Persistent Plaques and Linear Pigmentation in Adult-Onset Still’s Disease

Abstract

A 25-year-old Japanese man presented with high spiking fever, arthralgia and a skin rash. A pruritic edematous erythema with persistent plaques was found mainly on the trunk; these lesions persisted even when the fever subsided, with prominent linear pigmentation. As marked neutrophilia and a high level of serum ferritin were detected, a diagnosis of adult-onset Still’s disease (AOSD) was made, even though the persistent eruption was not characteristic of the disease. Oral prednisolone, together with low-dose methotrexate, was given with good results. In the literature, a similar atypical rash has been reported in 11 cases in Japan. All of them required high-dose administration of corticosteroids or other immunosuppressive agents. Severe systemic complications were seen in 3 patients, and 2 cases died of the disease. Persistent plaques and linear pigmentation are some of the manifestations of AOSD, which cannot be overlooked. This appearance could be an indication that suggests an increased risk of systemic complications and a prolonged time to clinical remission.

Flagellate Erythema in a Patient With Fever

“…This variant manifests as nonevanescent pruritic erythematous papules and plaques with slight scale that often presents with a flagellate erythemalike presentation with linear configurations on the trunk and extremities. [4][5][6][7][8] The linearity may represent a Koebner phenomenon or scratching because the eruption characteristically spares the mid-back. Differential diagnosis of flagellate erythema includes bleomycin, dermatomyositis, and shiitake mushroom dermatitis.…”

“…9 This nonclassic variant also has unique histopathologic features including dyskeratosis in the upper layers of the epidermis extending into the stratum corneum without involvement of the basal layer and a superficial dermal infiltrate with neutrophils and lymphocytes. [4][5][6][7][8] While this nontypical variant was first described by Kaur et al 4 in 1994, it may be an underrecognized cutaneous manifestation of AOSD. In the largest case series of persistent pruritic papules and plaques published to date, Lee et al 6 found that 65% (11 of 17) of their patients with AOSD had this variant.…”

Adult-onset Still’s disease in an elderly woman with atypical presentation

“…However, recent findings show that not only typical evanescent rash but also various skin lesions are associated in AOSD [11][12][13][14][15] . The representative characteristic skin lesion among the non-classical skin rash is called persistent pruritic papules and plaques which presents erythematous, slightly scaly papules with linear configuration on the trunk and proximal extremities [12] . Other non-classical skin lesions include urticarial [13] .…”

“…Other non-classical skin lesions include urticarial [13] . Interestingly, persistent pruritic papules and plaques show unique histological features such as peculiar, distinctive distribution of dyskeratotic keratinocytes in the cornified layers as well as in the epidermis [12,14] . Our dermatologist diagnosed her cutaneous symptom as toxicoderma at the initial presentation which was improved by the prednisolone administration.…”

Adult-onset Still’s disease

“…Soy 23 Erythema chronicum migrans Saito et al 24 Persistent plaques and linear pigmentation Suzuki et al 25 Non-caseating dermal granulomas…”