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Juvenile rheumatoid arthritis (JRA) is a generalized systemic disease of unknown etiology characterized by a transient macular, papular, or urticarial rash and ensuing fever, lymphadenopathy (LAD), hepatosplenomegaly (HSM), anemia, and arthralgia.


There are four classical evanescent eruptions: (1) urticaria, (2) erythema marginatum (of acute rheumatic fever), (3) the rash of Still's disease, and (4) serum sickness.


JRA has three major types:

  1. Systemic onset (15%): Still's disease, fevers, rash, LAD, HSM, serositis, polyarthritis

  2. Polyarticular (35%): Five or more joints: hands, feet > knees/wrists/ankles

  3. Oligo-/Pauciarticular (50%): Four or fewer joints: knees > ankles

SYNONYMS Still's disease, juvenile idiopathic arthritis, juvenile chronic arthritis.


AGE Peaks: 1 to 4 years; adolescence.

GENDER Systemic JRA: M = F. Others: F > M, 2:1.


GENETICS Polymorphisms for the genes encoding tumor necrosis factor-α (TNF-α), migratory inhibitory factor, interleukin-6 (IL-6), interleukin-10 (IL-10), and TAP genes (transporter of antigenic peptides) have been detected in this patient population. Several HLA alleles have also been associated with the subtypes of JRA, with the strongest genetic associations seen in early-onset pauciarticular JRA.



Cytokines TNF-α, migratory inhibitory factor, and IL-6 parallel the fever spikes, but the pathogenesis of JRA is still not understood. JRA tends to be precipitated by emotional, infectious, or surgical stress.


The onset of JRA may be sudden or insidious, depending on the age of the patient (the younger the patient, the more severe the systemic manifestations on average). Cutaneous eruptions occur in 90% of patients and may be the initial presentation. The rash of JRA is evanescent and can be macular or urticarial. Systemically, there may be associated fever, adenopathy, splenomegaly, anemia, and arthralgias.


Skin Findings

TYPE Macules, papules, urticarial plaques (Fig. 17-1).

Juvenile rheumatoid arthritis

Transient macular rash on the body characteristic of juvenile rheumatoid arthritis.

SIZE 2–6 mm to 8–9 cm.

COLOR Salmon pink to red, with a zone of pallor (Fig. 17-2).

Juvenile rheumatoid arthritis

Faint erythematous, urticarial plaques on the torso of a child.

DISTRIBUTION Areas of trauma/heat: axilla, waist, olecranon process/ulnar forearm, dorsal hands, knees, ears, scapula, sacrum, buttocks, and heels.

OTHER Palms and soles: thenar and hypothenar eminences may be erythematous. Periungual telangiectasias (5% of patients). Spindling fingers (50%, spindle-shaped deformity of fingers because proximal interphalangeal involvement > distal interphalangeal involvement).


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