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Zohreh Akhoundi Meybodi et al. ts, knees and ankles specially her left wrist and left good persistent clinical response. knee with limitation of range of motion and active synovitis. Other physical examinations were within DIScuSSIOn
Diagnosis of Adult-Onset Still's Disease (AOSD) is usually challenging especially among non-rheuma- tologists. Although we have useful clues to diagnosis of AOSD such as persistent fever; along with polyar- thritis that could be associated with skin rashes, sore throat and leukocytosis. Sore throat is remarkably diagnostic in AOSD that is presented in more than 90-80% of patients. Characteristically sore throat has no sign of local infection or inflammation. Elevation of liver enzymes, increased acute phase reactants (CRP, ESR) and very high titers of ferritin are useful lab indices of AOSD. Normal or low titer RF and ANA could be seen. Although the typical rash of AOSD is defined as an erythematous, non-persistent urticarial eruption with- out itching or minimally pruritic that occurs at night and during febrile episodes 2, but several atypical types of skin lesions were reported as well.6 Atypical rashes that are frequently reported were persistent pruritic maculopapular or papules and plaques, scaly papules, erythematous linear form, ur- ticaria rash and demographic lesions.5 Figure 1, persistent skin rash resembling dermographism
Paulo Ricardo Criado in 2011 reported 25 patients in our patient.
of AOSD with urticaria rash and demographic le- Her lab data showed high titer C-reactive protein sions who had a good clinical response to glucocor- (CRP) (95.3mg/dl) and increased erythrocyte sedi- ticoid and antihistamine agents. Dermatographism mentation rate (ESR) (80 mm/h). She had leukocyto- frequently was an accompaniment phenomenon in sis with neutrophilia; anemia, without thrombocyto- patients with AOSD. It could be seen frequently in penia and no evidence of microangiopatic hemolytic anemia. Rheumatoid factor (RF), Anti-nuclear anti- Dermatographism was also reported as a good clin- body (ANA) and brucellosis tests were all negative. ical sign in AOSD by Owlia et al. in 2008.3 Coagulation profile was normal. Blood and urine cul- In our patient, there was pruritic, scaly maculopap- tures were normal and was no evidence of bacterial, ular skin rashes with erythematous background con- fungal or viral infection. Chest radiograph was also verting to patches and plaques. The fixed red scaly lesions with irregular lines were like children's draw- At first, we thought that these new skin lesions are attributable to SSZ hypersensitivity. However hold- AOSD could be considered as RA variant, howev- ing the drug for a week along with high dose oral an- er, systemic signs and symptoms are severe. SSZ can tihistamine had no beneficial results. cause maculopapoler skin lesions but she had been According to previous history of RA, persistent given the drug for a long period of time without any skin rashes, leukocytosis and no finding of infection skin problem. Although, skin reactions may be seen and other differential diseases and no responses to in any course of treatment, however, persistent le- SSZ cessation, the diagnosis of AOSD was suggested sions even after discontinuation of drug therapy could for this patient. be due to condition other than drug hypersensitivity. On admission, the patient received intravenous Ong Ping Seung in 2011 reported one case that was pulsed methylprednisolone 500 mg and continued diagnosed and treated as RA who presented with sore with oral prednisolone 20 mg per day. throat, fever, arthritis, evanescent rash, raised liver She responded well and fever, arthritis and itchy le- enzymes and hyperferritinemia finally diagnosed as sions got improved, but symptoms recurred when oral AOSD after the exclusion of other potential differen- corticosteroid was getting tapered. Oral prednisone tial diagnoses.7 increased to 30 mg per day and hydroxychloroquine She was discharged with good general condition on Diagnosis of AOSD is a tricky one in more instanc- treatment with oral prednisolone 30mg per day then es and needs adequate clinical experience. Atypical was tapered to 5 mg, cyclosporine 50 mg, hydroxy- skin lesions in AOSD may mimic some drug hyper- chlorquine 200 mg/day and MTX 10 mg weekly with Journal of Case Reports in Practice (JCRP) 2014; 2(2): 48-50 Persistent pruritic skin rashes 4. Pouchot J, Sampalis JS, Beaudet F, et al. Adult 1. Elsa lanty ME, Genecov DG. Bone grafts in cra- Still's disease: manifestations, disease course, and niofacial surgery.Craniomaxillofac Trauma Reconstr. outcome in 62 patients. Medicine 1991;70:118-36.
2009; 2(3): 125–134.
5. Yamamoto T. Cutaneous manifestations associated 1. Dechant C, Kruger K. [Adult-onset Still's disease]. with adult-onset Still's disease: important diagnostic Dtsch Med Wochenschr 2011;136:1669-73.
values. Rheumatology international 2012;32:2233-7.
2. Owlia MB, Mehrpoor G. Adult-onset Still's dis- 6. Criado PR, de Carvalho JF, Ayabe LA, Brandt HR, ease: a review. Indian journal of medical sciences Romiti R, Maruta CW. Urticaria and dermographism in patients with adult-onset Still's disease. Rheuma- 3. Mehrpoor G, Owlia MB, Soleimani H, Ayatollahi tology international 2012;32:2551-5.
J. Adult-onset Still's disease: a report of 28 cases and 7. Seung OP, Sulaiman W. Adult-Onset Still's Dis- review of the literature. Modern rheumatology / the ease: A Case Report. Oman medical journal 2011;26.
Japan Rheumatism Association 2008;18:480-5.
Journal of Case Reports in Practice (JCRP) 2014; 2(2): 48-50

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