Adult-onset Still disease with peculiar persistent plaques and papules

Abstract

Adult-onset Still disease (AOSD) is a systemic inflammatory disorder characterized clinically by high spiking fever, polyarthralgia/arthritis, a salmon-pink evanescent rash, predominantly neutrophilic leucocytosis, lymphadenopathy, liver dysfunction, and splenomegaly. Recently, a nonclassic, nonevanescent skin rash has been reported. We report a 27-year-old woman with AOSD showing persistent pruritic papular lesions. Histologically, dyskeratotic keratinocytes were seen in the upper epidermis. We describe this case in detail and review the previous literature. Nonclassic pruritic eruptions with characteristic dyskeratotic keratinocytes might provide an important clue for the diagnosis of AOSD.

Adult-onset Still's disease with atypical cutaneous manifestations

“…However, in recent years, other atypical cutaneous manifestations of AOSD have been reported; these rare presentations are not routinely recognized [6–38] Atypical cutaneous features often present in addition to the typical evanescent rash, but in 43% of the cases they are the only skin manifestation. [6,8,11,14,15,17,25,28–33,37,38] . A delayed diagnosis in these cases is still common, as the cutaneous lesions are often misdiagnosed as an allergic reaction to drugs, usually NSAIDs prescribed for joint symptoms or fever.…”

“…Thus, most patients required medium or high doses of glucocorticoids (including intravenous methylprednisolone pulse therapy in some cases) and, in nearly 40%, a more potent or maintenance immunotherapy consisting of immunosuppressant drugs (including methotrexate, azathioprine, cyclosporine A, and hydroxychloroquine) [6,8–10,12,15,16,18–20,24,25,28,29,31,34,39,40]  and/or biologic agents (mainly anakinra or tocilizumab) [8,10,15,21] to control or manage symptoms because they had an intermittent/polycyclic or chronic systemic course. The development of atypical cutaneous manifestations seems to be associated with a potentially worse prognosis (especially those with persistent pruritic papules and plaques with dermatomyositis-type appearance), with a mortality rate that reached 8% primarily because of infectious complications related to the immunosuppressive therapy [6,10,24] .…”

Typical evanescent and atypical persistent polymorphic cutaneous rash in an adult Brazilian with Still’s disease: a case report and review of the literature

“… Prednisone, Chloroquine, Methotrexate, Nagai et al [ 34 ] 18/16-60/F Fever, arthralgia, sore throat, splenomegaly, lymphadenopathy Leukocytosis, increased CRP, liver enzymes & ferritin Evanescent salmon-pink maculopapular eruption, persistent papules and plaques with linear erythema similar to prurigo pigmentosa, edema of eyelids mimicking dermatomyositis Parakeratosis and necrotic keratinocytes in epidermis, inflammatory infiltrates of lymphocytes in the papillary and mid-dermis Corticosteroids, Methotrexate, Mizoribine Cyclosporin, Cyclophosphamide Lee et al [ 14 ] 30/17-67/F 6/17-67/M Fever, arthralgia, sore throat, splenomegaly, lymphadenopathy Leukocytosis, elevated liver enzymes & ferritin Evanescent rash, persistent pruritic urticarial, violaceous papules and plaques, dermatographism-like, prurigo pigmentosa-like and dermatomyositis-like eruption on trunk, neck, face, limbs Normal epidermis with perivascular infiltrate of neutrophils, nectrotic keratinocytes in upper epidermis. NSAIDs, Corticosteroids, Methotrexate, Azathioprine Yoshifuku et al [ 16 ] 1/27/F Fever, polyarthralgia, sore throat, hepatosplenomegaly lymphadenopathy Leukocytosis, increased CRP, elevated liver enzymes & ferritin Pruritic pigmented erythematous plaques and dark-reddish papules on abdomen and back Mild hyperkeratosis, and presence of dyskeratotic keratinocytes in upper epidermis Corticosteroids, Cyclosporin, Said et al [ 37 ] 1/23/M Fever, sore throat, myopericarditis, arthralgia, hepatosplenomegaly Leukocytosis, increased ESR &CRP, elevated ferritin, raised cardiac enzymes Urticated and erythematous plaques and papules on the dorsum of right hand and fingers Superficial and deep perivascular infiltrates of lymphocytes and neutrophils Corticosteroids, NSAIDs, Azathioprine Cho et al [ 24 ] 1/38/F Fever, polyarthralgia, sore throat hepatosplenomegaly lymphadenopathy Leukocytosis, elevated liver enzymes & ferritin …”

Wissler-Fanconi syndrome and related diagnoses: a case report

“…Two or more criteria must be major, which are: fever >39°C lasting ≥1 week, arthralgia or arthritis lasting ≥2 weeks, typical rash, and leukocytosis >10,000/mm 3 with >80% polymorphonuclear cells. Minor criteria are sore throat, recent development of significant lymphadenopathy, hepatomegaly or splenomegaly, abnormal liver function tests, and negative tests for antinuclear antibody and rheumatoid factor (immunoglobulin M) 4,5. Historically, in children with fever and polyarthritis, the differentiation between Still’s disease and rheumatic fever was of particular diagnostic importance because of the requirement for prophylactic antibiotic drugs in cases of acute rheumatic fever.…”