Letters to the Editor patient‑‑response to oral retinoid treatment. Clin Exp Dermatol 1994;19:67‑9. 12. Kronthal HL, Pomeranz JR, Sitomer G. Fox‑Fordyce disease: Treatment with an oral contraceptive. Arch Dermatol 1965;91:243‑5. 13. Ahmed Al‑Qarqaz F, Al‑Shannag R. Fox‑Fordyce disease treatment with fractional CO2 laser. Int J Dermatol 2013;52:1571‑2. 14. Tetzlaff MT, Evans K, DeHoratius DM, Weiss R, Cotsarelis G, Elenitsas R. Fox‑Fordyce disease following axillary laser hair removal. Arch Dermatol 2011;147:573‑6. 15. Chavoin JP, Charasson T, Bernard JD. Surgical treatment of hidradenitis and Fox‑Fordyce disease of the nipples. Ann Chir Plast Esthet 1994;39:233‑8. Access this article online Quick Response Code:

Figure 2: Monomorphic papules over the right axilla with no terminal hair growth

hair removal have been shown to induce Fox‑Fordyce disease.[14] Surgical intervention is a definitive treatment and includes areolar dermal detachment safeguarding the nipple, apocrine sweat gland excision and fixation of the areola like a flap.[15]

Anisha George, Anuradha Bhatia, Emy Thomas Department of Dermatology, Christian Medical College, Ludhiana, Punjab, India Address for correspondence: Dr. Anisha George, Department of Dermatology, Christian Medical College, Ludhiana ‑ 141 008, Punjab, India. E‑mail: [email protected]

REFERENCES 1. Kamada A, Saga K, Jimbow K. Apoeccrine sweat duct obstruction as a cause for Fox‑Fordyce disease. J Am Acad Dermatol 2003;48:453‑5. 2. Böer A. Patterns histopathologic of Fox‑Fordyce disease. Am J Dermatopathol 2004;26:482‑92. 3. Ozcan A, Senol M, Aydin NE, Karaca S, Sener S. Fox‑Fordyce disease. J Eur Acad Dermatol Venereol 2003;17:244‑5. 4. Kassuga LE, Medrado MM, Chevrand NS, Salles Sde A, Vilar EG. Fox‑Fordyce disease: Response to adapalene 0.1%. An Bras Dermatol 2012;87:329‑31. 5. Sandhu K, Gupta S, Kanwar AJ. Fox fordyce disease in a prepubertal girl. Pediatr Dermatol 2005;22:89‑90. 6. Patrizi A, Orlandi C, Neri I, Fanti PA, Mazzanti L. Fox‑Fordyce disease: Two cases in patients with Turner syndrome. Acta Derm Venereol 1999;79:83‑4. 7. Pock L, Švrčková M, Macháčková R, Hercogová J. Pimecrolimus is effective in Fox–Fordyce disease. Int J Dermatol 2006;45:1134‑5. 8. Milcic D, Nikolic M. Clinical effects of topical pimecrolimus in a patient with Fox‑Fordyce disease. Australas J Dermatol 2012;53:e34‑5. 9. Feldmann R, Masouyé I, Chavaz P, Saurat JH. Fox‑Fordyce disease: Successful treatment with topical clindamycin in alcoholic propylene glycol solution. Dermatology 1992;184:310‑3. 10. Miller ML, Harford RR, Yeager JK. Fox‑Fordyce disease treated with topical clindamycin solution. Arch Dermatol 1995;131:1112‑3. 11. Effendy I, Ossowski B, Happle R. Fox‑Fordyce disease in a male 88

Website: www.ijdvl.com DOI: 10.4103/0378-6323.148597 PMID: *****

Hailey–Hailey disease with skin lesions at unusual sites and a good response to acitretin Sir, Hailey–Hailey disease is a bullous disorder characterized by the development of flexural erosions, blisters and warty papules. It has a relapsing and remitting course. Skin lesions can be induced or exacerbated by friction, sweating, burns, contact dermatitis and local infections. A 45‑year‑old woman from Himachal Pradesh presented with itchy lesions below the left breast for two weeks and a solitary fluid‑filled lesion on the left forearm for three days. She developed intense itching below her left breast following which she scratched the site and developed erosions. The solitary bulla on the left forearm had spontaneously ruptured to form an erosion. She used to get similar lesions below both breasts and in the groin for the past 10 years. She consulted a physician and was treated with multiple courses of antifungals, antibiotics, corticosteroids and dapsone, with only partial relief. These lesions used to increase every summer and get aggravated on sweating. The associated itching was severe enough to disturb sleep. There was no family history suggestive of any bullous disorder.

