From the Guidelines
Dermatitis herpetiformis in pediatric patients should be diagnosed and treated with a strict gluten-free diet and symptomatic relief using dapsone, prioritizing the reduction of morbidity, mortality, and improvement of quality of life. The diagnosis of dermatitis herpetiformis is based on the presence of granular IgA deposits in the dermal papillae of uninvolved perilesional skin as shown by direct immunofluorescence 1. The primary treatment is a strict gluten-free diet, which should be implemented immediately upon diagnosis and maintained lifelong, as it can lead to significant improvement within weeks to months and reduce the risk of lymphomas 1.
For symptomatic relief, dapsone is the first-line medication, typically started at 0.5-1 mg/kg/day and adjusted based on response, with most children requiring 25-100 mg daily 1. Before starting dapsone, baseline laboratory tests including complete blood count, liver function, and G6PD levels are essential as the medication can cause hemolytic anemia. Regular monitoring every 1-2 weeks initially, then monthly, and eventually every 3-6 months is necessary to check for side effects.
Key considerations in the management of dermatitis herpetiformis in pediatric patients include:
- Implementing a strict gluten-free diet immediately upon diagnosis and maintaining it lifelong
- Using dapsone for symptomatic relief, with careful monitoring for side effects
- Evaluating for other autoimmune conditions and nutritional deficiencies associated with celiac disease
- Providing adequate education, motivation, and support to achieve and maintain a gluten-free diet, including consultation with an experienced dietician and referral to a support group 1.
The condition typically presents with intensely pruritic papules, vesicles, and excoriations symmetrically distributed on extensor surfaces like elbows, knees, buttocks, and scalp. With proper treatment and dietary compliance, most children can achieve good control of their symptoms and reduce the risk of long-term complications.
From the FDA Drug Label
Dermatitis herpetiformis: The dosage should be individually titrated starting in adults with 50 mg daily and correspondingly smaller doses in children. If full control is not achieved within the range of 50 - 300 mg daily, higher doses may be tried. Dosage should be reduced to a minimum maintenance level as soon as possible In responsive patients there is a prompt reduction in pruritus followed by clearance of skin lesions. There is no effect on the gastrointestinal component of the disease. A strict gluten free diet is an option for the patient to elect, permitting many to reduce or eliminate the need for Dapsone; the average time for dosage reduction is 8 months with a range of 4 months to 2 1/2 years and for dosage elimination 29 months with a range of 6 months to 9 years
The diagnosis of dermatitis herpetiformis (DH) is not explicitly stated in the provided drug labels. The treatment of DH in pediatric patients involves:
- Dapsone therapy, with individually titrated doses starting with correspondingly smaller doses than the adult dose of 50 mg daily 2
- A strict gluten-free diet as an option to reduce or eliminate the need for Dapsone 2
From the Research
Diagnosis of Dermatitis Herpetiformis (DH) in Pediatric Patients
- The diagnosis of DH is established clinically, histologically, immunopathologically, and serologically 3.
- DH is characterized by subepidermal bullae on hematoxylin and eosin staining and granular immunoglobulin A deposits in the dermal papillae using the direct immunofluorescence method 4.
- Antibodies to tissue transglutaminase and epidermal transglutaminase can be measured serologically, although biopsy is still required for definitive diagnosis of DH 4.
- The finding of granular deposits of IgA along the dermal-epidermal junction is pathognomonic of DH 5.
Treatment of Dermatitis Herpetiformis (DH) in Pediatric Patients
- A gluten-free diet (GFD) is the treatment of choice for patients with DH 6, 4, 3, 5, 7.
- Dapsone and/or other drugs should be used during the period until the GFD is effective 3.
- Combined therapy with GFD and dapsone is an initial treatment of choice to control the cutaneous manifestations of DH 4.
- The main treatment of DH today is a strict, life-long gluten-free diet 7.
- Untreated DH patients should be regularly monitored for malabsorption and lymphomas 7.