Abstract:
BACKGROUND:Epidermolysis bullosa (EB) is a genetic, heterogeneous, trauma-induced blistering disease. Patients with laminin-332-deficient non-Herlitz junctional EB (JEB-nH) can have impaired wound healing witnessed by persistent, small, deep ulcers on the hands and feet that adversely affect the quality of life. OBJECTIVE:We sought to present the results of punch grafting in patients with laminin-332-deficient JEB-nH, and to discuss its therapeutic value. METHODS:Retrospective analysis of the Dutch EB Registry revealed 4 patients with laminin-332-deficient JEB-nH who were treated with punch grafting. Punch grafting was performed according to protocol, and the patients were followed up. RESULTS:In the past 10 years we have treated 23 ulcers in 4 patients with JEB-nH using punch grafting without any complications or adverse effects. The ulcers had on average persisted 6 years before treatment. Healing rate after punch grafting was 70% (n = 16), with a mean healing time of 2 months. Thirty percent (n = 7) of the treated ulcers did not completely heal, but did show improvement. The recurrence rate after 3 months was 13% (n = 2), and was a result of renewed blistering. LIMITATIONS:Limitations of the study are the retrospective design, small number of patients, absence of a control group, and follow-up and ulcer measurement that were not standardized. CONCLUSIONS:Punch grafting can be used as a first-line treatment in small persistent ulcers in patients with JEB-nH. The method is easy, is inexpensive, has little risk of complications, and results in significant healing rates and improvement in quality of life.
journal_name
J Am Acad Dermatoljournal_title
Journal of the American Academy of Dermatologyauthors
Yuen WY,Huizinga J,Jonkman MFdoi
10.1016/j.jaad.2012.04.012subject
Has Abstractpub_date
2013-01-01 00:00:00pages
93-7, 97.e1-2issue
1eissn
0190-9622issn
1097-6787pii
S0190-9622(12)00435-5journal_volume
68pub_type
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