Cutaneous ulceration, primarily of the lower extremities, is frequently found in patients with chronic, debilitating disease (Oien et al., 2000; Margolis et al., 2004). Lower extremity ulceration in patients with RA may be due to an associated disease process such as RV or pyoderma gangrenosum (PG). If in
CV the large vessels are affected, skin ulceration develops, often on lower extremities, or where skin is exposed to pressure, for example, in interphalangeal joints of the toes, over bunions, ankles, elbows and, in bedridden patients, the buttocks. These ulcerations may be very painful, come in crops, and tend to grow and become chronic. Superinfection frequently occurs, particularly being a great risk for patients with joints prostheses. In severe cases, ulcerations may form over subcutaneous nodules. CV or leucocytoclastic vasculitis, seen as palpable purpura, heals as a rule and responds to conventional DMARDs (Heurkens et al., 1991). Large-vessel vasculitis requires prompt treatment with cor-ticosteroids and cytotoxic drugs. In addition to drug treatment, pressure ulcers should be treated by eliminating pressure, frequent bathing in soda or beta-dine water, and careful local bandage therapy (Dowsett, 2005). It is difficult to determine whether pressure is the only cause of such ulcers, and vasculitis should be regarded in case of any irregularly shaped purpuric lesions and non-healing ulcers.
Symmetric lower extremity ulcerations in patients with RA, resembling necrobiosis lipoidica diabeti-corum, have been called superficial ulcerating rheumatoid necrobiosis. More common causes of lower extremity ulceration should also be considered, such as venous stasis ulcers, granulocytopenia, and splenomegaly in patients with RA is diagnostic for Felty syndrome (Goldberg and Pinals, 1980; Balint and Balint, 2004).
10.2.3. Felty's syndrome Felty's syndrome is defined as RA in combination with splenomegaly and leucopaenia. It is seen in less than 1% of hospital RA patients and occurs in the same frequency in males and females (Goldberg and Pinals, 1980; Balint and Balint, 2004). Clinically, it is characterized by severe joint destruction contrasting with moderate or absent joint inflammation and severe extra-articular disease, including a high frequency of rheumatoid nodules, lymphadenopathy, hepatopathy, vasculi-tis, leg ulcers, skin pigmentation, etc. The management of Felty's syndrome is still under investigation. In a review, MTX haematopoietic growth factors, and splenectomy appeared to be the most efficacious in increasing granulocyte count and improving clinical outcome (Rashba et al., 1996; Wassenberg et al., 1998; Hellmich et al., 1999). Treatment with DMARDs might improve cytopaenia and reduce the susceptibility to infection (Breedveld et al., 1987).
The neutrophilic dermatoses are a group of related cutaneous disorders that frequently have systemic manifestations or associations (Callen, 2002; Wallach, 2005). Several neutrophilic dermatoses occur in patients with RA (Wallach, 2005). These include PG, RND, acute febrile neutrophilic dermatosis (Sweet's syndrome), erythema eleva-tum diutinum, subcorneal pustular dermatitis (Sneddon-Wilkinson's disease), and neutrophilic lobular panniculitis. Sweet syndrome is a reactive dermatological disease characterized by fever, leuko-cytosis, and tender, erythematous, well-demarcated papules and plaques, which show dense neutrophilic infiltrates and papillary dermal edema (Joe, 2003). It may occur in the absence of other diseases but is often associated with other systemic autoimmune diseases, infections, and myelodysplastic malignancies (Gouliaris et al., 2003; Theng et al., 2003). Its association with RA has been well described
(Wallach, 2005). Clinical overlap between these disorders led to the concept of 'the neutrophilic dermatosis'. In addition, patients with a neutrophilic skin disorder may also suffer from extra-cutaneous aseptic neutrophilic infiltrates. The mechanisms underlying the inappropriate activation of polymorp-honuclears are poorly understood (Wallach, 2005). Hematopoietic growth factors and adhesion molecules are believed to play a role in the pathophysio-logy of the neutrophilic dermatoses.
PG is a condition characterized by acute, nec-rotizing, rapidly expanding cutaneous ulcers (Mani, 2002; Groves, 2004). These ulcers may occur on cutaneous site, yet they most commonly occur on the lower extremities and abdomen. Lesions begin as erythematous papulopustules. They quickly expand to become fluctuant, painful ulcerations with dusky, undermined borders. When the lesions heal, they demonstrate a characteristic atrophic 'papyraceous' scar. PG can be seen in association with multiple systemic diseases such as CD, ulcerative colitis, RA, and malignancies, especially hemato-logic malignancies (Brown et al., 2001; Tavarela Veloso, 2004; Harati et al., 2005). The ulcers of patients with PG seem more refractory to treatment than the ulcers of patients with PG alone. Those with PG ulcers represent a refractory subset of patients, and the ulcers are possibly secondary to unique pathophysiological features. In patients with RA, the lesions have often been confused with venous stasis ulcers, necrobiosis lipoidica dia-beticorum, and bacterial pyoderma. No histologic pattern is pathognomonic for PG and it is ultimately a clinical diagnosis of exclusion. However, histopatologic examination often reveals neutro-phils in the dermis with signs of endothelial swelling and deposits of immunoglobulins and complement factors in the dermal blood (Jorizzo et al., 1988; Magro and Crowson, 2003).
10.3.2. Rheumatoid neutrophilic dermatosis RND is a rare cutaneous manifestation seen in patients with severe RA (Brown et al., 2001; Defaria and Kroumpouzos, 2004). All have had severe disease and a RF positive, but recently the literature reports RND as presenting sign of seronegative arthritis. This presents as chronic, erythematosus, 'urticaria-like' plaques and papules over the extensor surfaces on the extremities and the trunk. The lesions are usually non-tender, non-scaling, and nomigratory. They may persist for weeks to months, and ulceration may occur. His-topathologiacally, a dense neutrophilic infiltrate is seen throughout the dermis without an associated vasculitis (Magro and Crowson, 2003). It may be difficult to distinguish RND from acute febrile neutrophilic dermatosis, which also occurs in patients with RA.
Patients with RA occasionally present with livedo reticularis, a reticulated vasodilatatory response that occurs predominantly on the trunk and on entire extremities (Cimmino et al., 2000). The lesions are non-palpaple and often quite subtle. The reticulated pattern results from the occlusion of distal vessels that supply the upper skin leading to a compensatory vasodilation of surrounding vessels. Ulceration and scarring are common. Patients with RA may also have livedoid vasculopathy. Also known as atrophie blanche, a chronic der-matological disorder associated with petechiae and recurrent, unusually shaped ulcers that heal to form hyperpigmentated areas and atrophie blanche (Schroeter and Harris, 1984; Cimmino et al., 2000).
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