Atypical Cutaneous Presentation of AOSD with Persistent Itchy Urticaria: A Case Report

Overview

Journal Medicine (Baltimore)

Specialty General Medicine

Date 2023 Dec 20

PMID 38115334

Authors

Jingfeng Lou

Xingping Zhang

Affiliations

Soon will be listed here.

Abstract

Rationale: Adult-onset Still's disease (AOSD) is a rare multisystem disorder considered a complex autoinflammatory syndrome. The clinical and biological features of AOSD typically include a high fever with arthritic symptoms, evanescent skin rash, sore throat, striking neutrophilic leukocytosis, hyperferritinemia, and abnormal liver function. The typical rash and fever are important diagnostic clues for AOSD. Here, we report a case of atypical rash manifesting as persistent itchy urticaria.

Patient Concerns: A 57-year-old female presented with a 6-day history of fever. During her hospital stay, she progressively developed rashes that were not associated with fever, primarily distributed on her back and the distal extremities, and associated with pronounced itching. The rash was initially suspected to be urticaria; however, the patient exhibited a poor response to antihistamines. After malignancies and other rheumatic diseases were excluded, the diagnosis leaned towards AOSD based on diagnostic criteria. The patient's fever was well controlled with the initiation of glucocorticoids, and no further rashes were observed.

Diagnoses: Although the patient exhibited atypical rashes, after ruling out malignancies and other rheumatic diseases, she met 2 major and 3 minor criteria. Based on Yamaguchi's criteria, the patient was diagnosed with AOSD.

Interventions: Initially, the patient was administered an intravenous infusion of methylprednisolone at 40 mg once daily. This was later transitioned to oral administration with gradual dose reduction.

Outcomes: Follow-up at 1 year showed no recurrence of the rash, with a stable condition and no relapse.

Lessons: This case provides valuable insights for the early diagnosis of AOSD, emphasizing the importance of considering this diagnosis even when presenting with atypical skin rash.

References

Criado P, de Carvalho J, Ayabe L, Brandt H, Romiti R, Maruta C . Urticaria and dermographism in patients with adult-onset Still's disease. Rheumatol Int. 2011; 32(8):2551-5. DOI: 10.1007/s00296-011-2025-5. View

Affleck A, Littlewood S . Adult-onset Still's disease with atypical cutaneous features. J Eur Acad Dermatol Venereol. 2005; 19(3):360-3. DOI: 10.1111/j.1468-3083.2004.01142.x. View

Fautrel B . Ferritin levels in adult Still's disease: any sugar?. Joint Bone Spine. 2002; 69(4):355-7. DOI: 10.1016/s1297-319x(02)00409-8. View

Macovei L, Burlui A, Bratoiu I, Rezus C, Cardoneanu A, Richter P . Adult-Onset Still's Disease-A Complex Disease, a Challenging Treatment. Int J Mol Sci. 2022; 23(21). PMC: 9655522. DOI: 10.3390/ijms232112810. View

Lubbe J, Hofer M, Chavaz P, Saurat J, Borradori L . Adult-onset Still's disease with persistent plaques. Br J Dermatol. 1999; 141(4):710-3. DOI: 10.1046/j.1365-2133.1999.03115.x. View

Jeong W, Kim J, Heo S, Kim J, Yoo J . Atypical cutaneous features in a patient with adult-onset Still's disease. Korean J Intern Med. 2020; 36(3):742-743. PMC: 8137391. DOI: 10.3904/kjim.2019.389. View

Li S, Zheng S, Tang S, Pan Y, Zhang S, Fang H . Autoinflammatory Pathogenesis and Targeted Therapy for Adult-Onset Still's Disease. Clin Rev Allergy Immunol. 2019; 58(1):71-81. DOI: 10.1007/s12016-019-08747-8. View

Ota T, Higashi S, Suzuki H, Eto S . Increased serum ferritin levels in adult Still's disease. Lancet. 1987; 1(8532):562-3. DOI: 10.1016/s0140-6736(87)90204-2. View

Akkurt Z, Bozkurt M, Ucmak D, Turkcu G, Ucak H . Atypical cutaneous features in adult onset Still's disease. Indian J Dermatol Venereol Leprol. 2014; 80(3):250-3. DOI: 10.4103/0378-6323.132254. View

10.

Tomaras S, Goetzke C, Kallinich T, Feist E . Adult-Onset Still's Disease: Clinical Aspects and Therapeutic Approach. J Clin Med. 2021; 10(4). PMC: 7918550. DOI: 10.3390/jcm10040733. View

11.

Kikuchi N, Satoh M, Ohtsuka M, Yamamoto T . Persistent pruritic papules and plaques associated with adult-onset Still's disease: report of six cases. J Dermatol. 2014; 41(5):407-10. DOI: 10.1111/1346-8138.12426. View

12.

Tomaru K, Nagai Y, Ohyama N, Hasegawa M, Endo Y, Tamura A . Adult-onset Still's disease with prurigo pigmentosa-like skin eruption. J Dermatol. 2006; 33(1):55-8. DOI: 10.1111/j.1346-8138.2006.00011.x. View

13.

Lee J, Hsu C, Liu M, Chao S . Evanescent and persistent pruritic eruptions of adult-onset still disease: a clinical and pathologic study of 36 patients. Semin Arthritis Rheum. 2012; 42(3):317-26. DOI: 10.1016/j.semarthrit.2012.05.003. View

14.

Wakai K, Ohta A, Tamakoshi A, Ohno Y, Kawamura T, Aoki R . Estimated prevalence and incidence of adult Still's disease: findings by a nationwide epidemiological survey in Japan. J Epidemiol. 1998; 7(4):221-5. DOI: 10.2188/jea.7.221. View

15.

Giacomelli R, Ruscitti P, Shoenfeld Y . A comprehensive review on adult onset Still's disease. J Autoimmun. 2018; 93:24-36. DOI: 10.1016/j.jaut.2018.07.018. View

16.

Gerfaud-Valentin M, Jamilloux Y, Iwaz J, Seve P . Adult-onset Still's disease. Autoimmun Rev. 2014; 13(7):708-22. DOI: 10.1016/j.autrev.2014.01.058. View

17.

Magadur-Joly G, Billaud E, Barrier J, Pennec Y, Masson C, Renou P . Epidemiology of adult Still's disease: estimate of the incidence by a retrospective study in west France. Ann Rheum Dis. 1995; 54(7):587-90. PMC: 1009940. DOI: 10.1136/ard.54.7.587. View

18.

Cozzi A, Papagrigoraki A, Biasi D, Colato C, Girolomoni G . Cutaneous manifestations of adult-onset Still's disease: a case report and review of literature. Clin Rheumatol. 2014; 35(5):1377-82. DOI: 10.1007/s10067-014-2614-2. View

19.

Yamaguchi M, Ohta A, Tsunematsu T, Kasukawa R, Mizushima Y, Kashiwagi H . Preliminary criteria for classification of adult Still's disease. J Rheumatol. 1992; 19(3):424-30. View

20.

Fautrel B, Zing E, Golmard J, Le Moel G, Bissery A, Rioux C . Proposal for a new set of classification criteria for adult-onset still disease. Medicine (Baltimore). 2002; 81(3):194-200. DOI: 10.1097/00005792-200205000-00003. View