eMedicine Specialties > Dermatology > Reactive & Inflammatory Dermatoses

Erythema Dyschromicum Perstans: Treatment & Medication

Author: Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Coauthor(s): Santiago A Centurion, MD, Staff Physician, Department of Dermatology, New Jersey Medical School, University of Medicine and Dentistry of New Jersey
Contributor Information and Disclosures

Updated: Aug 31, 2009

Treatment

Medical Care

Many therapeutic options are available for erythema dyschromicum perstans (EDP), but few have been effective, except for clofazimine. In one series of 8 patients, 7 had a good or excellent response to clofazimine administered either 100 mg every other day to patients weighing less than 40 kg or 100 mg every day to patients weighing more than 40 kg. This medication was continued for 3 months, then reduced to 200 mg/wk and 400 mg/wk, respectively. The one remaining patient had only a marginal response. One study found some improvement in early cases, but no cures were reported. This medication seems to have a valuable effect on the inflammatory phase of erythema dyschromicum perstans.

Clofazimine is a lipophilic rhimophenazine dye with both antimicrobial and anti-inflammatory properties originally developed to treat tuberculosis. Although its mechanism of action is unclear, it seems to exert its main effect upon neutrophils and monocytes in a variety of ways, such as stimulating phagocytosis and release of lysosomal enzymes.18

Clofazimine is administered orally, with improved bioavailability when taken with food. It concentrates in lipid-rich tissues, including the reticuloendothelial system, the intestine, the breasts, and the liver. Its half-life is 70 days. Isoniazid increases its serum levels and enhances its urinary excretion. Its most common adverse effects are in the skin, the gut, and the eye. It gives a temporary orange discoloration of the skin and the eye (ie, cornea, conjunctivae); it also may produce ichthyosis. Its most serious adverse effect is crystal deposition in the gut that produces a potentially fatal enteropathy. This rare complication is associated with months of high-dose (>100 mg/d) therapy. Nausea and diarrhea are more common. Splenic infarction and eosinophilic enteritis are also rare adverse effects.

Many other therapeutic modalities have been attempted, none with satisfactory results. These include ultraviolet exposure, ultraviolet avoidance, antibiotics, antihistamines, griseofulvin, chemical peels, antibiotics, corticosteroids, vitamins, isoniazid, chloroquine, and psychotherapy.

A patient from Turkey was described to have responded remarkably well to treatment with dapsone.19

Medication

The goals of pharmacotherapy are to reduce morbidity and to prevent complications.

Leprostatics

Have both antimicrobial properties and anti-inflammatory properties. They were originally developed to treat tuberculosis.


Clofazimine (Lamprene)

Lipophilic rhimophenazine dye with antimicrobial and anti-inflammatory properties originally developed to treat tuberculosis. Inhibits mycobacterial growth, binds preferentially to mycobacterial DNA. Has antimicrobial properties, but mechanism of action unknown.
Although its mechanism of action is unclear, it seems to exert its main effect upon neutrophils and monocytes in a variety of ways (eg, stimulating phagocytosis and release of lysosomal enzymes).

Adult

<40 kg: 100 mg PO qod; continue for 3 mo then reduce to 200 mg/wk
>40 kg: 100 mg PO qd; continue for 3 mo then reduce to 400 mg/wk

Pediatric

Not established

Dapsone may inhibit anti-inflammatory activity; avoid concurrent administration of clofazimine with aluminum-magnesium-containing antacids due to decreased absorption; concurrent use of phenytoin and clofazimine may result in reduced phenytoin efficacy; concurrent administration of small quantities of orange juice with clofazimine may result in modest reduction of clofazimine relative bioavailability

