Etiologies include infections, particularly from upper respiratory or gastrointestinal sources; inflammatory and autoimmune diseases; pregnancy; malignancy; drug; and idiopathic. polychondritis, drug-induced lupus, and development of antineutrophil cytoplasmic antibodies (ANCAs). However, a combination of all these features has not been reported. Herein, we report such a patient. == 2. Case Report == An 86-year-old female with bipolar disorder was admitted with anxiety, insomnia, fatigue, and acute renal failure. Although lithium levels were normal, lithium had been discontinued and replaced with carbamazepine 100 mg daily 2 days prior to admission. She was also taking hydralazine 100 mg three times daily for hypertension for 2 years with no dosage change in 8 months. On hospital day 8, she developed fever and conjunctivitis followed by oral erosions and painful lesions on her nose, ears, back, and fingers. On examination, she appeared acutely ill and was febrile (38.4C). Bilateral conjunctivitis with exudative discharge and periorbital edema was noted. Tense vesicles and bullae with surrounding erythema were noted on her scalp, nose, and back. The skin overlying the cartilaginous portions of both ears was erythematous and edematous with focal bullous change. The noncartilaginous lobes appeared normal. Erosions were noted around the hard palate and gingival mucosa (Determine 1). Tender hemorrhagic bullae were prominent on distal and lateral fingers (Determine 2). == Determine 1. == (a) Drug-induced Fairly sweet Syndrome Nomegestrol acetate demonstrating tense vesicles and bullae with surrounding erythema over nose. (b) Tense inflammatory vesicles and bullae over central back. (c) Hemorrhagic bullae of distal finger pads and lateral fingers. (d) Erythema, edema, and focal bullous change overlying the cartilaginous portions of both ears. The non-cartilagenous lobes demonstrated no inflammatory changes. (e) Bullous lesions and erosions around the hard palate. == Determine 2. == 3 mm punch biopsy-upper back Nomegestrol acetate skin with focal subepidermal vesicles with neutrophilic microabscesses, perivascular and interstitial neutrophilic dermal infiltrate with leukocytoclasis 10x. Laboratory testing revealed elevated C-reactive protein at 14 mg/dl (normal = 0 to 1 1 mg/dL) and erythrocyte sedimentation rate of 72 mm/hour (normal = 0 to 17 mm/hour). Her white blood cell count was normal at 5.5/mm3(normal = 4.1 to 10.9/mm3) and hemoglobin was low at 9.7 gm/dL (normal = 11.7 to 15.5 gm/dL). Serum Nomegestrol acetate creatinine was 2.1 mg/dl (normal = 11.7 to 15.5 gm/dL). Serum creatinine was 2.1 mg/d and urinalysis demonstrated a new proteinuria (30 mg/dl) with hematuria (51 to 100 red blood cells/hp). Further labs showed positive AntiNuclear Antibody (HEp-2) with homogenous pattern of 1 1 : 640 (normal < 1 : 160). Anti-histone antibodies were elevated at 3.7 models (positive >1.5 units, Mayo Medical Laboratories). Perinuclear antineutrophil cytoplasmic antibodies (pANCAs) were positive to myeloperoxidase and proteinase 3 at 200 models/ml (normal = 0 to 9 models/ml) and 48.5 units/ml (normal = 0 to 3.5 models/ml), respectively. Anti-double-stranded DNA, anti-Smith, anti-RNP, SSA, SSB, SCL-70, or JO-1 antibodies were not detected, and complement levels were normal. Blood and urine cultures were unfavorable. Serum protein electrophoresis showed acute phase reaction pattern. Three-millimeter punch biopsies from the back and finger demonstrated focal subepidermal vesicles with neutrophilic microabscesses, perivascular and interstitial neutrophilic dermal infiltrate, and leukocytoclasis without vasculitis (Determine 2). Perilesional direct immunoflourescence (DIF) was unfavorable. The patient declined ear cartilage biopsy, but anti-type II collagen antibodies were positive (47.6 EU/ml; normal <20 EU/ml; Mayo Medical Laboratories). Drug-induced SS was suspected. Both carbamazepine and hydralazine were discontinued, and the patient was started on oral prednisone 60 mg daily. The patient declined renal biopsy. Her renal function, skin lesions, and mucosal lesions improved on prednisone, and she was discharged on a tapering dose. Skin lesions were resolved three weeks following discharge (Determine 3) Her MPO and PR-3 titers (57.7 models/ml and 11.1 models/ml, respectively) also decreased as her symptoms improved. The patient died of unknown causes 2 months following discharge, Rabbit Polyclonal to ABHD12B still on a tapering prednisone dose. == Determine 3. == Clearing of affected areas 3 weeks following discharge on tapering.