Indian Journal of Dermatology, Venereology, and Leprology | January-February 2015 | Vol 81 | Issue 1

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Dermatological examination showed crusted erosions with excoriations below the left breast. Post inflammatory hyperpigmentation was present below the right breast symmetrical with the active lesion on the left side. A solitary erosion was present on the left forearm with surrounding erythema. Further cutaneous examination revealed a few papules and crusted lesions on the sides of her neck, axillae, and bilateral inguinal regions. There was no nail, mucosal or scalp involvement. With a clinical suspicion of Hailey–Hailey disease, a biopsy was done from the inframammary lesion on the left side and erosion on the forearm. She was given oral amoxicillin and clavulanate potassium combination 1 gm twice daily and was asked to report back after a week along with the biopsy report. By the next visit, her skin lesions had worsened. Dermatological examination revealed extensive crusted lesions below the breast, in the axillae, and in the inguinal regions bilaterally [Figure 1]. She also had a crusted plaque with erythematous margins on the left thigh. Investigations revealed normal hemogram, liver, renal functions, and serum lipid profile. Skin biopsy from the inframammary region and the left forearm showed similar features: there was loss of epidermis in a few areas, suprabasal clefting with acantholytic cells, chronic inflammatory cells, and serous fluid and a dilapidated brick wall appearance was evident [Figure 2]. Dermal edema was present, along with perivascular mixed inflammatory cells and extravasated red blood cells. Direct immunofluorescence examination was negative for immunoreactants. A diagnosis of Hailey–Hailey disease was confirmed on the basis of histopathology and clinical examination and the

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patient was started on oral acitretin, 25 mg once daily. The lesions started responding in 4 weeks and there was complete regression after 3 months [Figure 3]. Acitretin was continued uninterrupted for a total of 5 months. She was monitored once monthly with clinical examination and lab investigations including complete hemogram, liver and renal function tests, and serum lipid profile. She had attained menopause and had undergone  tubectomy, so pregnancy tests were not required. She tolerated the medication well without any side effects and has been followed up for 2 years with no relapse. Hailey–Hailey disease is an autosomal dominant disorder with a prevalence of 1:50,000. It usually begins in the third or fourth decade of life. The disease is caused by mutation in the calcium‑dependent adenosine triphosphatase ATP2C1 gene which encodes an adenosine triphosphate‑powered Ca2+ pump in the Golgi apparatus.[1,2] The primary skin lesions in Hailey–Hailey disease are flaccid vesicles or bullae on otherwise normal‑appearing skin that rupture easily leaving macerated or crusted erosions. The lesions may progress to become thickly crusted and may be misdiagnosed as impetigo or eczema. Sometimes, the erosions spread peripherally with an active inflammatory border producing circinate or configurate patterns, as in our case.[3] The lesions appear in crops and usually regress in a few weeks, although some lesions follow a chronic course. Moist, malodorous lesions with painful fissures are also common. The lesions remain localized to the neck, axillae, and groins in most patients. Other sites which are less commonly involved are the scalp, antecubital or popliteal fossa, and trunk. The lesions tend to recur at original sites. Rare presentations include segmental, extensive,

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Figure 1: Skin lesions at the time of exacerbation: Vesicles, pustules, and crusted plaques on (a) left axilla, (b) right axilla, (c) groin, (d) inframammary region, and (e) left upper thigh

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Figure 2: Regression of skin lesions post treatment with acitretin at (a) inframammary region, (b) right axilla, (c) left axilla, and (d) groin

