Hailey Hailey disease-a rare case report

Authors

  • Kumar Prateek Department of Dermatology, Rajendra Institute Of Medical Sciences, Ranchi, Jharkhand, India
  • Meena Rinoo Banwarilal Department of Dermatology, Rajendra Institute Of Medical Sciences, Ranchi, Jharkhand, India
  • Shyam Sundar Chaudhary Department of Dermatology, Rajendra Institute Of Medical Sciences, Ranchi, Jharkhand, India
  • Meenakshi Garg Department of Dermatology, Rajendra Institute Of Medical Sciences, Ranchi, Jharkhand, India

DOI:

https://doi.org/10.18203/issn.2455-4529.IntJResDermatol20162833

Keywords:

ATPC2 gene, Dilapidated brick wall appearance, Hailey Hailey disease

Abstract

Hailey-Hailey disease is an autosomal dominant acantholytic disorder relatively uncommon in India. It is characterized by painful, pruritic, foul smelling vesicles and bullous lesions with erosions in intertriginous areas such as the neck, axillae, inframammary areas and groin. The defect in ATPC2 gene leads to calcium channel dysfunction which results in defect in epidermis. Fungal infection, intertrigo, psoriasis, extramammary Paget's disease, acanthosis nigricans, pemphigus vegetans and Darier's disease are some of the dermatosis from which it needs to be differentiated. Histopathology has a key role in diagnosis of Hailey-Hailey disease with characteristic ‘dilapidated brick wall' appearance. Hailey-Hailey disease can be easily misdiagnosed by general physicians due to lack of knowledge of this uncommon disease as it resembles dermatosis involving intertriginous areas. Due to its relapsing and remitting course there is a need to have effective treatment options. We report a case of 45 year old middle aged female who presented with on and off itchy papules & vesicles over right inframammmary region around the umbilicus & medial aspect of right thigh. Skin biopsy revealed a diagnosis of Hailey-Hailey disease. This case is being reported due to its rare occurrence.

Author Biography

Kumar Prateek, Department of Dermatology, Rajendra Institute Of Medical Sciences, Ranchi, Jharkhand, India

Department of dermatology, Postgraduate student

References

Persić-Vojinović S, Milavec-Puretić V, Dobrić I, Rados J, Spoljar S. Disseminated Hailey-Hailey disease treated with topical tacrolimus and oral erythromycin: Case report and review of the literature. Acta Dermatovenerol Croat. 2006;14:253–7.

Schirren H, Schirren CG, Schluppen EM, Volkenandt M, Kind P. Exacerbation of the Hailey-Hailey disease with herpes simplex virus. Detection with polymerase chain reaction. Hautarzt. 1995;46:494-7.

Valia AR, Ramesh V, Jerajani HR, Fernandez RJ. In: R G Valia, Ameet R Valia, eds. IADVL Textbook of Dermatology Blistering Disorder. 3rd Ed, Vol 1, Bhalani Publishers; 2008: 1127-1128.

Sangoram SS, Indurkar VA, Amin-Hon VS, Nishigandh. Hailey-Hailey Disease- A Case Report. Pravara Med Rev. 2010;2:31-2.

Cheng TS, Jong KK. A man with familial flexural rash: a case of Hailey-Hailey; Disease. Hong Kong J Dermatol Venereol. 2005;13:98-100.

Michel B. "Familial benign chronic pemphigus" by Hailey and Hailey, April 1939. Commentary: Hailey-Hailey disease, familial benign chronic pemphigus. Arch Dermatol. 1982;118:774-83.

Warycha M, Patel R, Meehan S, Merola JF. Familial benign chronic pemphigus (Hailey- Hailey disease). Dermatol Online J. 2009;15:15.

Mashiko M, Akiyama M, Tsuji-Abe Y, Shimizu H. Bacterial infection-induced generalized HaileyHailey disease successfully treated by etretinate. Clin Exp Dermatol. 2006;31:57-9.

Chlebicka I, Jankowska-Konsur A, Maj J, Plomer-Niezgoda E, Cezary Szepietowski J. Generalized Hailey-Hailey disease triggered by nonsteroidal antiinflammatory drug-induced rash: case report. Acta Dermatovenerol Croat. 2012;20(3):201-3.

Thapa DP, Jha AK, Pudasaini S, Kharel C, Shrestha S. Genital Hailey-Hailey disease: A case report. Our Dermatol Online. 2013;4:87-8.

Botvinick I. Familial benign pemphigus with oral mucous membrane lesions. Cutis. 1973;12:371-3.

Holst VA, Fair KP, Wilson BB, Patterson JW. Squamous cell carcinoma arising in Hailey-Disorders of epidermal maturation and keratinization. In: Weedon D. Skin Pathology 2nd ed. Churchill Livingstone; 2002: 298-299.

