ContentsSynopsisCodesLook ForDiagnostic PearlsDifferential Diagnosis & PitfallsBest TestsManagement PearlsTherapyDrug Reaction DataReferencesView all Images (17)
Dermatitis herpetiformis in Child
Other Resources UpToDate PubMed

Dermatitis herpetiformis in Child

Contributors: Erin X. Wei MD, Lauren Strazzula MD, Belinda Tan MD, PhD, Susan Burgin MD
Other Resources UpToDate PubMed

Synopsis

Dermatitis herpetiformis (DH) is a chronic, pruritic autoimmune blistering disorder associated with gluten-sensitive enteropathy. DH can rarely present in childhood, with slight female predilection, in contrast to adult DH, which shows male predilection. The pathogenesis involves the deposition of IgA immune complexes in the papillary dermis. The associated autoantigen is an epidermal transglutaminase (transglutaminase 3, or TG3). There is a genetic predisposition to the disease, as it is associated with certain human leukocyte antigen (HLA) haplotypes, HLA class II DQ2.

The cutaneous lesions seen in childhood are similar to those in adults, with symmetric papulovesicular eruption on the extensor surfaces and buttocks that is extremely pruritic and may wax and wane in severity. Facial involvement has been reported. Pruritus and nonpruritic, noninflammatory papules and pruritic purpuric macules / petechiae on the palms can be a rare presentation with or without the typical papulovesicular eruption.

About 80%-95% of children with DH have gluten-sensitive enteropathy on small intestine biopsy. Most are asymptomatic and undiagnosed at presentation of DH; only 10% of children carry a previous diagnosis of celiac disease.

There is an increased incidence of autoimmune diseases in patients with DH. The most common associated autoimmune conditions include thyroid dysfunction, particularly Hashimoto thyroiditis, type 1 diabetes mellitus, and pernicious anemia. Patients with DH are at an increased risk of developing enteropathy-associated T-cell lymphoma. However, a gluten-free diet may be protective against cancer development.

For more information, see OMIM.

Codes

ICD10CM:
L13.0 – Dermatitis herpetiformis

SNOMEDCT:
111196000 – Dermatitis herpetiformis

Look For

Subscription Required

Diagnostic Pearls

Subscription Required

Differential Diagnosis & Pitfalls

Best Tests

Subscription Required

Management Pearls

Subscription Required

Therapy

Subscription Required

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.

Subscription Required

References

Subscription Required

Last Reviewed:02/06/2020
Last Updated:03/06/2020
Copyright © 2021 VisualDx®. All rights reserved.
Dermatitis herpetiformis in Child
Dermatitis herpetiformis : Buttocks, Elbow, Excoriation, Extensor distribution, Knee, Skin erosion, Vesicle, Pruritus
Clinical image of Dermatitis herpetiformis
A close-up of excoriated papules / vesicles near the knee.
Copyright © 2021 VisualDx®. All rights reserved.