Journal of Tissue Viability (2013) 22,92e102

www.elsevier.com/locate/jtv

Review Connective tissue ulcers

Ganary Dabiri a, Vincent Falanga a,b,* a Department of Dermatology and Skin Cancer, Roger Williams Medical Center, 50 Maude Street, Providence, RI 02908, USA b Departments of Dermatology and Biochemistry, Boston University School of Medicine, 609 Albany Street, Room J-607, Boston, MA 02118, USA

KEYWORDS Abstract Connective tissue disorders (CTD), which are often also termed collagen Ulcers; vascular diseases, include a number of related inflammatory conditions. Some of Connective tissue dis- these diseases include rheumatoid arthritis, systemic lupus erythematosus, sys- ease; temic sclerosis (scleroderma), localized scleroderma (morphea variants localized Vasculitis; to the skin), Sjogren’s syndrome, dermatomyositis, polymyositis, and mixed con- Connective tissue ul- nective tissue disease. In addition to the systemic manifestations of these diseases, cers there are a number of cutaneous features that make these conditions recognizable on physical exam. Lower extremity ulcers and digital ulcers are an infrequent but disabling complication of long-standing connective tissue disease. The exact fre- quency with which these ulcers occur is not known, and the cause of the ulcerations is often multifactorial. Moreover, a challenging component of CTD ulcerations is that there are still no established guidelines for their diagnosis and treatment. The morbidity associated with these ulcerations and their underlying conditions is very substantial. Indeed, these less common but intractable ulcers represent a major medical and economic problem for patients, physicians and nurses, and even well organized multidisciplinary wound healing centers. ª 2013 Tissue Viability Society. Published by Elsevier Ltd. All rights reserved.

Introduction inflammatory conditions. Some of these diseases include rheumatoid arthritis, systemic lupus ery- Connective tissue disorders (CTD), which are often thematosus, systemic sclerosis (scleroderma), also termed collagen vascular diseases, (will go localized scleroderma (morphea variants localized forward referring to them as connective tissue to the skin), Sjogren’s syndrome, dermatomyositis, disorders (CTD)), include a number of related polymyositis, and mixed connective tissue disease. The various CTD are distinct entities but they have features that are common, notably they share * Corresponding author. Department of Dermatology, Boston autoantibodies, but each disease also has their University School of Medicine, 609 Albany Street, Room J-607, own specific autoantibody (Table 1). Despite the Boston, MA 02115, USA. Tel.: þ1 617 638 5500. E-mail address: [email protected] (V. Falanga). hallmark physical exam findings that lead

0965-206X/$36 ª 2013 Tissue Viability Society. Published by Elsevier Ltd. All rights reserved. http://dx.doi.org/10.1016/j.jtv.2013.04.003 Collagen vascular diseases 93 clinicians to a diagnosis or narrow differential Table 2 Distinctive clues observed away from the diagnosis (Table 2), one cannot make a diagnosis of ulcer location. (Copyrighted V. Falanga, 2010). a CTD based on the presence of ulceration alone. The ulcerations that occur in CTD are late-onset Clinical clues Conditions and due to a variety of factors, most notably in- Hard fingers, face Scleroderma flammatory vasculitis and thrombotic nonvasculitic Facial butterfly rash Lupus erythematosus causes [1]. Vasculitides are not only associated Muscle weakness, Dermatomyositis with autoimmune forms of CTD but they them- eyelid rash Ear redness with Relapsing selves can be classified as a CTD. sparing of ear lobe polychondritis When patients present with an ulceration, it Livedo reticularis of Occlusion of small becomes imperative to establish the primary cause microlivedo blood vessels, from of the ulceration. Cutaneous ulcers of CTD often vasculitis, coagulopathy, have unusual shapes and can be mistakenly cryoglobulin, thought of as factitial (Fig. 1a) [2]. This is why a cryofibrinogen complete history and physical exam including Rheumatoid hands Rheumatoid ulcers extensive review of systems, family history, and Swelling of face, Cushingoid appearance current and relevant past medications is impera- posterior neck (steroids) tive for diagnosis and management [3]. Having a CTD does not mean that a patient cannot have joints of the hand and feet, ulnar deviation, swan other concomitant vascular/neuropathic insuffi- neck, and boutonniere deformity [2]. Although RA ciency like venous or pressure ulcers. This causes primarily affects the joints, extraarticular mani- confusion because there is overlap. Here we re- festations are frequent, such as rheumatoid nodules view the main autoimmune connective tissue ul- and skin ulcerations. The cause of leg ulcerations in cers, their presentation, pathology, pathogenesis, RA is multifactorial, including, vasculitis, para- and treatment and management. proteinemias, anticardiolipin antibodies, venous insufficiency, toxic effects of medications, superfi- cial ulcerating rheumatoid necrobiosis, pressure Connective tissue disorders ulcers, neuropathic ulcers, and pyoderma gan- grenosum [1,2]. The ulcers as seen on physical exam Rheumatoid arthritis have an angular configuration or an undulating border (Fig. 1b) [2]. Rheumatoid arthritis (RA) is a chronic, inflammatory Venous insufficiency can be a complication of autoimmune disorder expressed most commonly as a impaired movement of the ankle joint as a result symmetrical, deforming arthropathy. Physical exam of RA because of poor muscle pump action [1,3]. findings include symmetric swelling of the small Toxic effects of medications include use of corti- costeroids that cause skin atrophy where minor Table 1 Serum markers associated with connective trauma leads to ulceration [2]. RA patients can be tissue disorders. debilitated from their disease and bedridden making them prone to pressure ulcers [2]. Super- Serum markers ficial ulcerating rheumatoid necrobiosis (SURN) Rheumatoid Rheumatoid factor, lesions are bilateral over pretibial areas and are arthritis anti-CCP (cyclic refractory to treatment. They are characterized citrullinated peptide) by yellow-red plaques that ulcerate [4]. A well- Scleroderma Anti-ssDNA, known cause of ulceration in RA is vasculitis. Ves- (localized) peripheral blood eosinophilia, sels of different sizes may be affected. These anti-histone include small to medium sized muscular arteries, Scleroderma Anti-Scl-70, arterioles, and venules. Small to medium size (generalized) anti-centromere vessels, when involved, can mimic polyarteritis Systemic lupus ANA, anti-dsDNA, nodosa and can be a severe rheumatoid vasculitis. erythematosus anti-Smith, anti-histone These patients will require systemic therapy (drug induced SLE) because mortality can be high [5]. Milder vasculitic Sjogren’s syndrome Anti-SSB(anti-La), disease also occurs in RA patients, where post- anti-SS-A (anti-Ro) capillary venules are affected. These patients Dermatomyositis CPK, aldolase, present with palpable purpura [6]. anti-Jo1 Workup for the patients should include com- Mixed connective tissue Anti-nRNP plete history and thorough physical exam, Download English Version: https://daneshyari.com/en/article/2670694

Download Persian Version:

https://daneshyari.com/article/2670694

Daneshyari.com