Cutaneous Manifestations of Rheumatoid Arthritis: Diagnosis and Treatment
Abstract
:1. Introduction
2. Methods
3. Results
3.1. Nodules
3.1.1. Rheumatoid Nodules
3.1.2. Rheumatoid Nodulosis
3.1.3. Accelerated Rheumatoid Nodulosis
Condition | Etiology | Histology | Affected Areas | Treatment |
---|---|---|---|---|
Rheumatoid nodules | -Positive RF -Smoking | -Palisading macrophages -Granulation tissue | -Hands, fingers, elbows, feet | -SSZ, TCZ, RIX, colchicine, DPA -Corticosteroid injections -Surgery |
Accelerated rheumatoid nodulosis | -MTX use -HLA-DRB1*0401 | -Palisading macrophages -Granulation tissue | -Hand, feet, ear | -Stop triggering drug -HCQ, DPA, SSZ, colchicine |
Rheumatoid nodulosis | -No erosive arthritis -Negative RF | -Palisading macrophages -Granulation tissue | -Pretibial areas, feet, scalp, malleoli | -Prednisone, NSAIDs -Topical TAC, HCQ, DPA, PN -Surgery |
3.2. Neutrophilic Dermatoses
3.2.1. Sweet Syndrome
3.2.2. Pyoderma Gangrenosum
3.2.3. Rheumatoid Neutrophilic Dermatitis
3.2.4. Palisaded Neutrophilic Granulomatous Dermatitis and Interstitial Granulomatous Dermatitis
Diagnosis | Clinical Characteristics | Histological Characteristics | Differential Diagnosis | Treatment |
---|---|---|---|---|
Sweet Syndrome | Acute-onset tender plaques or nodules, fever, arthralgia, ophthalmologic manifestations, headaches, oral or genital lesions | -Diffuse, dense neutrophilic infiltrate in the reticular dermis with interstitial leukocytoclastic nuclear debris -Epidermis often spared, but spongiosis and subcorneal pustules may be present | -Clinically: infection -Histologically: bowel bypass syndrome, erythema elevatum diutinum, granuloma faciale, halogenoderma, leukemia cutis, leukocytoclastic vasculitis, lobular neutrophilic panniculitides, neutrophilic eccrine hidradenitis, pyoderma gangrenosum, rheumatoid neutrophilic dermatitis | -First-line: systemic or topical corticosteroids, potassium iodide, colchicine -Second-line: indomethacin, clofazimine, cyclosporine, dapsone -Reported success in refractory cases: rituximab, anti-TNF-α agents, IL1 receptor antagonists, and granulocyte and monocyte adsorption apheresis |
Pyoderma Gangrenosum | -Painful skin ulcers with undermined borders and peripheral erythema -Lesions can rapidly progress to blistered or necrotic ulcers | Intense neutrophilic infiltrate with neutrophilic pustules and abscess formation | Clinically: other causes of cutaneous ulceration including infections, malignancy, vascular ulceration, and systemic conditions such as systemic lupus erythematosus or Wegner’s granulomatosis -No definitive histopathological criteria | -Treatment includes anti-inflammatory therapies and proper wound care -First-line: fast-acting immunosuppressive drugs including topical or systemic corticosteroids and/or cyclosporine -Second-line: infliximab, other biologic TNF-α inhibitors, dapsone, minocycline -Refractory disease: IV immune globulin and alkylating agents |
Rheumatoid neutrophilic dermatitis | Erythematous papules, nodules, plaques, and urticaria-like lesions | Dermal neutrophilic infiltrate with variable degree of leukocytoclasis in the absence of vasculitis | -Clinically: Sweet syndrome, pyoderma gangrenosum, Behcet disease, bowel bypass syndrome, rheumatoid nodules -Histologically: Sweet syndrome (difficult to distinguish based on similar histopathologic presentations) | -Topical or systemic corticosteroids, dapsone or colchicine -Efficacy demonstrated with cyclophosphamide and hydroxychloroquine |
