Colchicine may assist in reducing granulation tissue in junctional epidermolysis bullosa.

Minhee Kim,Dedee F Murrell,Adam G Harris,Swaranjali Jain

doi:10.1016/j.ijwd.2016.04.001

Minhee Kim, Dedee F Murrell + Show 2 more

Open Access

https://doi.org/10.1016/j.ijwd.2016.04.001

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Abstract

Epidermolysis bullosa (EB) is a rare, inherited blistering genodermatosis. Patients with junctional EB (JEB) due to LAMB3 mutations have widespread blisters and erosions of skin, mucosae, and nails, creating significant physical, emotional, and psychosocial burdens. Here we report the use of colchicine for ameliorating hypergranulating wounds in a 41-year-old female with JEB generalized intermediate. Her skin wounds and granulation tissue gradually exacerbated under silicone dressings such that she became profoundly anemic. Subsequently, she was commenced on colchicine 500 μg daily on the basis that it may inhibit cell proliferation and be anti-inflammatory. After a 6-month trial of colchicine, she had an objective and subjective improvement in her validated EB Disease Activity and Scarring Index activity and damage scores and Quality Of Life in EB score with less skin erosions, granulation tissue, and erythema. In addition, her anemia resolved. She denied any gastrointestinal side effects. The exact mechanism of colchicine in assisting reduction of the blistering, erosions, and granulation in JEB is unclear, but the anti-inflammatory and antimitotic properties of colchicine may be partially responsible for this process.

Highlights

Epidermolysis bullosa (EB) is a rare, inherited blistering genodermatosis
Based on the level within which blisters arise, EB is divided into 4 major subtypes named EB simplex (EBS), junctional EB (JEB), dystrophic EB (DEB), and Kindler syndrome caused by 18 different gene mutations (Fine et al, 2014)
Use of colchicine to treat inflammation in EB wounds has not been reported in the literature, to our knowledge

Summary

Introduction

Epidermolysis bullosa (EB) is a rare, inherited blistering genodermatosis. The hallmark feature of EB is extreme skin fragility and recurrent vesiculobullous eruptions following mild mechanical injury. Over the last 2 years, she developed multifactorial anemia (hemoglobin fell in from 122 g/L in February 2013 to 89 g/L in February 2014) resulting from iron deficiency and chronic inflammation, weight loss, and nutritional deficiency She had multiple blood transfusions for the severe anemia, which kept recurring within a few weeks, and skin infections with methicillin sensitive staphylococcus aureus and Group B streptococcus requiring 4 units of packed red blood cell transfusion, iron infusion, and long-term intravenous antibiotic administration. There were fewer skin erosions and granulation tissue and erythema (Fig. 1, a–c) Her EBDASI score improved from 106 total (30 for activity and 76 for damage) at the baseline to 60 (23 for activity and 37) for damage after 6 months of colchicine treatment (Fig. 2). A steady decline of her elevated CRP level was observed (Table 1)

Discussion

Conclusion