Linear IgA Dermatosis associated with ulcerative colitis: complete and\nsustained remission after total colectomy
A 13-year-old boy sought dermatological consultation because of itching skin lesions\nlasting one month. He had had UC diagnosed 11 months earlier and was in continuous use\nof adalimumab, mesalazine and prednisone (10mg/d). On physical examination, erosions\nwith some peripherally disposed vesicles over a reddish background on the forehead and\nglabella were seen (). Some impetigo-like\npustular lesions were found on the chin and around the nose. There was also oral\ninvolvement, some crusted clustered papules in the precordium, and some large erosions\nwith hemorrhagic exudate in the back, lumbar region and buttocks ( and ). In the\nintergluteal region, a large eroded plaque covered by a whitish membrane was observed\n(). The patient denied use of any\nmedication previously associated to drug induced-LAD such as vancomycin, penicillin,\nceftriaxone, metronidazole, captopril, phenytoin, diclofenac, somatostatin, amiodarone,\nlithium, cefamandole, cyclosporin, interleukin-2, interferon-γ etc. Histopathology revealed a subepidermal\nblister and a superficial inflammatory infiltrate consisting of neutrophils arranged in\ncollections on dermal papillae ( and\n). Direct immunofluorescense of perilesional\nskin depicted a strong linear deposition of IgA along the basement membrane and a focal\ngranular positiveness of C3 (non-specific finding) (). IgG, IgM and fibrinogen were negative. The diagnosis of LAD was\ntherefore established and treatment with dapsone 50 mg/day started. In two weeks there\nwas regression of all the skin lesions, leaving only erosions inside the mouth, which\nwere handled with triancinolone acetonide 0.1% ointment. At this point in time oral\nprednisone was suspended. One month later there was a relapse of perineal and\nintergluteal lesions, with bleeding. Oral prednisone was reinitiated in the dose of\n20mg/day. A new attempt of tapering prednisone resulted again in a worsening of the skin\nlesions and the dose of dapsone was increased to 100mg/d. LAD remained partially but\nunsatisfactorily controlled over the next 2.5 months, when the patient underwent total\ncolectomy with ileorectal anastomosis for UC and experienced a substantial improvement\nof skin lesions, allowing reduction of prednisone and dapsone to 10 mg and 50 mg every\nother day, respectively. After 3 months, the patient returned with complete remission of\nboth LAD and UC and all medications were suspended. There was no relapse in two years of\nfollow-up.