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A6931 - An Steven Johnson Patient with Pulmonary and Cardiac Involvement
Author Block: W. Rodriguez1, A. Candelario2, L. Gonzalez1, O. J. Cantres3; 1Pulm/CCM/Sleep, VA Caribbean Healthcare System, San Juan, PR, United States, 2VA Caribbean Healthcare System, San Juan, PR, United States, 3Veterans Affair, San Juan, PR, United States.
A 61-year-old female came to emergency room after developing blistering in the neck lips and forehead after being exposed to Moxifloxacin for flu like symptoms. Physical examination showed conjunctival injection and multiple red-purple macules with central duskiness, denudatuion with positive Nikolsky sign initially localized along the face, neck and anterior torso, which advanced to affect lower abdomen and genitalia. She required endotracheal intubation and mechanical ventilation due to abundant oral secretions and mucosal edema. She developed acute kidney injury, elevation of liver enzymes (SGOT 124 U/L, SGPT 62 U/L) and markedly elevated amylase levels (2576). The patient was hypotense, and required vasopressor therapy. Cardiovascular evaluation revealed ST elevations on lateral and inferior and increased cardiac markers. Bedside echocardiogram demonstrated new onset severe LV systolic dysfunction with estimated ejection fraction of 10 to 15% and apical dyskinesis, suggestive of Takotsubo Cardiomyopathy. Steven Johnson’s syndrome/Toxic epidermal necrolysis (SJS/TEN) with multiorgam failure was diagnosed. Endotracheal tube intubation was remarkable for massive amount of blood. Bronchoscopy was remarkable for superficial ulcerations without active bleeding throughout trachea, up to carina. She received aggressive fluid and empiric broad-spectrum antibiotics and was started on continuous renal replacement therapy. She received supportive management and wound care with vaselinated gauzes with interval improvement in skin lesions. Cardiac function improved after initial acute phase.
SJS and TEN are rare severe mucocutaneous reactions, which may occur secondary to rheumatologic, infectious, or drug related causes. In Drug-induced SJS/TEN it is believed that the drug may stimulate the major histocompatibility complex I and the T-cell receptors by directly binding to them, leading to production of drug-specific cytotoxic T cells which lead to cell death. Although ocular and cutaneous diseases are commonly associated as with SJS/TEN, pulmonary complications have been documented as well. Bronchial involvement may present bronchial hypersecretion, dyspnea, or as production of non-purulent sputum; in some cases, although this may occur in only a small amount of cases, may present with hemoptysis. The patient also developed multiorgan involvement, including severe cardiomyopathy, and renal failure. Cardiac involvement is not common, with atrial fibrillation and pericarditis previously reported in rare cases.
This case illustrate a severe case of SJS/TEN with multiorgan involvement, including pulmonary mucosal involvement with hemoptysis and severe cardiomyopathy.