Adult-Onset Still’s Disease with Distinctive Skin Signs and Elevated LDH

Adult-onset Still's disease (AOSD) is a rare, inflammatory disorder characterised by the appearance of fever and salmon-patch like lesions, that may affect multiple organs and joints, including the liver and spleen.The disease responds to the administration of steroids, although biological agents have been used in the treatment of recalcitrant cases.Recently, some cases presenting atypical skin signs have been reported in literature.Herein, we describe such a case which, apart from the typical salmon-patch rash, presents erythematous urticarial papules lesions that coalesce to form plaques in the extremities.In addition, elevated LDH in the serum is a laboratory finding suggestive of a worse prognosis.A 45-year-old woman was hospitalised in the Department of Internal Medicine with a 39 o C fever and a rash that had appeared 4 days prior to the consultation.No intake of medications was reported by the patient.Their family history includes systemic lupus erythematosus in the mother and coeliac disease in two of the children -a son and a daughter.A dermatological consultation was requested.The clinical examination revealed salmon-like patches on the back (Figure 1) and erythematous papules that coalesce to form urticarial-like plaques on both thighs (Figure 2).Pruritus and arthralgias were not reported.The laboratory findings were leukopenia, abnormal hepatic enzymes, abnormal ESR, abnormal CRP.Haemocultures and urine cultures were sterile.The strep test from the throat and antistreptolysin-O (ASTO) were negative.Serology for Ebstein-Barr, Coxsackie, Echo, HIV1,2 and COVID-19 also proved negative.Anti-nuclear antibody and rheumatoid factor were negative (Table 1).Thorax radiography was normal.Echography of the abdomen revealed splenomegaly.