title: Cutaneous manifestations of adult-onset Still's disease: a case report and review of literature.
abstract: Adult onset Still's disease (AOSD) is a systemic inflammatory disorder of unknown etiology and pathogenesis characterized by high spiking fever, arthralgia or arthritis, sore throat, lymphadenopathy, hepatosplenomegaly, serositis, and transient cutaneous manifestations. Although more common in children, cases are seen also in adults. Cutaneous involvement is common and may be suggestive for the diagnosis. A case of AOSD in a 35-year-old man is reported here, presenting with urticarial maculopapular rash of trunk, high spiking fever, acute respiratory distress syndrome, and myopericarditis. Skin biopsy showed interstitial and perivascular mature CD15(+) neutrophils. A comprehensive review of literature showed that cutaneous involvement occurs in about 80 % of patients, with various clinical presentations. The most common skin manifestation is an evanescent salmon pink or erythematous maculopapular exanthema, predominantly on the trunk and proximal limbs, with rare involvement of face and distal limbs. Less common manifestations include persistent erythematous plaques and pustular lesions. A constant histopathologic finding is the presence of interstitial dermal neutrophils aligned between the collagen bundles. This pattern may provide an easy accessible clue for the definitive diagnosis of AOSD and exclude other diagnosis such as drug eruptions or infectious diseases.
authors.affiliation: Department of Medicine, Section of Dermatology and Venereology, University of Verona, Piazzale A. Stefani 1, 37126, Verona, Italy.
authors.forename: Alessandra
authors.initials: A
authors.lastname: Cozzi
authors.affiliation: Department of Medicine, Section of Dermatology and Venereology, University of Verona, Piazzale A. Stefani 1, 37126, Verona, Italy.
authors.forename: Anastasia
authors.initials: A
authors.lastname: Papagrigoraki
authors.affiliation: Department of Medicine, Section of Rheumatology, University of Verona, Piazzale A. Stefani 1, 37126, Verona, Italy.
authors.forename: Domenico
authors.initials: D
authors.lastname: Biasi
authors.affiliation: Department of Pathology and Diagnostics, Section of Pathology, University of Verona, Piazzale A. Stefani 1, 37126, Verona, Italy.
authors.forename: Chiara
authors.initials: C
authors.lastname: Colato
authors.affiliation: Department of Medicine, Section of Dermatology and Venereology, University of Verona, Piazzale A. Stefani 1, 37126, Verona, Italy. giampiero.girolomoni@univr.it.
authors.forename: Giampiero
authors.initials: G
authors.lastname: Girolomoni
mesh_terms: D000328:Adult; D001706:Biopsy; D005076:Exanthema / Q000473:pathology*; D006801:Humans; D008297:Male; D009504:Neutrophils / Q000473:pathology; D012867:Skin / Q000473:pathology*; D016706:Still's Disease, Adult-Onset / Q000175:diagnosis* / Q000473:pathology; D014581:Urticaria / Q000473:pathology*
keywords: Adult onset Still’s disease; Neutrophilic urticarial dermatosis; Neutrophils; Skin
references.citation: Medicine (Baltimore). 2009 Jan;88(1):23-31
references.pmid: 19352297
references.citation: Orphanet J Rare Dis. 2010 Dec 08;5:38
references.pmid: 21143856
references.citation: Clin Rheumatol. 2000;19(5):389-91
references.pmid: 11055830
references.citation: Dermatology. 2001;202(4):333-5
references.pmid: 11455148
references.citation: Rheumatol Int. 2010 May;30(7):855-62
references.pmid: 20020138
references.citation: Ann Rheum Dis. 2006 May;65(5):564-72
references.pmid: 16219707
references.citation: JAMA Dermatol. 2013 Dec;149(12):1425-6
references.pmid: 24080790
references.citation: Dermatology. 1994;188(3):241-2
references.pmid: 8186519
references.citation: J Rheumatol. 1992 Mar;19(3):424-30
references.pmid: 1578458
references.citation: Br J Dermatol. 2001 Jul;145(1):187-8
references.pmid: 11453943
references.citation: J Rheumatol. 1987 Dec;14(6):1139-46
references.pmid: 3325642
references.citation: J Rheumatol. 1990 Aug;17(8):1058-63
references.pmid: 2213780
references.citation: Q J Med. 1986 Nov;61(235):1055-65
references.pmid: 3659248
references.citation: Rheumatol Int. 2012 Aug;32(8):2233-7
references.pmid: 22198666
references.citation: J Am Acad Dermatol. 2010 Apr;62(4):557-70; quiz 571-2
references.pmid: 20227577
references.citation: Indian J Dermatol. 2013 Sep;58(5):405
references.pmid: 24082197
references.citation: J Eur Acad Dermatol Venereol. 2005 May;19(3):360-3
references.pmid: 15857467
references.citation: J Cutan Med Surg. 2006 Mar-Apr;10(2):99-103
references.pmid: 17241583
references.citation: Int J Dermatol. 2003 Oct;42(10):824-5
references.pmid: 14521701
references.citation: Br J Dermatol. 1999 Oct;141(4):710-3
references.pmid: 10583122
references.citation: Eur J Dermatol. 2006 Sep-Oct;16(5):593-4
references.pmid: 17101495
references.citation: Am J Dermatopathol. 2007 Apr;29(2):194-6
references.pmid: 17414447
references.citation: Semin Arthritis Rheum. 2012 Dec;42(3):317-26
references.pmid: 22717204
references.citation: Medicine (Baltimore). 1991 Mar;70(2):118-36
references.pmid: 2005777
references.citation: Clin Rheumatol. 2004 Feb;23(1):92
references.pmid: 14749997
references.citation: Acta Derm Venereol. 2014 Jan;94(1):102-3
references.pmid: 23624720
references.citation: Br J Dermatol. 1998 May;138(5):906-8
references.pmid: 9666847
references.citation: Ann Dermatol Venereol. 2005 Aug-Sep;132(8-9 Pt 1):693-6
references.pmid: 16230922
references.citation: J Korean Med Sci. 2002 Dec;17(6):852-5
references.pmid: 12483016