eMedicine Specialties > Dermatology > Bullous Diseases

Pemphigus Erythematosus

Author: Rakesh Bharti, MD, MBBS, Consultant Dermatologist and HIV Specialist, BDC Research Centre, India
Coauthor(s): Rossitza Lazova, MD, Associate Professor of Dermatology and Pathology, Director of Dermatopathology Residency and Fellowship Program, Yale University School of Medicine; Consulting Pathologist/Dermatopathologist, Veterans Affairs Medical Center, West Haven, Connecticut
Contributor Information and Disclosures

Updated: Oct 20, 2008

Introduction

Background

The various types of pemphigus include pemphigoid, pemphigus vegetans, Hailey-Hailey disease, and pemphigus foliaceus.

Pemphigus erythematosus, also known as Senear-Usher syndrome, is an overlap syndrome with features of lupus erythematosus (LE) and pemphigus foliaceus. Pemphigus is demonstrated by acantholysis and immunoglobulin deposits in the interkeratinocyte substance. The lupus component is demonstrated by circulating antinuclear antibodies (ANA) and sometimes by immunoglobulin and complement deposits at the dermoepidermal junction. The disease has a better prognosis than pemphigus foliaceus, but it can be chronic.

For a thorough description and introduction to the possible causes of pemphigus, see the article " Pemphigus: An Acronym for a Disease with Multiple Causes ", published by the International Pemphigus Society.

Pathophysiology

Patients present with vesiculobullae or superficially eroded lesions, which may ooze and crust, particularly in sun-exposed areas, such as the face, the upper part of the chest, and the back. Pemphigus may be photoactivated. LE is the classic autoimmune disease that demonstrates photosensitivity. It appears that a genetic predisposition to autoimmunity combines with a sensitivity to ultraviolet light leading to an overlap of these 2 diseases in rare cases. Now these are called immunobullous disease.

Frequency

United States

The incidence in the United States is estimated to be similar to that internationally.

International

The incidence of pemphigus is 0.5-3.2 cases per 100,000 population per year. Patients with pemphigus erythematosus comprise only a small subgroup of those with pemphigus. Kumar from India, in a 2008 article, reported a high prevalence (4.4 cases per million population), with a high preponderance (61.5%) in females.1

Mortality/Morbidity

With timely diagnosis and treatment, the disease typically has a good prognosis. Some patients may ultimately develop symptoms classified as criteria for systemic lupus erythematosus (SLE) by the American Rheumatism Association (ARA).

Race

Pemphigus erythematosus, like other variants of pemphigus erythematosus and LE, may be increased in patients who express specific human leukocyte antigen (HLA) haplotypes. Those identified to have pemphigus erythematosus are positive for human leukocyte antigen A10 (HLA-A10) or human leukocyte antigen A26 (HLA-A26) and human leukocyte antigen DRW6 (HLA-DRW6).

Sex

Reports generally find no difference in occurrence between the 2 sexes, although some studies from India suggest a male preponderance.

Age

Pemphigus erythematosus may occur at any age, but it is unusual in children.

Clinical

History

  • Onset and progression are typically slow.
  • Although the distribution of the lesions should suggest induction by sunlight, the patient may be completely unaware of the photosensitive nature of the disorder.

Physical

  • Lesions typically involve the scalp, the face, the upper part of the chest, and the back.
  • Patients with pemphigus erythematosus classically present with small, flaccid bullae with scaling and crusting. Occasionally, the appearance may suggest a papulosquamous disorder.
  • Secondary infection may occur, resulting in impetiginization, in healing with pigment changes, and in scarring.
  • On the face, pemphigus erythematosus presents on the bridge of the nose and on the malar areas as in the butterfly distribution seen in LE.
  • With extensive involvement, patients may present with an exfoliative erythroderma.
  • The skin may be tender.
  • Patients with pemphigus erythematosus do not typically develop mucous membrane involvement, which can be seen in some other variants of pemphigus.
  • Electrolyte imbalance and loss of temperature control can occur with extensive skin involvement.

Causes

  • Patients with pemphigus develop an autoimmune response directed against desmosomes. In patients with pemphigus foliaceus and its variant, pemphigus erythematosus, the target antigen is desmoglein 1. Desmogleins are desmosomal proteins important in keratinocyte adhesion. The binding of autoantibodies is postulated to result in a cascade of biochemical intracellular events that eventuates in the loss of desmosome function.
  • Certain HLA haplotypes (A10 or A26, DRW6) are thought to be associated, suggesting a genetic predisposition.

More on Pemphigus Erythematosus

Overview: Pemphigus Erythematosus
Differential Diagnoses & Workup: Pemphigus Erythematosus
Treatment & Medication: Pemphigus Erythematosus
Follow-up: Pemphigus Erythematosus
References
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References

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Further Reading

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Keywords

Senear-Usher syndrome, pemphigus seborrheic, lupus erythematosus, pemphigus foliaceus

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Contributor Information and Disclosures

Author

Rakesh Bharti, MD, MBBS, Consultant Dermatologist and HIV Specialist, BDC Research Centre, India
Rakesh Bharti, MD, MBBS is a member of the following medical societies: International AIDS Society
Disclosure: Nothing to disclose.

Coauthor(s)

Rossitza Lazova, MD, Associate Professor of Dermatology and Pathology, Director of Dermatopathology Residency and Fellowship Program, Yale University School of Medicine; Consulting Pathologist/Dermatopathologist, Veterans Affairs Medical Center, West Haven, Connecticut
Rossitza Lazova, MD is a member of the following medical societies: American Academy of Dermatology, American Society of Dermatopathology, and International Society of Dermatopathology
Disclosure: Nothing to disclose.

Medical Editor

Ponciano D Cruz Jr, MD, Vice-Chair, JB Shelmire Professor, Department of Dermatology, University of Texas Southwestern Medical Center
Ponciano D Cruz Jr, MD is a member of the following medical societies: Texas Medical Association
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

Julia R Nunley, MD, Professor, Program Director, Dermatology Residency, Department of Dermatology, Virginia Commonwealth University Medical Center
Julia R Nunley, MD is a member of the following medical societies: American Academy of Dermatology, American College of Physicians, American Society of Nephrology, International Society of Nephrology, Medical Dermatology Society, Medical Society of Virginia, National Kidney Foundation, Phi Beta Kappa, and Women's Dermatologic Society
Disclosure: Johnson and Johnson stock holder dividends; Amgen stock holder dividends; Forest Lab, Inc stock holder dividends; Galaxo Smith Klein stock holder dividends; Covidien stock holder dividends; Novartis Grant/research funds Consulting; Biolex  sub-investigator

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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