Herlitz Junctional Epidermolysis Bullosa:A Case Report and Review of Current Diagnostic Methods | Zendy

Basarab T. | Zendy; Dunnill M. G. S. | Zendy; Eady R. A. J. | Zendy; RussellJones R. | Zendy

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Herlitz Junctional Epidermolysis Bullosa:A Case Report and Review of Current Diagnostic Methods

Author(s) -

Basarab T.,

Dunnill M. G. S.,

Eady R. A. J.,

RussellJones R.

Publication year - 1997

Publication title -

pediatric dermatology

Language(s) - English

Resource type - Journals

SCImago Journal Rank - 0.542

H-Index - 73

eISSN - 1525-1470

pISSN - 0736-8046

DOI - 10.1111/j.1525-1470.1997.tb00965.x

Subject(s) - junctional epidermolysis bullosa (veterinary medicine) , hemidesmosome , medicine , epidermolysis bullosa , dermoepidermal junction , dermatology , lamina lucida , pathology , genodermatosis , basement membrane , laminin , ultrastructure , extracellular matrix , biochemistry , chemistry , basal lamina , gene , dermis , biology , microbiology and biotechnology

We report an infant with Herlitz junctional epidermolysis bullosa (JEB) presenting at birth with erosions on the scalp, thigh, and periumbilical area in addition to nail abnormalities. Ultrastructural studies demonstrated a split through the lamina lucida with poorly formed hemidesmosomes and no clearly defined subbasal dense plates. Indirect immunofluorescence staining with antibodies GB3 (antiiaminin 5) and 19‐DEJ‐1 (antiuncein) was totally absent. These findings, in combination with the clinical picture, favor a diagnosis of Herlitz JEB, Immunohistochemistry findings greatly facilitated an accurate diagnosis, which is essential in view of the poor prognosis for patients with this form of junctional epidermoiysis bullosa.

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