Other Rheumatologic Diseases

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Other Rheumatologic Diseases

Systemic-Onset Juvenile Idiopathic Arthritis (SoJIA; Still’s Disease) and Adult-Onset Still’s Disease (AoSD)

• Among the major types of juvenile idiopathic arthritis (JIA; formerly juvenile rheumatoid arthritis [JRA]), cutaneous manifestations are most common in SoJIA, psoriatic arthritis (see Chapter 6), and rheumatoid factor (RF)-positive polyarthritis (rheumatoid nodules and other findings similar to rheumatoid arthritis [RA]; see below).

• SoJIA can develop at any age prior to 16 years and affects both sexes equally; AoSD has peaks in the second and fourth decades and affects women more often than men.

• Both SoJIA and AoSD are characterized by daily spiking fevers (especially in the late afternoon/early evening) accompanied by an evanescent eruption of salmon-pink macules and slightly edematous papules and plaques (Fig. 37.1A,B); these lesions are usually asymptomatic and favor sites of pressure or trauma, often occurring in a linear array.

Fig. 37.1 Cutaneous findings in Still’s disease. A Evanescent pink papules and plaques in a child. Note the linear array of some of the lesions. B Multiple pink macules and urticarial papules that developed during a fever spike in a woman. C Flagellate array of pinkish-tan, scaly persistent plaques on the upper back in a teenage girl. A, Courtesy, Carlos Nousari, MD; B, Courtesy, Diane Davidson, MD; C, Courtesy, Julie V. Schaffer, MD.

• Less common skin findings include periorbital edema and persistent pruritic papules and plaques that are scaly, violaceous to reddish brown in color, and linear in configuration (Fig. 37.1C).

• Additional features include a prodromal sore throat and arthralgias/myalgias, arthritis (usually polyarticular; ± carpal ankylosis in AoSD), lymphadenopathy, hepatosplenomegaly, and serositis; occasionally patients may develop macrophage activation syndrome (also characterized by markedly elevated ferritin).

• Leukocytosis with neutrophilia, thrombocytosis, anemia, elevated ESR/CRP, and extremely high serum ferritin levels (e.g. >4000 mg/ml) are common laboratory findings, whereas ANA and RF are usually absent.

• DDx: infections (e.g. parvovirus B19), rheumatic fever, serum sickness-like reactions, urticarial vasculitis, Schnitzler’s syndrome (in adults; see Chapter 14), other autoimmune connective tissue diseases, hereditary periodic fever syndromes (see Table 3.2).

• Rx: NSAIDs (for mild disease), systemic CS, methotrexate, antagonists of IL-1 (e.g. anakinra, canakinumab) or IL-6 (e.g. tocilizumab), and TNF inhibitors (the latter especially for arthritis).

Rheumatoid Arthritis

• Chronic inflammatory disorder characterized primarily by destructive arthritis.

• Affects 1–3% of adults, with a female : male ratio of 2–3 : 1 and a peak onset between 35 and 60 years of age.

• RF and anti-cyclic citrullinated peptide (CCP) antibodies represent serologic markers of RA.

• Cutaneous manifestations of RA are presented in Table 37.1 and Fig. 37.2; these findings can serve as diagnostic clues or signs of serious systemic disease.

Fig. 37.2 Cutaneous findings in rheumatoid arthritis. A Rheumatoid nodules in a periarticular location on the hands. B Rheumatoid papules presenting as coalescing red-brown lesions, some with scale-crust, on the lower extremity. C Superficial ulcerating rheumatoid necrobiosis manifesting as shiny, yellow-brown plaques with red-brown borders and areas of ulceration; these clinical findings are reminiscent of necrobiosis lipoidica, although both this entity and rheumatoid papules are categorized as forms of palisaded neutrophilic and granulomatous dermatitis. D Pyoderma gangrenosum resulting in recurrent ulcerations on the lower extremities. E Bywater’s lesions. These tender purpuric papules on the distal fingers are characterized histologically by leukocytoclastic vasculitis. F Methotrexate-induced nodulosis. A, Courtesy, Kalman Watsky, MD; B, C, E, Courtesy, Jeffrey Callen, MD; D, Courtesy, Carlos Nousari, MD; F, Courtesy, Jean L. Bolognia, MD.

Interstitial Granulomatous Dermatitis (IGD) and Palisaded Neutrophilic and Granulomatous Dermatitis (PNGD)

• Two clinicopathologic patterns of granulomatous dermatitis that occur in patients (women > men) with RA and other autoimmune disorders.

• IGD presents as annular erythematous plaques or linear cords (‘rope sign’) that favor the lateral and upper trunk, axillae, medial thighs, and buttocks (Fig. 37.3); patients often have RA or seronegative arthritis.

