Hailey-Hailey Disease Responding to Thalidomide

Familial benign chronic pemphigus or Hailey-Hailey disease (HHD) is a rare autosomal dominant disorder characterized by the development of recurrent blisters and erosions in the intertriginous areas. Various topical and systemic treatment options include corticosteroids, topical 5-fluorouracil, topical vitamin D analogs, topical zinc oxide, dapsone, psoralen plus ultraviolet A, systemic retinoids, cyclosporine, methotrexate, and photodynamic therapy. In recalcitrant cases, further options including, invasive methods such as grenz ray therapy, carbon dioxide laser abrasion, and erbium: YAG laser ablation, dermabrasion, electron beam therapy, botulinum toxin, and full-thickness excision of affected skin with repair by split-thickness grafting have been reported as useful in treatment of HHD. We describe a case of HHD who was treated with several treatment modalities including antibiotics, corticosteroids, and dapsone earlier and when presented to us had a severe recalcitrant disease. Thalidomide, as a modality of treatment has been successfully used in few cases earlier. Our patient responded well to thalidomide.     

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.     

超脉冲二氧化碳激光治疗家族性良性慢性天疱疮2例并文献复习

本文报告2例家族性良性慢性天疱疮（HHD）超脉冲二氧化碳激光有效。2例HHD患者经超脉冲二氧化碳激光治疗1次后症状明显改善,无明显瘢痕,随访6个月未复发。结合本文病例对既往文献进行了复习。     

Pemphigus chronicus benignus familiaris (M. Hailey-Hailey)

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.     

Familial benign chronic pemphigus (Hailey-Hailey disease). Treatment with carbon dioxide laser vaporization.

BACKGROUND--Familial benign chronic pemphigus (Hailey-Hailey disease) is characterized by recurrent blistering lesions, mainly in the neck and intertriginous areas. No controlled studies on laser treatment of this condition have been published. OBSERVATIONS--We used carbon dioxide laser vaporization to treat skin lesions in eight patients. In the six patients with symmetrical skin lesions, one lesion was chosen for CO2 laser vaporization and a corresponding lesion of similar location, size, and severity on the other side of the body was left untreated. Laser vaporization was performed in defocus in four to five layers. In five of six patients with symmetrical control lesions, laser-treated lesions showed substantial improvement in comparison with the untreated lesions. A similar improvement was seen in the treated lesions of the two patients without control lesions. During the mean follow-up of 20 months (range, 10 to 27 months), skin lesions recurred in the laser-treated areas in three patients, but not in the other five. Histopathologic studies performed on two patients indicated that the effect of the CO2 treatment might be due to fibrosis of the upper dermal tissues. The adnexal glands were unaffected. CONCLUSIONS--Carbon dioxide laser vaporization is an effective treatment for familial benign chronic pemphigus.     

Treatment of angiofibromas of tuberous sclerosis with carbon dioxide laser vaporization

The therapeutic approach to angiofibromas in tuberous sclerosis is a challenge for the Dermatologist. The latest treatment modalities include use of argon and carbon dioxide lasers. We describe the ease of an 18-year-old Caucasian boy with multiple angiofibromas on the face, treated with CO₂ laser with good cosmetic results.     

Hailey-Hailey disease with dissemination and eczema herpeticatum in therapy with etretinate

We report a 39-year-old patient with Hailey-Hailey disease localized mainly in the genito-femoral and scrotal areas. During hospital treatment the condition worsened due to superinfection with Candida albicans and gram-negative bacteria. Treatment with systemic steroids followed by aromatic retinoids was tried: eczema herpeticum developed in the affected areas, and dissemination of Hailey-Hailey disease ultimately occurred with papulo-vesicular and histologically characteristic lesions.     

激光和强脉冲光治疗酒渣鼻的治疗进展

酒渣鼻是常见皮肤病,其临床特点包括皮肤潮红、持久性红斑、丘疹脓疱和毛细血管扩张.激光是治疗酒渣鼻毛细血管扩张和鼻赘期的理想选择,最常用的激光包括脉冲染料激光(波长585/595 nm)、长脉宽Nd:YAG激光(波长1064 nm)、CO2激光、铒激光和点阵激光等.强脉冲光、脉冲染料激光用于改善酒渣鼻红斑和毛细血管扩张,安全性高,停工期短.长脉宽Nd:YAG激光仅用于治疗较粗血管和难治性毛细血管扩张.CO2激光、铒激光和点阵激光用于改善酒渣鼻鼻赘.临床上多种激光联合治疗效果更佳.     

