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 Table of Contents  
CASE REPORT
Year : 2016  |  Volume : 2  |  Issue : 2  |  Page : 96-98

Hailey-Hailey disease: Botulinum toxin treatment


1 Vida and Sade Hospital Association, Santa Rosa, RS, Brazil
2 Faculty of Medicine of the Federal University of Santa Maria, Santa Maria, RS, Brazil
3 Faculty of Medicine of the Metropolitan University of Santos, SP, Brazil

Date of Web Publication20-Dec-2016

Correspondence Address:
Rafael Werlang Donadel
Rua Dom Joo VI, 213, Centro, Santa Rosa, RS
Brazil
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2455-3972.196170

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  Abstract 

Hailey-Hailey disease, also known as familial chronic benign pemphigus, is characterized by flaccid blisters and erosions, especially in the axillary and inguinal areas. Friction associated with local factors such as heat, moisture, microbial colonization, and secondary infections induce the typical appearance of the lesions, especially in intertriginous areas. Traditionally, the treatment is done with corticosteroids associated with topical or systemic antibiotics. We report the case of a patient with the disease presenting in the inguinal region, which has made use of different conventional drug treatments for 20 years without results. Treatment with application of botulinum toxin type A showed a significant improvement after about 20 days. The satisfactory response has been linked to decreased local sweating caused by botulinum toxin. which would lead to a less local irritation factors and lower colonization of microrganisms.

Keywords: Botulinum toxin, familial benign pemphigus, Hailey-Hailey disease


How to cite this article:
Donadel KW, Donadel MW, Donadel RW, dos Santos BO. Hailey-Hailey disease: Botulinum toxin treatment. Indian J Drugs Dermatol 2016;2:96-8

How to cite this URL:
Donadel KW, Donadel MW, Donadel RW, dos Santos BO. Hailey-Hailey disease: Botulinum toxin treatment. Indian J Drugs Dermatol [serial online] 2016 [cited 2021 Apr 14];2:96-8. Available from: https://www.ijdd.in/text.asp?2016/2/2/96/196170


  Introduction Top


Familial chronic benign pemphigus is also known as Hailey-Hailey disease. This rare chronic genetic skin disease leads to the formation of vesicles and erythematous plaques with overlying crusts in the axillary and inguinal regions. [1] Treatment of the disease is usually done with antibiotics and topical or systemic corticosteroids although both have limited results, it is highly unusual for cases to present so much resistance to the standard treatment, With the patient taking treatment over 20 years in the past without success. Treatment with botulinum toxin type A is proposed due to its action by reducing sweat production. [2],[3],[4]


  Case report Top


A 60-year-old Caucasian woman with a suspected diagnosis of Hailey-Hailey disease since 20 years, presented with recurrent lesions in the inguinal region characterized by erythematous plaques with flaccid bullae and erosion, maceration, and intense fetid odor [Figure 1]. Patient has had limited response to a large number of classic treatments already administered including different associations of both corticosteroids and topical and systemic antibiotics such as prednisone, erythromycin, clindamycin, sulfonamide, tetracycline, imidazole, and lymecycline. The differential diagnosis kept were pemphigus vulgaris, Darier's disease, simple intertrigo, and dermatophytosis, and the diagnosis of Hailey-Hailey's disease was established by clinical examination and histopathological findings. Given the years of treatment without satisfactory response, many times associated with intolerance to conventional treatment regimens, the patient was submitted to applications of 50 units (U) of botulinum toxin type A, on points with a distance of about one centimeter between one another, 2U being injected at each point. All other treatments were suspended following the application, and after 20 days, the patient returned with a significant improvement and has not shown recurrence after 60 days of application [Figure 2].
Figure 1: Lesions before application of botulinum toxin type A

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Figure 2: Lesions after 20 days of application of botulinum toxin type A

