ABSTRACT
Dapsone hypersensitivity syndrome (DHS) is a rare adverse effect of the commonly prescribed drug dapsone. We present a case of a 35-year-old male who was referred to us from the gastroenterologist with complaints of rash, nausea, vomiting, and jaundice since 2 days with a provisional differential diagnosis of infectious mononucleosis or viral exanthema. On enquiry patient gave history of taking dapsone a week prior for refractory urticaria. After thorough investigations we diagnosed him with DHS. This syndrome occurs in a relatively small proportion of patients, but it is associated with considerable morbidity and mortality. The reason for presenting this case is to remind physicians of the unpredictability and potential severity of this reaction which makes it a major concern in clinical practice.
ABSTRACT
Systemic sclerosis is a chronic autoimmune condition with a complex pathogenesis and a high rate of mortality and morbidity. Internal organ involvement requires interdisciplinary approach in individual patient management. New discoveries in the pathogenesis of scleroderma herald a drastic change in the traditional outlook to therapy and have led to the development of the target-based approach in management. The challenge at present is to translate these advances in molecular mechanisms into well-designed clinical trials that will recognize potential disease-modifying therapies. This article is an evidence-based review of prevailing treatment options and future therapeutic targets in systemic sclerosis.
Subject(s)
Antirheumatic Agents/therapeutic use , Fibrosis/drug therapy , Immunologic Factors/therapeutic use , Molecular Targeted Therapy/methods , Scleroderma, Systemic/drug therapy , Vasodilator Agents/therapeutic use , Clinical Trials as Topic , Evidence-Based Medicine , Hematopoietic Stem Cell Transplantation , Humans , Rheumatology/methods , Rheumatology/trends , Scleroderma, Systemic/geneticsABSTRACT
We present a 73 year old female with intractable pruritus and nonspecific cutaneous rash for a period of 9 months. She had recieved symptomatic therapy with no improvement. A complete examination revealed axillary and abdominal lymphadenopathy. A biopsy confirmed the diagnosis of Hodgkins lymphoma with Langerhans cell histiocytosis. She received 5 cycles of chemotherapy with resolution of pruritus and reduction in axillary and abdominal lymphadenopathy. The patient presented 6 months later with relapse and succumbed to the illness. Simultaneous occurrence of Langerhans cell histiocytosis and Hodgkins lymphoma may lead to misdiagnosis. The awareness of such an association is important to make an accurate diagnosis and guide appropriate therapy.