Clinical follow‐up study of adult‐onset Still’s disease

Abstract

Eighteen patients with adult-onset Still's disease have been followed up for 3-22 years in our department. Initial manifestations were fever with skin rash in 14 patients, fever, skin rash and sore throat in two, skin rash in one and arthralgia in one. During the follow-up period, typical skin rash was seen in all patients, of them five patients (29%) revealed atypical skin rash simultaneously. Atypical rash included persistent erythema with pigmentation in two, persistent plaques and papules with linear erythema in two and edema of the eyelids mimicking dermatomyositis in one. Persistent papules and plaques revealed histologically characteristic features, such as dyskeratotic keratinocyte and liquefaction degeneration as well as a sparse superficial dermal infiltrate containing scattered neutrophils. In patients of chronic articular type and polycyclic systemic type, atypical skin rash, lymphadenopathy and hyperferritinemia were noted to be significantly higher than those of monocyclic type. These factors might be prognostic factors of adult-onset Still's disease in our study.

Atypical cutaneous features in adult onset Still′s disease

“…[7,12,13] Although the typical rash has a relatively high sensitivity and specificity, some forms of atypical rashes may have prognostic significance. For instance, persistent plaques, linear pigmentation and dermatomyositis-like eruptions have been associated with a more severe clinical course [12,14] and suggested to be a clinical marker of disease severity.…”

Adult-Onset Still's Disease: Persistent Pruritic Erythematous Plaques and Papules

“…6 Nagai et al reported that five of 80 patients (29%) with AOSD revealed atypical skin rash. 7 Persistent pruritic rash of histopathological findings show dyskeratosis confined to the upper epidermis and superficial dermal infiltrate containing scattered neutrophils. [7][8][9] Skin biopsy was not obtained from the patient.…”

“…7 Persistent pruritic rash of histopathological findings show dyskeratosis confined to the upper epidermis and superficial dermal infiltrate containing scattered neutrophils. [7][8][9] Skin biopsy was not obtained from the patient. Laboratory findings in AOSD are similar to those in other inflammatory events including leucocytosis (mostly neutrophils), anemia, acute phase proteins (CRP, ESR, liver enzymes and ferritin), which were present in our patient.…”

“…The different cutaneous features such as pruritic eruptions associated with AOSD have been reported as case report or case series. [5][6][7][8][9] The prevalance of atypical cutaneous features among AOSD patients was 14% in a review. 6 Nagai et al reported that five of 80 patients (29%) with AOSD revealed atypical skin rash.…”

“…Various atypical cutaneous manifestations have been reported among AOSD patients, persistent pruritic eruptions such as urticarial erythema, flagellate erythema,erythematous papules,and /or plaques on trunk and extremities may occur in some patients. [5][6][7][8][9] The recognition of this clinical variant is necessary for diagnosis.…”