Carbon dioxide laser treatment for Hailey-Hailey disease: a retrospective chart review with patient-reported outcomes.

BACKGROUND: Hailey-Hailey disease (HHD) is an autosomal dominant genodermatosis that leads to skin breakdown and blister formation, usually in intertriginous areas. Laser ablation is a known surgical treatment for HHD. OBJECTIVES: We report outcomes in a series of patients with HHD treated with carbon dioxide (CO2 ) laser ablation. METHODS: A retrospective chart review of data for 13 patients who underwent CO2 laser ablation for HHD was conducted. Questionnaires assessing effectiveness, pain, healing time, recurrence, new disease, complications, additional ablation, improvement in quality of life (QoL), and overall satisfaction were completed by all patients. RESULTS: All patients had been recalcitrant to previous treatments prior to CO2 laser ablation. Anatomic sites treated and anesthesia techniques for the procedure varied. The mean ± standard deviation (SD) score for the effectiveness of CO2 laser ablation for HHD was 9.3 ± 0.9 (maximum: 10). All patients reported improved QoL. No patient had recurring disease within the treatment field. Five patients underwent additional CO2 laser treatments at new sites. One patient reported postoperative infection. No other complications were identified. The mean ± SD score for overall satisfaction with CO2 laser ablation was 8.9 ± 1.1. CONCLUSIONS: Treatment with CO2 laser ablation is very effective and can lead to prolonged or permanent remission in most HHD patients. Patients are highly satisfied with the results and report a substantial improvement in QoL.

Laser therapy for the treatment of Hailey-Hailey disease: a systematic review with focus on carbon dioxide laser resurfacing.

Benign familial chronic pemphigus, or Hailey-Hailey disease (HHD), is a recurrent bullous dermatitis that tends to have a chronic course with frequent relapses. Long-term treatment options include surgery with skin grafting or dermabrasion. Both are highly invasive and carry significant risks and complications. More recently, 'laser-abrasion' has been described as a less invasive option with a better side-effect profile. In this article, we systematically review the safety and efficacy of carbon dioxide laser therapy as a long-term treatment option for HHD, as well as provide a review of other lasers that have been reported with this goal. A total of 23 patients who had been treated with a carbon dioxide laser were identified. After treatment, 10 patients (43%) had had no recurrence, 10 (43%) had greater than 50% improvement, 2 (8%) had less than 50% improvement and 1 (4%) patient had no improvement at all (follow-up period ranged from 4 to 144 months). Laser parameter variability was wide and adverse effects were minimal, including dyspigmentation and scarring. Reviewed evidence indicates this therapy offers a safe, effective treatment alternative for HHD with minimal risk of side-effects. Larger, well-designed studies are necessary to determine the optimal treatment parameters.

Carbon dioxide laser treatment in Hailey-Hailey disease: a series of 8 patients.

BACKGROUND: Hailey-Hailey disease (HHD), or benign familial pemphigus, is a rare autosomal dominant genodermatosis characterized by the formation of blisters. Eruptions are usually located in large skin folds, and when blisters become infected the condition can be very painful and disabling. HHD is difficult to treat. Many topical and systemic treatments have been used to bring exacerbations under control, but none have achieved medium- to long-term remission. PATIENTS AND METHODS: Retrospective study of 8 patients with HHD treated with carbon dioxide laser therapy in our hospital between 1999 and 2011. The patients' mean age was 50.7 years. The 4 men and 4 women were followed for between 1 and 12 years. RESULTS: Satisfactory outcomes were achieved for 6 of the patients. Clinical improvement was observed in more than 75% of the affected area in 4 patients and in 50% to 75% of the area in 2 patients. The effect of treatments was maintained over time. The poorest outcomes were observed in patients treated at lower potencies. When blistering recurred after treatment, a second laser session achieved a good response. We observed no adverse events other than slight changes in skin texture and pigmentation. CONCLUSIONS: Carbon dioxide laser therapy was safe and effective in producing medium- to long-term improvement in HHD symptoms that were refractory to conventional treatments.

Effective treatment of Hailey-Hailey disease with a long-pulsed (5 ms) alexandrite laser.

