Familial Benign Pemphigus (Hailey-Hailey Disease) Medication

Updated: Aug 20, 2020
  • Author: Thomas N Helm, MD; Chief Editor: Dirk M Elston, MD  more...
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Medication

Medication Summary

The goals of pharmacotherapy in familial benign pemphigus are to reduce morbidity and to prevent complications. Reportedly, [13, 14] off-label use of low-dose botulinum toxin type A injection may be of benefit to control hyperhidrosis, which aggravates familial benign pemphigus. Etretinate (Tegison), which is not available in the United States, is a retinoic acid analog that is used only after other medicines have been tried and failed.

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Immunosuppressants

Class Summary

Immunosuppressants are used in refractory cases. They 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, Trexall)

Methotrexate is an antimetabolite that inhibits dihydrofolate reductase, thereby hindering DNA synthesis and cell reproduction.

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Corticosteroids

Class Summary

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

Prednisone is a glucocorticoid (adrenocortical steroid) that is absorbed easily into the GI tract. It may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity.

Triamcinolone topical (Kenalog, Oralone, Triderm, Zytopic)

Triamcinolone treats inflammatory dermatosis that is responsive to steroids. It decreases inflammation by suppressing the migration of polymorphonuclear leukocytes and reversing capillary permeability. Use 0.1% cream.

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Retinoid-like Agents

Class Summary

Retinoid-like agents inhibit sebaceous gland function and modify keratinization.

Isotretinoin (Amnesteem, Claravis, Sotret)

Isotretinoin decreases sebaceous gland size and sebum production. It may inhibit sebaceous gland differentiation and abnormal keratinization. Isotretinoin is used to treat severe cystic acne.

A US Food and Drug Administration–mandated registry is now in place for all individuals prescribing, dispensing, or taking isotretinoin. For more information on this registry, see iPLEDGE. This registry aims to further decrease the risk of pregnancy and other unwanted and potentially dangerous adverse effects during a course of isotretinoin therapy.

Acitretin (Soriatane)

Acitretin is a retinoic acid analog, similar to etretinate and isotretinoin. Etretinate is primary metabolite and has demonstrated clinical effects close to those seen with etretinate. Its mechanism of action is unknown.

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Antibiotics, Other

Class Summary

Antibiotics are used to eliminate microorganisms. Use these agents for possible secondary bacterial infections. Also, some antimicrobials have immunomodulatory effects.

Erythromycin (E.E.S., Ery-Tab, Erythrocin)

Erythromycin inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes causing RNA-dependent protein synthesis to arrest. Erythromycin is used for the treatment of staphylococcal and streptococcal infections. In children, age, weight, and severity of infection determine the proper dosage. When twice-daily dosing is desired, half the total daily dose may be taken every 12 hours. For more severe infections, double the dose.

Clindamycin hydrochloride (Cleocin)

Clindamycin is a lincosamide used for the treatment of serious skin and soft-tissue staphylococcal infections. It is also effective against aerobic and anaerobic streptococci (except enterococci). It inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes causing RNA-dependent protein synthesis to arrest.

Dapsone

Dapsone is bactericidal and bacteriostatic against mycobacteria; its mechanism of action is similar to that of sulfonamides where competitive antagonists of PABA prevent the formation of folic acid, inhibiting bacterial growth. Its anti-inflammatory mechanism of action most likely relates to the inhibition of neutrophils through the suppression of the halide-myeloperoxidase system.

Tetracycline

Tetracycline treats gram-positive and gram-negative organisms, as well as mycoplasmal, chlamydial, and rickettsial infections. It inhibits bacterial protein synthesis by binding with 30S and possibly 50S ribosomal subunit(s).

Clindamycin phosphate solution 10 mg/mL (Cleocin T, Evoclin, Clindagel, ClindaMax)

Clindamycin is a lincosamide used for the treatment of serious skin and soft-tissue staphylococcal infections when taken systemically. It is useful in the treatment of acne when applied topically. It inhibits bacterial growth, possibly by blocking dissociation of peptidyl tRNA from ribosomes causing RNA-dependent protein synthesis to arrest.

Ketoconazole topical (Extina, Xolegel)

Ketoconazole is an imidazole that inhibits the synthesis of ergosterol, thereby affecting cell membrane integrity and resulting in fungal cell death.

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Astringents

Class Summary

Astringents are drying agents used in the management of hyperhidrosis.

Aluminum chloride topical (Drysol, Certain Dri, Hypercare, Xerac)

This agent is aluminum chloride hexahydrate 20% in absolute alcohol. Its antiperspirant mechanism of action is not known, although the creation of aluminum-containing casts within the sweat duct has been postulated.

Aluminum acetate solution

Dissolve aluminum acetate tablets in water to attain a 1:20 solution. It has a drying effect on vesicular or wet dermatoses.

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