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Letters to the Editor Table 1: Treatment options in Hailey-Hailey disease

a

Type

Treatment options

Topical

Corticosteroids, antibiotics (clindamycin, erythromycin, soframycin), antifungals, retinoids, calcipotriol, tacrolimus, 5‑fluorouracil, topical cadexomer iodine, zinc oxide

Systemic

Corticosteroids, antibiotics, antifungals, retinoids (etretinate, acitretin, alitretinoin), dapsone, cyclosporine, methotrexate, biologicals (alefacept), narrow band ultraviolet B therapy, photodynamic therapy, thalidomide

Surgical/cosmetic

Botulinum toxin type A, dermabrasion, CO2 laser, Erbium: YAG laser

Local measures

Wearing loose clothing, staying in cool environments, local soothing compresses

YAG: Yttrium Aluminum Garnet laser

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Figure 3: Histopathology showing (a) dilapidated brick wall appearance with suprabasal clefts and acantholytic cells (H and E, 10×); (b and c) high-power view of the same (H and E, 40×)

mucosal, perianal, perineal, and photoexposed area involvement.[4‑6] An unusual presentation with lesions over the upper chest and upper arms with no intertriginous involvement has also been reported.[7] Our patient had a solitary bulla forming an erosion over the the forearm and a crusted plaque on the upper thigh, unusual sites of involvement. The clinical differential diagnosis of Hailey–Hailey disease includes intertrigo, erythrasma, pemphigus vulgaris, pemphigus foliaceus, dermatophytosis, and Darier’s disease. Histopathology is characterized by suprabasal acantholysis leading to bulla formation. Dyskeratosis may be present. Widespread partial loss of the intercellular bridges between keratinocytes gives a dilapidated brick wall appearance to the epidermis. Bullous Darier’s disease can mimic Hailey–Hailey disease very closely, both clinically and histopathologically. The later onset of lesions and rapid appearance and disappearance of lesions with recurrence differentiates Hailey–Hailey disease from bullous Darier’s disease clinically. Histologically, acantholysis is more incomplete and there are fewer dyskeratotic cells in Hailey–Hailey disease as compared to bullous Darier’s disease. Management of this condition is difficult and existing treatments do not provide a long lasting positive therapeutic benefit. Superinfection with bacteria, fungi, and viruses plays an important role in 90

exacerbations and persistence of lesions. The various therapeutic options are given in Table 1. Etretinate has been reported to be beneficial in some cases[8] and oral alitretinoin is a newer retinoid that has been used for treatment.[9] We were able to find two previous reports of acitretin for the treatment of Hailey–Hailey disease in the literature.[10,11] Our case indicates that some patients may develop a long‑term remission following acitretin therapy for Hailey–Hailey disease.

Biju Vasudevan, Rajesh Verma1, Sonia Badwal , Shekar Neema1, Debdeep Mitra1, T. Sethumadhavan2 2

1

Department of Dermatology, INHS Asvini, Department of Dermatology, Command Hospital, Pune, 2 Department of Pathology AFMC, Pune, India

Address for correspondence: Dr. Biju Vasudevan, Department of Dermatology, INHS Asvini, Near RC Church, Colaba, Mumbai - 400 005, Maharashtra, India. E-mail: [email protected]

REFERENCES 1. 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. 2. Sudbrak R, Brown J, Dobson‑Stone C, Carter S, Ramser J, White J, Healy E, et al. Hailey‑Hailey disease is caused by mutations in ATP2C1 encoding a novel Ca2+pump. Hum Mol Genet 2000;9:1131‑40. 3. Odom RB, James WD, Berger TG. Andrew’s Diseases of the Skin: Clinical Dermatology. Philadelphia, PA: WB Saunders Co; 2000. p. 27. 4. Ewald K, Gross G. Perianal Hailey‑Hailey disease: An unusual differential diagnosis of condylomata acuminata. Int J STD AIDS 2008;19:791‑2. 5. Burge SM. Hailey–Hailey disease: The clinical features, response to treatment and prognosis. Br J Dermatol 1992;126:275‑82. 6. Rao AG. Hailey‑Hailey disease on sun‑exposed areas. Indian J Dermatol 2013;58:412. 7. Hunt R, O’Reilly K, Ralston J, Kamino H, Shupack JL. Familial benign chronic pemphigus (Hailey‑Hailey disease). Dermatol Online J 2010;16:14.

