Treatment algorithm

Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups: see disclaimer

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all patients

1st line – gluten-free diet

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ONGOING
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1st line – 

gluten-free diet

Advise all patients with confirmed dermatitis herpetiformis to follow a strict and lifelong gluten-free diet.[2][11][20][21]

  • Dermatitis herpetiformis is the specific cutaneous manifestation of celiac disease and gluten is the main trigger for both conditions; a lifelong gluten-free diet is necessary to switch off the autoimmune process causing the disease and may help to prevent potential complications, such as lymphoma.[2][43][44]

Around 15% to 20% of people with dermatitis herpetiformis show gastrointestinal symptoms resembling those of celiac disease.[2][11]​ A gluten-free diet alone is usually able to induce the resolution of intestinal signs and symptoms in people with dermatitis herpetiformis (and those with celiac disease) within 3-6 months.[21][50]

  • If intestinal symptoms persist following 6 months of gluten-free diet or recur despite adherence to diet, referral for exclusion of other gastrointestinal conditions should be considered.

By contrast, its effects on resolving cutaneous lesions and pruritus is slower, in some instances taking several years; an average of 1-2 years on a gluten-free diet may be required for complete resolution of skin lesions.[21][51]

  • Deposited immunoglobulin A (IgA) autoantibodies may be detected in the dermis for up to 10 years after starting a strict gluten-free diet.[2]

  • A study of 237 patients with dermatitis herpetiformis showed that 38% of patients continued to have cutaneous symptoms after 2 years of a gluten-free diet, while 14% had skin symptoms after longer term (mean duration 24 years) gluten-free diet.[52]

Adherence to gluten-free diet is challenging, with one study reporting dietary lapses in approximately 25% of long-term treated patients with dermatitis herpetiformis.[53] In addition, gluten contamination can be present in food that is intended to be gluten free; one meta-analysis of gluten contamination in naturally gluten-free foods, gluten-free labeled foods, and meals prepared in food services found the overall prevalence of gluten contamination in these foods to be over 15%.[54]

For these reasons, other therapeutic strategies are often required in people with dermatitis herpetiformis.

Plus – dietary counseling

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ONGOING
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all patients
Plus – 

dietary counseling

Treatment recommended for ALL patients in selected patient group

Refer all patients starting a gluten-free diet to a dietitian with training in gluten-free diets.[2][20][45] Dietary counseling is important because a gluten-free diet has been associated with:

  • Potential vitamin B deficiency[46][47]

  • Reduced fiber intake

  • Risk of rebound weight gain.[48]

Advise all patients on a gluten-free diet to follow a healthy and well balanced diet.[2]

  • Micronutrient and vitamin supplementation may also be a consideration on a case-by-case basis.[2]

Some experts recommend avoidance of excessive iodine intake (i.e., via excessive seafood consumption/iodine supplementation), as this may increase risk of relapse in patients with dermatitis herpetiformis.[2]

  • Although an inconsistent manifestation, potassium iodide (ingested or applied topically) may induce a flare-up of symptoms in some patients with dermatitis herpetiformis.[49] When applied topically, potassium iodide may cause lesions similar to dermatitis herpetiformis (note these may also occur with trauma and other chemical irritants, due to a mechanism known as the Koebner phenomenon).

Consider – dapsone

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all patients
Consider – 

dapsone

Treatment recommended for SOME patients in selected patient group

Consider starting dapsone in addition to a lifelong gluten-free diet in patients who:[2][11][20][21]

  • Have intolerable or severe skin involvement at initial presentation, or

  • Continue to have skin signs and symptoms despite adhering to a strict gluten-free diet.

Dapsone is also an alternative treatment option in patients who cannot adhere to/cannot tolerate a gluten-free diet.[2]

Despite the lack of randomized clinical trials on its efficacy, dapsone is considered to be an essential treatment option in these patients as per current guidelines and general expert consensus, with treatment often resulting in the clearance of skin lesions and reduction in pruritus within a few days of treatment.[2][11][21][44][55] Dapsone has no effect on gastrointestinal signs and symptoms.

In patients starting dapsone, continue treatment until cutaneous signs and symptoms have completely resolved.[2]​ Monitor the patient closely for adverse effects related to dapsone therapy.

  • Dapsone may cause hemolytic anemia. Measure serum glucose-6-phosphate-dehydrogenase (G6PD) levels before treatment as severe hemolytic anemia is possible in patients with G6PD deficiency and dapsone should be avoided or used with caution in these patients.

  • All patients should also have complete blood count (CBC) with differential, liver function tests, renal function tests, urinalysis, and reticulocyte count performed at baseline and then periodically during treatment to monitor for dapsone-related adverse effects.[2][20][21]​ CBC with differential should be checked weekly for the first month, monthly for 6 months, and semi-annually thereafter, when feasible. Discontinue dapsone if there is a significant reduction in leukocytes, platelets, or hemopoiesis.

  • The majority of dapsone-related adverse effects (including methemoglobinemia, peripheral neuropathy, dizziness, headache, fatigue, nausea, and vomiting) are dose-dependent and for the most part can be rapidly reverted with dose reduction/discontinuation of therapy.

  • In about 1% to 2% of patients, and usually within the first few weeks of treatment, dapsone can cause a potentially life-threatening idiosyncratic hypersensitivity syndrome resembling drug reaction with eosinophilia and systemic symptoms (DRESS), characterized by fever, skin rash, and organ involvement.[56] Close monitoring of the patient with a particular focus on liver function is advised.

  • A rare adverse effect of dapsone therapy is idiosyncratic agranulocytosis, which may lead to life-threatening neutropenic sepsis; if affected, the patient typically presents with a fever and evidence of infection 1-3 months following initiation of dapsone.[57]​ Regular monitoring of CBC with differential aims to detect early phases of agranulocytosis before severe effects of neutropenia such as fever and infection occur.

