Clinical Case Summary

1

Successful Treatment of Lesions in Hailey-Hailey Disease

Hailey-Hailey disease is an autosomal dominant condition as a result of mutations in theATP2C1gene (encoding the human secretory pathway Ca2/Mn2ATPase) found on chromosome 3q21.1.2It is characterized histologically by suprabasal acantholysis resulting in oozing erosions in the flexures. It is particularly difficult to control during the hot summer months when sweat and friction aggravate the eruption. In addition, the eroded lesions of HHD commonly develop secondary infection.

Adtec plasma is an innovative new medical technique that demonstrated its beneficial use in a patient with HHD. Case Report A 56-year-old man with a 25-year history of HHD presented with recurrent erythematous, erosive lesions in the axillae, groin, and neck region. He reported aggravation of symptoms during summer with strenuous physical activity and from local irritation from the seams of clothing. The lesions were associated with stinging, burning, and sometimes pruritus. On examination in late spring, there were erythematous, oozing skin lesions in the right axilla and right side of the groin. These had failed to heal with topical fusidic acid/betamethasone

Right axilla. A, Before plasma treatment. B, Clinical improvement seen after 8 cold plasma treatments. Treatment Regime

Given the recalcitrant nature of the patient’s disease, novel therapy with cold atmospheric argon plasma was attempted for 2 reasons: to treat the fungal and bacterial superinfection and to assess the effect of reactive oxygen species (ROS) produced by the plasma on the course of the disease.

In addition to treatment with Fucicort Cream, on weekdays the right axilla was treated with 5 minutes of argon plasma therapy. After 4 plasma treatments. the skin lesions in the axilla healed, with relief of the stinging sensations, leaving only residual erythema.

2

Clinical Outcome

Initially, the right side of the groin was treated only with Fucicort Cream without improvement on 4 consecutive days. Plasma treatment was therefore added after the rapid improvement in the axilla. The affected area of the groin also improved with symptomatic relief, but some minor erosions persisted. In total, the patient received 8 plasma treatments to the axilla and 4 to the groin. After a total of 11 additional plasma treatments, both areas healed, leaving only residual erythema. All burning sensations stopped.

Right side of the groin. (left), Appearance of the right side of the groin after 4 cold plasma treatments. (right), Appearance of the right side of the groin after 11 cold plasma treatments with only residual erythema remaining.

Despite the hot, humid summer weather, the patient remained clear of active HHD lesions for 3 weeks until, after playing tennis in the heat, he reported mild discomfort with minor erosions. These responded quickly to an additional 11 daily plasma treatments. Over the remaining summer and warm autumn months, he remained asymptomatic and did not require further treatment.

The treatment itself is not painful, and no local anesthesia is necessary; patients describe the sensation of a mild, warm (22°C-34°C) wind in treated areas. No adverse effects have been observed in clinical trials.

Full Study Details can be obtained from the following paper: Cold atmospheric plasma: A successful treatment of lesions in Hailey-Hailey disease, G. Isbary, G. Morfill, J. Zimmermann, T. Shimizu and W. Stolz, Archives of Dermatology 147(4):388-390 (2011).