Fig 1. Vegetating, eroded, and malodorous friable pla-ques with purulent discharge involving the right cheek.

Fig 2. Suprabasal acantholysis extending focally to thesuperficial epidermis, which is characteristic of HaileyeHailey disease.

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VOLUME 65, NUMBER 1Letters 223

8. Yakubu A, Mabogunje OA. Squamous cell carcinoma of the

skin in Africans. Trop Geogr Med 1995;47:91-3.

9. Singh B, Bhaya M, Shaha A, Har-El G, Lucente FE. Presentation,

course, and outcome of head and neck skin cancer in African

Americans: a case-control study. Laryngoscope 1998;108(8 pt

1):1159-63.

10. McCall CO, Chen SC. Squamous cell carcinoma of the legs in

African Americans. J Am Acad Dermatol 2002;47:524-9.

11. Dieng MT, Diop NN, D�eme A, Sy TN, Niang SO, Ndiaye B.

Squamous cell carcinoma in black patients: 80 cases [in

French]. Ann Dermatol Venereol 2004;131:1055-7.

12. Ochicha O, Edino ST, Mohammed AZ, Umar AB, Atanda AT.

Pathology of peripheral lymph node biopsies in Kane, North-

ern Nigeria. Ann Afr Med 2007;6:104-8.

13. Adigun IA,BuhariMO,AyorindeRO.Malignant skin tumor inblacks:

experience in a teaching hospital. West Afr J Med 2006;25:276-8.

14. Mulwafu WK, Fagan JJ, Jessop S. Squamous cell carcinoma in

black patients with discoid lupus erythematosus. S Afr J Surg

2006;44:144-6.

15. Ribeiro AM, Curado MP, Filho JB. Cutaneous squamous cell

carcinoma of the lower limbs in Goiania, Goi�as. Brazil. Int

J Dermatol 2006;45:1039-42.

16. Adegbidi H, Yedomon H, Atadokpede F, Balley-Pognon MC, do

Ango-Padonou F. Skin cancers at the National University Hospital

ofCotonou from1985 to2004. Int JDermatol 2007;46(suppl):26-9.

17. Byrd-Miles K, Toombs EL, Peck GL. Skin cancer in individuals of

African, Asian, Latin-American, and American-Indian descent:

differences in incidence, clinical presentation, and survival

compared to Caucasians. J Drugs Dermatol 2007;6:10-6.

18. Hu S, Soza-Vento RM, Parker DF, Kirsner RS. Comparison of

stage at diagnosis of melanoma among Hispanic, black, and

white patients in Miami-Dade County, Florida. Arch Dermatol

2006;142:704-8.

19. Gloster HM Jr, Neal K. Skin cancer in skin of color. J Am Acad

Dermatol 2006;55:741-60.

doi:10.1016/j.jaad.2009.11.019

Ectopic facial Hailey-Hailey disease

To the Editor: HaileyeHailey disease, also known asfamilial benign chronic pemphigus, is an autosomaldominant disease that is characterized by vegetatingand malodorous intertriginous plaques with recur-rent blistering and erosions. Mutations in the ATP2C1gene, which encodes aGolgi apparatus Ca21-ATPaseprotein, lead to the inappropriate processing ofdesmosomal proteins. This results in deficient celladhesion with acantholysis occurring in intertrigi-nous sites that are prone to heat and friction.1

Variants of classic HaileyeHailey disease havebeen reported. These include segmental unilateraldisease,2 scalp lesions mimicking seborrheic derma-titis,3 and perianal disease simulating condylomaaccuminata.4 A review of the literature reveals noreports of facial involvement. One report describes apatient with facial lesions and classic HaileyeHaileylesions elsewhere. However, the diagnosis of ‘‘pho-tosensitive HaileyeHailey disease’’ was based onbiopsy specimens taken from the forearm and scro-tum only.5 We report a case of ectopic, biopsy-confirmed facial HaileyeHailey disease.

A 47-year-old African American man presentedwith a 4-year history of painful and malodorouslesions involving the cheeks and axillae. He hadbeen treated for more than 2 years with topicalbenzoyl peroxide preparations for a presumed diag-nosis of acne. Based on recurrent and painful lesionsin both axillae, he hadpreviously undergone bilateralaxillary excision for a presumed diagnosis of hidrad-enitis suppurativa. The patient reported similar inter-triginous lesions in his paternal grandmother and twobrothers. His father was unaffected and informationwas unavailable about the remainder of his family.

