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Dermatitis herpetiformis in Child
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Dermatitis herpetiformis in Child

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Contributors: Lauren Strazzula MD, Susan Burgin MD, Belinda Tan MD, PhD, Craig N. Burkhart MD, Dean Morrell MD, Nancy Esterly MD
Other Resources UpToDate PubMed

Synopsis

Dermatitis herpetiformis (DH) is a chronic pruritic autoimmune blistering disorder associated with gluten-sensitive enteropathy. Although more common in adults, DH can present in childhood, and it is more commonly seen in girls than boys. The pathogenesis involves the deposition of IgA in the papillary dermis, and the associated autoantigen is a gluten-transglutaminase complex.

The cutaneous lesions seen in childhood are similar to those in adults. The disease manifests as a symmetric papulovesicular eruption on the extensor surfaces and buttocks that is extremely pruritic and that may wax and wane in severity. Facial lesions have been reported. Rare presentations include nonpruritic inflammatory papules and pruritic purpuric macules and papules on the palms.

In children with DH, 80%-95% will have an associated gluten-sensitive enteropathy on small intestine biopsy. Interestingly, the enteropathy is typically asymptomatic upon presentation. Only 10% of children carry a previous diagnosis of celiac sprue.

There is an increased incidence of autoimmune diseases in patients with DH. The most common associated autoimmune conditions include Hashimoto thyroiditis, type I diabetes mellitus, and pernicious anemia. Patients with both celiac sprue and DH are at an increased risk of developing enteropathy-associated T-cell lymphoma. However, a gluten-free diet is protective against cancer development.

For more information, see OMIM.

Codes

ICD10CM:
L13.0 – Dermatitis herpetiformis

SNOMEDCT:
111196000 – Dermatitis herpetiformis

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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 Updated: 03/27/2019
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Dermatitis herpetiformis in Child
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Dermatitis herpetiformis : Buttocks, Elbow, Extensor distribution, Knee, Skin erosion, Vesicle, Pruritus
Clinical image of Dermatitis herpetiformis
A close-up of excoriated papules / vesicles near the knee.
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