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1/36. 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. ( info)

2/36. Human papillomavirus types 16 and 39 in a vulval carcinoma occurring in a woman with Hailey-Hailey disease.

    A woman with Hailey-Hailey disease, suffering from carcinoma of the vulva, was examined by histology and for the presence of human papillomavirus (HPV) DNA by polymerase chain reaction (PCR) and in situ hybridization. Our diagnosis by histological examination revealed the vulval carcinoma to be a squamous cell carcinoma (SCC), adjacent to lesions of Hailey-Hailey disease and severe dysplasia/carcinoma in situ [vulval intraepithelial neoplasia (VIN) III]. The PCR with consensus primers for the L1 region (L1-PCR) successfully amplified HPV DNA using total DNA extracted from formalin-fixed and paraffin-embedded tissue specimens. Restriction fragment length polymorphism analysis and sequencing of L1-PCR products revealed HPV types 16 and 39. HPV 16-specific primers for the E6 region identified HPV 16 DNA. in situ hybridization analysis with biotinylated HPV 16 and 39 dna probes revealed the presence of the HPV 39 genome in the nuclei of the tumour cells in the SCC. These results indicate that HPV 16 and 39 are associated with lesions in vulval carcinoma. Regarding the patient's susceptibility to infection in the case of Hailey-Hailey disease, there is a possibility that HPV was inoculated into the lesions of Hailey-Hailey disease and induced those of VIN III and SCC. ( info)

3/36. 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. ( info)

4/36. Squamous cell carcinoma arising in Hailey-Hailey disease of the vulva.

    A 61-year-old woman, who was known to have Hailey-Hailey disease, presented with increasing vulval soreness. biopsy showed vulval intraepithelial neoplasia (VIN) 3 and subsequent histology from a vulvectomy specimen showed extensive VIN with early invasive squamous cell carcinoma. This may be another example of chronic inflammation of the vulval area leading to the development of squamous cell carcinoma. However, in this case, chronic human papillomavirus may also have played a part, leading to VIN and reactivation of the Hailey-Hailey disease. We can find no previous reports of squamous cell carcinoma developing in the setting of Hailey-Hailey disease. ( info)

5/36. Botulinum toxin type A for the treatment of axillary Hailey-Hailey disease.

    BACKGROUND: Hailey-Hailey disease is an inherited acantholytic disorder affecting the intertriginous areas of the body which is exacerbated by sweat, moisture, and friction. The disease is frequently resistant to conventional nonsurgical treatment. OBJECTIVE: To evaluate whether chemodenervation of sweat glands would improve the course of the disease in a patient with Hailey-Hailey. methods: We used low-dose treatment of the left axilla with botulinum toxin type A, the right axilla being used as a control, followed by treatment of both axillae with the optimal dose routinely used for the treatment of axillary hyperhidrosis. RESULTS: After one treatment with a low dose of botulinum toxin type A, we observed partial improvement of the treated axilla. With subsequent treatment of both axillae with the recommended dose for axillary hyperhidrosis, we observed a sustained complete remission of the disease in the treated axillae. CONCLUSION: Botulinum toxin type A may be an effective and safe nonsurgical alternative for the treatment of benign familial pemphigus in intertriginous areas such as the axillae. ( info)

6/36. 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. ( info)

7/36. Squamous cell carcinoma arising in Hailey-Hailey disease.

    Hailey-Hailey disease is a recurrent, autosomal dominant vesiculobullous dermatotis with a predilection for intertrigenous areas. We report what we believe to be the first case of squamous cell carcinoma arising de novo in a skin lesion of Hailey-Hailey disease. The occurrence of malignant neoplasms arising in the skin lesions of Hailey-Hailey disease and other acantholytic dermatoses is reviewed. ( info)

8/36. A case of generalized Hailey-Hailey disease with fatal liver injury.

    We report a case of a 59-year-old man with a severe generalized form of Hailey-Hailey disease that was complicated by fatal liver injury. Erosive lesions were first noted in the axillary and perianal regions at 15 year of age, and Hailey-Hailey disease was diagnosed based on the clinical features and histologic findings in skin biopsy specimens. The patient was treated with at first topical steroids and later a low dose of a corticosteroid, but the skin lesions gradually became generalized. At 45 years of age liver dysfunction was detected after azathioprine and vinblastine treatment for the generalized skin lesions. The liver injury gradually progressed and finally the patient died. The gene responsible for Hailey-Hailey disease was recently identified as ATP2C1, and it encodes a Ca(2 )-transport ATPase with broad expression, including in skin and liver. This finding suggests that mutation of the ATP2C1 gene may give rise to an extracutaneous phenotype, such as the liver dysfunction observed in severe cases, including our own. Further accumulation of cases is necessary to determine whether this is true. ( info)

9/36. Acute generalized Hailey-Hailey disease.

    A patient with extensive histologically proven Hailey-Hailey disease is described whose initial clinical presentation was suggestive of erythema multiforme or toxic epidermal necrolysis. This potentially misleading morphology of acute proven Hailey-Hailey disease has not been described previously and may be a consequence of bacterial infection exacerbating acantholysis. ( info)

10/36. Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign pemphigus (Hailey-Hailey disease).

    Familial benign pemphigus is a chronic, recurrent, autosomal dominant blistering disease that may significantly affect quality of life. Surgical methods are often needed to control flares in difficult cases. We describe the use of photodynamic therapy (PDT) with topical 5-aminolevulinic acid (ALA) followed by irradiation with incoherent light (ALA-PDT) in 2 patients with chronic, recalcitrant familial benign pemphigus. ( info)
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