Fig. 37.3 Interstitial granulomatous dermatitis (IGD). A Large, symmetric pink patches and thin plaques that resemble patch-type granuloma annulare. B Annular lesions on the medial thighs. C Firm linear cord along the axillary line in a patient with rheumatoid arthritis. A, C, Courtesy, Kathryn Schwarzenberger, MD; B, Courtesy, Jeffrey Callen, MD.

• PNGD presents as skin-colored to erythematous, umbilicated papulonodules that are often crusted and favor the elbows (see Fig. 19.11B) and extensor hands/fingers; associated with RA, SLE, and ANCA-positive systemic vasculitides (e.g. Churg–Strauss syndrome).

• Because interstitial granulomatous drug reactions can have clinical and histologic features similar to those of IGD and PNGD, a review of all medications should be performed; culprit drugs include calcium channel blockers, angiotensin-converting enzyme inhibitors, statins, and TNF inhibitors.

• Histologically, a dense pandermal infiltrate is seen in both entities; IGD features rosettes of palisading histiocytes surrounding tiny foci of degenerated collagen, while findings in PNGD range from a predominance of neutrophils with foci of leukocytoclastic vasculitis to well-developed palisaded granulomas surrounding basophilic degenerated collagen.

• DDx: interstitial granulomatous drug reaction (see above); for IGD – patch-type granuloma annulare (GA), morphea (inflammatory stage), mycosis fungoides and leprosy; for PNGD – papular GA, rheumatoid nodules/nodulosis, perforating disorders.

• Rx: high-potency topical or intralesional CS, dapsone (if prominent neutrophils); may improve with treatment of the underlying disorder.

Sjögren’s Syndrome

• Autoimmune disorder primarily affecting the lacrimal and salivary glands, resulting in xerophthalmia and xerostomia; may coexist with other AI-CTDs (e.g. LE).

• Affects ~0.5% of the general population, with a female : male ratio of 9 : 1; peak onset is in the fourth and fifth decades of life, but can occur at any age.

• Cutaneous manifestations include xerosis, palpable purpura due to small vessel vasculitis (including cryoglobulinemic vasculitis), hypergammaglobulinemic purpura of Waldenström, urticarial vasculitis, annular erythema, and Raynaud’s phenomenon.

• Additional features can include vaginal dryness, arthritis, peripheral neuropathy, internal organ involvement (e.g. kidneys), and development of B-cell lymphoma (often extranodal marginal zone).

• Majority of patients have anti-Ro/SS-A and anti-La/SS-B antibodies, a positive RF, and an elevated ESR.

• Diagnostic criteria for Sjögren’s syndrome are presented in Table 37.2.

Table 37.2

American College of Rheumatology 2012 classification criteria for Sjögren’s syndrome.

Other possible causes of similar findings include chronic GVHD, primary systemic amyloidosis, sarcoidosis, and radiation therapy of the head and neck.

^* Using fluorescein and lissamine green to stain the cornea (score of 1–6) and conjunctiva (score of 1–3), respectively; other assessment methods include Schirmer and Rose Bengal tests.

• Rx: mostly symptomatic – use of artificial tears and saliva (e.g. methylcellulose drops), chewing sugar-free gum, meticulous dental hygiene, and treatment of superimposed oral candidiasis; sicca symptoms may improve with cyclosporine eye drops or muscarinic receptor agonists (e.g. pilocarpine), and vasculitis or internal involvement often require systemic CS, conventional immunosuppressive agents, or rituximab.

Relapsing Polychondritis

• Uncommon autoimmune condition that affects cartilaginous structures; pathogenesis is thought to involve a reaction against type II collagen.

• Most patients develop erythema, swelling and pain of the cartilaginous portion of the auricle, with sparing of the earlobe (Fig. 37.4A).

Fig. 37.4 Cutaneous findings in relapsing polychondritis. A Erythema and swelling of the ear with sparing of the earlobe. B Small vessel vasculitis presenting as palpable purpura in a patient who also had myelodysplastic syndrome. A, Courtesy, Kalman Watsky, MD; B, Courtesy, Jean L. Bolognia, MD.

• Other manifestations include cutaneous small vessel vasculitis (Fig. 37.4B), aphthae (oral or genital; may overlap with Behçet’s disease), nasal chondritis (potentially producing a saddle nose deformity), involvement of cartilage of the respiratory tract (e.g. larynx, trachea, bronchi) and costochondral junctions, arthritis, ocular inflammation, audiovestiblar damage, and myelodysplastic syndrome.

• DDx: early phase – erysipelas, cellulitis, infectious chondritis or zoster; nasal destruction – Wegener’s granulomatosis, nasal natural killer/T-cell lymphoma, infections (e.g. mucocutaneous leishmaniasis) (see Table 19.3).

• Rx: prednisone (for acute flares), dapsone, immunosuppressive agents, TNF inhibitors; surgical repair of damaged cartilaginous structures.