The physical examination revealed brown, vege-tating, eroded, and malodorous friable plaques witha purulent discharge involving the cheeks, the largestmeasuring approximately 5 cm (Fig 1), on a back-ground of acne scars and comedones. Both axillaehad similar macerated and vegetating malodorousplaques within and around a surgical scar.A complete skin examination was otherwise unre-markable. Two biopsy specimens taken from thecheeks revealed suprabasal acantholysis extendingfocally to the superficial epidermis, which is charac-teristic of HaileyeHailey disease (Fig 2). Directimmunofluorescence was negative.

Fig 1. A, Reddish papules on the chest before treatment(overall involvement: 15% of the body surface with a20% density). B, Reddish papules on the chest after 14months of treatment with imatinib mesylate (overallinvolvement: 15% of the body surface with a 10%density). The extent of surface involvement was assessedas in burns. The density was calculated in the mostaffected area as percent lesional skin surface to total skinsurface.

Fig 2. Radiographic scan of the left humerus showingnonhomogeneously distributed multiple areas of osteoly-sis emphasized in the proximal and distal third of thehumerus. Arrows show osteolysis.

J AM ACAD DERMATOL

JULY 2011224 Letters

HaileyeHailey disease is classically confined tointertriginous areas. This is attributed to minortrauma, in the form of maceration, heat, and friction,disrupting compromised desmosomal adhesion pro-teins. The development of disseminated and ectopicdisease secondary to a myriad of stressors has beenreported.2-5 In our case, inflammation from coexis-tent acne vulgaris may have been the responsiblestressor. HaileyeHailey disease koebnerizing to skinwith acne vulgaris has not been reported.

Numerous treatment regimens including warmcompresses followed by gentle debridement, hydro-cortisone 1% cream, gentamycin ointment, lidocaine2% gel, and a course of oral dicloxacillin wereminimally effective in controlling our patient’s dis-ease. Treatment with acitretin 25 mg daily met with amoderate, rapid response, perhaps because of im-provement in both the HaileyeHailey disease andthe background acne vulgaris.

Zeina A. W. Dajani, MD, and Diya F. Mutasim, MD

Department of Dermatology, University of Cincin-nati College of Medicine, Cincinnati, Ohio

Funding sources: None.

Conflicts of interest: None declared.

Correspondence to: Diya F. Mutasim, MD, Depart-ment of Dermatology, University of CincinnatiCollege of Medicine, PO Box 670592, Cincin-nati, OH 45267-0592

E-mail: diya.mutasim@uc.edu

REFERENCES

1. Dhitavat J, Fairclough RJ, Hovnanian A, Burge SM. Calcium

pumps and keratinocytes: lessons from Darier’s disease and

Hailey-Hailey disease. Br J Dermatol 2004;150:821-8.

2. Hwang LY, Lee JB, Richard G, Uitto JJ, Hsu S. Type I segmental

manifestation of Hailey-Hailey disease. J Am Acad Dermatol

2003;49:712-4.

3. Marren P, Burge S. Seborrhoeic dermatitis of the scalp—a

manifestation of Hailey-Hailey disease in a predisposed indi-

vidual? Br J Dermatol 1992;126:294-6.

4. Langenberg A, Berger TG, Cardelli M, Rodman OG, Estes S,

Barron DR. Genital benign chronic pemphigus (Hailey-Hailey

disease) presenting as condyloma. J Am Acad Dermatol

1992;26:951-5.

5. Choi DJ, Oh CW, Yoon TJ, Kim TH. Hailey-Hailey disease on sun-

exposed areas. Photodermatol Photoimmunol Photomed

2002;18:214-5.

doi:10.1016/j.jaad.2009.11.023

Imatinib mesylate for aggressive systemicmastocytosis with long bone osteolysis

To the Editor: Imatinib mesylate is a tyrosine kinaseinhibitor that binds the mast cell c-Kit receptor and

reduces symptoms in patients with aggressive sys-temic mastocytosis (SM).1 We present a rare case ofaggressive SMwith longbone involvement. Treatmentwith imatinib mesylate appeared to inhibit furtherprogression of long bone osteolysis in this patient.