DRESS Syndrome: A Practical Approach for Primary Care Practitioners

Seth, Divya; Kamat, Deepak; Montejo, Jenny M.

doi:10.1177/0009922808320703

Cited by 31 publications

(36 citation statements)

References 21 publications

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“…DRESS syndrome is a multisystem condition that involves most internal organs, most commonly the heart, liver, kidneys, lung involvement with subsequent breathlessness and neurological involvement leading to seizure activity 9. The hypersensitivity reaction occurs between 2 and 8 weeks from exposure to the causative agent 10. A range of pharmaceuticals is associated with DRESS syndrome, including antiepileptics such as carbamazepine, lamotrigine and phenytoin, in addition to allopurinol and sulphonamides, in particular sulfasalazine 11.…”

Section: Discussionmentioning

confidence: 99%

Is this still just sarcoidosis, or should we a-DRESS a different diagnosis?

et al. 2015

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An Afro-Caribbean woman presented with worsening breathlessness, weight loss, lethargy and fevers, developing a bilateral florid erythematous rash on her legs. She was recently diagnosed with rheumatoid arthritis and bilateral hilar lymphadenopathy was found on thoracic CT imaging. She was tachycardic and investigations revealed pancytopenia, eosinophilia, raised serum ACE, acute kidney injury and deranged liver function tests. Biopsy of the lymphadenopathy revealed mixed lymphoid cells and liver biopsy revealed extramedullary haematopoiesis, with hypercellular marrow found on bone marrow biopsy. Cardiac MRI was normal, excluding cardiac sarcoid. The patient developed status epilepticus and phenytoin was started. She subsequently developed skin desquamation, in keeping with toxic epidermal necrosis. Skin biopsies revealed atypical granulomas and multinucleated giant cells, which subsequently resolved on steroid treatment. This case highlights an overlap syndrome, with an unclear diagnosis between sarcoidosis, drug reaction or rash with eosinophilia and systemic symptoms and/or hypereosinophilic syndrome and Still's disease. Hence varied serological and clinical features can complicate the distinction between diagnoses.

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Section: Discussionmentioning

confidence: 99%

Is this still just sarcoidosis, or should we a-DRESS a different diagnosis?

et al. 2015

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“…A temporal relationship between medicine use and onset of the syndrome is the most important indicator of causality. [17] CLINICAL FEATURES DHS is characterized by a constellation of symptoms involving various organs and organ systems, particularly the skin, liver and hematologic system being most commonly involved, with cutaneous changes being the most apparent. Correct diagnosis of DHS may be difficult because of the wide variety of possible clinical and laboratory abnormalities and manifestations and because the syndrome may mimic infectious, neoplastic or collagen vascular disorders.…”

Section: Nomenclaturementioning

confidence: 99%

Drug hypersensitivity syndrome

Kumari¹,

Timshina²,

Thappa³

2011

Indian J Dermatol Venereol Leprol

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Drug hypersensitivity syndrome (DHS) is an adverse drug reaction commonly associated with the aromatic antiepileptic drugs (AEDs), viz., phenytoin (PHT), carbamazepine (CBZ), phenobarbital (PB), lamotrigine, primidone, etc. It can also be caused by other drugs, such as sulfonamides, dapsone, minocycline, gold derivatives, cyclosporine, captopril, diltiazem, terbinafine, azathioprine and allopurinol. Diagnosis of DHS may be difficult because of the variety of clinical and laboratory abnormalities and manifestations and because the syndrome may mimic infectious, neoplastic or collagen vascular disorders. The risk for developing hypersensitivity within 60 days of the first or second prescription in new users of PHT or CBZ was estimated to be 2.3-4.5 per 10,000 and 1-4.1 per 10,000, respectively. The syndrome is defined by the fever, skin rash, lymphadenopathy and internal organ involvement within the first 2-8 weeks after initiation of therapy. Internal manifestations include, among others, agranulocytosis, hepatitis, nephritis and myositis. Insufficient detoxification may lead to cell death or contribute to the formation of antigen that triggers an immune reaction. Cross-reactivity among PHT, CBZ and PB is as high as 70%-80%. Management mainly includes immediate withdrawal of the culprit drug, symptomatic treatment and systemic steroids or immunoglobulins.

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“…[1][2][3] The syndrome is most commonly associated with the aromatic anticonvulsants (i.e., phenobarbital, phenytoin and carbamazepine) and has an incidence of 1:1000 to 1:10 000 exposures to these drugs. 2,3 The exact incidence of DRESS caused by sulfonamides is unknown.…”

mentioning

confidence: 99%

“…Symptoms of DRESS may progress to multiorgan failure; deaths are reported in up to 10% of instances. 2 The pathogenesis of DRESS syndrome is not clear.…”

mentioning

confidence: 99%