Tratamiento de la enfermedad de Hailey-Hailey con láser de dióxido de carbono: tratamiento en 8 pacientes

Introduction and objectivesHailey-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 methodsRetrospective 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.ResultsSatisfactory 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.ConclusionsCarbon dioxide laser therapy was safe and effective in producing medium- to long-term improvement in HHD symptoms that were refractory to conventional treatments.     

Carbon dioxide laser ablation for trichoepitheliomas: The largest reported series

Trichoepitheliomas are benign cutaneous tumours often occurring on the face and can lead to considerable psychological distress to its sufferers. Treatment is often difficult, and surgery is limited by the obvious scars and multiple numbers of lesions. Carbon dioxide laser ablation can be used safely with good cosmetic outcome and low recurrence rate, and in this article, we describe our experience in the treatment of these tumours with the carbon dioxide laser. This is the largest reported series in the literature.     

Ablative laser resurfacing: high-energy pulsed carbon dioxide and erbium:yttrium-aluminum-garnet

The development of the short-pulsed high-energy carbon dioxide laser in the mid 1990's led to the emergence of laser skin resurfacing. Used in the continuous mode, the CO(2) laser can cut and coagulate simultaneously. Used in the pulsed mode, the CO(2) laser is a powerful tool for epidermal ablation in many different contexts both therapeutic and cosmetic. Both the CO(2) and Erbium YAG lasers emit light in the infrared spectrum. Energy is preferentially absorbed by intracellular water creating rapid heating and vaporization of tissue. Because of the wavelength of the Er:YAG laser (2940 nm) more closely approximates the absorption peak of water (3000 nm) the target chromophore than the CO(2) laser (10,600 nm) nearly all of the energy is absorbed in the epidermis and papillary dermis yielding superficial ablation and less underlying thermal damage. The advantages, disadvantages, and applications of each type of laser resurfacing will be discussed. Despite proven efficacy, the public acceptance of laser resurfacing has declined with the emergence of new laser systems that cause dermal remodeling without ablating the overlying epidermis dramatically reducing recovery time. In the absence of blinded comparison studies, it remains unclear whether the clinical results of the newer 'nonablative' laser systems compare with their ablative predecessors.     

Nevus sebaceus

A 35-year-old woman presented with cosmetically disturbing facial plaques, which had been present since birth and were consistent with nevus sebaceus on histopathologic examination. Nevus sebaceus is a congenital, hairless plaque, which is found most commonly on the scalp and face and typically becomes thicker and more verrucous in appearance as the patient enters adulthood. There is an association with benign and malignant neoplasms. Typically, these lesions are either removed surgically or the patients are followed clinically. Other therapeutic modalities utilized for cosmesis include carbon dioxide laser and photodynamic therapy.     

Radiofrequency ablation: A safe and economical modality in treatment of Brooke-Spiegler syndrome

Brooke-Spiegler syndrome is an uncommon disease. Patients have a predisposition to develop cutaneous adnexal neoplasms such as cylindromas, trichoepitheliomas, spiradenomas, trichoblastomas, basal cell carcinomas, follicular cysts, and organoid nevi. Malignant transformation of preexisting tumors also occurs in these individuals. Various techniques have been used for the treatment of trichoepitheliomas and cylindromas including excision, electrocautery, carbon dioxide laser ablation, cryosurgery, and radiotherapy. In our case, cylindromas were ablated by radiofrequency in multiple sittings. Trichoepitheliomas were ablated using coagulation mode with power # 3 to 3.5. Cosmetically acceptable results were obtained in 100 percent of the cylindromas and 70 percent of the trichoepitheliomas (Visual Analog Scale). The radiofrequency ablation technique under different modes can be used in both large tumors as well as smaller ones, especially in developing countries because it is very cost effective and easily accessible.     

Seborrhoeic dermatitis of the scalp—a manifestation of Hailey–Hailey disease in a predisposed individual?

A 59-year-old man was found to have typical Hailey-Hailey disease of the back, neck and axillae. In addition, he had fine white scaling in the scalp and postauricular areas. Despite the clinical appearance of seborrhoeic dermatitis, a biopsy of his scalp showed prominent suprabasal epidermal separation with acantholysis. We propose that in a genetically predisposed individual, Hailey-Hailey disease can assume atypical and non-specific appearances.     

