Laryngeal edema in drug reaction with eosinophilia and systemic symptoms syndrome due to sulfasalazine

We present a case of a 47-year-old man with drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome. The patient had been diagnosed with rheumatoid arthritis and sulfasalazine was prescribed 4 weeks before admission. Initial symptoms with fever and rash worsened even after a discontinuation of the medication, and concomitant symptoms developed including typical manifestations of facial rash and edema sparing the periorbital area, as well as atypical laryngeal edema. Rheumatologists should be aware that sulfasalazine is derived from sulfonamide and can possibly induce DRESS syndrome, one of the life-threatening drug eruptions.     

The importance of vancomycin in drug rash with eosinophilia and systemic symptoms (dress) syndrome

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome characterized by fever, rash, eosinophilia, atypical lymphocytes, and multiorgan involvement has a significant mortality. Inpatient vancomycin use is increasing and appears to be emerging as an important etiology of DRESS syndrome. This study highlights the importance of vancomycin as a cause of DRESS syndrome. We reviewed all cases of DRESS syndrome among inpatients consulted by the Allergy & Immunology service at Massachusetts General Hospital (MGH) from July 2009 through December 2010. We also reviewed the use of inpatient parenteral vancomycin over the past 4 years at MGH. Six patients fulfilled clinical criteria for DRESS syndrome, including rash, fever, eosinophilia, and hepatitis, with five (83%) having vancomycin as the attributable cause. Onset of symptoms varied from 12 days to 4 weeks after start of vancomycin treatment. Systemic findings included atypical lymphocytes, lymphadenopathy, nephritis, hypotension, tachycardia, and pharyngitis. Treatment with corticosteroids was required in three cases. Recurrence of peripheral eosinophilia was a marker of disease relapse. In three of the five patients (60%), elevated human herpesvirus 6 (HHV6) IgG titers correlated with greater systemic involvement and prolonged time to resolution. MGH pharmacy records indicate a progressive increase in the number of patients treated with parenteral vancomycin over the last 4 years. Causative agents for DRESS syndrome in an inpatient setting is likely different from that seen in the general population. With increasing use of vancomycin, we are likely to see more cases of DRESS syndrome caused by vancomycin. Recognition of vancomycin as a common cause of inpatient DRESS syndrome is important.     

First case of drug rash eosinophilia and systemic symptoms due to boceprevir

Boceprevir and telaprevir are 2 specific inhibitors of the hepatitis C (HCV) serine protease 3. Cutaneous side effects have been reported with high frequency, essentially rash, and dry skin. We report a case of drug rash with eosinophilia and systemic symptoms (DRESS) due to boceprevir. A 56-year-old African woman with chronic hepatitis C complicated with cirrhosis and cryoglobulinemia received pegylated interferon alfa-2a (PegIFN) and ribavirin (RBV) for 4 weeks and then boceprevir was added. She was also co-infected with HIV state A2. Eight weeks after adding boceprevir she developed a generalized maculopapular exanthema with fever, facial oedema, apparition of lymph node and alteration of the general state. She presented an eosinophilia (up to 3.0 × 10⁹ cells/L), no biological inflammatory syndrome. The computed tomography revealed several lymph nodes located in the abdominal and inguinal areas. The cutaneous biopsy was consistent with a drug rash reaction. The HCV treatment was stopped and the patient was treated with topical steroids. Cutaneous and systemic symptoms disappeared in few weeks. Boceprevir was considered the culprit drug. We report to our knowledge the first case of DRESS due to boceprevir.     

Hydroxychloroquine-induced DRESS syndrome

The authors describe the first case of drug rash with eosinophilia and systemic symptoms syndrome caused by hydroxychloroquine treatment in a male patient affected by seronegative arthritis.     

Hypersensitivity syndrome (DRESS) and meningoencephalitis associated with nevirapine therapy.

