Management of familial benign chronic pemphigus

Figures & data

Table 1 Topical agents used to treat HHD

References	Agents	Notes
Topical steroids: used for acute exacerbations
^Citation6,Citation9,Citation11,Citation14	Moderate- to high-intensity steroids; creams or ointments depending on severity of flare	Twice a day, up to 2–16 weeks. May use alternating intervals until remission. Caution with skin atrophy and contact dermatitis
Topical steroid sparing agents: typically used for maintenance
^Citation15^–^Citation18	Tacrolimus 0.1% ointment Pimecrolimus (cream)	Twice a day, up to 2 weeks. May alternate with topical steroids for better outcomes
Topical antimicrobials: used for mild superficial infections
^Citation11^–^Citation13	Clindamycin 1% lotion or cream Gentamicin 0.1% cream Mupirocin 2% cream Adjunct of chlorhexidine gluconate 4% wash Bleach baths (1/2 cup bleach in 40 gal bath tub) 5-minute to 10-minute application of topical compresses with 1:40 diluted aluminum acetate Ketoconazole 2% cream (or other azole creams)	Two to four times a day for 2–4 weeks
Other topical options: refractory to topical treatments listed earlier
^Citation28,Citation29	Calcitriol topical	Twice a day, up to 4 weeks. A 3-month remission reported
^Citation30	Tacalcitol	Twice a day for 3 months. Better results compared to topical steroids
^Citation31	Topical 5-FU	Three times a week for 3 months, then once a week for 3 months. Remission documented at 9-month follow-up
^Citation32	Topical cadexomer	May see response within 1 month of treatment and near-complete resolution near 10 months
Other local options: typically used when topical treatments have failed
^Citation11	Intralesional steroids	Can be considered if topical steroids fail
^Citation23,Citation25^–^Citation27	BTA	As needed, one case reported 50 units per axilla. Needs reinjections for maintenance. Concern for development of neutralizing antibodies

Abbreviations: HHD, Hailey–Hailey disease; 5-FU, 5-fluorouracil; BTA, botulinum toxin type A; gal, gallon.

Table 2 Systemic agents used in HHD

References	Agents	Notes
Antimicrobials
^Citation6,Citation33	Erythromycin, penicillins	May be used in addition to usual treatment options to treat severe superficial infections or not responsive to topical treatments
^Citation34	Doxycycline 100 mg PO daily, consider low-dose maintenance	At least 3 months. Five of six relapse within days to months, two of six no relapse within 5 years
^Citation16	Erythromycin plus topical tacrolimus	2 weeks. No recurrence
^Citation52	Oral dapsone	One of three complete remissions with maintenance after treatment dose was completed
Retinoids
^Citation37	Etretinate	Successful. Careful with teratogenicity of retinoids
^Citation38	Alitretinoin 30 mg/d and NB-UVB twice a week	Clearance in 6 weeks, alitretinoin was then used as maintenance
^Citation36	Acitretin 25 mg PO daily	6 months. Significant improvement
DMARDs
^Citation45	MTX 15 mg per week	3 months. Clearance for 2 years
^Citation46	MTX maintenance 7.5 mg IM	Weekly for up to 16 weeks
^Citation43	Thalidomide 100 mg TID with topical steroids and antimicrobials	Used in conjunctions with topical steroids and antimicrobials. Lesions in remission after discontinuation
^Citation44,Citation46^–^Citation49	Cyclosporine	Risk of flare upon discontinuation
^Citation51	Glycopyrrolate 1 mg daily, topical mometasone ointment 0.1% once daily, and oral minocycline 50 mg daily	Improvement after 1 month of treatment and remission after 6 months, xerostomia as side effect

Abbreviations: HHD, Hailey–Hailey disease; PO, per os; NB-UVB, narrowband ultraviolet B; DMARDs, disease-modifying antirheumatic drugs; MTX, methotrexate; IM, intramuscularly; TID, three times a day.

Table 3 Laser, light, and surgical therapies

References	Methods	Notes
Laser and light therapies
^Citation1,Citation58,Citation59,Citation61,Citation63	Carbon dioxide laser: settings vary among case reports; power range: 5–25 W; spot size 1–9 mm; modes: continuous, defocused, and pulsed; 1–5 passes	Significant improvement or remission of disease achieved in the majority of cases. Scarring and hyperpigmentation are side effects
^Citation54	Alexandrite 12–20 J/cm^2 and spot size 10–15 mm. Cooling techniques used	Up to 13 treatments needed to achieve complete clearance, with five maintenance treatments at 3-month intervals postclearance. Minimal- to-no recurrence was observed at 2-year follow-up. Postinflammatory hyperpigmentation observed
^Citation22,Citation64	Er:YAG	Partial-to-compete remission at follow-up 8–12 months
^Citation55,Citation56	PDT with topical methyl aminolevulinic acid and red light. The light 630 nm and 37 J/cm^2 for 7.5 minutes. Only 590–700 nm and 120 mW/cm^2 for 30 minutes	Postoperative pruritus and pain. Variable results; no improvement to clearance
^Citation65	PDL 7–10 mm spot size, 7–10 J/cm^2 fluence, 6–10 ms pulse duration	Five treatments were performed at 2-week to 4-week intervals. Ineffective in one to six patients. The others had improvement with different degrees of relapse
^Citation63	1,450 nm diode laser. 6 mm spot size 14 J/cm^2	Not effective after three treatment sessions. Painful. Decrease in sweating and odor
Surgical therapies
^Citation40,Citation66,Citation67	Dermabrasion	Used in refractory disease. Reepithelialization takes ~10 days absence of relapse for long period of time (up to 42 months)
^Citation68^–^Citation70	Excision with split-thickness skin grafting	Up to 8 years documented without recurrence of disease

Abbreviations: Er:YAG, erbium-doped yttrium aluminum garnet; PDT, photodynamic therapy; PDL, pulsed dye laser.

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