Bronchiolitis obliterans associated with Stevens-Johnson Syndrome: histopathological bronchial reconstruction of the whole lung and immunohistochemical study

Abstract

This study presents an extremely rare case of constrictive bronchiolitis obliterans (BO) associated with Stevens-Johnson Syndrome (SJS) provides the morphological and immunohistochemical features using histopathological bronchial reconstruction technique. A 27-year-old female developed progressive dyspnea after SJS induced by taking amoxicillin at the age of 10. Finally, she died of exacerbation of type II respiratory failure after 17 years from clinically diagnosed as having BO. Macroscopic bronchial reconstruction of the whole lungs at autopsy showed the beginning of bronchial obliterations was in the 4th to 5th branches, numbering from each segmental bronchus. Once they were obliterated, the distal and proximal bronchi were dilated. Microscopic bronchial reconstruction demonstrated the localization of obliteration was mainly from small bronchi to membranous bronchioli with intermittent airway luminal narrowing or obliteration. Moreover, CD3-, CD20-, and CD68-positive cells were found in the BO lesions. CD34- and D2-40-positive cells were mainly distributed in the peribronchiolar lesions and bronchiolar lumens, respectively. SMA- and TGF-β-positive cells were seen in the fibrous tissue of BO lesions.The virtual slidesThe virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/1071703140102601.

Clinical features of Stevens–Johnson syndrome and toxic epidermal necrolysis

“…noted that the risk factors for BO are infection and SJS . The pathogenesis of SJS/TEN‐induced pulmonary involvement is not completely clear, but could reflect severe bronchial inflammation induced not only by immune response but also by respiratory infection …”

“…19 The pathogenesis of SJS/TEN-induced pulmonary involvement is not completely clear, but could reflect severe bronchial inflammation induced not only by immune response but also by respiratory infection. 20 There is no evidence-based treatment for SJS/TEN. 21 Early diagnosis and the discontinuation of suspected drugs are the most important for the general treatment approach.…”

Bronchiolitis Obliterans Associated with Stevens-Johnson Syndrome: A Case Report

“…A few cases of BO after SJS with a histologic confirmation have since been reported. 5 , 7 , 8 In the autopsied lungs of patients with BO after SJS, Sugino, et al 7 noted macroscopically extensive occlusion of the bronchi at the 4th or 5th branches, more distal bronchi than the segmental bronchus. The occlusive lesions were sporadically and intermittently located from the small bronchi to the membranous bronchioli.…”

Complications of Stevens–Johnson syndrome beyond the eye and skin

“…Chronic pulmonary complications of SJS/TEN, were first described in 1983, wherein an 8-year-old girl died of chronic obliterative bronchitis 10 months after the resolution of her rash [23]. Since the 1990s, there have been over 20 case reports and series on the chronic histopathologic and pathophysiologic pulmonary changes seen in SJS/TEN [20,[24][25][26][27][28][29][30]. Obstructive lung diseases including bronchiolitis obliterans, bronchiectasis, and chronic obliterative bronchitis account for most documented longterm pulmonary complications in SJS.…”