Dermatologic Manifestations of Rheumatoid Arthritis - Rheumatology Advisor

Dermatologic Manifestations of Rheumatoid Arthritis

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  • Rheumatoid arthritis (RA) should be suspected in the adult patient who presents with inflammatory polyarthritis. The initial evaluation of such patients requires a careful history and physical examination, along with selected laboratory testing to identify features that are characteristic of RA or that suggest an alternative diagnosis. A thorough medical history with close attention to joint involvement and a complete physical examination to assess for possible joint range-of-motion limitation as well as possible presence of synovitis and extraarticular disease manifestations is crucial to including RA in the differential diagnosis and to prompt further laboratory testing.

    Introduction

    Rheumatoid arthritis (RA) should be suspected in the adult patient who presents with inflammatory polyarthritis. The initial evaluation of such patients requires a careful history and physical examination, along with selected laboratory testing to identify features that are characteristic of RA or that suggest an alternative diagnosis. A thorough medical history with close attention to joint involvement and a complete physical examination to assess for possible joint range-of-motion limitation as well as possible presence of synovitis and extraarticular disease manifestations is crucial to including RA in the differential diagnosis and to prompt further laboratory testing.

  • Rheumatoid neutrophilic dermatitis (RND), first described in 1978, occurs most frequently in middle-aged women with seropositive RA. The eruption typically consists of erythematous, urticarial-like papules, plaques, or nodules < 2 cm in diameter. These nonpruritic lesions are usually symmetrical and are commonly located on the extremities, neck, and trunk. Histopathologically, there is a dense dermal neutrophilic infiltrate without the presence of vasculitis. The infiltrate can extend to the panniculus.

    Rheumatoid Neutrophilic Dermatitis (RND)

    Rheumatoid neutrophilic dermatitis (RND), first described in 1978, occurs most frequently in middle-aged women with seropositive RA. The eruption typically consists of erythematous, urticarial-like papules, plaques, or nodules < 2 cm in diameter. These nonpruritic lesions are usually symmetrical and are commonly located on the extremities, neck, and trunk. Histopathologically, there is a dense dermal neutrophilic infiltrate without the presence of vasculitis. The infiltrate can extend to the panniculus.

  • Aletaha D et al. Arthritis Rheum. 2010;62:2569-2581. Chu P et al. Arch Dermatol. 1994;130:1278-1283. Ginsberg MH et al. Arthritis Rheum. 1975;18:49-58. Highton J et al. Rheumatology (Oxford). 2007;46:1385-1376. Klareskog L et al. Lancet. 2009;373:659-672. Makol A et al. Rheumatology (Oxford). 2014;53:890-899. Nyhäll-Wåhlin BM et al. Scand J Rheumatol. 2011;40:81-87. Sangueza OP et al. J Am Acad Dermatol. 2002;47:251-257. Sayah A et al. J Am Acad Dermatol. 2005;53:191-209. Scherbenske JM. Arch Dermatol. 1989;125:1105-1108. Tomasini C et al. J Am Acad Dermatol. 2002; 46:892. Turesson C et al. Scand J Rheumatol. 2004;33:65-72.

    References


    Aletaha D et al. Arthritis Rheum. 2010;62:2569-2581.

    Chu P et al. Arch Dermatol. 1994;130:1278-1283.

    Ginsberg MH et al. Arthritis Rheum. 1975;18:49-58.

    Highton J et al. Rheumatology (Oxford). 2007;46:1385-1376.

    Klareskog L et al. Lancet. 2009;373:659-672.

    Makol A et al. Rheumatology (Oxford). 2014;53:890-899.

    Nyhäll-Wåhlin BM et al. Scand J Rheumatol. 2011;40:81-87.

    Sangueza OP et al. J Am Acad Dermatol. 2002;47:251-257.

    Sayah A et al. J Am Acad Dermatol. 2005;53:191-209.

    Scherbenske JM. Arch Dermatol. 1989;125:1105-1108.

    Tomasini C et al. J Am Acad Dermatol. 2002; 46:892.

    Turesson C et al. Scand J Rheumatol. 2004;33:65-72.

