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Journal of the American Academy of Dermatology
Volume 57, n° 3
pages 463-466 (septembre 2007)
Doi : 10.1016/j.jaad.2007.02.023
Dermatopathology

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

Reuven Bergman, MD a, , Avikam Harel, MD b, Eli Sprecher, MD, PhD a
a From the Department of Dermatology, Rambam Medical Center and the Bruce Rappaport Faculty of Medicine, Technion-Israel, Institute of Technology 
b Pediatric Dermatology Unit, Dana’s Children’s Hospital, Sourasky Medical Center 

Reprint requests: Reuven Bergman, MD, Department of Dermatology, Rambam Medical Center, Rehov Aliya 19, POB 9602, Haifa 31096, Israel.

Haifa, Israel

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.

The full text of this article is available in PDF format.

Abbreviations used : DM, EB, EBS, K, KRT, MIM, WC



 Supported in part by a grant provided by the Bureau for Economic Growth, Agriculture, and Trade, Office of Economic Growth and Agricultural Development, US Agency for International Development, under the terms of Award No, TA-MOU-01-M21-023.
Conflicts of interest: None declared.



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