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Dermatitis herpetiformis (dh)
- 2. WHAT IS DERMATITIS HERPETIFORMIS ? • A chronic, recurrent. Intensely pruritic eruption occurring symmetrically on the extremities and the trunk. • Consists of tiny vesicles, papules, and urticarial plaques that are arranged in groups. • Associated with gluten-sensitive enteropathy (GSE). • Characterized histologically by papillary collection of neutrophils. • Granular lgA deposits In paralesional or normal skin are diagnostic. • Responds to sulpha drugs and, to a lesser extent a gluten-free diet. • This was first described by Louis Adolphus Duhring in 1884
- 3. EPIDEMIOLOGY.. • Prevalence more in Caucasians, varies from 10 to 39 per 100,000 persons. • AGE OF ONSET - Most common at 30 to 40 years; may occur in children. • SEX - Male: female ratio is 2:1.
- 4. ETIOLOGY • Exact etiology is not known. • Agent can be Epidermal transgluataminase predominant autoantigen. • Strong association with HLA – DR3, DQ2, DQ8. • Environmental Factor: * Gluten sensitive enteropathy, antibodies to gliadin, antigenic component to gluten is not present in these pt. . * Gluten is a protein present in grasses of the species Triticeae, which includes wheat, rye, barley. • Triggering factors can be dermal pressure or trauma, sun exposure and topical or oral Iodine administration.
- 5. PATHOGENESIS • The leading theory for dermatitis herpetiformis is that a genetic predisposition for gluten sensitivity, coupled with a diet high in gluten, leads to the formation of IgA antibodies against gluten-tissue transglutaminase (t-TG), which is found in the gut. These antibodies cross-react with epidermal transglutaminase (e-TG). eTG is highly homologous with tTG. Serum from patients with gluten-sensitive enteropathy, with or without skin disease, contains IgA antibodies to both skin and gut types. Deposition of IgA and epidermal TG complexes in the papillary dermis cause the lesions of dermatitis herpetiformis. • In patients with gluten-sensitive enteropathy, levels of circulating antibodies to tissue and epidermal transglutaminase have been found to correlate with each other, and both appear to correlate with the extent of enteropathy.
- 7. CLINICAL MANIFESTATION • Pruritus, intense, episodic; burning or stinging of the skin; rarely, pruritus may be absent. • Symptoms often precede the appearance of skin lesions by 8 to 12 h. • Ingestion of iodides and overload of gluten are exacerbating factors. • SYSTEMS REVIEW: Laboratory evidence of small bowel malabsorption is detected in 10 to 20% of patients. GSE occurs in nearly all patients and is demonstrated by small-bowel biopsy. There are usually no systemic symptoms. • Skin Lesions: Erythematous papules or wheal like plaques ; tiny firm- topped vesicles, sometimes haemorrhagic. • Strained bullae. Diameter of bullae is around 0.5 to 2 cm. Lesions are arranged in groups (hence the name herpetiformis). Scratching results in excoriations, crusts post inflammatory hyper- and hypo-pigmentation at sites of healed lesions.
- 9. SITES OF PREDICTION • Typical and almost diagnostic: Extensor areas-elbows, knees. Strikingly symmetrical Buttocks, scapular, and sacral areas. Here, often in a "butterfly"' fashion. Scalp, face. and hairline.
- 11. • Continuous pruritus of different gradation, sometimes esthesia of heat, tingling and pain appears. • True polymorphism of hives and may be accompanied by false polymorphism • Herpetiform group of hives components. • Chronicity which is interrupted by complete or incomplete remission of 3 months to 1 year duration or more. DIACRISIS OF DH
- 13. DIFFERENTIAL DIAGNOSTIC CRITERIA • May occur at any age. • Start of the disease: The skin is damaged • Bulla’s nature: Strained and round shape • Hive's : Grouped • Anabrosis: Small • Process of epithelialisation of Anabrosis: Fast • Present antibodies of IgA class • Nikolsky’s sign + Hansen’s Symptom: Absent
- 14. DIAGNOSIS OF DH • DH is best diagnosed with a skin biopsy. • A Direct Immunofluorescence Test is done which shows the presence of IgA antibody deposits. • Blood test to check the presence of the antibodies. • An Intestinal biopsy to check the presence of damage due to celiac disease.
- 16. TREATMENT AND PREVENTION • No cure, but medication can help to heel the rash. • Dapsone (diaminodiphenyl sulfone) :– 0.05 – 0.1 g twice per day in cycle of 5-6 days with interval of 2-3 days. • Course of dose depends on effectiveness and tolerance of the drug. • Oral vitamin E – to protect against dapsone induced haemolysis. • Sulfasalazine – 0.5 g three times a day increased to 2g/d(when sensitivity to dapsone).
- 17. PREVENTIVE MEASURES • Colchicines – when dapsone or Sulfasalazine or gluten free diet cannot be taken. • Heparin with / out tetracycline + Nicotinamide can be used in case of intolerance to dapsone or sulphonamides. • Others – Cyclosporine, Azathioprine, Prednisolone. • Gluten Free Diet. • Strict avoidance of wheat, rye, barley • If dietary restriction are discontinued then the rash returns after an average of 3 months • Also sometimes Iodine restirction.