  • Rheumatoid vasculitis (RV) is destructive inflammation of the blood vessel wall that adds substantial morbidity to a diagnosis of RA. RV usually develops late in the course of RA, when disease burden is high. RV may affect a wide range of blood vessel types, from medium-sized muscular arteries to somewhat smaller arterioles to postcapillary venules. RV leads to necrosis, blood vessel occlusion, and tissue ischemia in a manner that resembles other forms of systemic vasculitis, particularly polyarteritis nodosa.

    Rheumatoid Vasculitis

    Rheumatoid vasculitis (RV) is destructive inflammation of the blood vessel wall that adds substantial morbidity to a diagnosis of RA. RV usually develops late in the course of RA, when disease burden is high. RV may affect a wide range of blood vessel types, from medium-sized muscular arteries to somewhat smaller arterioles to postcapillary venules. RV leads to necrosis, blood vessel occlusion, and tissue ischemia in a manner that resembles other forms of systemic vasculitis, particularly polyarteritis nodosa.

  • The rheumatoid nodule is the most common cutaneous and extraarticular manifestation of RA. Though most commonly found on pressure points such as on the olecranon process, they can also occur at other sites. Subcutaneous nodules are located proximally to joints, are firm to palpation, and usually are noted to have overlying intact skin. Histologically, rheumatoid nodules are characterized by a central area of necrosis with concentric layers of macrophages and lymphocytes.

    Diagnosis

    The rheumatoid nodule is the most common cutaneous and extraarticular manifestation of RA. Though most commonly found on pressure points such as on the olecranon process, they can also occur at other sites. Subcutaneous nodules are located proximally to joints, are firm to palpation, and usually are noted to have overlying intact skin. Histologically, rheumatoid nodules are characterized by a central area of necrosis with concentric layers of macrophages and lymphocytes.

  • The term interstitial granulomatous dermatitis describes a form of granulomatous dermatitis in which annular plaques or rope-like cords appear on the lateral trunk and skin folds in patients with RA. The lesions tend to be symmetrically distributed on the trunk and tend to be largely asymptomatic. The histopathologic findings are notable for areas of degenerated collagen with palisading granulomas composed of histiocytes.

    Interstitial Granulomatous Dermatitis

    The term interstitial granulomatous dermatitis describes a form of granulomatous dermatitis in which annular plaques or rope-like cords appear on the lateral trunk and skin folds in patients with RA. The lesions tend to be symmetrically distributed on the trunk and tend to be largely asymptomatic. The histopathologic findings are notable for areas of degenerated collagen with palisading granulomas composed of histiocytes.

  • Palpable nodules in the subcutaneous tissues have been reported at initial presentation in 7% of patients with RA and are found at some time during the course of RA in 30% to 40% of cases. Most patients with nodules will test positive for RF. It is widely reported that patients with rheumatoid nodules will undergo a severe disease course, with more rapid progression of joint destruction than those patients without nodules. Patients with RA who present with nodules are also more likely to later develop vasculitis. Pictured here is a rheumatoid nodule on the elbow.

    Rheumatoid Arthritis Subcutaneous Nodule

    Palpable nodules in the subcutaneous tissues have been reported at initial presentation in 7% of patients with RA and are found at some time during the course of RA in 30% to 40% of cases. Most patients with nodules will test positive for RF. It is widely reported that patients with rheumatoid nodules will undergo a severe disease course, with more rapid progression of joint destruction than those patients without nodules. Patients with RA who present with nodules are also more likely to later develop vasculitis. Pictured here is a rheumatoid nodule on the elbow.

  • Granulomatous dermatitis in the context of autoimmune disease and RA presents in 2 major clinical and histopathologic patterns: palisaded neutrophilic granulomatous dermatitis (PNGD) and interstitial granulomatous dermatitis. Interstitial granulomatous dermatitis is considered a type of PNGD by some authors, while other researchers have classified these conditions separately. PNGD may present as symmetrically distributed, umbilicated papules that favor the extensor surfaces of the extremities, particularly the elbows and fingers. The most frequent disease association is RA. The histopathologic findings of early lesions include a small-vessel leukocytoclastic vasculitis and a dense neutrophilic infiltrate. Shown here is light micrograph of section through a rheumatoid nodule, showing area of palisading histiocytes.

