A Pruritic Linear Urticarial Rash, Fever, and Systemic Inflammatory Disease in Five Adolescents: Adult‐Onset Still Disease or Systemic Juvenile Idiopathic Arthritis sine Arthritis?

Abstract

The characteristic rash of systemic juvenile idiopathic arthritis is a transient erythematous eruption associated with a quotidian spiking fever. Usually asymptomatic, it can be pruritic, with dermatographism at sites of scratching or pressure. An illness similar to this entity in adults is designated adult-onset Still disease. The relationship between the pediatric and adult disease is uncertain and differences in case definition have evolved. Specifically, a sustained arthritis for at least 6 weeks is required for a diagnosis of systemic juvenile idiopathic arthritis, whereas transient arthritis and arthralgia are accepted criteria in adult-onset Still disease. We describe five patients less than 16 years of age who presented with an acute illness characterized by fever and a distinctive skin eruption. Intense pruritus and linear erythematous lesions flared with a spiking fever, usually in the late afternoon and evening. Periorbital edema/erythema and nonlinear urticarial lesions were also seen. Two children had splinter hemorrhages of the nail beds and one girl developed a fixed, scaling, pigmented, linear eruption. Severe malaise, myalgia, arthralgia, and leukocytosis were present in every patient. Other systemic manifestations included sore throat, transient arthritis, abdominal pain, lymphadenopathy, hepatomegaly, splenomegaly, hyperferritinemia, and hepatic dysfunction. No patient had a sustained arthritis. The course of the disease was variable. One patient, diagnosed with macrophage activation syndrome, recovered on oral naproxen. Two patients responded to systemic corticosteroid therapy. One girl developed status epilepticus and died from aspiration and asphyxia. A boy with severe hepatitis developed renal failure and thrombotic thrombocytopenic purpura and was treated with plasmapheresis, dialysis, and systemic corticosteroids; he had recurrent episodes of rash and fever into adult life. These children did not fulfill the case definition of systemic juvenile idiopathic arthritis because they lacked a persistent arthritis. Adolescent and adult patients with the same clinical and laboratory findings are described under the rubric of adult-onset Still disease. Recognition of the distinctive urticarial skin eruption and spiking fever is important in the diagnosis of a disease with severe morbidity and potentially life-threatening complications.

Urticaria and dermographism in patients with adult-onset Still’s disease

“…The three reported cases had atypical cutaneous eruption. Patients 1 and 3 presented persistent urticarial lesions, a rare manifestation previously reported in a few cases [8,[14][15][16][17][18][19][20][21][22]. Patient 2 had urticarial papules and persistent erythema which was aggravated during fever spikes.…”

“…However, only about 25 cases of AOSD are described in the literature which had a clinical presentation with urticaria or angioedema [8,[14][15][16][17][18][19][20][21][22].…”

Unique Histopathologic Findings in a Patient With Adult-Onset Still Disease

“…[3][4][5] A recent case series demonstrated that it is characterized by necrotic keratinocytes in the mid and upper epidermis, including in the cornified layer. 2 In addition, a separate case report has noted marked diffuse mucinosis, confirmed with Alcian blue staining, that corresponded to similar clinical findings on the upper arms and proximal thighs.…”

Adult-onset Still’s disease: a report of 28 cases and review of the literature

“…In some studies, dermatographism and Köebner phenomenon have been described as objective features of AOSD [5,6,9,19]. Various atypical rashes have been reported in AOSD such as persistent dermal plaques, [20][21][22][23] urticaria [19,24], and vesicolupustular lesions on the hands and feet [25].…”

“…Dermatographism could be seen in 1.5-5% of the healthy population [26,27]. Pathogenesis of dermatographism is unclear [19]. It is usually idiopathic, but it may be associated with systemic use of penicillin, famotidin, and atorvastatin [27,28].…”