Archives of Dermatological Research

, Volume 272, Issue 3–4, pp 393–399 | Cite as

Epidermolysis bullosa simplex generalisata: Importance of immunofluorescence studies in early diagnosis

  • Harvey Baker
Article

Summary

An unusual case of severe generalized epidermolysis bullosa (EB) simplex is described. Its severity, oral involvement and early milia formation suggested a dystrophic form of the disease, but early immunofluorescence studies on skin biopsy material using bullous pemphigoid (BP) serum clearly showed the level of cleavage to be superficial to the dermo-epidermal junction and microscopy confirmed this. The diagnosis of EB simplex was thus quickly established allowing conservative treatment to be pursued with confidence, and preventing unnecessary exposure of the child to systemic corticoid or phenytoin therapy.

Key words

Epidermolysis bullosa simplex Bullous pemphigoid Immunofluorescence studies Dermoepidermal junction 

Zusammenfassung

Es wird ein ungewöhnlicher Fall von schwerer generalisierter Epidermolysis bullosa simplex beschrieben. Die Schwere der Erkrankung, die Beteiligung der Mundschleimhaut und die frühzeitige Bildung von Milien ließen an die dystrophische Form der Erkrankung denken. Durch Immunfluoreszenzuntersuchungen an Hautbiopsien, wobei Serum von Patienten mit bullösem Pemphigoid werwendet wurde, konnte die Ebene der Spaltbildung oberhalb der dermo-epidermalen Junktionszone dargestellt werden. Dies wurde durch die Histologie bestätigt. Dadurch war es möglich, die Diagnose Epidermolysis bullosa simplex rechtzeitig zu stellen, so daß eine konservative Therapie versucht wurde und dem Kind die systematische Anwendung von Kortikoiden oder Hydantoin erspart blieb.

Schlüsselwörter

Epidermolysis bullosa simplex Bullöses Pemphigoid Immunfluoreszenzuntersuchungen Dermo-epidermale Junktionszone 

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Copyright information

© Springer-Verlag 1982

Authors and Affiliations

  • Harvey Baker
    • 1
  1. 1.Department of DermatologyThe London HospitalLondonEngland

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