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  Table of Contents  
Year : 2016  |  Volume : 7  |  Issue : 2  |  Page : 147-148  

Hailey-Hailey disease

1 Department of Dermatology, Venereology and Leprosy, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra, India
2 Department of Pathology, Mahatma Gandhi Institute of Medical Sciences, Wardha, Maharashtra, India

Date of Web Publication4-Mar-2016

Correspondence Address:
Prof. Sumit Kar
Department of Dermatology, Venereology and Leprosy, Mahatma Gandhi Institute of Medical Sciences, Sewagram - 442 102, Wardha, Maharashtra
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.178090

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How to cite this article:
Yadav N, Madke B, Kar S, Prasad K, Gangane N. Hailey-Hailey disease. Indian Dermatol Online J 2016;7:147-8

How to cite this URL:
Yadav N, Madke B, Kar S, Prasad K, Gangane N. Hailey-Hailey disease. Indian Dermatol Online J [serial online] 2016 [cited 2020 May 28];7:147-8. Available from: http://www.idoj.in/text.asp?2016/7/2/147/178090

   Discussion Top

The prevalence of Hailey–Hailey disease (HHD) also known as familial benign chronic pemphigus is 1:50,000.[1] It is an autosomal dominant disorder caused due to mutation in ATP2C1 gene.[2],[3] The gene encodes an adenosine triphosphate–powered calcium pump in the Golgi apparatus of epidermal cells.[4] Faulty calcium pump action leads to disorganized function of desmogleins, which are calcium-dependent adherence proteins (cadherins). Flaccid vesicles or bullae are the primary lesions in Hailey–Hailey disease. These lesions rupture easily leaving behind macerated erosions. Most commonly involved sites are the neck, axillae, and groins. Sometimes lesions can also appear on scalp, antecubital or popliteal fossa, and trunk. Conjunctiva, mucosa, and vulva involvement is rarely seen. A positive family history of HHD is present in many patients.[5] The management of HHD is challenging. At present, there is no reported cure for HHD. The treatment is primarily aimed at symptomatic relief. A concoction of topical antibiotics; antifungal agents; as well as systemic, topical, and intralesional corticosteroids have been found to be useful in the management of HHD in many cases.[5],[6] Other drugs that have also proved to be effective are cyclosporine, retinoids, botulinum toxin A, and dapsone.[1] Recalcitrant plaques in HHD give better result with ablative lasers such as carbon dioxide lasers and erbium:YAG laser.[7]
Figure 1: Photographs showing macerated hyperpigmented plaques over the groins

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Figure 2: Photographs showing macerated hyperpigmented plaques over the neck

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Figure 3: Histopathology slide showing suprabasal clefting with a few acantholytic cells and dilapidated brick wall appearance H and E, X10

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   References Top

Vasudevan B, Verma R, Badwal S, Neema S, Mitra D, Sethumadhavan T. Hailey-Hailey disease with skin lesions at unusual sites and a good response to acitretin. Indian J Dermatol Venereol Leprol 2015;81:88-91.  Back to cited text no. 1
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de Aquino Paulo Filho T, deFreitas YK, da Nóbrega MT, Lima CB, Carriço BL, Silva MA, et al. Hailey-Hailey disease associated with herpetic eczema-the value of the Tzanck smear test. Dermatol Pract Concept 2014;4:29-31.  Back to cited text no. 2
van Beek N, Patsatsi A, Gupta Y, Möller S, Freitag M, Lemcke S, et al. A family with atypical Hailey–Hailey disease: Is there more to the underlying genetics than ATP2C1? PLoS One 2015;10:e0121253.  Back to cited text no. 3
Hu Z, Bonifas JM, Beech J, Bench G, Shigihara T, Ogawa H, et al. Mutations in ATP2C1, encoding a calcium pump, cause Hailey–Hailey disease. Nat Genet 2000;24:61-5.  Back to cited text no. 4
James WD, Berger TG, Elston DM. Familial benign chronic pemphigus (Hailey-Hailey Disease). In: James WD, Berger TG, Elston DM, editors. Andrews' Diseases of the Skin: Clinical Dermatology. 10th ed. Toronto: Elsevier Inc.; 2006. p. 559-60.  Back to cited text no. 5
D'Errico A, Bonciani Bonciolini V, Verdelli A, Antiga E, Fabbri P, et al. Hailey-Hailey disease treated with methotrexate. J Dermatol Case Rep 2012;6:49-51.  Back to cited text no. 6
Ortiz AE, Zachary CB. Laser therapy for Hailey-Hailey disease: Review of the literature and a case report. Dermatol Reports 2011;3:e28.  Back to cited text no. 7


  [Figure 1], [Figure 2], [Figure 3]


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