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Journal of Case Reports in Practice (JCRP) 2014; 2(2): 48-50 CASE REPORT
Persistent pruritic skin rashes masquerading sulfasalazine sensitivity in
a newly diagnosed Adult-Onset Still's Disease (AOSD)
ohreh Akhoundi Meybodi 1 and SinA owliA 1Department of Medicine, Shahid Sadoughi University of medical sciences, Yazd, Iran Adult-Onset Still's Disease (AOSD) is a rare clinical entity with unknown etiology characterized by arthritis, fever, evanescent rash and other systemic presentations. This report describes a 30-year-old woman presented first with classic picture of rheumatoid arthritis since six years ago on oral prednisolone, sulfasalazine and methotrex- ate and was recently admitted with refractory sore throat, fever, arthritis, persistent pruritic rash and leukocytosis. Adverse drug reactions to sulfasalazine was the working clinical diagnosis. Discontinuation of sulfasalazine did not show any clinical benefit. The patient responded fully to combine pulsed methylprednisolone and higher doses of oral prednisolone and systemic disease-modifying anti-rheumatic drugs.
Key words: adult-onset Still's disease, persistent pruritic rash, dermatographism, rheumatoid arthritis, dermogra-
We reported here a case of AOSD rash that was Adult-Onset Still's Disease (AOSD) is a rather subject to a misdiagnosis of sulfasalazine hypersen- rare systemic inflammatory disorder characterized by fever, cutaneous eruption and arthralgia or arthritis. Sore throat, lymphadenopaty, hepatomegaly, spleno- cASE rEPOrt
megaly, elevation of serum ferritin and leukocytosis We report a 30-year-old woman; a known case of are seen in absence of rheumatoid factor and antinu- chronic rheumatoid arthritis (RA) with recent joint clear antibodies.1,2 exacerbation and pruritic maculopapular rash in- The first definition of AOSD in adult patient with volving trunk and extremities. Systemic symptoms signs and symptoms of this, was reported in 1896. including fever, malaise, sweating, sore throat, an- Then in 1897 George Still described it in 22 patients orexia, generalized myalgia, headache and arthralgia with similar presentation, and used the term AOSD.3 of shoulders, elbows, wrists, interphalangeal joints, Genetic factors and various infectious agents have knees and ankles has been developed 10 days before been postulated as possible predisposing factors. In a admission. Searching for an occult infection did not series of 62 patients, an association between Human show any source. Intense pruritus did not respond Leukocytes Antigen (HLA) and AOSD was reported.4 to oral hydroxyzine 25 mg three times a day so oral Differential diagnoses of AOSD are wide and in- prednisolone increased to 30 mg/day for 5 days. clude infectious, neoplastic, and other collagen vas- She has had seronegative RA since 6 years ago and cular disorders namely systemic lupus erythematosus, has been treated with low dose oral prednisolone and which should be ruled out before diagnosing AOSD.3 methotrexate (MTX) 10mg/week and SSZ 1000 mg Classic cutaneous manifestations are transient salm- on-colored maculopapular patches without itching On physical examination, she was ill and febrile but or minimally pruritic. Dermographism and intensely not toxic. She looks a little pale but not icteric. Her pruritic and erythematous papules/plaques are report- body temperature was 39°C orally. She had a pulse ed frequently.1,5 rate of 100 beats/min and a blood pressure of 115/65 Sulfasalazine (SSZ) is a frequently used agent in mmHg. Her respiratory rate was 18/min. Her throat treatment of rheumatoid arthritis (RA) with cutane- was not congested. No lymphadenopathy and hepato- ous reactions as a common adverse reaction, howev- splenomegaly were observed.
er, these demonstrations are easily differentiated from There were pruritic maculopapular skin rashes with typical skin rashes in AOSD in most cases. erythematous background involving the trunk, neck and extremities developing to patches and plaques with typical dermatographism (Fig. 1) Shahid Sadoughi University of medical sciences, School Musculoskeletal examination revealed tenderness of Medicine, Yazd 8915173143, Iran. on her shoulders, elbows, wrists, interphalangeal join-

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.
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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.
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Journal of Case Reports in Practice (JCRP) 2014; 2(2): 48-50

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