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Dr. Andrew Rynne

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Article Home Adult and Senior Health Keratolysis Exfoliativa Congenita

Keratolysis Exfoliativa Congenita

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Keratolysis exfoliativa congenita is a rare skin disorder characterized by superficial peeling of the skin of the palms and soles in the absence of inflammation.

 

Keratolysis Exfoliativa Congenita also called as Peeling skin syndrome is an extremely rare inherited disorder characterized by continual, spontaneous skin peeling (exfoliation). Other findings may include reddening of the skin (erythema) and itching (pruritus).It starts as pinhead-sized white spots that gradually extend peripherally, forming anular thin scales, in the absences of inflammation.

Synonyms

  • Keratolysis Exfoliativa Congenita
  • Familial Continuous Skin Peeling
  • Deciduous Skin
  • Acral Peeling Skin Syndrome
  • Skin Peeling Syndrome 

Types

One group working on this disorder distinguishes between it as two types of Acral Peeling Skin Syndrome:

  • a non-inflammatory form called type A
  • an inflammatory form known as type B

Type B is associated with congenital erythroderma, a condition in which the skin has an intense red color. There is mounting evidence that the inflammatory type B is a variant of Netherton syndrome.

Signs and symptoms

  • White spot on palms and soles
  • Thin annular scaling
  • Reddening of the skin
  • Itching
  • Absence of Inflammation 

Treatment

There is no specific treatment for this disease supportive and symptomatic treatment is given.