The DRESS (drug rash with eosinophilia and systemic symptoms) syndrome is a serious condition that has been reported in association with various drugs, such as allopurinol, sulfonamides and aromatic anticonvulsants. Recently the condition has been described in HIV-infected patients taking antiretroviral agents. We report the first case, to our knowledge, of DRESS syndrome complicated by meningoencephalitis associated with nevirapine therapy.     

Vancomycin‐associated drug reaction with eosinophilia and systemic symptoms syndrome

Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a rare but potentially life‐threatening multi‐system disorder characterised by the delayed onset of fever, rash and internal organ involvement following the administration of a drug. We report three definite cases of vancomycin‐associated DRESS syndrome occurring and review the literature regarding this syndrome.     

Leflunomide-induced DRESS syndrome with renal involvement and vasculitis

DRESS or drug reaction (or rash) with eosinophilia and systemic symptoms belongs to the severe cutaneous adverse reaction group and is characterized by hematological abnormalities and visceral organ involvement. Although most often related with anticonvulsant and sulfonamide use, it is reported with numerous other drugs. We report an unusual case of DRESS syndrome due to Leflunomide, also complicated by renal involvement in the form of granulomatous interstitial nephritis and vasculitis. On a review of the literature, eight similar cases were found, and these are discussed.     

Bullous dermatosis associated with vancomycin extravasation

Cutaneous side effects related to vancomycin therapy have been reported including histamine-related reactions, linear IgA bullous dermatosis, Stevens-Johnson syndrome, maculopapular rash and drug rash with eosinophilia and systemic symptoms. In all instances, these reports were due to the systemic administration of vancomycin and subsequent immunological reactions to the medication. Drug extravasation into soft tissues can result in a variety of clinical outcomes usually related to physiochemical properties of the drug extravasated and its diluents or pharmacologic effects on the vasculature and tissue. The authors report a patient who experienced vancomycin extravasation that resulted in a localized bullous eruption resembling linear IgA bullous dermatosis, a phenomenon not previously described in the literature.     

Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome: suspected association with titanium bioprosthesis

Drug rash with eosinophilia and systemic symptoms (DRESS), also known as hypersensitivity syndrome, is an idiosyncratic drug reaction presenting with fever, diffuse lymphadenopathy, exfoliative dermatitis, and visceral involvement, which may include hepatitis, pneumonitis, pericarditis, myocarditis, nephritis, and colitis. This report describes a 19-year-old, previously healthy man with manifestations of hypersensitivity (DRESS) syndrome after acquiring a titanium bioprosthesis for a spinal fracture. To our knowledge, there have been no prior reports of DRESS syndrome in association with titanium bioprosthetic implants.     

Dress syndrome and fulminant hepatic failure induced by lamotrigine

Lamotrigine is a non-aromatic antiepileptic drug. Drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is a severe idiosyncratic reaction to drugs, especially anti-epileptic drugs. Associated clinical features include cutaneous eruption, fever, multiple peripheral lymphadenopathies, and potentially life-threatening damage of one or more organs. We report a case of DRESS syndrome induced by lamotrigine presenting with a hypersensitivity syndrome and fulminant hepatic failure requiring liver transplant. A 21-year old female patient presented an episode of seizure with loss of conscience. CT and EEG studies performed were normal. Treatment with lamotrigine was prescribed. In the course of 30 days, the patient developed skin lesions, pruritus, cholestatic hepatitis, and systemic symptoms-fever, lymphadenopathies, extensive exfoliative erythematous maculopapular rash, and jaundice. Serologic and laboratory tests showed no other causes responsible for the clinical spectrum. Hematologic tests revealed peripheral eosinophilia. Fulminant hepatic failure was diagnosed and an orthotopic liver transplant was performed. Histologic sections of the ex-planted liver demonstrated submassive hepatic necrosis, with the remnant portal spaces and lobules showing a mixed inflammatory infiltrate with lymphocytes and eosinophils. Lamotrigine treatment has been associated with multiorgan failure, DRESS syndrome, acute hepatic failure, and disseminated intravascular coagulation. In conclusion, we suggest that these potentially fatal side effects should be considered in any patient with clinical deterioration following administration of this drug.     

