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Available from: Calogero Pagliarello, Aug 17, 2015
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    ABSTRACT: 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.
    Indian Journal of Dermatology 03/2014; 59(2):190-2. DOI:10.4103/0019-5154.127684
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    ABSTRACT: 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.
    Journal of the European Academy of Dermatology and Venereology 11/2014; 29(6). DOI:10.1111/jdv.12875 · 2.83 Impact Factor
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    ABSTRACT: Hailey-Hailey disease, also called benign familial pemphigus, is a late-onset blistering disorder that affects the flexures. There are typically painful erosions and cracks in affected areas. Lesions generally begin between 20 and 40 years of age. In two third of all cases, positive family history is detected. In pathogenesis, there is a defect in keratinocyte adhesion due to ATP2 C1 gene mutation. The result of the desmosomal decomposition is acantholysis. Menstruation, pregnancy, skin infections, physical trauma, excessive sweating and exposure to ultraviolet radiation are important triggering factors. Histopathologic changes are suprabasal acantholysis and formation of intraepidermal bullae. In the epidermis, a partial acantholysis that looks like broken bricks is observed. Copyright © 2015 Elsevier Inc. All rights reserved.
    Clinics in Dermatology 04/2015; 33(4). DOI:10.1016/j.clindermatol.2015.04.006 · 2.47 Impact Factor