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Pemphigus, IgA: Treatment & Medication

Author: Lawrence Chan, MD, Department Head and Director of Skin Immunology Research, Professor, Departments of Dermatology and Microbiology/Immunology, University of Illinois College of Medicine
Contributor Information and Disclosures

Updated: Sep 24, 2008

Treatment

Medical Care

Medical therapy should be directed towards reducing inflammation, since IgA pemphigus represents a group of autoimmune blistering skin diseases manifested clinically as chronic inflammation. Since IgA pemphigus is uncommon, therapy is based on the mechanism of disease and anecdotal reports.

  • Generally, corticosteroids are the mainstay of treatment.
  • Dapsone also may be helpful because of its antineutrophilic effects.
  • Rapid response to treatment with adalimumab and mycophenolate mofetil was reported in 2005.20

Activity

Usually, no restrictions are placed on patient activities; however, advise patients to avoid contact sports during the active disease state.

Medication

In general, corticosteroids are the mainstay of treatment. Dapsone, a medication with antineutrophilic effects, also may be helpful.

Anti-inflammatory agents

IgA pemphigus is characterized histologically by inflammatory cell (neutrophil) infiltration in the upper epidermis. Anti-inflammatory agents theoretically block the inflammatory process and improve the disease conditions.21,22


Prednisone (Deltasone)

Effective treatment for IgA pemphigus. Immunosuppressant for treatment of autoimmune disorders; may decrease inflammation by reversing increased capillary permeability and suppressing PMN activity. Stabilizes lysosomal membranes and suppresses lymphocytes and antibody production. Consult the pediatrician before prescribing medication in children.

Adult

0.5-2 mg/kg/d PO; taper as condition improves; single morning dose is safer for long-term use but divided doses have more anti-inflammatory effect

Pediatric

Administer as in adults

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; connective tissue infections; fungal or tubercular skin infections; GI tract disease

Pregnancy

B - Fetal risk not confirmed in studies in humans but has been shown in some studies in animals

Precautions

Abrupt discontinuation may cause adrenal crisis; hyperglycemia, edema, osteonecrosis, myopathy, peptic ulcer disease, hypokalemia, osteoporosis, euphoria, psychosis, myasthenia gravis, growth suppression, and infections may occur


Dapsone (Avlosulfon)

Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to sulfonamides in which competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth. Used alone or in conjunction with other anti-inflammatory medications for treating IgA pemphigus. Consult the pediatrician before prescribing medication in children.

Adult

75-100 mg/d PO
Alternatively, 50-300 mg PO qd

Pediatric

25-50 mg/d PO

May inhibit anti-inflammatory effects of clofazimine; hematologic reactions may increase with folic acid antagonists, eg, pyrimethamine (monitor for agranulocytosis during second and third months of therapy); probenecid increases dapsone toxicity; trimethoprim with dapsone may increase toxicity of both drugs; because of increased renal clearance, dapsone levels may significantly decrease when administered concurrently with rifampin

Documented hypersensitivity; G-6-PD deficiency

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

Perform weekly blood counts (first month), then perform WBC counts monthly (6 mo), then semiannually; discontinue if significant reduction in platelets, leukocytes, or hematopoiesis is seen; caution in methemoglobin reductase deficiency, G-6-PD deficiency, or hemoglobin M because of high risk for hemolysis and Heinz body formation; caution in patients exposed to other agents or conditions (eg, infection, diabetic ketosis) capable of producing hemolysis; peripheral neuropathy can occur (rare); phototoxicity may occur when exposed to UV light


Acitretin (Soriatane)

Retinoic acid analog. Mechanism of action is unknown. Used effectively in several reported cases of IgA pemphigus.

Adult

25-50 mg/d PO

Pediatric

Not established

Toxicity may occur with beta carotene coadministration; absorption increased with milk; increases toxicity of methotrexate (avoid concomitant use); interferes with effects of microdosed progestin minipill

Documented hypersensitivity; pregnancy; women of childbearing age

Pregnancy

X - Contraindicated; benefit does not outweigh risk

Precautions

Do not use in patients with severe obesity; women of childbearing age must be capable of complying with effective contraceptive measures; perform AST, ALT and LDH tests prior to initiation of therapy and at 1- to 2-wk intervals until stable and thereafter at intervals as clinically indicated; perform retinal ophthalmoscopic examination because acitretin-associated maculopathy has been reported

More on Pemphigus, IgA

Overview: Pemphigus, IgA
Differential Diagnoses & Workup: Pemphigus, IgA
Treatment & Medication: Pemphigus, IgA
Follow-up: Pemphigus, IgA
Multimedia: Pemphigus, IgA
References
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References

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Further Reading

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Keywords

IgA pemphigus, pemphigus IgA, pemphigus, intraepidermal neutrophilic IgA dermatosis, intercellular IgA vesiculopustular dermatosis, intercellular IgA dermatosis, intraepidermal IgA pustulosis, IgA pemphigus

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Contributor Information and Disclosures

Author

Lawrence Chan, MD, Department Head and Director of Skin Immunology Research, Professor, Departments of Dermatology and Microbiology/Immunology, University of Illinois College of Medicine
Lawrence Chan, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, Association of Professors of Dermatology, Chicago Dermatological Society, Dermatology Foundation, Illinois State Medical Society, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

Medical Editor

Takeji Nishikawa, MD, Emeritus Professor, Department of Dermatology, Keio University School of Medicine; Director, Samoncho Dermatology Clinic; Managing Director, The Waksman Foundation of Japan Inc
Disclosure: Nothing to disclose.

Pharmacy Editor

David F Butler, MD, Professor of Dermatology, Texas A&M University College of Medicine; Chair, Department of Dermatology, Director, Dermatology Residency Training Program, Scott and White Clinic
David F Butler, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, American Society for Dermatologic Surgery, American Society for MOHS Surgery, Association of Military Dermatologists, and Phi Beta Kappa
Disclosure: 3M Pharmaceutical Grant/research funds Other; Graceway Pharmaceuticals Grant/research funds Other

Managing Editor

Edward F Chan, MD, Clinical Assistant Professor, Department of Dermatology, University of Pennsylvania School of Medicine
Edward F Chan, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and Society for Investigative Dermatology
Disclosure: Nothing to disclose.

CME Editor

Catherine Quirk, MD, Clinical Assistant Professor, Department of Dermatology, Brown University
Catherine Quirk, MD is a member of the following medical societies: Alpha Omega Alpha and American Academy of Dermatology
Disclosure: Nothing to disclose.

Chief Editor

Dirk M Elston, MD, Director, Department of Dermatology, Geisinger Medical Center
Dirk M Elston, MD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

 
 
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