Engraftment syndrome: a common cause for rash and fever following autologous hematopoietic stem cell transplantation for multiple sclerosis

Autologous hematopoietic stem cell transplantation (HSCT) is currently being evaluated as a therapy for patients with progressive multiple sclerosis (MS) at risk of debilitating neurological impairment. While preliminary results from a few studies have been reported, little is known about toxicities or outcome of HSCT for MS. We report a relatively frequent triad of non-infectious fever, rash and fatigue or lassitude that may also be associated with pruritis, pulmonary symptoms, and eosinophilia and frequently occurs around engraftment. This syndrome occurred in 26% of our series of patients (5/19) undergoing HSCT for multiple sclerosis. The engraftment syndrome is usually self-limited but may require intervention with systemic corticosteroids.     

Severe drug rash with eosinophilia and systemic symptoms after treatment with minocycline

Introduction

Lung involvement is rarely observed in the DRESS syndrome (Drug rash with eosinophilia and systemic symptoms). We report here a severe minocycline induced hypersensitivity syndrome with initial respiratory distress.

Case report

A 19 year old man was admitted to the intensive care unit for acute respiratory distress with fever (40⁰C), lymph node enlargement, hepatomegaly, splenomegaly and eosinophilia (1640/mm³). Bilateral alveolar opacities were observed on the chest x-ray. Sedation and mechanical ventilation rapidly became necessary because of severe hypoxaemia (47 mm Hg) and the sudden onset of severe aggressive behaviour. The diagnosis of DRESS was immediately suspected as the patient had been treated for acne with minocycline for 28 days, and IV corticosteroids (2 mmg/kg/day) were initiated. Skin lesions were delayed and appeared 3 days later. The outcome was uncertain for the following 6 weeks with serious disturbance of hepatic and renal function. Serology for human herpes virus (HHV6) was initially negative but became positive. One year later, after progressive withdrawal of corticosteroid therapy, the patient had made a complete recovery with no sequelae.

Conclusion

The DRESS syndrome can cause considerable morbidity with multiple, severe visceral functional disturbances. Respiratory physicians should be aware of this syndrome as lung involvement can be serious and may precede cutaneous symptoms.     

Progressive systemic sclerosis with eosinophilia and a fulminating course

A case of progressive systemic sclerosis, with blood and pleural fluid eosinophilia and a fulminating course, is presented. Wide-mouth colonic diverticula developed within 10 weeks. Death from renal failure occurred five and a half months after the onset of symptoms. The possibility of eosinophilia as a marker of severe disease in progressive systemic sclerosis is raised.     

Early detection of circulating anodic antigen (CAA) in a case of acute schistosomiasis mansoni with Katayama fever.

A 34-year-old male developed acute Katayama fever with fever, diarrhoea, joint pains, headache, urticarial rash and eosinophilia 18 days after falling into and spending 15 min in the water during water-skiing in the outlet of the Volta river. Low anti-schistosomal antibody titres were found by the immunofluorescence assay after 4 weeks, and the first Schistosoma mansoni eggs were found in faeces after 6 weeks. Both symptoms and eosinophilia increased the first days after treatment with oxamniquine, after which he improved gradually. Examination of frozen sera by the newly developed Magnetic Beads Antigen Capture-EIA (MBAC-EIA) later demonstrated a peak in schistosomal circulating anodic antigen (CAA) levels of diagnostic significance already 4 weeks after he was infected.     