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

Most noticeable adverse effect is skin discoloration (red to purple-black), which fades slowly on withdrawal; secretions discolored; urine becomes red; ichthyosis of shins and forearms may be prominent
Adverse effects include liver disease, stomach or intestinal disease, and skin problems (instruct patients to inform health care provider as soon as possible if symptoms develop); bleeding in the digestive system (symptoms may include blood in stools, black tarry stools, vomiting blood, and vomit that looks like coffee grounds); intestinal obstruction (symptoms may include abdominal pain and cramping, vomiting blood, abdominal distention, and diarrhea); liver damage (symptoms may include abdominal pain for more than a few days, dark urine, fatigue, general discomfort, uneasiness or ill feeling [malaise], loss of appetite, pale or clay-colored stools, and yellow eyes or skin); doses >100 mg/d should only be given for as short a period as possible and only under close medical supervision
Severe abdominal symptoms with rare reports of splenic infarction, bowel obstruction, and GI bleeding; autopsy reveals crystalline deposits of clofazimine in tissues, including intestinal mucosa, spleen, liver, and mesenteric lymph nodes


Dapsone (Avlosulfon)

Bactericidal and bacteriostatic against mycobacteria; mechanism of action is similar to that of sulfonamides, in which competitive antagonists of PABA prevent formation of folic acid, inhibiting bacterial growth

Adult

50-300 mg PO qd

Pediatric

Not established

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 toxicity; trimethoprim with dapsone may increase toxicity of both drugs; because of increased renal clearance, levels may significantly decrease when administered concurrently with rifampin

Documented hypersensitivity; known 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

Associated with a variety of systemic toxicities, including agranulocytosis, anemia, methemoglobinemia, hepatitis, and neuropathy; patients may experience headache and/or GI distress on initiation of therapy; perform weekly blood counts (first mo), then monthly WBC counts (6 mo), then semiannual WBC counts; discontinue if a significant reduction in platelets, leukocytes, or hematopoiesis occurs; 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; pancreatitis may occur; various forms of renal complications including acute renal failure, acute tubular necrosis, and oliguria have occurred with dapsone use

More on Erythema Dyschromicum Perstans

Overview: Erythema Dyschromicum Perstans
Differential Diagnoses & Workup: Erythema Dyschromicum Perstans
Treatment & Medication: Erythema Dyschromicum Perstans
Follow-up: Erythema Dyschromicum Perstans
Multimedia: Erythema Dyschromicum Perstans
References
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References

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Further Reading

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Keywords

erythema dyschromicum perstans, EDP, ashy dermatosis, dermatosis cenicienta, erythema chronicum figuratum melanodermicum, lichen planus

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Contributor Information and Disclosures

Author

Robert A Schwartz, MD, MPH, Professor and Head, Dermatology, Professor of Pathology, Pediatrics, Medicine, and Preventive Medicine and Community Health, UMDNJ-New Jersey Medical School
Robert A Schwartz, MD, MPH is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and Sigma Xi
Disclosure: Nothing to disclose.

Coauthor(s)

Santiago A Centurion, MD, Staff Physician, Department of Dermatology, New Jersey Medical School, University of Medicine and Dentistry of New Jersey
Santiago A Centurion, MD is a member of the following medical societies: American Academy of Dermatology, American Medical Association, and Sigma Xi
Disclosure: Nothing to disclose.

Medical Editor

Shyam Verma, MBBS, DVD, FAAD, Adjunct Clinical Assistant Professor, Department of Dermatology, University of Virginia, State University of New York at Stonybrook, Penn State University
Shyam Verma, MBBS, DVD, FAAD is a member of the following medical societies: American Academy of Dermatology
Disclosure: Nothing to disclose.

Pharmacy Editor

Michael J Wells, MD, Associate Professor, Department of Dermatology, Texas Tech University Health Sciences Center
Michael J Wells, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American Medical Association, and Texas Medical Association
Disclosure: Nothing to disclose.

Managing Editor

Jeffrey P Callen, MD, Professor of Medicine, Chief, Division of Dermatology, University of Louisville School of Medicine
Jeffrey P Callen, MD is a member of the following medical societies: Alpha Omega Alpha, American Academy of Dermatology, American College of Physicians, and American College of Rheumatology
Disclosure: Amgen Honoraria Consulting; Abbott Honoraria Consulting; Electrical Optical Sciences Honoraria Consulting; Centocor Honoraria Consulting; Medicis Honoraria Consulting; Celgene Honoraria Consulting

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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