benign familial pemphigus (Hailey-Hailey disease)
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Epidemiology
- rare
Pathology
- disruption of intracellular Ca+2 transport
- defect in keratinocyte cohesion
- suprabasal cell separation (acantholysis) of the epidermis
* image[1]
Genetics
- autosomal dominant
- associated defects in ATP2C1
Clinical manifestations
- pruritic & painful rash
- intermittent & persistent blisters
- blistering in the intertriginous areas
- hyperpigmentation, hypopigmentation, multiple acrochordons, malodorous crust, areas of maceration & erosion (case report)[1]
- involvement of neck & axilla (case report)[1]
- pseudofolliculitis barbae
- seasonal variation, warm weather exacerbation
Laboratory
Diagnostic procedures
- skin biopsy for diagnosis[1]
Complications
- superimposed bacterial infection or fungal infections
- fatal eczema herpeticum with herpes simplex virus
Differential diagnosis
- intertrigo
- inverse psoriasis
- seborrheic dermatitis
- erythrasma
- acanthosis nigricans
- Darier disease
- Galli-Galli disease
- autoimmune pemphigus[1]
Management
- topical corticosteroid & a topical antimicrobial
- triamcinolone (0.1% cream), clindamycin (1% lotion)
- naltrexone 3 mg daily may alleviate disease refractory to other treatments[2]
- laser ablation may provide long-term remission for patients with symptoms refractory to medical management[2]
More general terms
Additional terms
References
- ↑ 1.0 1.1 1.2 1.3 1.4 1.5 1.6 Haley A, Mui UM, Tyring SK. (image) A 58-Year-Old Man With a Macerated Rash of the Neck and Axilla. JAMA. 2018;319(14):1499-1500. April 10, 2018 PMID: https://www.ncbi.nlm.nih.gov/pubmed/29634814 https://jamanetwork.com/journals/jama/fullarticle/2677996
- ↑ 2.0 2.1 2.2 2.3 Adusumilli NC, Friedman AJ Benign Familial Pemphigus JAMA Dermatol. Published online January 12, 2022 PMID: https://www.ncbi.nlm.nih.gov/pubmed/35019935 https://jamanetwork.com/journals/jamadermatology/fullarticle/278788
- ↑ 3.0 3.1 Harris JB, Werbel T, Saikaly SK Images in Dermatology Benign Familial Pemphigus in Skin of Color. JAMA Dermatol. 2023;159(3):331 PMID: https://www.ncbi.nlm.nih.gov/pubmed/36630125 https://jamanetwork.com/journals/jamadermatology/fullarticle/2800237