Rare Diseases Symptoms Automatic Extraction

Punch grafting of chronic ulcers in patients with laminin-332-deficient, non-Herlitz junctional epidermolysis bullosa.

[junctional epidermolysis bullosa]

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.We sought to present the results of punch grafting in patients with laminin-332-deficient JEB-nH, and to discuss its therapeutic value.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.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 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.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.

Diseases presenting "absence of a control group" symptom

  • junctional epidermolysis bullosa

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