Hypertrophic scar formation following carbon dioxide laser ablation of plantar warts in cyclosporin-treated patients

We present four renal transplant patients who developed hypertrophic scars following carbon dioxide laser ablation of recalcitrant plantar warts. All of the patients were on long-term treatment with cyclosporin, which we believe to be responsible. We discuss several possible mechanisms by which cyclosporin may influence wound healing and scarring.     

Vesiculobullous Hailey-Hailey disease: Successful treatment with oral retinoids

A 56-year-old male presented with a pruritic, generalized vesiculobullous eruption. His past history revealed classical symptoms of limited Hailey-Hailey disease for 34 years. Clinically, vesicles, bullae and occasional pustules were present and multiple biopsies confirmed this to be an unusual presentation of Hailey-Hailey disease. Various therapeutic modalities including topical and oral antibiotics, oral prednisone and dapsone failed to achieve sustained remission. Treatment with lowdose oral etretinate (25 mg daily) produced marked clinical improvement with complete suppression of new vesicle formation after 6 weeks.     

Mycosis fungoides palmaris et plantaris: successful treatment with the carbon dioxide laser

Mycosis fungoides palmaris et plantaris (MFPP) is an uncommon form of mycosis fungoides with main involvement on palms and soles. The lesions may be quite hyperkeratotic. A 56-year-old patient with MFPP was successfully treated with the carbon dioxide (CO₂) laser. There was no recurrence of disease at treated sites and excellent cosmetic results were obtained. Treatment with the CO₂ laser may be considered a therapeutic option.     

Spontaneous regression of subcutaneous in‐transit malignant melanoma deposits of the lower leg after treatment with the carbon dioxide laser

Primary malignant melanoma (MM) is one of the few cancers that can show spontaneous regression, and although this is well described, the mechanisms behind this are unclear. Spontaneous regression of secondary metastatic deposits of melanoma is rare and there are only a handful of case reports supporting this phenomenon. We report a patient with in‐transit MM metastatic disease of the leg, who underwent carbon dioxide (CO₂) laser ablation, and subsequently developed remission of untreated deposits. We discuss the possible immunological mechanism for this phenomenon.     

A novel splice mutation in the ATP2C1 gene in a woman with concomitant psoriasis vulgaris and disseminated Hailey-Hailey disease

Concurrent psoriasis vulgaris and Hailey-Hailey disease is very rare. The clinical and pathologic findings of widespread Hailey-Hailey disease in a 48-year-old woman with pre-existing generalized psoriasis vulgaris were described. In our patient, the vesicular eruption of Hailey-Hailey disease was obscured clinically by the psoriatic lesions. The diagnosis of both diseases was confirmed pathologically. The patient had a total of five skin biopsies performed over the neck, flank area, back, pubic area, and a finger. Acantholytic dyskeratosis, changes suggestive of Hailey-Hailey disease, were found in four specimens, psoriasis in two specimens, and both diseases in one specimen. The diagnosis of Hailey-Hailey disease was further confirmed by detecting a novel splice mutation (832G>A) in the ATP2C1 gene. Our case illustrated that diagnosis of disseminated Hailey-Hailey disease may easily be missed in a patient with a pre-existing generalized pruritic eruption, such as psoriasis. The appearance of eczematous vesicular eruption or eroded intertrigo-like lesions in a patient with pre-existing generalized eruption should raise a suspicion of Hailey-Hailey disease.     

Outcome of carbon dioxide laser therapy for persistent cutaneous viral warts

Twenty-two adults with persistent, symptomatic viral warts on the hands or feet which had failed to respond to conventional therapy were treated by carbon dioxide (CO2) laser ablation under local anaesthesia. Four patients did not return for follow-up treatment. Of the remaining 18 patients, 12 had plantar warts, the other six had warts on the hands that were mainly periungual. The median duration of the warts prior to laser treatment was 5 years. The areas treated were solitary in eight and multiple in 10 cases. After an average follow-up period of 10 months (minimum 6 months), all of the treated areas were clear of warts in 10 patients (56%), some had cleared in three, and all the treated warts had relapsed in the other five patients. Side-effects were commonly observed, especially post-operative pain, temporary loss of function of the treated part and scarring. The use of the CO2 laser may clear otherwise resistant viral warts but a significant morbidity may occur with this method of treatment.