Literature DB >> 26123453

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

Sophie Rolls1, Catherine Hyams1, Michael Sheaff2, Terence C O'Shaughnessy1.   

Abstract

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. 2015 BMJ Publishing Group Ltd.

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Year:  2015        PMID: 26123453      PMCID: PMC4488709          DOI: 10.1136/bcr-2014-207778

Source DB:  PubMed          Journal:  BMJ Case Rep        ISSN: 1757-790X


  27 in total

Review 1.  Autoantibodies and overlap syndromes in autoimmune rheumatic disease.

Authors:  E C Jury; D D'Cruz; W J Morrow
Journal:  J Clin Pathol       Date:  2001-05       Impact factor: 3.411

2.  Anti-cyclic citrullinated peptide antibodies in patients with sarcoidosis.

Authors:  Senol Kobak; H Ylmaz; F Sever; A Duran; N Sen
Journal:  Sarcoidosis Vasc Diffuse Lung Dis       Date:  2014-10-20       Impact factor: 0.670

Review 3.  'Overlap' syndromes.

Authors:  R Cervera; M A Khamashta; G R Hughes
Journal:  Ann Rheum Dis       Date:  1990-11       Impact factor: 19.103

4.  Preliminary criteria for classification of adult Still's disease.

Authors:  M Yamaguchi; A Ohta; T Tsunematsu; R Kasukawa; Y Mizushima; H Kashiwagi; S Kashiwazaki; K Tanimoto; Y Matsumoto; T Ota
Journal:  J Rheumatol       Date:  1992-03       Impact factor: 4.666

5.  Variability in the clinical pattern of cutaneous side-effects of drugs with systemic symptoms: does a DRESS syndrome really exist?

Authors:  S H Kardaun; A Sidoroff; L Valeyrie-Allanore; S Halevy; B B Davidovici; M Mockenhaupt; J C Roujeau
Journal:  Br J Dermatol       Date:  2007-03       Impact factor: 9.302

Review 6.  Sarcoidosis.

Authors:  Michael C Iannuzzi; Benjamin A Rybicki; Alvin S Teirstein
Journal:  N Engl J Med       Date:  2007-11-22       Impact factor: 91.245

7.  DRESS syndrome: a practical approach for primary care practitioners.

Authors:  Divya Seth; Deepak Kamat; Jenny Montejo
Journal:  Clin Pediatr (Phila)       Date:  2008-07-22       Impact factor: 1.168

Review 8.  Overlap syndromes with sarcoidosis.

Authors:  D G James; O P Sharma
Journal:  Postgrad Med J       Date:  1985-09       Impact factor: 2.401

9.  Sarcoidosis presenting with erythema multiforme-like cutaneous lesions.

Authors:  B E Beacham; J Schuldenfrei; S S Julka
Journal:  Cutis       Date:  1984-05

Review 10.  Sarcoidosis.

Authors:  Jennifer J Wu; Karin Rashcovsky Schiff
Journal:  Am Fam Physician       Date:  2004-07-15       Impact factor: 3.292

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