Atypical Skin Rash in a Patient with Adult-Onset Still's Disease: A Case Report

Abstract

Adult-onset Still's disease (AOSD) is an uncommon idiopathic disorder with various clinical manifestations. The absence of specific serological and pathological findings often makes the disease difficult to diagnose. The presence of skin lesions is important to the correct diagnosis of the disease. Various atypical skin lesions have been reported in association with Adult-onset Still's disease. We present a 52-year-old male who had atypical cutaneous manifestations of Adult-onset Still's disease. The rash manifested as persistent, pruritic, dark reddish, confluent, erythematous maculopapules and plaques on his chest, abdomen, upper back and proximal extremities. He suffered high, spiking fevers and had marked elevations of ferritin, C-reactive protein, and the erythrocyte sedimentation rate, which are characteristic of Adult-onset Still's disease. The fever and systemic symptoms improved after the administration of intravenous pulse methylprednisolone therapy. The skin lesions improved after prednisolone and methotrexate combination therapy. Therefore, to make the correct diagnosis, it is important to understand that patients with Adult-onset Still's disease may present with various types of skin lesions.

Cutaneous manifestations in a patient with adult-onset Still’s disease

“…The rash can be triggered by skin irritation, for example by rubbing it vigorously with a towel or wearing improper clothing, or by heat, such as when taking a hot bath [1]. W piśmiennictwie można znaleźć opisy różnych morfologicznie zmian w przebiegu AOSD, między innymi zmian rumieniowych, zmian linijnych z towarzyszącym świądem, grudek na podłożu rumieniowym, które mogą być pokryte łuskami, zmian pokrzywkowych, pokrzywkopodobnych, osutki pęcherzykowo-krostkowej przypominającej zmiany trądzikopodobne lub trwałych przebarwień [2,[6][7][8]. Do chorób dermatologicznych, które mogą towarzyszyć chorobie Stilla u dorosłych, zalicza się: obrzęk naczynioruchowy, zespół Sweeta, łysienie oraz guz- kowe zapalenie tętnic [7].…”

“…In the literature, there are reports of a variety of cutaneous manifestations associated with the disease, including erythematous lesions, linear changes accompanied by pruritus, rash consisting of papules on an erythematous base (with or without scaling), urticarial rash or urticaria-like skin lesions, vesiculopustular lesions resembling acne-like rash, and permanent skin discoloration [2,[6][7][8]. Dermatological conditions which may accompany adult-onset Still's disease include angioedema, Sweet's syndrome, alopecia, and polyarteritis nodosa [7]. However, it is not apparent from the literature review whether comorbidities are linked to Still's disease.…”

Extremely High Serum Ferritin: An Instrumental Marker of Masquerading Adult-Onset Still’s Disease with Hemophagocytic Syndrome

“…Various atypical rashes in AOSD have been reported, such as disseminated erythematous papular, verrucous plaques with vasculitic features, persistent papular characterized by noncaseating dermal granulomas, or peau d’orange-like skin infiltration due to cutaneous mucinosis, although Yamaguchi’s criteria includes only typical salmon-pink rash [ 5 , 22 – 24 ]. Our patient had only atypical rashes without the typical salmon-pink rash, which was a confusing aspect.…”

“…It is predominantly found on the proximal limbs and trunk [ 1 ]. However, various atypical rashes have been reported in some case studies of AOSD [ 5 ]. Moreover, there are some reports of AOSD without arthritis [ 6 ].…”

“…Moreover, there are some reports of AOSD without arthritis [ 6 ]. The diagnosis of atypical AOSD can be extremely difficult because of its non-specific serological and pathological findings in common with many other diseases, including infections, malignancies, and other connective tissue diseases, which should be excluded [ 1 , 5 , 7 ].…”