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
