INTRODUCTION
Benign Familial Pemphigus, or Hailey-Hailey Disease (HHD), affects intertriginous areas of the skin causing epidermal blistering and vesicles that coalesce into weeping and crusting plaques.1
Only 8 cases in the literature have been published demonstrating the utility of the 595 nm pulsed-dye laser (PDL) to treat recalcitrant HHD.2,3 We present an additional case of recalcitrant HHD successfully treated with PDL.
The loss-of-function mutation of the ATP2C1 gene causes a disruption in calcium homeostasis of keratinocytes. The resulting dysfunction in desmosomes and cell-cell adhesion causes acantholysis.1 Lesions are often found in areas such as the groin, inguinal folds, axilla, and intramammary folds, and there is an increased risk of squamous cell carcinoma arising within areas of HHD.1 Symptoms are often remitting and relapsing with eruptions exacerbated by heat, sweat, and friction.
Only 8 cases in the literature have been published demonstrating the utility of the 595 nm pulsed-dye laser (PDL) to treat recalcitrant HHD.2,3 We present an additional case of recalcitrant HHD successfully treated with PDL.
CASE REPORT
A 62-year-old Asian female presented with a painful rash in the groin, inner thighs, and buttocks for 20 years. The rash flared multiple times a year and was exacerbated by heat, sweating, and walking. Physical exam revealed hyperpigmented inactive patches in bilateral axillae as well as well-defined erythematous macerated plaques with a peripheral collarette of scale on the bilateral inner thighs, vulva, perineum, and perianal skin. A 4 mm punch biopsy from the right upper inner thigh revealed an acantholytic dyskeratotic dermatosis consistent with HHD.
Over the next 6 years, she was treated with various topical regimens, including topical steroids, topical antifungals, tetracycline antibiotics, and tacrolimus ointment. While her
Over the next 6 years, she was treated with various topical regimens, including topical steroids, topical antifungals, tetracycline antibiotics, and tacrolimus ointment. While her
axillary disease remained well-controlled, she required multiple courses of minocycline 100 mg bid, higher potency topical steroids (clobetasol and betamethasone), and intralesional triamcinolone injections for persistent lesions in her groin.
In August 2022, PDL was started to treat recalcitrant plaques on both inner thighs and vulva. Each treatment was completed using the Candela Vbeam Perfecta 595 nm laser at a setting of fluence 7 J/cm2, pulse duration of 10 msec, and spot size of 10 mm. A single pass with 10-20% overlap was performed over the entirety of each active plaque. There was marked decrease in erythema, maceration, itching, and pain after just one session. Relapse occurred after approximately 4 weeks, prompting retreatment. At the time of publication, she had received a total of 6 PDL sessions at 6-8 week intervals with significant improvement but not complete remission. Topical maintenance with triamcinolone 0.1% ointment mixed with nystatin cream Monday, Wednesday, and Friday alternating with tacrolimus 0.1% ointment was continued throughout the treatment period. There were no other changes in therapy or laser-related complications.
