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Pemphigus foliaceus in Adult
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Pemphigus foliaceus in Adult

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Contributors: Tyler Werbel, Susan Burgin MD
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Synopsis

Pemphigus foliaceus is an autoimmune skin disorder characterized by acantholysis of the epidermis (dissolution of the bridges between epidermal cells) resulting in blister formation. The autoantibody in question is an immunoglobulin G (IgG) directed against a cell adhesion molecule, desmoglein 1 (although an IgA form has been reported). Pemphigus foliaceus is associated with human leukocyte antigen (HLA) class II alleles DR1, DR4, and DR14. It occurs in all races and equally in both sexes.

Pemphigus foliaceus may be seen in any age group but tends to be a disease of older adults. An exception is the endemic subtype of pemphigus foliaceus that often affects children and young adults in Brazil, other parts of Latin America, and in Tunisia (fogo selvagem). Recent sun exposure or ionizing radiation can exacerbate the condition. It is generally a more benign form of pemphigus, and most patients do not fall severely ill. However, in rare cases, lesions can progress to exfoliative erythroderma, potentially causing metabolic derangements. Oral lesions are rare, but skin lesions can persist for a period of years.

There are several subtypes of pemphigus foliaceus, including fogo selvagem and pemphigus erythematosus. Fogo selvagem is thought to occur in response to an environmental trigger. Pemphigus erythematosus is considered a less severe form of the disease that typically only affects the malar region. Pemphigus erythematosus may coexist with other autoimmune disorders, such as myasthenia gravis or lupus erythematosus. It has also been reported in association with medications such as penicillamine. See Drug Reaction Data below for more information.

Codes

ICD10CM:
L10.2 – Pemphigus foliaceous

SNOMEDCT:
35154004 – Pemphigus foliaceus

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: 07/09/2018
Last Updated: 10/09/2018
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Pemphigus foliaceus in Adult
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Pemphigus foliaceus : Bullae, Crust, Desquamation, Erythema, Face, Scalp, Skin erosion, Trunk
Clinical image of Pemphigus foliaceus
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