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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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Corresponding Author: Christopher Haley, MD, Center for Clinical Studies, 451 N Texas Ave, Webster, TX 77598 (firstname.lastname@example.org).
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.
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