Primary options

dapsone: children: consult specialist for guidance on dose; adults: 25-50 mg orally once daily initially, increase gradually according to response, maximum 100-200 mg/day

Consider – topical corticosteroid

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ONGOING
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all patients
Consider – 

topical corticosteroid

Treatment recommended for SOME patients in selected patient group

For patients with localized disease, consider the use of an ultrapotent topical corticosteroid (e.g., clobetasol propionate) alongside dapsone and a gluten-free diet for short-term symptomatic relief of pruritus and to reduce the appearance of new lesions.[8][11][17][20][21]

Topical corticosteroids can be used in children; however, higher potency corticosteroids should be used with caution.

Primary options

clobetasol topical: (0.05%) children ≥12 years of age and adults: apply sparingly to the affected area(s) twice daily for up to 2 weeks, maximum 50 g/week

2nd line – sulfasalazine

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ONGOING
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all patients
2nd line – 

sulfasalazine

Sulfasalazine is a second-line option if dapsone is contraindicated or not effective in controlling the disease despite adherence to a gluten-free diet.[2][11][20][21]

Sulfasalazine is considerably less effective than dapsone and only anecdotal case reports are available to support its use in this setting.[2][11][20][21][58][59][60][61]

Primary options

sulfasalazine: children ≥6 years of age: 40-60 mg/kg/day orally given in divided doses every 4-8 hours initially, followed by 30 mg/kg/day given in divided doses every 6 hours, maximum 4 g/day; adults: 1 g orally every 6-8 hours initially, followed by 500 mg every 6-12 hours, maximum 4 g/day

Plus – gluten-free diet

Back
ONGOING
|
all patients
Plus – 

gluten-free diet

Treatment recommended for ALL patients in selected patient group

Advise all patients with confirmed dermatitis herpetiformis to follow a strict and lifelong gluten-free diet.[2][11][20][21]

  • Dermatitis herpetiformis is the specific cutaneous manifestation of celiac disease and gluten is the main trigger for both conditions; a lifelong gluten-free diet is necessary to switch off the autoimmune process causing the disease and may help to prevent potential complications, such as other autoimmune diseases and lymphoma.[2][43][44]

Around 15% to 20% of people with dermatitis herpetiformis show gastrointestinal symptoms resembling those of celiac disease.[2][11]​ A gluten-free diet alone is usually able to induce the resolution of intestinal signs and symptoms in people with dermatitis herpetiformis (and those with celiac disease) within 3-6 months.[21][50]

  • If intestinal symptoms persist following 6 months of gluten-free diet or recur despite adherence to diet, referral for exclusion of other gastrointestinal conditions should be considered.

By contrast, its effects on resolving cutaneous lesions and pruritus is slower, in some instances taking several years; an average of 1-2 years on a gluten-free diet may be required for complete resolution of skin lesions.[21][51]

  • Deposited immunoglobulin A (IgA) autoantibodies may be detected in the dermis for up to 10 years after starting a strict gluten-free diet.[2]

  • A study of 237 patients with dermatitis herpetiformis showed that 38% of patients continued to have cutaneous symptoms after 2 years of a gluten-free diet, while 14% had skin symptoms after longer term (mean duration 24 years) gluten-free diet.[52]

Adherence to gluten-free diet is challenging, with one study reporting dietary lapses in approximately 25% of long-term treated patients with dermatitis herpetiformis.[53] In addition, gluten contamination can be present in food that is intended to be gluten free; one meta-analysis of gluten contamination in naturally gluten-free foods, gluten-free labeled foods, and meals prepared in food services found the overall prevalence of gluten contamination in these foods to be over 15%.[54]

For these reasons, other therapeutic strategies are often required in people with dermatitis herpetiformis.

Plus – dietary counseling

Back
ONGOING
|
all patients
Plus – 

dietary counseling

Treatment recommended for ALL patients in selected patient group

Refer all patients starting a gluten-free diet to a dietitian with training in gluten-free diets.[2][20][45] Dietary counseling is important because a gluten-free diet has been associated with:

  • Potential vitamin B deficiency[46][47]

  • Reduced fiber intake

  • Risk of rebound weight gain.[48]

Advise all patients on a gluten-free diet to follow a healthy and well balanced diet.[2]

  • Micronutrient and vitamin supplementation may also be a consideration on a case-by-case basis.[2]

Some experts recommend avoidance of excessive iodine intake (i.e., via excessive seafood consumption/iodine supplementation), as this may increase risk of relapse in patients with dermatitis herpetiformis.[2]

  • Although an inconsistent manifestation, potassium iodide (ingested or applied topically) may induce a flare-up of symptoms in some patients with dermatitis herpetiformis.[49] When applied topically, potassium iodide may cause lesions similar to dermatitis herpetiformis (note these may also occur with trauma and other chemical irritants, due to a mechanism known as the Koebner phenomenon).

Consider – topical corticosteroid

Back
ONGOING
|
all patients
Consider – 

topical corticosteroid

Treatment recommended for SOME patients in selected patient group

For patients with localized disease, consider the use of an ultrapotent topical corticosteroid (e.g., clobetasol propionate) alongside sulfasalazine and a gluten-free diet for short-term symptomatic relief of pruritus and to reduce the appearance of new lesions.[8][11][17][20][21]

Topical corticosteroids can be used in children; however, higher potency corticosteroids should be used with caution.

Primary options

clobetasol topical: (0.05%) children ≥12 years of age and adults: apply sparingly to the affected area(s) twice daily for up to 2 weeks, maximum 50 g/week

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Please note that formulations/routes and doses may differ between drug names and brands, drug formularies, or locations. Treatment recommendations are specific to patient groups. See disclaimer

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