Pemphigus and BMZ diseases in people: presentations and ...

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• Subepidermal blistering disease • Age > 60 years • May start as an urticarial eruption (very pruritic) • Tense blisters, common locations: abdomen, flexor surfaces of forearms, inner thighs • (-) Nikolsky sign Histology • Subepidermal blister with prominent eosinophilic infiltration IF - see table 1 Management • Topical steroids or systemic corticosteroids +/- other immunosuppressives • Tetracycline +/- Nicotinamide, Azathioprine, Mycophenolate mofetil, Methotrexate, Cyclophosphamide Course and prognosis • Self-limited with good prognosis • 50% enter remission within 2-6 years Mucous Membrane Pemphigoid (Cicatricial Pemphigoid) Clinical • Erosive lesions of skin and mucous membranes • Skin involvement in 1/3 of patients, mostly mucosal • Heals with scarring (i.e. conjunctival scarring) • Eye involvement may lead to blindness • Mucosal involvement may lead to dysphagia or even oesophageal stenosis requiring dilatation • (-) Nikolsky sign Histology • Subepidermal blister with mixed inflammatory cell infiltration IF - see table 1 In addition, antiepiligrin cicatricial pemphigoid circulating autoantibodies bind to dermal side of salt-split skin Management • Topical steroids or systemic corticosteroids +/- other immunosuppressives • Tetracycline +/- Nicotinamide, Azathioprine, Mycophenolate mofetil, Methotrexate, Cyclophosphamide Course and prognosis • Chronic, progressive Pemphigoid Gestationis (Herpes gestationis) Clinical • Rare, autoimmune disease of pregnancy • Extremely pruritic, polymorphic bullous dermatosis with urticarial plaques • Usually starts on the abdomen spreading peripherally sparing the face, palms, soles, mucous membranes • Exacerbations after delivery common • Babies born to these mothers may have transient blistering after delivery • Heals with scarring (i.e. conjunctival scarring) Histology • Subepidermal blister with eosinophilic infiltration IF - see table 1 33
Management • Topical steroids or systemic corticosteroids if required Course and prognosis • Maternal mortality rate is unaffected • Regresses without scarring a few days to weeks after delivery • May recur in subsequent pregnancies Linear IgA Disease (Chronic Bullous Disease of Childhood) Clinical • Often in patients > 30 years;
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Page 3: Management • Systemic corticoster

Pemphigus and BMZ diseases in people: presentations and ...

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