eMedicine Specialties > Dermatology > Bullous Diseases
Familial Benign Pemphigus (Hailey-Hailey Disease): Treatment & Medication
Updated: Aug 13, 2008
- Overview
- Differential Diagnoses & Workup
- Treatment & Medication
- Follow-up
- Multimedia
Treatment
Medical Care
Familial benign pemphigus waxes and wanes in intensity. Soothing compresses (aluminum acetate 1:40 dilution) followed by intermittent use of mild corticosteroid preparations (class V or class VI corticosteroids) and topical antibiotics (clindamycin or erythromycin) result in transient improvement. More widespread flares of familial benign pemphigus may require systemic antibiotics to suppress protease activation and acantholysis. Erythromycin and tetracycline are favored. Bacterial culture and sensitivity can help guide appropriate therapy.
In patients with refractory cases of familial benign pemphigus (Hailey-Hailey disease), dapsone, systemic corticosteroids, methotrexate, retinoids (isotretinoin or acitretin),5 and etretinate have been tried and have been reported to be of value in some anecdotal reports. Most patients with familial benign pemphigus at the author's institution respond well to anti-infective therapy and short courses of corticosteroids, and other immunosuppressive agents have only rarely been helpful in the author's experience. Topical tacrolimus ointment has been a valuable addition to the treatment regimen and has been able to control familial benign pemphigus well, even without the adjunctive use of topical corticosteroids.
Topical tacrolimus ointment has been found to be helpful in familial benign pemphigus,6 and photodynamic therapy with 5-aminolevulinic acid has been used for recalcitrant cases.7
Reports8,9 indicate that low-dose botulinum toxin type A injection may be of benefit for familial benign pemphigus. Control of hyperhidrosis, which aggravates familial benign pemphigus (Hailey-Hailey disease), may be the mechanism for this off-label, novel approach.
Isolated reports of oral acitretin10 therapy or intramuscular alefacept11 leading to improvement in familial benign pemphigus warrant further study.
Surgical Care
Dermabrasion, carbon dioxide laser ablation, and pulsed dye laser therapy have been tried in the treatment of familial benign pemphigus, with variable success.12,13,14
Diet
To help minimize friction, it is recommended that patients with familial benign pemphigus maintain their weight at appropriate levels.
Activity
Instruct patients with familial benign pemphigus (Hailey-Hailey disease) to select cool and comfortable clothing that reduces heat, moisture, and friction. Patients should avoid fabrics or clothing styles that rub or irritate affected areas. Washing new shirts may soften the collars. In some cases, pain may limit physical activities.
Medication
The goals of pharmacotherapy in familial benign pemphigus are to reduce morbidity and to prevent complications. Reportedly,8,9 off-label use of low-dose botulinum toxin type A injection may be of benefit to control hyperhidrosis, which aggravates familial benign pemphigus.
Immunosuppressants
Used in refractory cases. Ameliorate symptoms of inflammation (eg, pain, swelling, stiffness). Use of systemic immunosuppressive therapy for familial benign pemphigus is controversial. No large-scale studies offer a clear evidence-based approach to immunosuppressive therapy in the management of familial benign pemphigus. Because adverse effects can be severe and even fatal at times, such therapies must be initiated cautiously and with adequate informed consent.
Methotrexate (Rheumatrex, Folex PFS)
Antimetabolite that inhibits dihydrofolate reductase, thereby hindering DNA synthesis and cell reproduction.
