Source

Department of Dermatology, School of Medicine, Keio University, 35 Shinanomachi, Shinjuku-ku, Tokyo 160-8582, Japan.

Abstract

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.

PMID: 11450592     Free full text

Source

Departments of Pathology and Dermatology, University of Virginia Health Sciences Center, Charlottesville, Virginia 22908-0214, USA.

Abstract

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.

PMID: 10901726

Source

Department of Dermatology, Barnsley District General Hospital, Gawber Rd, Barnsley S75 2EP, U.K.

Abstract

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.

PMID: 10735968

Source

Department of Dermatology, Nihon University, School of Medicine, 30-1 Oyaguchi-Kamimachi, Itabashi-Ku, Tokyo, Japan.

Abstract

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.

PMID: 10233276

Source

Department of Dermatology, Wilford Hall Medical Center, Lackland AFB, Texas, USA.

Abstract

Hailey-Hailey disease (chronic benign familial pemphigus) is a blistering disease characterized by episodic maceration and erosions in areas subject to friction and trauma such as the neck, axilla, and other intertriginous areas. We report a case in which a griseofulvin-induced drug eruption led to a generalized vesiculobullous eruption requiring treatment in a burn treatment center. This emphasizes that Hailey-Hailey disease, usually confined to relatively small areas, has the potential to cause blistering throughout the epidermis under certain conditions.

PMID: 8565613

Source

Department of Dermatology, Royal South Hants Hospital.

Abstract

Benign familial chronic pemphigus, or Hailey-Hailey disease, is a rare hereditary condition characterized by development of blisters at sites of friction such as the neck, axillae and groin. Contact sensitivity to topical medications is reported to be common and routine patch testing has been strongly advocated. We report a case of Hailey-Hailey disease in a 43-year-old veterinary surgeon who presented with an acute exacerbation of his disease caused by herpes simplex virus type 1 (HSV). Patch testing was carried out to exclude a coexistent contact dermatitis and was complicated by severe local blistering. We are reporting this case to remind clinicians that HSV is a recognized cause of exacerbations of this disease and to warn that patch testing is not without hazard.

PMID: 1451302

Source

Department of Dermatology, Slade Hospital, Oxford, U.K.

Abstract

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.

PMID: 1532504