Familial benign pemphigus (Hailey-Hailey disease) is often resistant to conventional treatments. This report describes a 35-year-old Asian American male with a 12-year history of recalcitrant Hailey-Hailey disease who was treated with a long-pulsed alexandrite laser. Fluences ranged from 12 to 20 J/cm with a 5-ms pulse duration (spot sizes: 10-15 mm). Cold air cooling was used during the sessions. There was 50% improvement noted after the first treatment. Within 10 treatments, there was 95% clearance. Complete resolution was achieved by the thirteenth treatment. The lesions have been clear or nearly clear (greater than 95%) for the past 2 years. Once clearance was achieved, five maintenance laser treatments were initiated at 3-month intervals and eventually discontinued for 12 months without relapse.

Segmental multilayered argon plasma coagulation: effective therapy option for perianal and scrotal Hailey-Hailey disease.

AIM: Hailey-Hailey disease (HHD) is a genodermatosis characterized by recurrent chronic weeping plaques at intertriginous sites. In severe cases, topical and systemic treatments are often insufficient. Extensive excisions are traumatic for patients and have a high morbidity. Superficial ablative procedures such as dermabrasion have become established, but are difficult to perform in the perianal region and scrotum. Therefore, alternative therapies are required. METHOD: Four patients with extensive anogenital HHD were treated by argon plasma coagulation (APC). This leads to a controlled coagulation of nearly constant depth. After dividing the affected areas into segments, a multilayered APC was performed in every second segment to the upper corium. The remaining segments were treated in the same way after healing of the initially treated parts. RESULTS: A disease-free period of up to 37 months was observed in all four patients. There was no case of reduced cutaneous sensitivity or extensive scarring. CONCLUSIONS: Argon plasma coagulation is effective and safe for perianal and scrotal HHD. The benefits include the accessibility to difficult anatomic sites and good control of ablation. The risk of anal stenosis is minimized by the segmental two-step procedure. The rapid re-epithelialization occurs from structures not affected by the disease and allows rapid mobilization of patients.

[Benign familial chronic pemphigus (Hailey-Hailey disease). Treatment with carbon dioxide laser]. / Pemphigus chronicus benignus familiaris (M. Hailey-Hailey). Therapie mit CO2-Laser.

Benign familial chronic pemphigus (Hailey-Hailey disease) is an autosomal dominant blistering disease caused by mutations in the ATP2C1 gene encoding a calcium-ATPase which is crucial for intercellular epidermal adhesion. We present a 37-year-old woman with erosions and crusts in both axillary areas. Based on clinical and histological findings, she was diagnosed with benign familial chronic pemphigus. The lesions healed after ablative carbon dioxide laser therapy; no relapse occurred within a follow-up of two years.

[Treatment of Hailey-Hailey disease with carbon dioxide laser vaporization]. / Traitement de la maladie de Hailey-Hailey par laser ablatif CO2.

INTRODUCTION: Only ablative methods lead to long term remission of areas affected by Hailey-Hailey disease: excision/skin graft, cryosurgery, dermabrasion... The method using the CO2 laser is a recent addition in the management of this dermatitis. We report our experience with this technique in 4 patients. PATIENTS AND METHODS: Carbon dioxide laser vaporization was proposed to 4 patients exhibiting Hailey-Hailey disease resistant to classical treatments. A test under local anesthesia was performed beforehand in all the patients. A 60 year-old man had an immediate reaction and refused to continue treatment. In the other 3 cases, the result of the test at 6 months was considered satisfactory. These patients were treated under general anesthesia in a single area of 50 to 70 cm2, and a half-body for comparison. The CO2 laser was used in pulse mode, with successive irradiations, until a homogenous, whitish-yellow aspect with first retraction was obtained. RESULTS: Although the healing delays were long (a mean of 1 month) and required major analgesics over the first few days, the cosmetic results were satisfactory and no abnormal scarring was observed. After a median follow-up of 27 months, no relapse of the disease other than punctiform elements was noted. All the patients wanted treatment of the other remaining affected areas be continued. In 2 patients, CO2 laser vaporization permitted treatment of areas not easily accessible to other ablative methods (around the mouth, the anus and the vulva) with anatomy and normal function spared. DISCUSSION: These results are globally good. Although the time to healing was long, the cosmetic and functional results were always satisfactory, without abnormal scarring. Moreover, in 2 of the patients, CO2 laser was able to treat areas inaccessible to other methods. The reason for the efficacy of ablative methods is debated. Re-epidermization with keratinocytes of appendices and not expressing the molecular defect, and the constitution of dermal cicatricial tissue, are two currently proposed hypotheses.

