Patient with gluten sensitive enteropathy has a lifelong background of periodic crops of intensely pruritic, grouped, papular or vesicular lesions on the elbows, knees, sacrum and shoulders. Since the vesicles are intensely pruritic, the patient regularly scratches the top off them, which relieves pruritis. What is the most probable diagnosis
High-Yield Explanation
Dermatitis Herpetiformis (Duhring's Disease) * Recurrent chronic pruritic disease associated with gluten-sensitive enteropathy * Gluten: general name for storage proteins found in wheat, rye, and barley * NOT found in rice, oats or corn * Gliadin: soluble fraction; likely antigenic component * Autoantigen: epidermal transglutaminase (TG-3), tissue transglutaminase (endomysial) Clinical features:- Erythematous grouped papules or vesicles over elbows, knees, buttocks; intensely pruritic, so primary lesions typically not visible due to excoriations Histology:- Neutrophilic microabscesses in dermal papillae, +- subepidermal vesicles DIF:- Granular IgA> C3 deposition in dermal papillae * IIF:- negative * Labs: anti-gliadin/anti-endomysial antibodies in DH/celiac disease Treatment:- Dapsone (immediate skin improvement), referral to GI (>90% with gluten-sensitive enteropathy and | risk of small bowel lymphoma) * | Incidence thyroid disease (Hashimoto's thyroiditis), IDDM, enteropathy-associated T cell lymphoma * Associated with HLA-DQ2 (strongest), HLA-B8. Ref:- Sima Jain; pg num:-132,134