report

et al. 2011

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Pseudohypoaldosteronism type 1 (PHA1) is a disease involving a state of renal tubular unresponsiveness to the action of aldosterone and characterized by excessive salt loss in the urine, hyperkalemia, and metabolic acidosis. In kidney, PHA1 may occur primarily by mutations in the subunits of the sodium channel or in the mineralocorticoid receptors, and secondarily by several renal disorders. Miliaria rubra and thrombocytosis are reported in a 6-month-old girl with PHA1. In patients with PHA1, miliaria rubra-like cutaneous eruptions are suggested to occur due to obstruction of eccrine sweat glands through inflammation caused by excessive sodium excretion in sweat during hyponatremic crises. The presence of thrombocytosis in patients with PHA1 has not been previously reported. A hypothesis is proposed suggesting that sympathetic activation which provides vascular tonus during sodium excretion in sweat and salt-depletion crisis may play a role in the development of eruptions and thrombocytosis in patients with PHA1.

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Seudohipoaldosteronismo tipo I múltiple: presentación neonatal y seguimiento

Cited by 3 publications

References 9 publications

Newborn with pseudohypoaldosteronism and miliaria rubra

Newborn with pseudohypoaldosteronism and miliaria rubra

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

Miliaria rubra and thrombocytosis in pseudohypoaldosteronism: Case report

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