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  Discussion Top


Hailey-Hailey disease is benign, but often undermines the physical and emotional well-being of patients. Pain, itching, unpleasant odor, and even the appearance of the lesions compromise the quality of life. For this reason, treatment even for cases with few lesions is recommended. [2] The disease usually starts between the second and third decades of life, evolves in spurts, has variable manifestations and the lesions heal without the formation of scar tissue. [5] There is a mutation in the gene located on chromosome ATP2C1 3q21q24, leading to loss of cell adhesion in epidermis. [6] Epidermal defect leads to acantholysis, either spontaneously or as a result of friction, heat, sweat, or infections. It manifests itself with papules, vesicles, and bullae that break down quickly, leaving erosions and crusts with or without itching or burning, often the lesions present are infected by bacteria, yeasts, and viruses, manifesting usually by fetid odor, pain, and Oozing. The infected tissue plays an important role in the exacerbation and persistence of the disease. The most affected regions by these lesions are the intertriginous areas such as the axillary folds, the inguinal and perineal region, and the sides of the Neck. Mucosal involvement is rare. [7] Histologically, the epidermis shows intraepidermal suprabasal acantholysis and pronounced dyskeratosis of keratinocytes with grains and round bodies. In more chronic lesions, epidermal hyperplasia, parakeratosis, and focal crusts can be observed. Direct immunofluorescence of the lesions is negative. Treatment is symptomatic with general measures to prevent friction and Sweat. For the treatment of secondary bacterial colonization use of the following drugs has been suggested: or al tetracycline, imidazole, sulfone, thalidomide, prednisone, methotrexate and topically cyclosporine, tacrolimus and topical corticosteroids are the most used. [8] Surgical treatment with wide excision, dermabrasion, erbium (yttrium aluminum garnet) or CO 2 lasers, and photodynamic therapy has also been suggested as possible treatments with varying degrees of success. [1] Botulinum toxin type A is a protein that produces chemical denervation, by blocking the release of acetylcholine from nerve endings. The proposed use of botulinum toxin is due to its action blocking cholinergic stimulation by the postganglionic sympathetic fibers, decreasing sweat production by the sweat glands. [3],[9],[10] Heat and sweat are exacerbating factors in patients with Hailey-Hailey Disease. Less sweating, with a reduction of local humidity, works to reduce local irritation factors important in the formation of the lesions and reduces colonization by microorganisms involved in exacerbations. The use of botulinum toxin type A can be considered a safe therapeutic option with simple application in patients with limited response or intolerant to conventional treatments.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
  References Top

1.
Hohl D, Mauro T. Darier disease and Hailey-Hailey disease. In: Bolognia JL, Jorizzo JL, Rapini RP, editors. Dermatology. 2 nd ed. New Haven: Mosby; 2008. p. 791-800.  Back to cited text no. 1
    
2.
Gisondi P, Sampogna F, Annessi G, Girolomoni G, Abeni D. Severe impairment of quality of life in Hailey-Hailey disease. Acta Derm Venereol 2005;85:132-5.  Back to cited text no. 2
    
3.
Bessa GR, Grazziotin TC, Manzoni AP, Weber MB, Bonamigo RR. Hailey-Hailey disease treatment with botulinum toxin type A. An Bras Dermatol 2010;85:717-22.  Back to cited text no. 3
    
4.
Kang NG, Yoon TJ, Kim TH. Botulinum toxin type A as an effective adjuvant therapy for Hailey-Hailey disease. Dermatol Surg 2002;28:543.  Back to cited text no. 4
    
5.
Azulay RD, Azulay DR. Genodermatosis and cutaneous disembriopathologies. In: Azulay RD, Azulay-Abulafia L, editors. Dermatologia. 5 th ed. Rio de Janeiro: Guanabara Koogan; 2008. p. 621-55.  Back to cited text no. 5
    
6.
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.  Back to cited text no. 6
    
7.
Sampaio SA, Rivitti EA. Bullous dermatoses and cutaneous disembriopathologies. In: Azulay RD, Azulay-Abulafia L, editors. Dermatologia. 5 th ed. Rio de Janeiro: Guanabara Koogan; 2008. p. 1063-74.  Back to cited text no. 7
    
8.
Burge SM. Hailey-Hailey disease: The clinical features, response to treatment and prognosis. Br J Dermatol 1992;126:275-82.  Back to cited text no. 8
    
9.
Lowe NJ, Yamauchi PS, Lask GP, Patnaik R, Iyer S. Efficacy and safety of botulinum toxin type a in the treatment of palmar hyperhidrosis: A double-blind, randomized, placebo-controlled study. Dermatol Surg 2002;28:822-7.  Back to cited text no. 9
    
10.
Lowe PL, Cerdan-Sanz S, Lowe NJ. Botulinum toxin type A in the treatment of bilateral primary axillary hyperhidrosis: Efficacy and duration with repeated treatments. Dermatol Surg 2003;29:545-8.  Back to cited text no. 10
    


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