Generic selectors
Exact matches only
Search in title
Search in content
Post Type Selectors
Filter by Categories
15th National Conference of the IAOMFP, Chennai, 2006
Abstracts from current literature
Acne in India: Guidelines for management - IAA Consensus Document
Art & Psychiatry
Association Activities
Association Notes
Award Article
Book Review
Brief Report
Case Analysis
Case Letter
Case Letters
Case Notes
Case Report
Case Reports
Clinical and Laboratory Investigations
Clinical Article
Clinical Studies
Clinical Study
Conference Oration
Conference Summary
Continuing Medical Education
Cosmetic Dermatology
Current Best Evidence
Current Issue
Current View
Derma Quest
Dermato Surgery
Dermatosurgery Specials
Dispensing Pearl
Do you know?
Drug Dialogues
Editor Speaks
Editorial Remarks
Editorial Report
Editorial Report - 2007
Editorial report for 2004-2005
Fourth All India Conference Programme
From Our Book Shelf
From the Desk of Chief Editor
Get Set for Net
Get set for the net
Guest Article
Guest Editorial
How I Manage?
IADVL Announcement
IADVL Announcements
IJDVL Awards
IJDVL Awards 2018
IJDVL Awards 2019
IJDVL Awards 2020
IJDVL International Awards 2018
Images in Clinical Practice
In Memorium
Inaugural Address
Knowledge From World Contemporaries
Leprosy Section
Letter in Response to Previous Publication
Letter to Editor
Letter to the Editor
Letter to the Editor - Case Letter
Letter to the Editor - Letter in Response to Published Article
Letter to the Editor - Observation Letter
Letter to the Editor - Study Letter
Letter to the Editor - Therapy Letter
Letter to the Editor: Articles in Response to Previously Published Articles
Letters in Response to Previous Publication
Letters to the Editor
Letters to the Editor - Letter in Response to Previously Published Articles
Letters to the Editor: Case Letters
Letters to the Editor: Letters in Response to Previously Published Articles
Medicolegal Window
Miscellaneous Letter
Net Case
Net case report
Net Image
Net Letter
Net Quiz
Net Study
New Preparations
News & Views
Observation Letter
Observation Letters
Original Article
Original Contributions
Pattern of Skin Diseases
Pediatric Dermatology
Pediatric Rounds
Presedential Address
Presidential Address
Presidents Remarks
Report of chief editor
Report of Hon : Treasurer IADVL
Report of Hon. General Secretary IADVL
Research Methdology
Research Methodology
Resident page
Resident's Page
Resident’s Page
Residents' Corner
Residents' Corner
Residents' Page
Review Article
Review Articles
Revision Corner
Self Assessment Programme
Seminar: Chronic Arsenicosis in India
Seminar: HIV Infection
Short Communication
Short Communications
Short Report
Special Article
Specialty Interface
Study Letter
Study Letters
Symposium - Contact Dermatitis
Symposium - Lasers
Symposium - Pediatric Dermatoses
Symposium - Psoriasis
Symposium - Vesicobullous Disorders
Symposium Aesthetic Surgery
Symposium Dermatopathology
Symposium-Hair Disorders
Symposium-Nails Part I
Symposium-Nails-Part II
Systematic Review and Meta-Analysis
Systematic Reviews and Meta-analyses
Systematic Reviews and Meta-analysis
Therapeutic Guideline-IADVL
Therapeutic Guidelines
Therapeutic Guidelines - IADVL
Therapy Letter
Therapy Letters
View Point
What’s new in Dermatology
View/Download PDF

Translate this page into:

Letter to the Editor
doi: 10.4103/0378-6323.66597
PMID: 20657133

Benign familial pemphigus (Hailey-Hailey disease) responsive to low dose cyclosporine

Arti Nanda, Fatima Khawaja, Rafat Harbi, Mousmee Nanda, Richard Dvorak, Qasem A Alsaleh
 As'ad Al-Hamad Dermatology Center, Al-Sabah Hospital, Kuwait

Correspondence Address:
Arti Nanda
P.O. Box: 6759, Salmiya, 22078, Salmiya
How to cite this article:
Nanda A, Khawaja F, Harbi R, Nanda M, Dvorak R, Alsaleh QA. Benign familial pemphigus (Hailey-Hailey disease) responsive to low dose cyclosporine. Indian J Dermatol Venereol Leprol 2010;76:422-424
Copyright: (C)2010 Indian Journal of Dermatology, Venereology, and Leprology