Adult
10-12.5 mg PO qwk
Pediatric
Not recommended
Oral aminoglycosides may decrease absorption and blood levels of concurrent oral MTX; charcoal lowers MTX levels; coadministration with etretinate may increase hepatotoxicity of MTX; folic acid or its derivatives contained in some vitamins may decrease response to MTX; coadministration with NSAIDs may be fatal; indomethacin and phenylbutazone can increase MTX plasma levels; may decrease phenytoin serum levels; probenecid, salicylates, procarbazine, and sulfonamides, including TMP-SMZ, may increase effects and toxicity of MTX; may increase plasma levels of thiopurines
Documented hypersensitivity; alcoholism; hepatic insufficiency; documented immunodeficiency syndromes; preexisting blood dyscrasias (eg, bone marrow hypoplasia, leukopenia, thrombocytopenia, significant anemia); renal insufficiency
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Monitor CBC counts monthly, and liver and renal function q1-3mo during therapy (monitor more frequently during initial dosing, dose adjustments, or when risk of elevated MTX levels, eg, dehydration); MTX has toxic effects on hematologic, renal, GI tract, pulmonary, and neurologic systems; discontinue if significant drop in blood counts; aspirin, NSAIDs, or low dose steroids may be administered concomitantly with MTX (possibility of increased toxicity with NSAIDs including salicylates has not been tested)
Corticosteroids
Have anti-inflammatory properties and cause profound and varied metabolic effects. In addition, these agents modify the body's immune response to diverse stimuli.
Prednisone (Deltasone, Orasone)
Glucocorticoid (adrenocortical steroid) absorbed easily into GI tract. May decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.
Adult
0.5-1 mg/kg/d PO prn for short periods
Pediatric
Administer as in adults
Patients on corticosteroid therapy should not be vaccinated against smallpox or immunized because of possible neurologic complications and lack of antibody response; coadministration with estrogens may decrease prednisone clearance; when used with digoxin, digitalis toxicity secondary to hypokalemia may increase; phenobarbital, phenytoin, and rifampin may increase metabolism of glucocorticoids (consider increasing maintenance dose); monitor for hypokalemia with coadministration of diuretics
Documented hypersensitivity; viral infection; peptic ulcer disease; hepatic dysfunction; severe osteoporosis; diabetes; connective tissue infections; fungal or tubercular skin infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Reduced resistance and masked signs of infections; prolonged use may result in cataracts and glaucoma; psychic changes may be exhibited, such as mood swings and depression
Triamcinolone (Aristocort)
Treats inflammatory dermatosis that is responsive to steroids. Decreases inflammation by suppressing migration of polymorphonuclear leukocytes and reversing capillary permeability. Use 0.1% cream.
Adult
Apply thin film bid/tid until favorable response
Pediatric
Apply as in adults
None reported
Documented hypersensitivity; fungal, viral, and bacterial skin infections
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Caution in pediatric patients; do not use in decreased skin circulation; prolonged use, applications over large areas, and use of potent steroids and occlusive dressings may result in systemic absorption; systemic absorption may cause Cushing syndrome, reversible HPA-axis suppression, hyperglycemia, and glycosuria
Retinoids
Inhibit sebaceous gland function and modify keratinization.
Isotretinoin (Accutane)
Decreases sebaceous gland size and sebum production. May inhibit sebaceous gland differentiation and abnormal keratinization. Used to treat severe cystic acne.
Adult
1 mg/kg/d PO
Pediatric
Not recommended
Toxicity may occur with beta carotene coadministration; pseudotumor cerebri or papilledema may occur; may reduce plasma levels of carbamazepine
Documented hypersensitivity; pregnancy or potential pregnancy (unless proper contraceptives used); sensitivity to paraben (preservative in gelatin capsule)
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Women should not be pregnant when on Accutane therapy; may decrease night vision; may be associated with development of hepatitis; occasional exaggerated healing response of acne lesions (excessive granulation with crusting) may occur; patients with diabetes may experience problems in controlling blood sugar while on isotretinoin; avoid exposure to UV light or sunlight until tolerance achieved; discontinue if rectal bleeding, abdominal pain, or severe diarrhea occur
Acitretin (Soriatane)
Retinoic acid analog, similar to etretinate and isotretinoin. Etretinate is primary metabolite and has demonstrated clinical effects close to those seen with etretinate. Mechanism of action is unknown.