PNGD and IGD | -PNGD: symmetric smooth, umbilicated, or crusted papules, often on the elbows and extremities with skin to erythematous color -IGD: shared clinical features with PNGD, with locations favoring the lateral trunk and skin folds | -IGD: lympho-histiocytic infiltrate -PNGD: Greater neutrophil infiltrate | -PNGD, histologically: leukocytoclastic vasculitis, Sweet syndrome, neutrophilic urticaria | -Management of underlying condition -Noted clinical improvement with topical or oral corticosteroids, oral dapsone, and hydroxychloroquine |
3.3. Vasculitis and Vasculopathy
3.3.1. Felty Syndrome
3.3.2. Rheumatoid Vasculitis
Diagnosis | Characteristics | Clinical Findings | Differential Diagnosis | Treatment |
---|---|---|---|---|
Felty Syndrome | Severe RA features (erosive joint disease and deformity), Neutropenia, splenomegaly, vasculitis, necrotizing skin lesions, portal HTN, increased risk of malignancies, anemia of chronic disease, myeloid hyperplasia in bone marrow | Severe joint pain, swelling and deformities (esp. small joints of hands and wrists), fatigue, respiratory tract and skin bacterial infections, splenomegaly, variceal bleeding | SLE, large granular lymphocytic leukemia, other hematologic malignancies, drug reactions, amyloidosis, sarcoidosis, HIV, EBV | -MTX, glucocorticoids -Targeted immunotherapies: rituximab, tocilizumab -IVIG adjuvant therapy -Splenectomy |
Rheumatoid Vasculitis | Severe RA features, cutaneous vasculitis, medium-sized artery necrotizing vasculitis, vasculitic peripheral neuropathy, ocular disease, cardiac disease, low serum complement C3 | RA symptoms, deep leg ulcers, palpable purpura, rash, sores around nails, constitutional symptoms (fever, fatigue, weight loss), sensory neuropathy, visual disturbances | -Non-vasculitic RA cutaneous syndromes: Sweet syndrome, pyoderma gangrenosum -Other vasculitides: PAN, ANCA-associated, cryoglobulinemic, paraneoplastic -Vasculitis mimics: Infection, malignancy, endocarditis, thromboembolic diseases | -Corticosteroids and immunosuppressive therapy -Targeted immunotherapies: rituximab, infliximab, etanercept, tocilizumab, peficitinib |
4. Conclusions
Funding
Institutional Review Board Statement
Informed Consent Statement
Data Availability Statement
Conflicts of Interest
References
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Diaz, M.J.; Natarelli, N.; Wei, A.; Rechdan, M.; Botto, E.; Tran, J.T.; Forouzandeh, M.; Plaza, J.A.; Kaffenberger, B.H. Cutaneous Manifestations of Rheumatoid Arthritis: Diagnosis and Treatment. J. Pers. Med. 2023, 13, 1479. https://doi.org/10.3390/jpm13101479
Diaz MJ, Natarelli N, Wei A, Rechdan M, Botto E, Tran JT, Forouzandeh M, Plaza JA, Kaffenberger BH. Cutaneous Manifestations of Rheumatoid Arthritis: Diagnosis and Treatment. Journal of Personalized Medicine. 2023; 13(10):1479. https://doi.org/10.3390/jpm13101479
Chicago/Turabian StyleDiaz, Michael J., Nicole Natarelli, Aria Wei, Michaela Rechdan, Elizabeth Botto, Jasmine T. Tran, Mahtab Forouzandeh, Jose A. Plaza, and Benjamin H. Kaffenberger. 2023. "Cutaneous Manifestations of Rheumatoid Arthritis: Diagnosis and Treatment" Journal of Personalized Medicine 13, no. 10: 1479. https://doi.org/10.3390/jpm13101479