    Palisaded neutrophilic granulomatous dermatitis (PNGD)

    Granulomatous dermatitis in the context of autoimmune disease and RA presents in 2 major clinical and histopathologic patterns: palisaded neutrophilic granulomatous dermatitis (PNGD) and interstitial granulomatous dermatitis. Interstitial granulomatous dermatitis is considered a type of PNGD by some authors, while other researchers have classified these conditions separately. PNGD may present as symmetrically distributed, umbilicated papules that favor the extensor surfaces of the extremities, particularly the elbows and fingers. The most frequent disease association is RA. The histopathologic findings of early lesions include a small-vessel leukocytoclastic vasculitis and a dense neutrophilic infiltrate. Shown here is light micrograph of section through a rheumatoid nodule, showing area of palisading histiocytes.

  • Rheumatoid nodulosis is characterized by multiple nodules on the hands and multiple subchondral bone cysts known as "geodes." These nodules tend to occur on extensor surfaces adjacent to joints, elbows, and fingers, as well as on the forearm, metacarpophalangeal and proximal interphalangeal joints, occiput, back, heel, and other areas. If the formation of accelerated nodulosis is suspected to be secondary to methotrexate treatment, discontinuation of the drug and consideration of other treatment including other DMARDs and biologic response-modifying agents (eg, anti-tumor necrosis factor [TNF] alpha agents). Pictured here are rheumatoid nodules.

    Rheumatoid Nodulosis

    Rheumatoid nodulosis is characterized by multiple nodules on the hands and multiple subchondral bone cysts known as "geodes." These nodules tend to occur on extensor surfaces adjacent to joints, elbows, and fingers, as well as on the forearm, metacarpophalangeal and proximal interphalangeal joints, occiput, back, heel, and other areas. If the formation of accelerated nodulosis is suspected to be secondary to methotrexate treatment, discontinuation of the drug and consideration of other treatment including other DMARDs and biologic response-modifying agents (eg, anti-tumor necrosis factor [TNF] alpha agents). Pictured here are rheumatoid nodules.

Rheumatoid arthritis (RA) is a symmetric and inflammatory peripheral polyarthritis of unknown etiology. Deformity results from the erosion of cartilage and bone from extension of tendons and ligaments secondary to inflammation. When not diagnosed early in the course of disease or not adequately treated, joint destruction leads to restricted joint range of motion and loss of the ability to participate in activities of daily living. Early diagnosis and treatment with disease-modifying antirheumatic drugs (DMARDs) is important in preventing further joint injury and long-term disability.

The diagnosis of RA can be made in a patient with inflammatory arthritis involving 3 or more joints, positive rheumatoid factor (RF) and/or anti-citrullinated peptide/protein (CCP) antibody, disease duration of more than 6 weeks, and elevated C-reactive protein (CRP) or erythrocyte sedimentation rate (ESR), but without evidence of diseases with similar clinical features.

However, the diagnosis of RA is not always clear-cut, as is the case with patients presenting with seronegative RA, clinically quiescent RA, and early-onset RA. In patients with early disease, the joint manifestations are often difficult to distinguish from other forms of inflammatory polyarthritis, thus making the recognition of the cutaneous manifestations of RA upon patient presentation critical.

View the images in this slideshow to learn the dermatologic hallmarks of this potentially debilitating disease.

Choice B (pleural effusion) is the correct answer. Pleural effusions are a common extra-articular manifestation of RA. However, it is not considered a poor prognostic factor for the disease. Poor prognostic factors for RA include elevated ESR and RF levels,...

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Choice B (pleural effusion) is the correct answer. Pleural effusions are a common extra-articular manifestation of RA. However, it is not considered a poor prognostic factor for the disease. Poor prognostic factors for RA include elevated ESR and RF levels, rheumatoid nodules, vasculitis (ulceration of hands and fingers), progressive synovitis, erosive lesions on radiographic examination, and HLA-DR4. The effusion of RA usually contains a glucose <30 mg/dL.

Of note is the phenomenon of methotrexate-induced lung injury, occurring weeks to months following initiation of methotrexate therapy. The acute presentation typically includes fever, chills, malaise, nonproductive cough, dyspnea, and chest pain. Risk factors for methotrexate-induced lung toxicity include age older than 60 years, rheumatoid pleuropulmonary involvement, previous use of DMARDs, hypoalbuminemia, higher weekly doses of methotrexate, pre-existing lung disease, and decreased elimination of methotrexate.

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