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J Am Acad Dermatol. 2007 Sep;57(3):463-6.

Dyskeratosis as a histologic feature in epidermolysis bullosa simplex-Dowling Meara.

Author information

  • 1Department of Dermatology, Rambam Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-Israel, Institute of Technology, Haifa 31096, Israel. r_bergman@rambam.health.gov.il

Abstract

BACKGROUND:

Intracellular keratin aggregation and clumping is a characteristic ultrastructural feature in epidermolysis bullosa simplex (EBS)-Dowling Meara (DM) yet without histologic correlates in routinely stained specimens.

OBJECTIVE:

We sought to detect histologic clues to keratin aggregation and clumping in the involved epidermis of EBS-DM.

METHODS:

Four cases of EBS-DM caused by dominant keratin (KRT)5 and KRT14 mutations were studied histologically and ultrastructurally. The histologic slides of 11 additional EBS cases (9 Weber-Cockayne subtypes and two Koebner subtypes) were also reviewed histologically.

RESULTS:

Intracytoplasmic aggregation and clumping of tonofilaments were observed ultrastructurally in all 4 EBS-DM cases. Intracytoplasmic eosinophilic homogenizations and inclusions (ie, dyskeratosis) in individual keratinocytes were detected histologically in 3 of the 4 EBS-DM cases, but in none of the 9 EBS-Weber-Cockayne cases or the two EBS-Koebner cases.

LIMITATIONS:

This was a relatively small studied group.

CONCLUSION:

The histopathological detection of dyskeratosis in individual keratinocytes may provide a valuable clue to keratin aggregation and clumping, and to the diagnosis in EBS-DM.

PMID:
17707151
DOI:
10.1016/j.jaad.2007.02.023
[PubMed - indexed for MEDLINE]
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