Adult
0.5-0.75 mg/kg/d PO
Pediatric
Not recommended
Increases toxicity of MTX (avoid concomitant use); interferes with effects of microdosed progestin minipill; coadministration with alcohol may enhance synthesis of etretinate, which has much longer half-life than acitretin (>120 d)
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Do not use in severe obesity; women of childbearing age must be capable of complying with effective contraceptive measures; recommended that contraception be continued for at least 3 y after stopping treatment with acitretin; etretinate may form from acitretin, which takes approximately 2-3 y to clear from the body; caution if impaired renal or liver function; perform AST, ALT, and LDH tests prior to initiation of acitretin therapy at 1- to 2-wk intervals until stable and thereafter at intervals as clinically indicated
Etretinate (Tegison)
Not available in the United States. Retinoic acid analog. Used only after other medicines have been tried and failed.
Adult
0.5-1 mg/kg/d PO divided bid
Pediatric
Not recommended
Increases MTX toxicity (avoid concomitant use); interferes with effects of microdosed progestin minipill
Documented hypersensitivity
Pregnancy
X - Contraindicated; benefit does not outweigh risk
Precautions
Do not use in severe obesity; women of childbearing age must be capable of complying with effective contraceptive measures; recommended that contraception be continued for at least 3 y after stopping treatment; etretinate takes approximately 2-3 y to clear from the body; caution if impaired renal or liver function; perform AST, ALT, and LDH tests prior to initiation of therapy at 1- to 2-wk intervals until stable and thereafter at intervals as clinically indicated
Antimicrobials
Used to eliminate microorganisms. Use for possible secondary bacterial infections. Also, some antimicrobials have immunomodulatory effects.
Erythromycin (E.E.S., E-Mycin, Ery-Tab)
Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes causing RNA-dependent protein synthesis to arrest. For treatment of staphylococcal and streptococcal infections. In children, age, weight, and severity of infection determine proper dosage. When bid dosing is desired, half-total daily dose may be taken q12h. For more severe infections, double the dose.
Adult
250 mg PO q6h or 500 mg PO q12h
Pediatric
20 mg/kg PO 2 h prior to procedure, followed by 10 mg/kg 6 h later
Coadministration may increase toxicity of theophylline, digoxin, carbamazepine, and cyclosporine; may potentiate anticoagulant effects of warfarin; coadministration with lovastatin and simvastatin, increases risk of rhabdomyolysis
Documented hypersensitivity; hepatic impairment
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Caution in liver disease; estolate formulation may cause cholestatic jaundice; adverse GI tract effects are common (give doses pc); discontinue use if nausea, vomiting, malaise, abdominal colic, or fever occur
Clindamycin hydrochloride (Cleocin)
Lincosamide for treatment of serious skin and soft tissue staphylococcal infections. Also effective against aerobic and anaerobic streptococci (except enterococci). Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes causing RNA-dependent protein synthesis to arrest.
Adult
150-300 mg PO q6h
Pediatric
8-16 mg/kg/d PO divided tid/qid
Increases duration of neuromuscular blockade induced by tubocurarine and pancuronium; erythromycin may antagonize effects of clindamycin; antidiarrheals may delay absorption of clindamycin; cyclosporine levels may decrease when administered concurrently; kaolin may reduce absorption
Documented hypersensitivity; regional enteritis; ulcerative colitis; hepatic impairment; antibiotic-associated colitis
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Adjust dose in severe hepatic dysfunction, no adjustment necessary in renal insufficiency; associated with severe and possibly fatal colitis; may result in colitis; occasionally results in overgrowth of nonsusceptible organisms (eg, yeast)
Dapsone (Avlosulfon)
Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to that of sulfonamides where competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Anti-inflammatory mechanism of action most likely relates to inhibition of neutrophils through suppression of the halide-myeloperoxidase system.
Adult
50-200 mg PO qd
Pediatric
2 mg/kg PO qd; not to exceed 100 mg/d
May inhibit anti-inflammatory effects of clofazimine; hematologic reactions may increase with folic acid antagonists, such as pyrimethamine (monitor for agranulocytosis during second and third mo of therapy); probenecid increases dapsone toxicity; trimethoprim with dapsone may increase toxicity of both drugs; because of increased renal clearance, dapsone levels may decrease significantly when administered concurrently with rifampin
Documented hypersensitivity; G-6-PD deficiency; anemia
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Sulfa allergy; history of liver, kidney, or heart disease; dapsone induces hemolysis and results in dose-related reduction in hemoglobin; myelosuppression and agranulocytosis have been reported, monitor patients frequently with CBC counts; methemoglobinemia, hepatitis, neuropathy, and headaches have been reported
Tetracycline (Sumycin)
Treats gram-positive and gram-negative organisms, as well as mycoplasmal, chlamydial, and rickettsial infections. Inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s).