A 43-year-old Egyptian male was referred to us with eight year history of recurrent painful vesico-pustular eruption followed by painful erosions affecting the groins, axillae and neck. He gave family history of similar disease in six other family members including his younger brother, father, paternal grandfather, paternal uncle, and one paternal cousin. Before coming to us, he had been treated with several treatment modalities including oral and topical antibiotics, topical antifungals, corticosteroids, and dapsone with a partial or temporary improvement. His condition had been steadily deteriorating. On examination, he was observed to have large denuded plaques studded with pus on the erythematous base along with erosions and crustations present on the neck, axillae [Figure - 1]a and b and groins. A skin biopsy from the affected skin revealed suprabasal cleft, acantholysis with dilapidated brick wall appearance, and presence of dyskeratotic cells [Figure - 2]a and b. In the upper dermis, there was mixed inflammatory cell infiltrate comprising lymphocytes, polymorphs and few eosinophils. A diagnosis of benign familial pemphigus (Hailey-Hailey disease) was done. He was started on acetretin 0.75 mg/kg per day in combination with topical pimecrolimus and topical antibiotics. His situation continued to worsen and a month later he was shifted to cyclosporine 2.5 mg/kg per day. Within a week of cyclosporine treatment, he showed 80% clearance of skin lesions with a complete clearance in three weeks time [Figure - 3]a and b. A week after complete clearance, the dose of cyclosporine was slowly tapered off by 0.5 mg/kg every month and was stopped over six months. During cyclosporine treatment, he was observed to have mild hypertension that was effectively controlled with calcium channel blockers. After discontinuing this treatment, his blood pressure reverted back to normal limits. During a follow-up two years later, he continues to be in comfortable condition with only minor recurrences that could be controlled with topical tacrolimus (0.1%), and topical and/or systemic antibiotics.

Benign familial pemphigus (Hailey-Hailey disease) is a rare autosomal dominant disorder characterized by development of recurrent blisters, erosions, and crustations in the intertrigenous areas. Mutations in ATP2C1 gene, which encodes the human secretary pathway Ca 2+ /Mn 2+ ATPase protein 1 (HSPCA) have been observed to be responsible for Hailey-Hailey disease. [1] Various treatment options include topical or systemic antibiotics, antifungals, corticosteroids in early stages, and dapsone, PUVA, systemic retinoids, cyclosporine, methotrexate, and photodynamic therapy for the recalcitrant cases. [2] Our patient had used several treatment modalities including antibiotics, antifungals, corticosteroids and dapsone earlier and when presented to us had a severe recalcitrant disease. He failed to show an improvement to acetretin tried for four weeks, Infact while on acetretin, his condition continued to deteriorate. We observed a favorable response to low dose of cyclosporine (2.5 mg/kg/day) with complete clearance in three weeks and a week after the clearance, the dose of cyclosporine could be further reduced and was stopped after six months. Cyclosporine in a dose of 2.8-5 mg/kg per day has been reported to be effective earlier in three patients of Hailey-Hailey disease. [3],[4] All patients achieved remissions but a rebound was observed soon after stopping cyclosporine. Adverse effects related to long term use of cyclosporine were described to restrict its use on long term basis. Tacrolimus has been recently used on the same basis to control some cases of Hailey-Hailey disease. [5],[6],[7] Due to a severe, progressive, and recalcitrant disease, a systemic therapy was indicated in our patient. Thus, we used cyclosporine A to control the acute activity followed by antibiotics and topical tacrolimus ointment to treat the minor recurrences. He showed improvement with a low dose of cyclosporine and continued to have sustained remission with only minor recurrences for two years even after stopping the cyclosporine. We feel, at least some patients with severe and recalcitrant disease can be controlled with a low dose cyclosporine and can be maintained in prolonged remission by slow tapering and thus avoiding the toxicity related to high dose or long term use of this drug. The exact mechanism of action, how cyclosporine and tacrolimus work in Hailey-Hailey disease, is not clear. The proposed mechanism of action is suppression of inflammation by their immunomodulatory action. [3],[6] Presumably various stimuli including heat, friction, UV light, etc are known to precipitate or aggravate the disease in these patients by inducing inflammation.

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.
[Google Scholar]
Ruiz-Rodriguez R, Alvarez JG, Jaιn P, Acevedo A, Cordoba S. Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign pemphigus (Hailey-Hailey disease). J Am Acad Dermatol 2002;47:740-2.
[Google Scholar]
Ormerod AD, Duncan J, Stankler L. Benign familial pemphigus responsive to cyclospoprin, a possible role for cellular immunity in pathogenesis. Br J Dermatol 1991;124:299-305.
[Google Scholar]
Berth-Jones J, Smith SG, Graham-Brown RA. Benign familial chronic pemphigus (Hailey-Hailey disease) responds to cyclosporine. Clin Exp Dermatol 1995;20:70-2.
[Google Scholar]
Rabeni EJ, Cunningham NM. Effective treatment of Hailey-Hailey disease with topical tacrolimus. J Am Acad Dermatol 2002;47:797-8.
[Google Scholar]
Sand C, Thomsen HK. Topical tacrolimus ointment is effective therapy for Hailey-Hailey disease. Arch Dermatol 2003;139:1401-2.
[Google Scholar]
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.
[Google Scholar]

Fulltext Views

PDF downloads
Show Sections