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Keratolysis exfoliativa (dyshidrosis lamellosa sicca): a distinct peeling entity.

AbstractBACKGROUND:
Keratolysis exfoliativa (KE), also known as dyshidrosis lamellosa sicca, is a palmoplantar dermatosis characterized by air-filled blisters and collarette desquamation. It has been regarded as a subtype of dyshidrotic eczema, a fungal infection or a dermatophytid reaction. KE may also resemble acral peeling skin syndrome and localized epidermolysis bullosa simplex. Although KE is a common disorder, it is a rarely reported and is an under-recognized dermatosis.
OBJECTIVES:
To delineate the characteristic features of KE.
METHODS:
We investigated the clinical, immunohistopathological, ultrastructural and molecular features of KE. Patients were included from the clinical records. Additional diagnostic research consisted of mutation analysis of the candidate genes TGM5, KRT5, KRT14, FLG, SPINK6 and SPINK9.
RESULTS:
A total of 24 patients with KE were identified, six with familial and 18 with sporadic KE. Lesions consisted of air-filled blisters only on palmoplantar skin, followed by collarette and lamellar peeling. Both light microscopy and electron microscopy showed cleavage and partially degraded corneodesmosomes within the stratum corneum, whereas immunofluorescence microscopy showed normal expression of corneodesmosomal components. No mutations were found in TGM5, KRT5/14 and SPINK6/9. There was no clear link with atopy or with FLG mutations.
CONCLUSIONS:
Our study suggests premature corneodesmolysis as the main pathological mechanism of this palmoplantar skin disorder. We conclude that KE appears to be a distinct peeling entity.
AuthorsY Y Chang, J van der Velden, G van der Wier, D Kramer, G F H Diercks, M van Geel, P J Coenraads, P L J M Zeeuwen, M F Jonkman
JournalThe British journal of dermatology (Br J Dermatol) Vol. 167 Issue 5 Pg. 1076-84 (Nov 2012) ISSN: 1365-2133 [Electronic] England
PMID23039091 (Publication Type: Journal Article, Research Support, Non-U.S. Gov't)
Copyright© 2012 The Authors. BJD © 2012 British Association of Dermatologists.
Chemical References
  • Biomarkers
  • FLG protein, human
  • Filaggrin Proteins
  • Keratins
Topics
  • Adolescent
  • Adult
  • Biomarkers (metabolism)
  • Child
  • DNA Mutational Analysis
  • Dermatitis, Exfoliative (diagnosis, genetics)
  • Diagnosis, Differential
  • Female
  • Filaggrin Proteins
  • Fluorescent Antibody Technique
  • Humans
  • Keratins (genetics)
  • Male
  • Microscopy, Electron
  • Middle Aged
  • Netherlands
  • Pedigree
  • Skin (ultrastructure)
  • Skin Diseases, Genetic (diagnosis, genetics)
  • Surveys and Questionnaires

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