What are some other connective tissue diseases with cutaneous manifestations? Autoimmune urticaria: May affect up to 30% or more of chronic idiopathic urticaria patients (>6 weeks duration of urticaria) with associated autoantibodies to the high-affinity IgE receptor or, less frequently, against IgE. - Juvenile idiopathic arthritis: High episodic fever daily for 2 weeks with symmetrical polyarthritis or oligoarthritis, as well as one of the following: exanthem, generalized adenopathy, hepatosplenomegaly, serositis.
- Adult-onset Still’s disease: Recurrent episodes of high spiking fevers, frequently in the late afternoon; a salmonpink exanthem that may demonstrate Koebnerization; arthritis with subsequent carpal ankylosis is characteristic.
- Sjögren’s syndrome: Xerostomia and xerophthalmia; arthritis; petechiae and purpura, urticarial vasculitis, and annular erythema.
- Relapsing polychondritis: Patients present with inflammation of the cartilaginous portions of the ear and nose, eventually causing a deformed ear or saddle-nose deformity. Acute involvement of the tracheal cartilage may cause collapse of the airway. Arthritis most often involves the costochondral joints. Other manifestations include audiovestibular damage, heart valve disease, and neurologic, ocular, and renal disease. Patients may die of rupture of the chordae tendineae of the heart valves.
- Rheumatoid arthritis: Patients present with arthritis. Cutaneous manifestations include rheumatoid nodules, rheumatoid neutrophilic dermatitis, palisaded neutrophilic and granulomatous dermatitis, leukocytoclastic vasculitis, and pyoderma gangrenosum.
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