Dermatitis herpetiformis in Child
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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.
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.
Codes
ICD10CM:
L13.0 – Dermatitis herpetiformis
SNOMEDCT:
111196000 – Dermatitis herpetiformis
L13.0 – Dermatitis herpetiformis
SNOMEDCT:
111196000 – Dermatitis herpetiformis
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Differential Diagnosis & Pitfalls
- Linear IgA bullous dermatosis of childhood
- Neurotic excoriations
- Scabies
- Papular urticaria
- Insect bite reactions
- Atopic dermatitis (eczematous dermatitis)
- Herpes simplex virus
- Epidermolysis bullosa simplex
- Chronic prurigo
- Polymorphous light eruption
- Pityriasis lichenoides et varioliformis acuta
- Prurigo pigmentosa
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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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Last Reviewed:02/05/2020
Last Updated:01/12/2022
Last Updated:01/12/2022