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Pemphigus foliaceus in Child
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Pemphigus foliaceus in Child

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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 tends to affect children and young adults in endemic regions of the world. Most patients affected by this condition work outdoors, commonly as farmers. They also tend to live in areas with poor housing conditions and have poor hygiene. Fogo selvagem is thought to occur in response to an environmental trigger. Specifically, it has been suggested that fogo selvagem may be associated with some insects, such as the Similium black fly, on the basis that the greatest number of cases occur at the end of the rainy season when insect multiplication is greatest. Despite its unique epidemiological profile, fogo selvagem shares the same clinical features as the non-endemic form.

Pemphigus erythematosus is considered a less severe form of the disease that typically only affects the malar region. Like pemphigus foliaceus, pemphigus erythematosus typically affects older adults, but several cases in children have been observed. Pemphigus erythematosus may coexist with other autoimmune disorders, such as myasthenia gravis or lupus erythematosus.

Codes

ICD10CM:
L10.2 – Pemphigus foliaceous

SNOMEDCT:
35154004 – Pemphigus foliaceus

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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: 05/07/2019
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Pemphigus foliaceus in Child
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Pemphigus foliaceus : Bullae, Crust, Desquamation, Erythema, Face, Scalp, Skin erosion, Trunk
Clinical image of Pemphigus foliaceus
A close-up of superficial erosions and crusts and some postinflammatory hyperpigmentation.
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