Smoothbeam laser treatment may help improve hidradenitis suppurativa but not Hailey-Hailey disease.

OBJECTIVE: The attributes of the Smoothbeam laser--a 1450 nm diode laser--were considered likely to improve two distinct chronic conditions of flexural skin: Hailey-Hailey disease (HHD) and hidradenitis suppurativa (HS). METHODS: A series of laser treatments were given to the axillae of one patient with each condition. RESULTS: Treatments were painful but tolerated. There were no adverse sequelae following laser treatment. For the patient with HS, partial improvement was achieved after four treatments and sweating was notably reduced. Intermittent laser treatment may be needed to maintain or build upon the improvements gained. Longstanding HS cases, however, with established sinuses and thick scar tissue are unlikely to be helped by this form of laser treatment. There was no subjective or objective improvement seen in the patient with HHD. The only notable beneficial effect was a marked reduction in sweating and elimination of the malodour. CONCLUSIONS: Subsurfacing laser treatment in contrast with resurfacing laser treatment has no benefit in the management of HHD. Why the Smoothbeam laser causes sweat reduction is not known--and its clinical value has yet to be established.

Type 2 segmental manifestation of Hailey-Hailey disease: poor therapeutic response to dermabrasion is due to severe involvement of adnexal structures.

In autosomal dominant skin conditions, two different types of segmental manifestation can be distinguished. Type 1 represents heterozygosity for a postzygotic mutation, resulting in a degree of severity similar to that of the nonmosaic phenotype. Type 2 reflects loss of heterozygosity and shows an excessively pronounced involvement superimposed on the ordinary nonsegmental phenotype. We describe the clinical, histopathological and therapeutic aspects of the first case of type 2 segmental manifestation of Hailey-Hailey disease (HHD). A 24-year-old woman with a family history of HHD comprising four generations, presented with lesions of erythema and blistering arranged in a unilateral pattern following the lines of Blaschko. The disorder was first noted at the age of 3 months. At the age of 24 years, additional scattered symmetrical lesions involving the axillary and inguinal folds were noted. Histopathological examination of the severely involved linear skin areas revealed pronounced acantholysis within the deep adnexal structures, whereas clinically unaffected skin showed the typical histopathological features of the heterozygous phenotype with suprabasal clefting and acantholysis sparing the adnexae. Dermabrasion was performed in the areas of segmental involvement. During a follow-up period of one year, no recurrence was noted, but 18 months after dermabrasion a recurrence was present in the left submammary and left perianal regions. This therapeutic resistance to dermabrasion may be explained by the presence of acantholysis within the adnexal structures of the skin as found in type 2 segmental HHD.

Treatment of Hailey-Hailey disease (or benign familial pemphigus) using short pulsed and short dwell time carbon dioxide lasers.

BACKGROUND: Surgical intervention of Hailey-Hailey disease (HHD) may be required to achieve prolonged remission or cure. Excisional surgery, dermabrasion, and continuous carbon dioxide (CO2) laser therapies have been utilized with success, though patients may experience considerable morbidity. OBJECTIVE: To evaluate the use of short pulsed and short dwell time CO2 lasers in the treatment of HHD. METHOD: Case report and review of the relevant literature. RESULTS: A 26-year-old woman with refractory axillary HHD was initially treated with a short dwell time CO2 laser. The right axilla was treated with two passes at a fluence of 25 J/cm2, and the left axilla with three passes at 28 J/cm2. Three years later, several foci within each axilla that periodically blistered were further treated with two passes of a short pulsed CO2 laser at a fluence of 15 J/cm2. At a 3.5-year follow-up, the patient reported continued resolution of her left axilla but disease persistence in her right axilla. CONCLUSION: HHD can be effectively treated with a short dwell time CO2 laser if appropriate laser parameters are used.

Efficacy of erbium:YAG laser ablation in Darier disease and Hailey-Hailey disease.