Adult
250-500 mg PO q6h
Mild-to-moderate infections: 500 mg PO bid or 250 mg PO qid for 7-14 d
Severe infections: 500 mg PO qid for 7-14 d
Pediatric
<8 years: Not recommended
>8 years: 25-50 mg/kg/d (10-20 mg/lb) PO qid
Bioavailability decreases with antacids containing aluminum, calcium, magnesium, iron, or bismuth subsalicylate; may enhance agents with neuromuscular blocking effect; can decrease effects of oral contraceptives, causing breakthrough bleeding and increased risk of pregnancy; tetracyclines can increase hypoprothrombinemic effects of anticoagulants
Coadministration with retinoids can cause increased intracranial pressure (coadministration contraindicated); administer tetracycline at least 1 h before or 4-6 h after colestipol or cholestyramine; if tetracycline administered concurrently with digoxin, monitor digoxin levels (dosage adjustment for digoxin may be required; risk of interaction may be reduced if given with Lanoxicaps)
Documented hypersensitivity; severe hepatic dysfunction
Pregnancy
D - Fetal risk shown in humans; use only if benefits outweigh risk to fetus
Precautions
Photosensitivity may occur with prolonged exposure to sunlight or tanning equipment; reduce dose in renal impairment; consider drug serum level determinations in prolonged therapy; tetracycline use during tooth development (last one half of pregnancy through age 8 y) can cause permanent discoloration of teeth; Fanconi-like syndrome may occur with outdated tetracyclines
Pseudotumor cerebri has been associated with tetracyclines, therefore, possibility for permanent sequelae exists
Clindamycin phosphate solution 10 mg/mL (Cleocin T, Clindets, Clinda-Derm)
Lincosamide for treatment of serious skin and soft tissue staphylococcal infections when taken systemically. Useful in treatment of acne when applied topically. Inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes causing RNA-dependent protein synthesis to arrest.
Adult
Apply to affected area bid
Pediatric
Administer as in adults
None with topical use; systemic use may enhance effects of neuromuscular blocking agents
Documented hypersensitivity
Pregnancy
B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals
Precautions
Systemic (not topical) use has been associated with severe colitis; GI tract disturbances rarely have been reported with topical use; prolonged use may result in overgrowth of nonsusceptible organisms resulting in gram-negative folliculitis; discontinue if superinfection occurs
Ketoconazole (Nizoral)
Imidazole that inhibits the synthesis of ergosterol, thereby affecting cell membrane integrity and resulting in fungal cell death.
Adult
Apply 2% cream to affected area qd/bid for 2-6 wk
Pediatric
Not established
None reported
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
Local reactions may occur including rash, irritation, burning, and pruritus; rarely, systemic absorption may occur
Astringents
Drying agents used in management of hyperhidrosis.
Aluminum chloride (Drysol)
Aluminum chloride hexahydrate 20% in absolute alcohol. Antiperspirant mechanism of action is not known, although creation of aluminum-containing casts within the sweat duct has been postulated.
Adult
Apply to affected area hs for 2-7 consecutive nights, then prn; to prevent irritation, completely dry area prior to application
Pediatric
Not established
None reported
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
External use only; not for application on irritated, broken, or recently shaved skin (some dermatologists use 5% aluminum acetate for hemostasis after shave biopsies)
Aluminum acetate 5% soak (Bite Rx)
Dissolve aluminum acetate tablets in water to attain a 1:20 solution. Has a drying effect on vesicular or wet dermatoses.