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Letters to the Editor 8. Hunt MJ, Salisbury EL, Painter DM, Lee S. Vesiculobullous Hailey‑Hailey disease: Successful treatment with oral retinoids. Australas J Dermatol 1996;37:196‑8. 9. Sárdy M, Ruzicka  T. Successful therapy of refractory Hailey‑Hailey disease with oral alitretinoin. Br J Dermatol 2014;170:209‑11. 10. Berger EM, Galadari HI, Gottlieb AB. Successful treatment of Hailey‑Hailey disease with acitretin. J Drugs Dermatol 2007;6:734‑6. 11. Moreno‑Suárez F, Bastante  ES, Burgos  FJ, Martínez PB. Response to acitretin in a patient with verrucoid forms of Hailey–Hailey disease and presence of human papillomavirus type 6 DNA. J Am Acad Dermatol 2014;70:AB87.

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Website: www.ijdvl.com DOI: 10.4103/0378-6323.148600 PMID: *****

Lack of effectiveness of keratin dressings in epidermolysis bullosa Sir, Keratin is the fundamental protein that creates cell structure in many tissues, particularly in the skin, hair, and nails.[1‑4] In recent years, emphasis has been placed on the role of keratin in healing wounds. The effects of keratin on cell multiplication and replacement, epithelialization and wound closure have been observed in many cases. Molecular analysis shows that keratin proteins have a positive effect on wound healing via increasing or accelerating epithelialization. This is caused by keratinocyte activation and migration, which is, in turn, caused by increasing keratin gene expression. The synthesis of fundamental proteins such as collagen 4, collagen 7, and collagen 17 increases in active and stimulated keratinocytes.[1] Keratin 17 production increases when the skin is damaged. It has been observed that wound healing does not occur

properly in patients with low amounts of keratin 17. As a result of this keratin, wound healing is hastened, and the frequency of blistering is reduced.[2] Taking the aforementioned into consideration, the effects of applying keratin to the skin as an exogen during wound healing have been studied, and keratin‑based care products have been developed in which the activity of keratin protein has been retained. Bandages with keratin content help provide keratins to wounds. They are well tolerated by patients’ tissues during rehabilitation due to their non‑immunogenic characteristics.[3] A gel containing keratin obtained from sheep’s wool, has been used in the treatment of EB patients.[4] A total of five epidermolysis bullosa (EB) patients, including three dystrophic EB (DEB) and two EB simplex (EBS) patients (case 1 = 9‑year‑old male with DEB; case 2 = 17‑year‑old female with DEB; case 3 = 3‑month‑old female with DEB; case 4 = 6‑year‑old male with EBS; case 5 = 2‑month‑old female with EBS), were regularly subjected to application of a thick hydrogel rich in keratin (Keragel T™, Keraplast Technologies, Lincoln, New Zealand). The gel was applied every other day. The bodies of the patients were entirely washed with lukewarm sterile water, and the gel was rubbed on after the skin dried. The skin was then wrapped with a non‑stick bandage (paraffin gauze bandage, Smith Medical Limited, Hull, England) after waiting for 15 minutes. The treatment was abandoned in case 3 at the fourth month of the treatment upon the request of the family because the number of blisters had increased significantly. Clinical photos of the fourth and fifth patients were taken before and after the treatment [Figures  1a and b, 2a and b] A significant decrease in the number of blisters was observed in all of our patients within the first two

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Figure 1: (a) Pre-treatment appearance: Eroded areas over left knee, mid-lateral tibial and ankles (Patient 2). (b) Post-treatment appearance: Worsening of erosions after three months of treatment

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Figure 2: (a) Pre-treatment appearance; There were eroded area on the back, right thigh extensor (Patient 1). (b) Post-treatment appearance; Worsening of erosions after three months of treatment

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Hailey-Hailey disease with skin lesions at unusual sites and a good response to acitretin.

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