Research promising for epidermolysis bullous

Issue: March 2011

Although current treatment of epidermolysis bullous remains palliative, research into treatment is ongoing, with some promising data emerging, according to a speaker at the 69^th Annual American Academy of Dermatology Annual Meeting.

Anna L. Bruckner, MD, of Lucile Packard Children’s Hospital at Stanford, said that epidermolysis bullous is best thought of as a family of mechanobullous disorders, which share the “common feature of loss of cohesion of the skin or mucosa after minor mechanical trauma.”

Following the publication of the Third International Consensus Report on the Diagnosis and Classification of epidermolysis bullous (EB) in 2008, EB is now classified into four types based on where the blistering may occur, including:

Simplex (EBS) – Intraepidermal cleavage
Junctional EB (JEB) – Intra-lamina lucida
Dystrophic EB (DEB) – Sub-lamina densa
Mixed (Kindler syndrome)

Palliative care for EB targets optimal wound healing and improving the patient’s quality of life, Bruckner said.

But laboratory research is showing that correction of the clinical features of EB is possible, and more recent experience in humans is further testing these hypotheses. Genetically corrected skin grafts have been used successfully in one adult with JEB-non-Herlitz, Bruckner said.

Another avenue of research includes bone marrow transplantation. However, Bruckner pointed out, while the initial results hold promise, long-term results are not clear, and this procedure obviously carries high risks.

“Much still needs to be learned, but there is good reason to be cautiously optimistic about the future of treatment for EB,” she said.

For more information:

Bruckner A. S015. Presented at: 69^th Annual American Academy of Dermatology Meeting. Feb. 4-8, 2011. New Orleans.

Disclosures: Dr. Bruckner reports no relevant financial disclosures.

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