Predictors of disease severity in drug reaction with eosinophilia and systemic symptoms

Background: Drug reaction with eosinophilia and systemic symptoms is an outcome of a complex interaction between specific drugs, certain herpesviruse types and the immune system of the affected individual and is characterized by an unpredictable course and recurrent flares even after withdrawal of the offending drug and administration of systemic steroids. Aims: To identify the predictors of disease severity in drug reaction with eosinophilia and systemic symptoms. Methods: After obtaining ethical clearance from the institutional ethics committee and a written informed consent from individual study participant, the first hundred patients who required inpatient care in Government Medical College, Kozhikode with drug reaction with eosinophilia and systemic symptoms from January 1st 2011 were included in this study aimed to identify the predictors of disease severity in drug reaction with eosinophilia and systemic symptoms. Results: Male-to-female ratio of the study group was 0.8:1. The presence of atypical cells in peripheral smear and advanced age were found to be predictors of disease severity in drug reaction with eosinophilia and systemic symptoms, whereas, sex, facial erythema and edema and absolute eosinophil count were found not to be predictors of the same. Limitations: The main limitation of this study was our inability to assess the role of human leukocyte antigen (HLA) association and herpes virus reactivation in disease severity in drug reaction with eosinophilia and systemic symptoms. This study was also not designed to evaluate the response to treatment given and the mortality caused by drug reaction with eosinophilia and systemic symptoms. Conclusions: Studies on the predictors of severity in drug reaction with eosinophilia and systemic symptoms in different population groups may enable us to identify the warning signs and help to formulate the standard therapeutic guidelines.

Drug reaction with eosinophilia and systemic symptoms (DRESS): A histopathology based analysis.

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.

Disseminated cutaneous phaeohyphomycosis due to Cladophialophora bantiana.

Cladophialophora bantiana is a neurotropic dematiaceous fungus which only rarely affects the skin. We report a case of disseminated cutaneous phaeohyphomycosis caused by Cladophialophora bantiana in an immunocompromised female who presented with multiple pyogenic granuloma‑like nodules, dermatophytosis‑like plaque, and subcutaneous cysts on the upper and lower extremities without systemic involvement. Biopsy revealed black yeasts resembling sclerotic bodies and culture yielded irregular, velvety, grey colonies with black reverse. Excisionof the nodules and treatment with oral itraconazole 100 mg twice daily resulted in complete clinical resolution within two months, following which itraconazole was administered for another 4 months.

Spontaneous regression in an ulcerated CK7 positive Merkel cell carcinoma.

Merkel cell carcinoma is an aggressive and frequently lethal tumor of the elderly, associated with sun exposure and immunosuppression which is less common in the dark-skinned. We report the case of a 40-year-old woman who presented with multiple slowly progressive, mildly itchy ulcerated plaques of size ranging from 2 × 3 cm to 5 × 7 cm on the left knee of 1 year duration. Skin biopsy showed diffuse dermal infi ltration by small round cells with molding of cells and lymphocyte infi ltration. The cells stained positive for cytokeratin (CK) 20, CK7, neuron-specifi c enolase, and chromogranin. The skin lesions underwent spontaneous regression within 1 month of skin biopsy and have not recurred during the past 2 years. The immune mechanisms triggered by biopsy possibly explain the spontaneous regression.

Drug reaction with eosinophilia and systemic symptoms: Observations from a tertiary care institution.

Background: Drug reaction with eosinophilia and systemic symptoms (DRESS) is a severe drug reaction which can mimic a viral infection, an autoimmune disease or a neoplastic disease. Aims: To study the clinical and epidemiological aspects of DRESS and to identify the precipitating drugs. Methods: All patients admitted to the dermatology ward of our tertiary care hospital from 1 st October 2010 to 30 th September 2013 with probable or definite DRESS as per the RegiSCAR scoring system were included in this prospective study. The clinical manifestations observed in the study population were studied and the common offending drugs were identified. Results: During the 3 year study period, 26 patients fulfilled criteria for probable or definite DRESS. In more than 50% of cases, the culprit drug was phenytoin. Most common symptoms observed were fever, rash and facial edema. Liver was the most common internal organ affected. Most of the patients responded to withdrawal of the drug and administration of steroids for 3-6 weeks. One patient with dapsone-induced DRESS died. Conclusions: Intense facial erythema and edema and an elevated eosinophil count were not found to be bad prognostic factors. In most instances the flare ups during the course of the disease could be managed with a slower tapering of steroids. More prospective studies on DRESS are required to assess the prognostic factors and to formulate better diagnostic criteria.

Rosai-Dorfman syndrome.

Rosai-Dorfman syndrome is characterized by sinus histiocytosis with massive lymphadenopathy. A 25-year-old woman presented with multiple erythematous and yellowish papules on the forehead, cheeks, chin and thigh. She had massive generalized, firm, non-tender and non-matted lymphadenopathy and mild hepatomegaly. Her hemogram was normal. A skin biopsy showed collections of histiocytes with emperipolesis and plenty of plasma cells. A lymph node biopsy showed partial loss of architecture, dilated sinuses filled with histiocytes, which showed lymphocytophagocytosis, anisonucleosis and a clear cytoplasm. She was referred to an oncologist for further management.