Necrotizing Fasciitis Associated with Hailey-Hailey Disease: A Rare Case.

Necrotizing fasciitis (NF) is a rare and life-threatening infection of soft tissue characterised by rapid and extensive destruction of the skin, subcutaneous fat, and fascia. Early diagnosis of NF is challenging, and it can be very difficult to distinguish NF from other infectious diseases of skin and subcutaneous tissue. Imaging studies and laboratory investigations are crucial diagnostic means for NF. We diagnosed a case of NF with multiple organ dysfunction and septic shock, and this is the first case of NF associated with Hailey-Hailey disease (HHD) to our knowledge. Clinicians should be alert to signs and symptoms of NF in HHD and other skin diseases with damaged skin barrier function such as pemphigus, pemphigoid, and all kinds of ulcers, especially in diabetic and immunosuppressed patients. Key Words: Necrotizing fasciitis, Genodermatosis, Hailey-Hailey disease.

Familial "benign" pemphigus? Erythroderma and fatal outcome

Abstract Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

Familial "benign" pemphigus? Erythroderma and fatal outcome.

Hailey-Hailey disease, or familial benign pemphigus, is a rare bullous genodermatosis that usually presents with flaccid blisters, erosions, and maceration limited to flexural areas, resulting in increased morbidity and reduced quality of life for affected patients. The authors report an unusual case of generalized Hailey-Hailey disease with erythroderma and fatal outcome.

Incidental Acantholysis in Hailey-Hailey Disease (Microscopic Nikolsky Sign): An Underappreciated Histologic Sign.

Identification of subtle disease-specific histologic changes may be of significant help in early diagnosis of acantholytic skin diseases. Hailey-Hailey disease (HHD) is an autosomal dominant genodermatosis characterized by vesiculoerosive lesions favoring the intertriginous areas. Histologically, HHD is characterized by full-thickness acantholysis of the spinous layer in association with dyskeratosis of individual keratinocytes; a pemphigus vulgaris-like suprabasal pattern of acantholysis may be observed in the earliest stages of disease. HHD is characterized by highly variable expressivity regarding the age at onset and severity of the disease. Patients may present with late-onset and/or only mild disease. We report the recurrent presence of incidental foci of variably extensive, subclinical acantholysis in multiple bioptic specimens taken from a patient with known HHD for dermatologic conditions other than HHD. Such histologic finding has gone underappreciated in the literature, despite being a likely frequent occurrence in skin biopsies from HHD patients; recognition of this finding might represent a valuable diagnostic clue in selected cases of HHD.

Hailey-Hailey Disease With Coexistent Herpes Virus Infection: Insights Into the Diagnostic Conundrum of Herpetic/Pseudoherpetic Features in Cutaneous Acantholytic Disorders.

The specific histopathologic diagnosis of a primary acantholytic disorder takes into account the distribution and extent of acantholysis, presence or absence of dyskeratosis, nature of the dermal inflammatory cell infiltrate, and immunofluorescence findings. Herpes virus infection is a common cause of secondary acantholysis where distinctive viral cytopathic changes aid in making it a clear-cut diagnosis in majority of cases. We present a case of coexistence of Hailey-Hailey disease and herpes simplex virus infection to compare and contrast their histopathologic features. This is imperative because acantholytic cells from primary acantholytic disorders may occasionally show cytological features traditionally associated with herpes virus infection (pseudoherpetic changes). The objective of this article is to create a greater awareness of pseudoherpetic changes and also to explore the clinical significance of coexistence of a primary acantholytic disorder and herpes virus infection, as in this case.

Persisting Ulcerating Lesions After Cyclosporine Therapy for Erosions Found in Flexural Sites.

