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  Table of Contents  
Year : 2011  |  Volume : 2  |  Issue : 1  |  Page : 43-45  

Hailey-Hailey disease: A case treated with topical tacrolimus

1 Department of Dermatology, Guru Gobind Singh Medical College and Hospital, Faridkot, Punjab, India
2 Department of Pathology, Guru Gobind Singh Medical College and Hospital, Faridkot, Punjab, India

Date of Web Publication21-Apr-2011

Correspondence Address:
Tejinder Kaur
Department of Dermatology, House No- 48, Medical College Campus, Faridkot, Punjab
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/2229-5178.79857

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How to cite this article:
Kaur T, Sandhu SK. Hailey-Hailey disease: A case treated with topical tacrolimus. Indian Dermatol Online J 2011;2:43-5

How to cite this URL:
Kaur T, Sandhu SK. Hailey-Hailey disease: A case treated with topical tacrolimus. Indian Dermatol Online J [serial online] 2011 [cited 2022 Jan 25];2:43-5. Available from: https://www.idoj.in/text.asp?2011/2/1/43/79857


A 50-year-old female presented with recurrent, painful, vesicular, erythematous, crusted malodorous lesions in all the intertriginous areas with extensive involvement of the groins since 15 years. The lesions were refractory to all kinds of treatment given in the past. There was no history of similar complaint in any family member. Physical examination showed erythematous, macerated plaques with multiple fissures, peripheral vesicles and crusts in the axillae, submammary and inguinal folds [Figure 1]. Few similar lesions were also present in the folds of the neck and antecubital fossae. All routine investigations were normal except for blood sugar levels, which were raised, and she was started on oral hypoglycemic agents after medical consultation. KOH smear for fungus was negative. Tzanck smear showed few acantholytic cells. Histopathological examination showed intraepidermal clefts of varying sizes with incomplete acantholysis giving dilapidated brick wall appearance [Figure 2]. Direct immunofluorescence was negative. With all these clinico-pathological findings diagnosis of  Hailey-Hailey disease More Details was made and patient was put on combination of topical and systemic antibiotics, antimycotics, and topical corticosteroids which yielded only transient relief. Subsequently, she was put on topical tacrolimus (0.03%) twice a day only to which patient responded well [Figure 3]. The lesions healed completely after about 10 weeks and there has been no relapse even after a follow up of more than a year.
Figure 1: Showing vesicles, fissuring, and erosions in the groins

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Figure 2: Histopathology showing intra-epidermal clefts with irregular acantholysis (H and E, ×40)

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Figure 3: (a,b) Lesions on treatment with topical tacrolimus

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Hailey-Hailey disease is an autosomal dominant, chronic disabling disease with recurrent remissions and relapses. It involves abnormal keratinocyte adhesion in supra basal layers of epidermis due to defect in gene ATP2C1 on chromosome 3q 21-24 which encodes Ca 2+ ATPase. [1] The Ca 2+ homeostasis plays a major role in epidermal cell to cell adhesions.

The treatment of Hailey-Hailey disease is very challenging. Numerous therapies have been tried in the past ranging from topical and systemic antibiotics, antimycotics, methotrexate, corticosteroids, dapsone, thalidomide, etretinate to surgical and CO 2 laser therapies in severe and refractory cases but all these therapies have offered only short-term control of disease. [2] There are very few recent reports of use of topical tacrolimus in Hailey-Hailey disease. The exact mechanism of action of tacrolimus in Hailey-Hailey disease is not clear. It has been suggested that tacrolimus. like cyclosporine. targets a calcium-activated phosphatase called calcineurin, thereby blocking the expression of cytokines, leading to recovery of ATP2C1 expression to levels sufficient to maintain homeostasis in the epidermis. [3] It has been suggested to act through inhibition of mainly proinflammatory cytokine IL6 production. [3] Almost all reports have shown tacrolimus to be effective in Hailey-Hailey disease. [1],[4] There is only single report of local irritation with topical tacrolimus in a patient with Hailey-Hailey disease leading to complete failure of therapy. [5] This side effect can be easily overcome by decreasing the concentration of the drug. No such side effect was noticed in our case.

To summarize, topical tacrolimus can be an effective alternative treatment for Hailey-Hailey disease for long-term control of disease.

   References Top

1.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.   Back to cited text no. 1
2.Freiman A, Hakim MD, Billick RC. Hailey-Hailey Disease (Benign Familial Pemphigus): Carbon Dioxide Laser Therapy. Mcgill J Med 2002;6:100-3.  Back to cited text no. 2
3.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.  Back to cited text no. 3
4.Sand C, Thomsen HK. Topical Tacrolimus Ointment Is an Effective Therapy for Hailey-Hailey Disease. Arch Dermatol 2003;139:1401-2.   Back to cited text no. 4
5.Laffitte E, Panizzon RG. Is Topical Tacrolimus Really an Effective Therapy for Hailey-Hailey Disease? Arch Dermatol 2004;140:1282.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]

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