Febarbamate-induced pulmonary eosinophilia: a case report

Subacute pulmonary hypersensitivity to febarbamate is reported, manifested by peripheral eosinophilia, skin rash and pulmonary bilateral infiltrates. Bronchoalveolar lavage showed an increased eosinophil count, and transbronchial biopsy an intraalveolar and interstitial eosinophil infiltration. The reaction cleared on cessation of the drug, and a rechallenge test with febarbamate induced peripheral eosinophilia and skin rash.     

Disease patterns of patients with systemic lupus erythematosus as shown by application of factor analysis.

Clinical and laboratory test data of 77 patients with systemic lupus erythematosus (SLE) were evaluated by factor analysis. Six factors representing disease patterns were extracted: cutaneous symptoms of alopecia, malar rash, rash and photosensitivity; renal involvement; the anticoagulant syndrome of phlebitis and partial thromboplastic time inversely related to platelet count; lymphopenia; viral or fibromyalgia symptoms of headache, nervousness, joint and muscle pain; and serology of anti-DNA antibodies and complement inversely related. Application of factor analysis reveals various clinical presentations of SLE.     

Drug neosensitization during anticonvulsant hypersensitivity syndrome.

Anticonvulsant hypersensitivity syndrome (AHS) is a rare, severe drug hypersensitivity reaction included in the drug-related rash with eosinophilia and systemic symptoms syndrome (DRESS), in which a transient state of immune suppression and reactivation of latent virus infections have been observed. We describe 5 patients who developed neosensitization to different drugs taken during a previous episode of anticonvulsant-related DRESS, in whom skin prick, intradermal and/or patch tests were performed to confirm the diagnosis of drug hypersensitivity. In 1 patient, transient hypogammaglobulinemia was observed during the AHS. Four of the 5 patients developed a delayed skin eruption or a delayed systemic hypersensitivity reaction after intake of a drug that they had also taken during a previous anticonvulsant DRESS which had occurred months or years earlier; in the fifth, a possible reaction was prevented thanks to the allergy workup. The diagnosis of drug allergy was demonstrated by positive delayed reaction to intradermal test with amoxicillin in 2 cases, positive patch tests to paracetamol and amitriptyline in 2 cases, and by clinical evidence of ceftriaxone erythroderma in one. The possibility of neosensitization to drugs administered during anticonvulsant-related DRESS should be considered. A transient state of immunosuppression induced during the anticonvulsant-related DRESS may trigger latent virus reactivation and massive nonspecific immune system response, which may lead to breakdown of tolerance to other drugs present at that time in the organism.     

Acute pulmonary hypersensitivity to carbamazepine.

Acute pulmonary hypersensitivity to carbamazepine (Tegretol) is reported, manifested by diffuse pulmonary infiltrates, skin rash, and eosinophilia. The reaction cleared on cessation of the drug. A lymphocyte transformation test was reactive to carbamazepine.     

Acute renal failure,hemolytic anemia and skin rash associated with captopril therapy

In a 71 year old white man with renovascular hypertension, a skin rash, Coombs' positive hemolytic anemia, peripheral eosinophilia and acute renal failure with eosinophiluria developed 47 days after the start of captopril therapy. The skin rash, eosinophilia and acute renal failure resolved promptly after the discontinuation of captopril therapy but the hemolytic anemia and positive Coombs, test persisted for eight weeks.     

Severe acute hepatitis in the dress syndrome: Report of two cases

The DRESS (drug rash, eosinophilia and systemic symptoms) syndrome, also known as DIHS (drug-induced hypersensitivity syndrome), is a severe idiosyncratic reaction to several drugs, mainly antiepileptics and antibiotics, which can occasionally produce acute liver failure. In this article we present two cases of the DRESS syndrome presenting with severe acute hepatitis, including the first case of DRESS associated with levetiracetam. Although both cases finally resolved with good outcomes, DRESS can lead to acute liver failure and has a bad prognosis when liver damage is present. Rapid diagnosis is crucial since withdrawal of the offending drug is the key of treatment, while the potential role of corticosteroids is discussed.