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Systemic Workup: FBC, Iron, B12, Folate, TFT Gastroenterology referral should be considered Villous biopsy - will show villous atrophy and crypt hyperplasia Management Strict lifelong gluten-free diet (GFD) Treats both skin and gut disease Reduces lymphoma risk Skin improvement takes months–years Dapsone (rapid symptomatic control) 25–50 mg daily → up to 100–200 mg Dramatic response usually seen within days If they fail to respond to dapsone reconsider the diagnosis Alternatives: Sulfapyridine Sulfasalizine Long-term prognosis: Many patients can discontinue dapsone with strict gluten free diet Monitor coeliac serology for compliance Prognosis: Excellent with adherence to GFD Increased lymphoma risk with poor dietary compliance Linear IgA disease Question 1 Metabolic: Porphyrias: Metabolic disorders caused by altered activity of enzymes in the heme biosynthesis pathway Porphyria can be subdivided into 3 main groups based upon clinical presentation: Acute intermittent (hepatic) porphyrias - neurological symptoms Cutaneous porphyria Acute and cutaneous - neurologic and cutaneous symptoms Acute hepatic: Acute intermittent porphyria Acute and cutaneous: Hereditary coproporphyria, variegate porphyria Cutaneous: PCT, CEP, EPP, Hepatoerytrhopoietic porphyria Three ways in which porphyrias present in the skin: Immediate painful photosensitivity: Erytrhopoeitic porphyria Mutilating photosensitivity Congenital erythropoeitic porphyria (Gunter’s disease) Other rare severe homozyghous porphyrias Bullous porphyrias - skin fragility and bullae Porphyria cutanea tarda Variegate porphyria What causes Porphyria