Adult
Apply as compress for 20-30 min 4-6 times/d
Pediatric
Administer as in adults
None reported
Documented hypersensitivity
Pregnancy
C - Fetal risk revealed in studies in animals but not established or not studied in humans; may use if benefits outweigh risk to fetus
Precautions
External use only
More on Familial Benign Pemphigus (Hailey-Hailey Disease) |
| Overview: Familial Benign Pemphigus (Hailey-Hailey Disease) |
| Differential Diagnoses & Workup: Familial Benign Pemphigus (Hailey-Hailey Disease) |
 Treatment & Medication: Familial Benign Pemphigus (Hailey-Hailey Disease) |
| Follow-up: Familial Benign Pemphigus (Hailey-Hailey Disease) |
| Multimedia: Familial Benign Pemphigus (Hailey-Hailey Disease) |
| References |
| « Previous Page | Next Page » |
References
Hailey H, Hailey H. Familial benign chronic pemphigus. Arch Dermatol. 1939;39:679-85.
Fairclough RJ, Dode L, Vanoevelen J, Andersen JP, Missiaen L, Raeymaekers L, et al. Effect of Hailey-Hailey Disease mutations on the function of a new variant of human secretory pathway Ca2+/Mn2+-ATPase (hSPCA1). J Biol Chem. Jul 4 2003;278(27):24721-30. [Medline].
Foggia L, Aronchik I, Aberg K, Brown B, Hovnanian A, Mauro TM. Activity of the hSPCA1 Golgi Ca2+ pump is essential for Ca2+-mediated Ca2+ response and cell viability in Darier disease. J Cell Sci. Feb 15 2006;119(Pt 4):671-9. [Medline].
Zhu YG, Yang S, Gao M, Chen JJ, Li W, Wang PG, et al. Two novel mutations of the ATP2C1 gene in Chinese families with Hailey-Hailey disease. J Dermatol Sci. May 2006;42(2):125-7. [Medline].
Hunt MJ, Salisbury EL, Painter DM, Lee S. Vesiculobullous Hailey-Hailey disease: successful treatment with oral retinoids. Australas J Dermatol. Nov 1996;37(4):196-8. [Medline].
Rabeni EJ, Cunningham NM. Effective treatment of Hailey-Hailey disease with topical tacrolimus. J Am Acad Dermatol. Nov 2002;47(5):797-8. [Medline].
Ruiz-Rodriguez R, Alvarez JG, Jaén P, Acevedo A, Córdoba S. Photodynamic therapy with 5-aminolevulinic acid for recalcitrant familial benign pemphigus (Hailey-Hailey disease). J Am Acad Dermatol. Nov 2002;47(5):740-2. [Medline].
Konrad H, Karamfilov T, Wollina U. Intracutaneous botulinum toxin A versus ablative therapy of Hailey-Hailey disease--a case report. J Cosmet Laser Ther. Dec 2001;3(4):181-4. [Medline].
Lapiere JC, Hirsh A, Gordon KB, Cook B, Montalvo A. Botulinum toxin type A for the treatment of axillary Hailey-Hailey disease. Dermatol Surg. Apr 2000;26(4):371-4. [Medline].
Berger EM, Galadari HI, Hassan I, Gottleib AB. Successful treatment of Hailey-Hailey disease with acitretin. Journal of Drugs in Dermatology. July 2007;6:734-6. [Medline].
Hurd DS, Johnston C, Bevins A. A case report of Hailey-Hailey disease treated with alefacept (Amevive). Br J Dermatol. Feb 2008;158(2):399-401. [Medline].
Don PC, Carney PS, Lynch WS, Zaim MT, Hassan MO. Carbon dioxide laserabrasion: a new approach to management of familial benign chronic pemphigus (Hailey-Hailey disease). J Dermatol Surg Oncol. Nov 1987;13(11):1187-94. [Medline].
Fisher GH, Geronemus RG. Improvement of familial benign pemphigus after treatment with pulsed-dye laser: a case report. Dermatol Surg. Jul 2006;32(7):966-8. [Medline].
Kartamaa M, Reitamo S. Familial benign chronic pemphigus (Hailey-Hailey disease). Treatment with carbon dioxide laser vaporization. Arch Dermatol. May 1992;128(5):646-8. [Medline].
Berth-Jones J, Smith SG, Graham-Brown RA. Benign familial chronic pemphigus (Hailey-Hailey disease) responds to cyclosporin. Clin Exp Dermatol. Jan 1995;20(1):70-2. [Medline].
