Christian Raúl
13.4K posts

Christian Raúl
@ChrisOceans
a veces quiero gritar y poder volar






Epidermolysis bullosa acquisita (EBA) EBA is a rare autoimmune subepidermal blistering disorder mediated by autoantibodies against type VII collagen. Histopath showed a subepidermal blister w/ neutrophils & eosinophils, while DIF showed linear IgG & C3 deposition along the basement membrane zone. Serology confirmed elevated collagen VII autoantibodies w/ -ve BP180 & BP230 antibodies, supporting the EBA dx. Despite tx w/ prednisone, MMF, colchicine, & topical corticosteroids, he continued to develop weekly blistering w/ severe pruritus. Off-label dupilumab (Dupixent®️) was initiated w/ tapering of prednisone & MMF. After months of tx w/ dupilumab, colchicine, & topical corticosteroids, he achieved marked improvement in pruritus & complete cessation of blister formation w/out significant adverse effects. Although collagen VII antibody titers remained elevated, indirect immunofluorescence converted to -ve, suggesting clinical response despite persistent seropositivity. DDx: bullous pemphigoid (especially given the tense bullae, pruritus, eosinophils, & linear BMZ IgG/C3 deposition), linear IgA bullous dermatosis (LABD), bullous lupus erythematosus. 📖Fitzpatrick’s Color Atlas and Synopsis of Clinical Dermatology






Hailey–Hailey disease (HHD) is a rare AD acantholytic dermatosis caused by ATP2C1 mutations & characterized by recurrent erosive intertriginous plaques. This middle-aged woman presented w/ a 4-yr hx of painful recurrent erosive plaques of the axillae, inframammary folds, & groin associated with malodor, fissuring, & functional impairment. FHx was notable for similar lesions in her father and two sisters. Exam showed macerated erythematous plaques w/ fissures & erosions in intertriginous areas. Bx demonstrated suprabasal acantholysis with a “dilapidated brick wall” appearance, confirming HHD. Prior tx, which included topical corticosteroids, tacrolimus, antibiotics, methotrexate, CO2 laser, & NB-UVB were ineffective or poorly tolerated. Combo therapy w/ topical 5-FU 3% cream & excimer lamp phototherapy led to marked improvement w/in 4 wks.































