Dyskeratosis as a histologic feature in epidermolysis bullosa simplex-Dowling Meara - 19/08/11
, Avikam Harel, MD b, Eli Sprecher, MD, PhD a
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.
Le texte complet de cet article est disponible en PDF.Abbreviations used : DM, EB, EBS, K, KRT, MIM, WC
Plan
| 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. |
Vol 57 - N° 3
P. 463-466 - septembre 2007 Retour au numéro
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