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Progressive symmetric erythrokeratodermia

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Title: Progressive symmetric erythrokeratodermia  
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Subject: Channelopathy, Erythrokeratodermia variabilis, Clouston's hidrotic ectodermal dysplasia, Spinocerebellar ataxia type-13, X-linked congenital stationary night blindness
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Progressive symmetric erythrokeratodermia

Progressive symmetric erythrokeratodermia
Classification and external resources
OMIM 133200
DiseasesDB 32838

Progressive symmetric erythrokeratodermia (also known as "Erythrokeratodermia progressiva symmetrica") is a rare, autosomal dominant skin condition that manifests soon after birth with erythematous, hyperkeratotic plaques that are symmetrically distributed on the extremities, buttocks, and face, but sparing the trunk.[1]:565 No other clinical symptoms nor mental or physical signs are usually associated with the condition. [2]

Skin plaques start to appear as reddened areas of inflammation, thus often leading to the mistaken diagnosis of Atopic Dermatitis. Following inflammation, the red areas start keratinization, eventually forming the definitive plaques that appear brownish, dry and scaled. Following quite a precise temporal pattern of evolution, the keratinized plaques last for weeks or months, eventually leading to periods of desquamation that leads to the uncovering of "normal" skin. Then, a new cycle usually begins, leaving a variable number of days-delay between the cycles.

Though environmental causes are not well understood, it seems clear that factors like sun exposure, wind and air conditioning add to the degree of skin inflammation that sets the start of a new cycle.

Definitive treatment does not exist at the moment. Palliative treatment are intended to alleviate the itching that often accompanies the skin inflammation and to moisture the dry skin to prevent excessive dryness and scaling of the plaques.

See also

References

  1. ^ James, William; Berger, Timothy; Elston, Dirk (2005). Andrews' Diseases of the Skin: Clinical Dermatology. (10th ed.). Saunders. ISBN 0-7216-2921-0.
  2. ^ Kan; Chung-Hong, Hu; Woan-Ruoh, Lee (2003). Shu-Feng' Progressive Symmetric Erythrokeratodermia - A case report. Dermatol Sinica, June 2003.
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