Weedon D. Disorders of epidermal maturation and keratinization. In: Weedon’s, Skin Pathology. David Weedon (Ed) 2nd ed. Edinburgh, London, New York, Oxford, Philadelphia, St. Louis, Sidney, Toronto: Churchill Livingstone; 2002: 298-299

Burge SM. Hailey–Hailey disease: The clinical features, response to treatmentand prognosis. Br J Dermatol. 1992;126:275-82.

Ikeda S, Suga Y, Ogawa H. Successful management of Hailey–Hailey disease with potent topical steroid ointment. J Dermatol Sci. 1993;5:205–11.

Galimberti RL, Kowalczuk AM, Bianchi O, Bonino MV, Garcia Garcia A. Chronic benign familial pemphigus. Int J Dermatol. 1988;27:495–500.

Marsch W, Stüttgen G. Generalized Hailey–Hailey disease. Br J Dermatol. 1978;99:553–60.

Defresne C, Adam C, De Marneffe K. Pemphigus chronique benin familial de Hailey–Hailey. Dermatologica. 1982;165:624–6.

Mayuzumi N, Ikeda S, Kawada H, Ogawa H. Effects of ultraviolet B irradiation, proinflammatory cytokines and raised extracellular calcium concentration on the expression of ATP2A2 and ATP2C1. Br J Dermatol. 2005;152:697–701.

Mayuzumi N, Ikeda S, Kawada H, Ogawa H. Effects of drugs and anticytokine antibodies on expression of ATP2A2 and ATP2C1 in cultured normal human keratinocytes. Br J Dermatol. 2005;152:920–4.

Leyden J, Marples R. Superinfection induced by antibiotics in familial benign chronic pemphigus. Acta Derm Venereol (Stockh). 1973;53:61–4.

Kellermayer R, Szigeti R, Keeling KM, Bedekovics T, Bedwell DM. Aminoglycosides as potential pharmacogenetic agents in the treatment of Hailey–Hailey disease. J Invest Dermatol. 2006;126:229–31.

Jitsukawa K, Ring J, Weyer U, Kimmig W, Radloff H. Topical cyclosporine in chronic benign familial pemphigus (Hailey–Hailey disease). J Am Acad Dermatol. 1992;27:625–6.

Cecchi R, Bartoli L, Brunetti L. Pemfi go familiare benigno (malattia di Hailey–Hailey) trattato con ciclosporina A orale. G Ital Dermatol Venereol. 1993;128:615–7.

Berth Jones J, Smith SG, GrahamBrown RAC. Benign familial chronic pemphigus(Hailey–Hailey disease) responds to cyclosporin. Clin Expt Dermatol. 1995;20:70–2.

Umar SA, Bhattacharjee P, Brodell RT. Treatment of Hailey–Hailey disease with tacrolimus ointment and clobetasol propionate foam. J Drugs Dermatol. 2004;3:200–3.

Rocha Paris F, Fidalgo A, Baptista J, Caldas LL, Ferreira A. Topical tacrolimus in Hailey–Hailey disease. Int J Tissue React. 2005;27:151–4.

Bianchi L, Chimenti MS, Giunta A. Treatment of Hailey–Hailey disease with topical calcitriol. J Am Acad Dermatol. 2004;51:475–6.

Rajpara SM, King CM. Hailey–Hailey disease responsive to topical calcitriol. BrJ Dermatol. 2005;152:816–7.

Hunt MJ, Salisbury EL, Painter DM, Lee S. Vesiculobullous Hailey–Hailey disease:successful treatment with oral retinoids. Australas J Dermatol. 1996;37:196–8.

Mashiko M, Akiyama M, Tsuji-Abe Y, Shimizu H. Bacterial infection-induced generalized Hailey–Hailey disease successfully treated by etretinate. Clin Exp Dermatol. 2006;31:57–9.

Berger EM, Galadari HI, Gottlieb AB. Successful treatment of Hailey–Haileydisease with acitretin. J Drugs Dermatol. 2007;6:734–6.

Norman R, Greenberg RG, Jackson JM. Case reports of etanercept in infl amatory dermatoses. J Am Acad Dermatol. 2006;4:139–42.

Lapiere JC, Hirsh A, Gordon KB, Cook B, Montalvo A. Botulinum toxin type A for the treatment of axillary Hailey–Hailey disease. Dermatol Surg. 2000;26:371–4.

Konrad H, Karamfi lov T, Wollina U. Intracutaneous botulinum toxin A versusablative therapy of Hailey–Hailey disease—a case report. J Cosmet Laser Ther. 2001;3:181–4.

Kang NG, Yoon TJ, Kim TH. Botulinum toxin type A as an effective adjuvanttherapy for Hailey–Hailey disease. Dermatol Surg. 2002;28:543.

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Published

2016-08-12

Issue

Vol. 2 No. 2 (2016): April-June 2016

Section

Case Reports