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Pemphigus erythematosus
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Pemphigus erythematosus

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Contributors: Sruthi Renati MD, Belinda Tan MD, PhD, Susan Burgin MD
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Synopsis

Pemphigus erythematosus, or Senear-Usher syndrome, is an autoimmune skin blistering disorder with an overlapping clinical presentation of pemphigus foliaceus and lupus erythematosus. It is generally regarded as a limited form of pemphigus foliaceus. Patients have cutaneous lesions consisting of flaccid blisters or superficial erosions that typically involve seborrheic areas such as the scalp, face, upper chest, and back. Patients also have crusting on the malar areas reminiscent of the butterfly rash seen in lupus erythematosus. Mucous membrane involvement is uncommon.

The age of onset and sex distribution are similar to that of pemphigus foliaceus (approximately 50 years, occurs equally in men and women). Like other forms of pemphigus, pemphigus erythematosus is very rare in children.

Pemphigus erythematosus may persist almost indefinitely as a localized disease. Despite its name, most patients with pemphigus erythematosus do not develop signs of systemic lupus erythematosus (SLE). Patients with pemphigus erythematosus who also meet the American College of Rheumatology criteria for systemic lupus are said to have both conditions simultaneously.

Both clinically and immunopathologically, pemphigus erythematosus combines features of pemphigus and lupus. Patients have intercellular autoantibodies and subcorneal acantholysis as seen in pemphigus foliaceus. Patients also frequently have positive antinuclear antibodies (ANA) and a positive lupus band on direct immunofluorescence.

Pemphigus erythematosus occurs with increased frequency in patients with a history of thymoma, myasthenia gravis, and other autoimmune diseases. Some of these patients also have autoantibodies directed against striated muscle. Certain HLA subtypes are represented more frequently in pemphigus erythematosus (HLA-A10, HLA-A26, HLA-DRW6).

Pemphigus erythematosus can be drug induced. The most notable medications associated with this condition are d-penicillamine and captopril. Both of these agents contain a highly negatively charged sulfhydryl group that has been implicated as the potential cause of acantholysis. There are also reports of drug-induced pemphigus erythematosus with ceftazidime, propranolol, pyritinol, and heroin.

Pemphigus erythematosus has been reported to occur in lesions of ultraviolet burns and thermal burns. More rarely, pemphigus erythematosus has been reported to occur in the setting of malignancy, most commonly bronchogenic carcinoma.

In a 2003 article describing an outbreak of endemic pemphigus in Northern Columbia, pemphigus erythematosus was the primary clinical phenotype. On examination, most of the patients showed keratotic follicular skin lesions resembling discoid lupus erythematosus, a high degree of photosensitivity, and a lupus band-like deposition of immunoglobulins and complement at the basement membrane zone.

Pemphigus erythematosus is less likely to rapidly progress than other forms of pemphigus and responds to similar immunosuppressant therapies.

Codes

ICD10CM:
L10.4 – Pemphigus erythematosus

SNOMEDCT:
36739006 – Pemphigus erythematosus

Look For

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Diagnostic Pearls

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Differential Diagnosis & Pitfalls

Best Tests

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Management Pearls

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Therapy

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Drug Reaction Data

Below is a list of drugs with literature evidence indicating an adverse association with this diagnosis. The list is continually updated through ongoing research and new medication approvals. Click on Citations to sort by number of citations or click on Medication to sort the medications alphabetically.

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References

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Last Reviewed: 05/22/2018
Last Updated: 10/09/2018
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Pemphigus erythematosus
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Pemphigus erythematosus : Cheek, Crust, Erythema, Nose, Photosensitivity, Skin erosions
Clinical image of Pemphigus erythematosus
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