ABSTRACT
Background: The data on the histology of cutaneous lesions of drug reaction with eosinophilia and systemic symptoms (DRESS) is limited. Aims: To study the histopathology of cutaneous lesions of drug reaction with eosinophilia and systemic symptoms (DRESS) and to identify any features with diagnostic or prognostic signifi cance. Methods: All patients admitted to the dermatology ward of government medical college, Kozhikode from January 1, 2014 to December 31, 2014 with probable or defi nite DRESS as per the RegiSCAR scoring system and who were willing to undergo skin biopsy were included in this prospective study. Results: The study population comprised of nine patients. The consistent histological fi nding documented was the predominantly lymphocytic dermal infl ammatory infi ltrate. Four of the fi ve patients whose histology revealed focal interface dermatitis and keratinocyte vacuolation with or without apoptotic keratinocytes, had elevated liver transaminases. Tissue eosinophilia was associated with disease fl ares. The presence of atypical lymphocytes in peripheral smear and histological evidence of dense dermal infl ammatory infi ltrate showed an association with hepatic involvement. Limitations: The main limitations of our study were the small sample size and our inability to carry out a detailed immunohistochemistry work-up. Conclusions: In the appropriate setting, varying combinations of epidermal hyperplasia, spongiosis, parakeratosis and individually necrotic keratinocytes in the background of lymphocyte predominant dermal infi ltrate (with some atypia) favor a diagnosis of drug reaction with eosinophilia and systemic symptoms. Female sex, the presence of atypical lymphocytes in peripheral smear, dense dermal infl ammatory infi ltrate, tissue eosinophilia and interface dermatitis with or without keratinocyte necrosis was associated with a poor prognosis.
ABSTRACT
Background and Aim: Infl ammatory myofi broblastic tumors (IMFTs) are uncommon neoplasms of the central nervous system (CNS) of intermediate grade biologic potential. Anaplastic lymphoma kinase (ALK-1), a diagnostic marker of anaplastic large cell lymphoma, is also expressed in a subset of IMFTs and appears to have prognostic signifi cance. Though, few studies have evaluated expression of ALK-1 in IMFTs of the CNS. This retrospective study was undertaken to evaluate the expression of ALK-1 expression in IMFT of CNS by immunohistochemistry and correlate with the clinical, radiological and pathologic features. Materials and Methods: Five cases diagnosed as IMFT/ infl ammatory pseudotumour/plasma cell granuloma, diagnosed in CNS over 10 year period (1998-2007) were retrieved from the archives of Department of Neuropathology of a tertiary referralcenter. The clinical profi le and imaging features were collected from the case records. Hematoxylin and eosin stained sections were reviewed with immunohistochemistry for smooth muscle actin (SMA), vimentin, desmin, ALK-1, p53, MIB-1, CD68, leukocyte common antigen, CD3, and CD20. Results: All fi ve cases of IMFTs presented as duralbased space occupying or en-plaque lesions. Histologically, four cases had combined plasma cell granuloma-fi brous histiocytoma morphology, and one had fi brous histiocytoma-like morphology. Immunohistochemically, SMA was strongly positive in spindle cell component of the tumors confi rming diagnosis. ALK-1 expression could not be detected by immunohistochemistry in any of the cases. Conclusion: Further studies analyzing ALK-1 gene mutation and rearrangements are required to determine pathogenetic role, if any, in CNS IMFTs.