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Dermatitis Herpetiformis
Aka: Dermatitis Herpetiformis
- See Also
- Bullous Disease
- Epidemiology
- Incidence: Up to 39 cases per 100,000
- Usual onset at 20 to 40 years
- Gender preponderance in men by 2:1 ratio
- Ethnicity
- Most commonly affects white patients
- Rarely affects black or asian patients
- Pathophysiology
- Idiopathic condition
- Usually associated with Gluten Sensitive Enteropathy
- Symptoms: Precede lesion onset by 8 to 12 hours
- Intense Pruritus
- Skin burning
- Signs
- Grouping of lesions may occur (herpetiform-like)
- Altered pigmentation at sites of healed lesions
- Polymorphous collection of lesions
- Urticarial wheals
- Vesicles
- Bullae
- Erythematous Papules
- Symmetric distribution of lesions on limbs and trunk
- Common sites
- Elbows and knees
- Buttocks and Shoulders
- Sacral area
- Uncommon sites
- Scalp and hairline
- Face and posterior neck
- Rare sites
- Palms and soles
- Mucus Membranes
- Differential Diagnosis
- Pemphigus hermetiformis
- Labs
- Complete Blood Count
- Eosinophilia
- Histology
- Dermal Papillae with Neutrophil microabscesses
- Dermal inflammatory infiltrate
- Subepidermal vessicles
- Blisters in the lamina lucida
- Lymphohistiocytic infiltrate at dermal vessels
- Immunofluorescence
- Granular IgA deposits in Dermal Papillae tips
- Other autoimmune lab associations variably present
- Antinuclear Antibody
- Antithyroid Microsomal Antibody
- Complications
- Gluten Sensitive Enteropathy associated conditions
- Steatorrhea
- Abnormal D-Xylose Absorption
- Anemia
- Atrophic Gastritis
- Achlorhydria
- Gastrointestinal Lymphoma
- Autoimmune Conditions
- Thyroid Disease
- Type I Diabetes Mellitus
- Systemic Lupus Erythematosus
- Vitiligo
- Sjogren's Syndrome
- Management
- Medications
- First-Line: Dapsone
- Alternative: Sulfapyradine
- Dietary Management
- Gluten-Free Diet (improvement within 6-12 months)
- Elemental Diet (improvement may be seen in weeks)
- Monitoring
- See Dapsone (requires G6PD and monitoring of CBC)
- Course
- Prolonged course over years
- Spontaneous remission in one third of patients
- References
- Bickle (2002) Am Fam Physician 65:1861-70 [PubMed]
- Cotell (2000) Am J Emerg Med 18(3):288-99 [PubMed]