Burge SM. Hailey-Hailey disease: the clinical features, response to treatment and prognosis. Br J Dermatol. Mar 1992;126(3):275-82. [Medline].
Cooley JE, Briggaman RA, Cronce DJ, Banes AJ, O'Keefe EJ. Hailey-Hailey disease keratinocytes: normal assembly of cell-cell junctions in vitro. J Invest Dermatol. Dec 1996;107(6):877-81. [Medline].
Dadban A, Guillot B. Oesophageal involvement in familial benign chronic pemphigus. Acta Derm Venereol. 2006;86(3):252-3. [Medline].
De Dobbeleer G, De Graef C, M'Poudi E, Gourdain JM, Heenen M. Reproduction of the characteristic morphologic changes of familial benign chronic pemphigus in cultures of lesional keratinocytes onto dead deepidermized dermis. J Am Acad Dermatol. Nov 1989;21(5 Pt 1):961-5. [Medline].
Fisher BK, Margesson LJ. Hailey-Hailey disease (familial benign chronic pemphigus). In: Genital Skin Disorders: Diagnosis and Treatment. St. Louis, Mo: Mosby-Year Book; 1998:68, 117.
Flint ID, Spencer DM, Wilkin JK. Eczema herpeticum in association with familial benign chronic pemphigus. J Am Acad Dermatol. Feb 1993;28(2 Pt 1):257-9. [Medline].
Galimberti RL, Kowalczuk AM, Bianchi O, Bonino MV, Garcia Garcia A. Chronic benign familial pemphigus. Int J Dermatol. Sep 1988;27(7):495-500. [Medline].
Gokdemir G, Gonen S, Kivanç-Altunay I, Köslü A. Familial pemphigus vulgaris: two siblings with unusual localized variant. Int J Dermatol. Feb 2006;45(2):170-2. [Medline].
Hernández-Pérez E. Familial benign chronic pemphigus. Cutis. Jan 1987;39(1):75-7. [Medline].
Ikeda S, Suga Y, Ogawa H. Successful management of Hailey-Hailey disease with potent topical steroid ointment. J Dermatol Sci. Jun 1993;5(3):205-11. [Medline].
Ishibashi Y, Kajiwara Y, Andoh I, Inoue Y, Kukita A. The nature and pathogenesis of dyskeratosis in Hailey-Hailey's disease and Darier's disease. J Dermatol. Aug 1984;11(4):335-53. [Medline].
Metze D, Hamm H, Schorat A, Luger T. Involvement of the adherens junction-actin filament system in acantholytic dyskeratosis of Hailey-Hailey disease. A histological, ultrastructural, and histochemical study of lesional and non-lesional skin. J Cutan Pathol. Jun 1996;23(3):211-22. [Medline].
Oguz O, Gökler G, Ocakoglu O, Oguz V, Demirkesen C, Aydemir EH. Conjunctival involvement in familial chronic benign pemphigus (Hailey-Hailey disease). Int J Dermatol. Apr 1997;36(4):282-5. [Medline].
Remitz A, Lauerma AI, Stubb S, Förström L, Reitamo S. Darier's disease, familial benign chronic pemphigus (Hailey-Hailey disease) and contact hypersensitivity. J Am Acad Dermatol. Jan 1990;22(1):134. [Medline].
Sehgal VN, Jain S. Chronic familial benign pemphigus. J Dermatol. Jun 1994;21(6):382-8. [Medline].
Starzycki Z, Chorzelski TP, Jablonska S. Familial pemphigus vulgaris in mother and daughter. Int J Dermatol. Mar 1998;37(3):211-4. [Medline].
Welsh EA, Ikeda S, Peluso AM, Bonifas JM, Bare JW, Woodley DT, et al. Hailey-Hailey disease is not allelic to Darier's disease. J Invest Dermatol. Jun 1994;102(6):992-3. [Medline].
Further Reading
Keywords
pemphigus, benign familial pemphigus, familial benign pemphigus, bullous disease, Hailey-Hailey disease
