Dermatitis Herpetiformis

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Monitor for potentially fatal dapsone-induced sulfone syndrome: fever, jaundice and hepatic necrosis, exfoliative dermatitis, lymphadenopathy, methemoglobinemia, and hemolytic anemia.
Can occur 48 hours or 6 months after treatment, most often 5 weeks after initiation

Pediatric Considerations

<2 years: Dosing is not established.
>2 years: 0.5 to 1.0 mg/kg/day

Pregnancy Considerations

Category C: Safety during pregnancy is not established.
Secreted in breast milk and will produce hemolytic anemia in infants
Adherence to a strict GFD 6 to 12 months before conception should be considered with the hope of eliminating need for dapsone during pregnancy.

Second Line

High-potency topical steroids can be used acutely to control symptoms until dapsone becomes effective (1)[C].
Sulfapyridine (1 to 2 g/day) is FDA approved for use in DH and is thought to be the active metabolite in sulfasalazine (2 to 4 g/day) (2,5)[B]. Common side effects include nausea, vomiting, and anorexia. Enteric-coated form may reduce side effects. Other side effects include agranulocytosis, hypersensitivity reactions, hemolytic anemia, proteinuria, and crystalluria (2,5).

ISSUES FOR REFERRAL

Over time, interdisciplinary treatment may involve a dermatologist, gastroenterologist, and registered dietician. Genetic counseling and testing should be considered on diagnosis (1,2).

ADDITIONAL THERAPIES

A single case report describes topical dapsone therapy as potential alternative treatment or as an adjunct to oral dapsone to decrease systemic exposure and risk of severe side effects. However, has not been studied extensively (6)[C].

ONGOING CARE

FOLLOW-UP RECOMMENDATIONS

Patient Monitoring

Every 6 to 12 months by physician and dietician to evaluate GFD adherence and recurrence of symptoms
Adherence to GFD can be monitored with serologic levels of anti-tTG, anti eTG, and EMA levels (1).
Patients on dapsone require lab monitoring weekly for the 1st month, biweekly for 2 months, then every 3 months for the duration of medication use (1,4).

DIET

GFD

Grains that should be avoided: wheat (includes spelt, kamut, semolina, and triticale), rye, and barley (including malt)
Safe grains (gluten-free): rice, amaranth, buckwheat, corn, millet, quinoa, sorghum, teff (an Ethiopian cereal grain), and oats
Care should be taken to avoid gluten-free grains that are contaminated with sources of gluten during processing such as oats.
Sources of gluten-free starches that can be used as flour alternatives
- Cereal grains: amaranth, buckwheat, corn, millet, quinoa, sorghum, teff, rice (white, brown, wild, basmati, jasmine), and montina
- Tubers: arrowroot, jicama, taro, potato, and tapioca
- Legumes: chickpeas, lentils, kidney beans, navy beans, pea beans, peanuts, and soybeans
- Nuts: almonds, walnuts, chestnuts, hazelnuts, and cashews
- Seeds: sunflower, flax, and pumpkin

PATIENT EDUCATION

Patients on dapsone should be made aware of potential hemolytic anemia and the signs associated with methemoglobinemia.
American Academy of Dermatology, 930 N. Meacham Road, P.O. Box 4014, Schaumberg, IL 60168-4014; (708) 330-0230
The University of Chicago Celiac Disease Center, 5841 S. Maryland Ave., Mail Code 4069, Chicago, IL 60637; (773) 702-7593; www.celiacdisease.net or http://www.cureceliacdisease.org/
Gluten Intolerance Group of North America, 31214-124 Ave. SE, Auburn, WA 98092; (206) 246-6652; fax (206) 246-6531; https://www.gluten.org/
The Celiac Disease Foundation, 13251 Ventura Blvd., #1, Studio City, CA 9160; (818) 990-2354; fax (818) 990-2379

PROGNOSIS

Lifelong disease with favorable prognosis
10- to 15-year survival rates do not seem to differ from general population.
Remission in 10-15%
Skin disease responds readily to dapsone. Occasional new lesions (2 to 3 per week) are to be expected and are not an indication for altering daily dosage.
Strict adherence to a GFD improves clinical symptoms and decreases dapsone requirement. GFD is the only sustainable method of eliminating cutaneous and GI disease.
Risk of lymphoma may be decreased in those who maintain a GFD.

COMPLICATIONS

Majority of complications are associated with GSE.
Malnutrition, weight loss, nutritional deficiencies (folate, B12, iron)
Abdominal pain, dyspepsia
Osteoporosis, dental abnormalities
Autoimmune diseases
Lymphomas

REFERENCES

11 Antiga E, Caproni M. The diagnosis and treatment of dermatitis herpetiformis. Clin Cosmet Investig Dermatol. 2015;8:257-265.22 Bolotin D, Petronic-Rosic V. Dermatitis herpetiformis. Part II. Diagnosis, management, and prognosis. J Am Acad Dermatol. 2011;64(6):1027-1033.33 Junkins-Hopkins JM. Dermatitis herpetiformis: pearls and pitfalls in diagnosis and management. J Am Acad Dermatol. 2010;63(3):526-528.44 Wozel G, Blasum C. Dapsone in dermatology and beyond. Arch Dermatol Res. 2014;306(2):103-124.55 Willsteed E, Lee M, Wong LC, et al. Sulfasalazine and dermatitis herpetiformis. Australas J Dermatol. 2005;46(2):101-103.66 Handler MZ, Chacon AH, Shiman MI, et al. Letter to the editor: application of dapsone 5% gel in a patient with dermatitis herpetiformis. J Dermatol Case Rep. 2012;6(4):132-133.

ADDITIONAL READING

Bolotin D, Petronic-Rosic V. Dermatitis herpetiformis. Part I. Epidemiology, pathogenesis, and clinical presentation. J Am Acad Dermatol. 2011;64(6):1017-1024.
Cardones AR, Hall RPIII. Management of dermatitis herpetiformis. Immunol Allergy Clin North Am. 2012;32(2):275-281.
Cardones AR, Hall RPIII. Pathophysiology of dermatitis herpetiformis: a model for cutaneous manifestations of gastrointestinal inflammation. Immunol Allergy Clin North Am. 2012;32(2):263-274.
K ¡rp ¡ti S. An exception within the group of autoimmune blistering diseases: dermatitis herpetiformis, the gluten-sensitive dermopathy. Immunol Allergy Clin North Am. 2012;32(2):255-262.
Paek SY, Steinberg SM, Katz SI. Remission in dermatitis herpetiformis: a cohort study. Arch Dermatol. 2011;147(3):301-305.

CODES

ICD10

L13.0 Dermatitis herpetiformis

ICD9

694.0 Dermatitis herpetiformis

SNOMED

111196000 Dermatitis herpetiformis (disorder)

CLINICAL PEARLS

DH is a chronic, relapsing, intensely pruritic rash that often presents with erosions, excoriations, lichenification, and pigmentary changes secondary to scratching and healing of old papulovesicular lesions.
Strong association with gluten-sensitive enteropathy
Diagnosis established with perilesional skin biopsy showing direct immunofluorescence demonstrating granular IgA deposits in the dermal papillae.
Serologic levels of IgA transglutaminase aid in diagnosis and monitoring of deviations from GFD.
Mainstay of treatment is a GFD with dapsone used primarily for short-term symptom relief.

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