Successful Botulinum Toxin (OnabotulinumtoxinA) Treatment of Hailey-Hailey Disease

January 2015 | Volume 14 | Issue 1 | Case Reports | 68 | Copyright © January 2015

Derek Ho BS,a and Jared Jagdeo MD MSa,b,c

aDermatology Service, Sacramento VA Medical Center, Mather, CA
bDepartment of Dermatology, University of California Davis, Sacramento, CA
cDepartment of Dermatology, State University of New York Downstate Medical Center, Brooklyn, NY

Abstract
Hailey-Hailey disease is a genetic disorder that affects flexural skin with scale, blisters, and maceration. Botulinum toxins have been previously used to treat Hailey-Hailey disease. Here, we present a patient who underwent one treatment of onabotulinumtoxinA and achieved excellent improvement that was sustained for three months post initial treatment.

J Drugs Dermatol. 2015;14(1):68-70.

CASE REPORT

A 62-year-old Caucasian man with a past medical history of Hailey-Hailey disease, basal cell carcinoma on right upper lip and right temporal, hyperlipidemia, and chronic obstructive pulmonary disease (COPD) presented to dermatology clinic with acute flare of Hailey-Hailey disease of the bilateral axilla. In the previous two months, he had worsening pruritus and painful blisters he rated 9/10 on pain scale at bilateral axilla. He stated that this acute episode was one of the most severe episodes he had experienced, and that the lesions worsened with heat and sweating.
Since his early 20s, his Hailey-Hailey disease was symptomatic. Previously, he was prescribed methotrexate, hydrocortisone, triamcinolone, and accutane as treatment for Hailey-Hailey disease, but he stopped these medications due to lack of improvement. Family history was significant for Hailey-Hailey disease. Physical examination revealed moderately well defined, erythematous, arcuate plaques with scale and maceration along the skin folds on bilateral axilla extending inferiorly to the superior flanks (Figure 1). From the history and physical examination, our patient's presentation was consistent with acute flare of Hailey-Hailey disease. He was treated in the axilla bilaterally with a total of 200 units of onabotulinumtoxinA. There were approximately 30 injections per location injected in a grid-like pattern of the involved skin.
The patient returned to dermatology clinic three months later for follow-up (Figure 2), and reported marked-improvement since one week post-treatment. Of note, the central area of the right axilla that was not involved on initial presentation and was not treated with onabotulinumtoxinA was flaring with Hailey-Hailey disease and treated at his return visit. He was satisfied with the results and will continue with quarterly onabotulinumtoxinA treatment to prevent recurrence and reduce his Hailey-Hailey disease symptoms.

DISCUSSION

We present this case of a patient with Hailey-Hailey disease, previously unresponsive to conventional topical and oral medications for many years, but showed marked improvement weeks after treatment with onabotulinumtoxinA treatment. Hailey-Hailey disease (familial benign pemphigus) is a rare hereditary skin disease.1 The defect involves a gene encoding calcium pump ATP2C1, which is located on chromosome 3q21-24.2 Hailey-Hailey disease often presents with blisters or vesicles and erythematous plaques with overlying scale and maceration in skin folds. Commonly involved areas include flexural or intertriginous areas such as the axilla, groin, neck, and inframammary folds where sweat, moisture, and friction play key roles in disease manifestation.1 Affected skin areas tend to macerate, resulting in painful epidermal fissures that are a nidus for secondary infection.
Medical treatment of Hailey-Hailey disease includes: topical and oral corticosteroids,3 oral retinoids,4 cyclosporine,5 and methotrexate.6 These medications often are associated with significant side effects that may manifest due to the prolonged need for continued treatment of this chronic disease.
In the medical literature, we found twelve published cases of Hailey-Hailey disease treated with botulinum toxin that resulted in satisfactory outcomes.7-14 OnabotulinumtoxinA is a neurotoxin that inhibits acetylcholine release from nerve terminals, which results in temporary chemical denervation of sweat glands.15