Treatment of Familial Benign Chronic Pemphigus With Superficial Radiotherapy.

IMPORTANCE: Hailey-Hailey disease (HHD) is a chronic genodermatosis with recurrent vesicles and erosions mainly in the intertriginous areas. Hailey-Hailey disease severely affects patient quality of life. Standard treatments attempt to control the flares, but often do not result in long-term remission of the disease. OBJECTIVE: To describe outcomes of treatment with superficial radiotherapy (SR) for severe treatment-refractory HHD. DESIGN, SETTING, AND PARTICIPANTS: This retrospective case-series included 13 patients with severe HHD with a mean (SD) duration of 24 (14) years whose treatments with SR and follow-up were conducted at the Department of Dermatology at Bispebjerg University Hospital (Copenhagen, Denmark) from January 2015 to April 2021. INTERVENTIONS: Patients were treated with SR (20 kilovolt; 8 fractions of 2 gray was equal to 1 cycle) with a total dose of 16 gray in each treatment cycle. Patients received 1 to 6 treatment cycles with 1 to 5 separate body areas treated in each cycle. Sixty-two separate body areas were treated with SR. MAIN OUTCOMES AND MEASURES: Complete long-term remission, defined as no relapse during follow-up of at least 12 months. RESULTS: For the 13 participants (mean [SD] age, 52 [18] years; 8 women [62%]), 56 of 62 treated areas (90%) achieved long-term remission, and the mean (SD) follow-up was 32 (12) months for the successfully treated areas. Nine of 13 patients (69%) responded with complete remission of all treated areas after the first treatment cycle and an additional 3 patients experienced complete remission after the second SR cycle. One patient with partial remission in 1 of 2 treated skin areas experienced such an improvement in HHD that they chose to abstain from retreatment. The treatment was followed by severe inflammation lasting for up to 1 month followed by temporary slight hyperpigmentation of the treated areas. The average Dermatology Life Quality Index score before treatment with SR was 22 (the disease having extremely large effect on the patient's life) and decreased to an average of 3 (small effect on the patient's life) after treatment with SR. CONCLUSIONS AND RELEVANCE: The results of this case series suggest that treatment with SR was associated with remission in patients with severe HHD and may provide a long-term improvement of treated skin areas.

Hailey-Hailey disease improved by fractional CO2 laser.

Hailey-Hailey disease (HHD), also known as benign familial pemphigus, is an autosomal dominant skin condition that affects the adhesion of epidermal keratinocytes. Although the initial manifestation of flaccid vesicles on erythematous or normal skin in flexure sites frequently goes unnoticed, large, macerated, exudative plaques of superficial erosions with crusting are observed at the time of diagnosis. There is no specific treatment for HHD, and most cases are symptomatically supported. However, infrared laser ablation has been somewhat helpful. We present a case successfully treated with fractional CO2 laser showing a long-term favourable outcome and no adverse effects. Thus, this modality could be an alternative to full ablation for this condition.

Successful treatment with narrow-band UVB therapy for a case of generalized Hailey-Hailey disease with a novel splice-site mutation in ATP2C1 gene.

Hailey-Hailey disease (HHD) is a rare autosomal dominant disorder characterized by development of recurrent blisters, erosions, and crustations in the intertriginous areas. The treatment of HHD is often challenging, and various methods have been tried. We report here a case of a 45-year-old woman with a generalized form of HHD that was dramatically improved and well controlled by narrow-band ultraviolet B phototherapy.

[Familial benign chronic pemphigus (Hailey-Hailey disease): successful treatment with carbon dioxide laser]. / Pemphigus chronicus benignus familiaris (Morbus Hailey-Hailey) : Erfolgreiche Behandlung mittels CO2-Laser.

Hailey-Hailey disease is a rare, autosomal dominantly inherited genodermatosis, which manifests with recurrent intraepidermal blistering and erythematous hyperkeratotic patches in intertriginous areas. Conventional therapeutic approaches include topical and systemic steroids, antibiotic agents, as well as oral retinoids. Alternative treatments include surgical interventions such as excision, dermabrasion and laser ablation. A 56-year-old woman presented with 15-year history of severe therapy-resistant Hailey-Hailey disease. We performed laser ablation with carbon dioxide laser under general anesthesia. Six weeks after treatment complete remission within ablated areas was achieved. Six-month follow-up showed no relapse. In our opinion carbon dioxide laser ablation represents an effective and safe therapeutic option for Hailey-Hailey disease.

Persistent improvement of previously recalcitrant Hailey-Hailey disease with electron beam radiotherapy.

Hailey-Hailey disease, or familial benign chronic pemphigus, is an autosomal dominant genodermatosis. Disease symptoms may contribute to an adverse impact on quality of life and functional limitation and disability. As Hailey-Hailey disease is chronic and frequently recalcitrant to treatment, multiple therapeutic approaches, including surgical options, have been attempted. We describe here three cases of recalcitrant Hailey-Hailey disease that showed long-term improvement with radiotherapy. Axillary lesions were treated with electron beam at an anti-inflammatory dose (energy 6-8 MeV). Patients received 20 Gy in 10 fractions to 90% isodose, at each axilla. No disease recurrence was observed during a 38 months follow-up of the treated sites. The effect of radiotherapy was thus considered to be locally beneficial, but without any positive influence on the general course of the disease.

Benign familial pemphigus: little benefit from superficial radiotherapy.

Benign familial pemphigus (Hailey-Hailey disease) is a rare relapsing-remitting epidermal blistering disease palliated by a multitude of medical and surgical treatments. There are limited reports of benefit from low-penetration X-rays. We describe two resistant cases that appeared to respond initially to superficial radiotherapy, a 66-year-old man who had multiple courses to the groins (4 x 2.00 Gy/5 x 3.00 Gy) and to the axillae and low back (10 x 2.00 Gy) and a 53-year-old man treated twice to the groins and perineum (5 x 3.00 Gy/10 x 2.00 Gy). However, on longer follow up, benefit was found to be questionable. We conclude that while superficial radiotherapy may offer temporary relief, there is little evidence that it alters the natural history of this disease.

Clinical analysis of 69 patients with familial benign chronic pemphigus.

OBJECTIVE: To analyze the clinical feature, efficacy of treatment and prognosis in familial benign chronic pemphigus (FBCP). METHODS: Sixty-nine cases of FBCP were retrospectively analyzed. RESULTS: The ratio of male to female is 3.93:1 in 69 patients (55 males, 14 females). The mean age at the onset was 29.09 years (3-60 years). There was familial history in 27 families in all of the cases. The lesion usually involved in genital area, neck, axillae and popliteal fossa. Erythemas and vesicles on the soles were seen only in 1 case. Histopathologically 44 cases had special features of FBCP, and immunopathologically 8 cases were direct immunofluorescence (DIF) negative, in which one case had C3 linear deposition along dermoepidermal junction. The combined regimen was more effective. The low-dose X-ray could improve the effect. CONCLUSION: The disease is transmitted as an irregular autosomal dominant trait. The condition in males is more frequent than that in females, probably owing to the different level of female hormone in both sexes. Our patients have the same clinical features as those reported in the literature, but the erythema, vesicle lesions on sole have not been documented in the literature. The combined therapy should be adopted in this condition.