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A 58-Year-Old Man With a Macerated Rash of the Neck and Axilla

Educational Objective
Based on this clinical scenario and the accompanying image, understand how to arrive at a correct diagnosis.
1 Credit CME

A 58-year-old overweight African American man presented with a 5-year history of a slightly pruritic and painful rash of the right lateral area of the neck and right axilla. It was worse during the summers, and blisters sometimes developed. Emollients were unhelpful. The patient was otherwise healthy. His sister had diabetes mellitus and a similar rash in her axillae. The patient performed indoor manual labor as a maintenance engineer and had no significant sun exposure. Physical examination revealed irregularly thickened skin of the right axilla (Figure 1, left) and right lateral area of the neck (Figure 1, right) containing hyperpigmentation, hypopigmentation, multiple acrochordons (skin tags), malodorous crust, and areas of maceration and erosion. Further examination of the skin revealed pseudofolliculitis barbae in the submental and mandibular regions bilaterally. The nails, oral cavity, and mucosal membranes appeared normal. A biopsy of right axillary skin was performed (Figure 2).

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Hailey-Hailey disease (benign familial pemphigus)

C. Prescribe a topical corticosteroid and a topical antimicrobial

The key to the correct diagnosis is the presence of an intertriginous rash with report of blistering, summertime exacerbation, and family history of a similar rash. First-line treatment for Hailey-Hailey disease is topical and should include a topical corticosteroid with or without a topical antimicrobial.1,2 Hailey-Hailey disease can have an appearance similar to that of acanthosis nigricans in dark-skinned patients, but acanthosis nigricans is typically asymptomatic and unlikely to vary with season. A crusted rash with seasonal variation could suggest seborrheic dermatitis or candidal intertrigo, which can be treated with ketoconazole. Seborrheic dermatitis, however, usually involves the face or scalp and worsens during winter. Lack of satellite lesions makes candidal intertrigo unlikely.

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Article Information

Corresponding Author: Christopher Haley, MD, Center for Clinical Studies, 451 N Texas Ave, Webster, TX 77598 (chaley1029@gmail.com).

Conflict of Interest Disclosures: All authors have completed and submitted the ICMJE Form for Disclosure of Potential Conflicts of Interest and none were reported.

Additional Contributions: We thank the patient for providing permission to share his information.

References
1.
Burge  SM.  Hailey-Hailey disease: the clinical features, response to treatment and prognosis.  Br J Dermatol. 1992;126(3):275-282.PubMedGoogle ScholarCrossref
2.
Arora  H, Bray  FN, Cervantes  J, Falto Aizpurua  LA.  Management of familial benign chronic pemphigus.  Clin Cosmet Investig Dermatol. 2016;9:281-290.PubMedGoogle ScholarCrossref
3.
Engin  B, Kutlubay  Z, Çelik  U, Serdaroğlu  S, Tüzün  Y.  Hailey-Hailey disease: a fold (intertriginous) dermatosis.  Clin Dermatol. 2015;33(4):452-455.PubMedGoogle ScholarCrossref
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Chiaravalloti  A, Payette  M.  Hailey-Hailey disease and review of management.  J Drugs Dermatol. 2014;13(10):1254-1257.PubMedGoogle Scholar
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Wolf  R, Oumeish  OY, Parish  LC.  Intertriginous eruption.  Clin Dermatol. 2011;29(2):173-179.PubMedGoogle ScholarCrossref
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Missiaen  L, Dode  L, Vanoevelen  J, Raeymaekers  L, Wuytack  F.  Calcium in the Golgi apparatus.  Cell Calcium. 2007;41(5):405-416.PubMedGoogle ScholarCrossref
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Burge  SM, Millard  PR, Wojnarowska  F.  Hailey-Hailey disease: a widespread abnormality of cell adhesion.  Br J Dermatol. 1991;124(4):329-332.PubMedGoogle ScholarCrossref
8.
Tomaszewska  KA, Gerlicz-Kowalczuk  Z, Kręgiel  M,  et al.  The coexistence of Darier’s disease and Hailey-Hailey disease symptoms.  Postepy Dermatol Alergol. 2017;34(2):180-183.PubMedGoogle ScholarCrossref
9.
de Aquino Paulo Filho  T, deFreitas  YK, da Nóbrega  MT,  et al.  Hailey-Hailey disease associated with herpetic eczema-the value of the Tzanck smear test.  Dermatol Pract Concept. 2014;4(4):29-31.PubMedGoogle Scholar
10.
Ibrahim  O, Hogan  SR, Vij  A, Fernandez  AP.  Low-dose naltrexone treatment of familial benign pemphigus (Hailey-Hailey disease).  JAMA Dermatol. 2017;153(10):1015-1017.PubMedGoogle ScholarCrossref
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