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Chapter 45: Dermatitis Herpetiformis

Steven Castellano; Anne Marano; Russell Hall

DERMATITIS HERPETIFORMIS

Therapeutic Approach

Dermatitis herpetiformis (DH) is a chronic, cutaneous, blistering disease of the extensor surfaces and scalp that is characterized by severely pruritic, symmetrically distributed papulovesicles. DH is associated with gluten-sensitive enteropathy, with essentially all patients showing evidence of disease activity on gastrointestinal biopsy, even if it is subclinical. DH is diagnosed by skin biopsy showing subepidermal vesiculation and neutrophils in the dermal papillae. Direct immunofluorescence of perilesional skin demonstrates granular deposits of IgA within the dermal papillae. The cornerstones of DH therapy are oral dapsone and/or strict adherence to a gluten-free diet; sulfasalazine is utilized in patients who are unable to tolerate dapsone, and ultra potent topical corticosteroids are first-line antipruritic therapies that are applied as needed during active flares. See Table 45-1.

Table 45-1Dermatitis Herpetiformis Treatment Table
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Table 45-1 Dermatitis Herpetiformis Treatment Table
MEDICATION NAME INDICATION MECHANISM OF ACTION DOSING ADVERSE EFFECTS SUGGESTED MONITORING LEVEL OF EVIDENCE (REFERENCE)
Topical Therapy
Ultra potent topical corticosteroids, typically class I or class II (eg, clobetasol proprionate 0.05%, flucinionide 0.05%, or betamethasone diproprinate 0.05% cream or ointment) Symptomatic relief of isolated new lesions, possible use as adjunct to gluten-free diet Anti-inflammatory Apply to affected areas BID Skin irritation, skin atrophy, striae, acne, skin pigmentation change, skin infections None IIA1
Dapsone 5% Gel Symptomatic relief of isolated new lesions, possible adjunct to gluten-free diet Anti-inflammatory: inhibits neutrophil chemotaxis Apply to affected areas BID Local irritation None III2,3
Systemic Therapy
Dapsonea Cutaneous signs and symptoms, pruritus Anti-inflammatory: inhibits neutrophil chemotaxis 25-200 mg QD

Physiologic (occurs in all patients): hemolytic anemia, methemoglobinemia (resulting in decreased peripheral oxygen saturation on pulse oximeter)

Idiosyncratic: agranulocytosis, hepatitis, peripheral motor neuropathy, dapsone-acute hypersensitivtiy (<1%), present in breast milk.

Of note, sulfa allergy to sulfonamides is not a contraindication to trying dapsone, which is a sulfone, while monitoring for cross reactivitiy

Baseline: G6PD, CBC with diff, LFT

Initiation: CBC with diff weekly × 4 wk, then qow for 4 wk

Monitoring: CBC with diff, LFTs, peripheral motor exam every 3-6 mo or with change in dosage

 IIA4-8
Sulfasalazine Cutaneous signs and symptoms, unable to tolerate dapsone Anti-inflammatory: inhibits neutrophil chemotaxis 500-1000 mg BID Anemia, agranulocytosis, hepatotoxicity, proteinuria, crystalluria Baseline and every 3-6 mo: CBC with diff, LFTs, UA IIB9,10
Sulfapyradine (not commercially available in the United States) Cutaneous signs and symptoms, unable to tolerate dapsone Anti-inflammatory: inhibits neutrophil chemotaxis 250-1000 mg QID Anemia, agranulocytosis, hepatotoxicity, proteinuria, crystalluria Baseline and every 3-6 mo: CBC with diff, LFTs, UA III11,12
Sulfamethoxypyridizine (not commercially available in the United States) Cutaneous signs and symptoms, unable to tolerate dapsone Anti-inflammatory: inhibits neutrophil chemotaxis 125-750 mg BID Anemia, agranulocytosis, hepatotoxicity, proteinuria, crystalluria Baseline and every 3-6 mo: CBC with diff, LFTs, UA III13
Tetracycline and niacinamide Unable to tolerate dapsone, inadequate control despite strict ...

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