In February 2012, a patient with a 15-year history of painful, pruritic eruptions presented with a severe exacerbation that had lasted for the previous week. The patient also reported severe headaches, a fever of 38.9°C (102.1°F), a cough, and diarrhea, which had now resolved. Physical examination showed erythematous macerated patches with erosions in the left submammary region, bilateral axillae, and vaginal area (Figure 1), and greasy scale covering the scalp. Despite lessening of the lesions in the submammary region and abdomen with the administration of minocycline 100 mg twice per day and cyclosporine 100 mg three times per day, the patient continued to complain of persistent painful ulcerations in the labia majora and perianal area (Figure 2). The patient was started on acyclovir 400 mg three times per day for 10 days for positive herpes simplex viral culture and ampicillin for Proteus infection of the area.

Hailey-Hailey Disease Presenting as Lichenoid Plaques on the Thigh.

A 40-year-old woman presented with multiple itchy violaceous papules and plaques on the medial aspects of both thighs. This was accompanied by a burning sensation on friction and increased itching that interfered with her daily activities, particularly in the summer. The family history was noncontributory. Cutaneous examination showed multiple flat-topped hyperkeratotic violaceous papules and plaques, appearing in linear fashion on the medial aspects of the thighs bilaterally (Figure 1). Histopathologic examination showed acanthosis, widespread suprabasal acantholysis resembling a "dilapidated brick wall," without any evidence of dyskeratosis. There was a mild dermal perivascular infiltrate (Figure 2). On the basis of the clinical and histologic findings, she was diagnosed as having localized Hailey-Hailey disease. She was treated conservatively with topical corticosteroids and antimicrobials. She was also advised to lose weight, to avoid friction, and to wear loose-fitting cotton garments. There was marked symptomatic relief, and the lesion showed mild improvement over the next 2 months.

Disqueratosis acantolítica papular de la vulva en una mujer con pénfigo benigno familiar / Papular acantholytic dyskeratosis of the vulva in a woman with benign familial pemphigus

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Koebnerization of Hailey-Hailey disease into a cutaneous drug eruption of acute generalized exanthematous pustulosis associated with systemic symptoms.

We describe a 65-year-old Caucasian female with well-controlled Hailey-Hailey disease (HHD) who developed acute generalized exanthematous pustulosis (AGEP) with severe systemic symptoms. Despite sparing of the patient's intertriginous skin, histopathologic evidence of HHD was observed in all biopsies, suggestive of a unique koebernization phenomenon of HHD to areas of cutaneous drug eruption. While internal organ involvement is less commonly reported in AGEP, there are an increasing number of patients with signs and symptoms suggestive of an AGEP/drug reaction with eosinophilia and systemic symptoms (DRESS) spectrum of cutaneous drug disorders. Early diagnosis of patients with AGEP and systemic symptoms is critical so that these patients may receive prompt and aggressive systemic therapy to decrease the risk of end organ damage and improve overall morbidity and mortality.

Papular acantholytic dyskeratosis of the vulva associated with familial Hailey-Hailey disease.

Papular acantholytic dyskeratosis (PAD) of the vulva is a rare, chronic disorder first described in 1984. It presents in young women as white to skin-coloured smooth papules over the vulva, which are persistent but asymptomatic. Histologically, there is hyperkeratosis and focal parakeratosis with acantholytic and dyskeratotic cells forming corps ronds and grains, placing PAD within Ackerman's spectrum of focal acantholytic dyskeratoses with Hailey-Hailey disease (HHD) and Darier disease. There have been 17 previous reports of PAD of the vulva, to our knowledge. Only one demonstrated a familial pattern, and none of the cases was associated with a family history of HHD. This is the first report of PAD and HHD in a single family, suggesting that PAD and HHD lie on a spectrum of disease and are genetically linked.

Acute generalized erythematous pustulosis occurring with Hailey-Hailey disease.

A 70-year-old woman urgently presented with severe eruptive skin dermatitis associated with fever and malaise 7 days after taking clindamycin for an unknown skin eruption. She had a 40-year-long history of well-controlled Hailey-Hailey disease. Physical examination revealed erythrodermic skin changes covering more than 80% of the patient's body surface, with hundreds of nonfollicular pustules. Many of the pustules fused into large bullae, involving the intertriginous as well as the extensor areas, sparing the mucosa. Her body temperature was 103 degrees F. Laboratory workup was significant for neutrophilia with a white cell count > 10,000/mm3.