BACKGROUND: Among different surgical approaches, dermabrasion and carbon dioxide laser vaporization have been used to treat Hailey-Hailey disease (HHD) (familial benign chronic pemphigus) and Darier disease (DD) (keratosis follicularis), with various results. Because of the erbium: YAG laser's unique absorption characteristics in tissue water, erbium:YAG laser ablation combines the advantages of both techniques, avoiding thermal injury of vaporization and also allowing selectively deeper tissue removal in the follicular lesions of DD. Therefore, good results should be expected in both types of acantholytic disorders. OBSERVATIONS: Four patients (2 with HHD and 2 with DD) with different affected areas were treated with laser ablation. During a follow-up period ranging from 8 to 20 months, complete remission was achieved in 3 patients--2 with DD and 1 with HHD--and significant improvement was achieved in 1 patient with HHD. Histological examination of control biopsy specimens after ablation in 1 patient with DD revealed no signs of the disease and only a slight fibrosis in the papillary dermis. CONCLUSIONS: Erbium:YAG laser ablation effectively removes lesions of both HHD and DD and can also yield excellent long-term results in chronic, recalcitrant cases.

Benign familial pemphigus (Hailey-Hailey disease). Treatment with the pulsed carbon dioxide laser.

BACKGROUND: Benign familial pemphigus (BFP) is a chronic blistering disease with significant morbidity. Surgical methods are often needed to control flares in difficult cases. OBJECTIVE: To describe the response of BFP to vaporization with a pulsed carbon dioxide (CO2) laser. METHODS: A 38-year-old woman with chest and axillary involvement unresponsive to conventional therapy was treated with the UltraPulse 5000 Laser (Coherent Medical Group, Palo Alto, CA). After active sites of BFP showed good response to treatment, we treated uninvolved skin of the left axilla to assess the efficacy of prophylactic therapy. RESULTS: Treatment of affected areas, except biopsy sites, resulted in clearing of active lesions after 1-2 weeks. We noted striking sparing of the treated areas from developing subsequent disease. The region that was later treated prophylactically has shown minor, asymptomatic recurrence of BFP in less than 5% of the area treated over an 18-month follow-up period. CONCLUSION: The pulsed carbon dioxide laser is a useful modality in treatment of BFP. In our patient, prophylactic treatment led to near complete eradication of disease in the treated area. A controlled, larger study is needed to confirm our results, and to determine optimal laser parameters. Long-term effects and duration of remission remain to be determined.

Hailey-Hailey disease. Eradication by dermabrasion.

BACKGROUND: Chronic benign familial pemphigus (Hailey-Hailey disease) is a rare dominant genodermatosis that bothers patients by chronic recalcitrant plaques occurring mainly in the intertriginous areas. We looked for a surgical treatment that is not impaired by the complications and disadvantages of radical excision and grafting of involved areas. In 10 patients with debilitating Hailey-Hailey disease (five men, five women; age, 36 to 57 years), nine different involved areas (neck, axilla, cubital area, inframammary area, periumbilical area, groin and adjacent inner thigh, scrotum, vulva, and the perineal/perianal area) were treated by dermabrasion for a total of 46 treated regions. The plane of abrasion was located in the upper to middle dermis as verified by random sampling before and after surgery, thus documenting removal of the entire epidermis. RESULTS: In most instances, reepithelialization was completed after 7 to 10 days followed by excellent functional and cosmetic long-term results. When examined 3 to 79 months (median, 42 months) later, all treated areas remained completely disease free since surgery, except for major recurrences in four sites and minor recurrences in another four sites. Four of these areas were dermabraded again without subsequent relapse. CONCLUSIONS: Our results show that dermabrasion may be considered an excellent treatment of Hailey-Hailey disease that is unresponsive to conservative therapy. As a probable explanation, the intrinsic defect of cell adhesion in Hailey-Hailey disease seems to be limited to epidermal keratinocytes but spares adnexal structures from which rapid reepithelialization occurs.

[Surgical treatment of Hailey-Hailey disease or benign chronic familial pemphigus. Apropos of a case of submammary localization]. / Traitement chirurgical de la maladie de Hailey-Hailey ou pemphigus chronique bénin familial. A propos d'une localisation sous-mammaire.

The authors report a case of Hailey-Hailey disease or familial benign chronic pemphigus and review the literature concerning the surgical treatments used for this disabling disease. The majority of authors recommend excision followed by split skin graft, while others prefer excision followed by directed healing or simple excision-suture. The case reported here is the first reported case of cure of Hailey-Hailey disease combined with mammaplasty in a single stage.