Skin Signs of Rheumatoid Arthritis and its Therapy-Induced Cutaneous Side Effects.

Rheumatoid arthritis (RA) is a systemic inflammatory disorder that primarily affects the joints, but may exhibit extra-articular, including cutaneous, manifestations such as rheumatoid nodules, rheumatoid vasculitis, granulomatous skin disorders, and neutrophilic dermatoses. A large burden of cutaneous disease may be an indication of RA disease activity and the need for more aggressive treatment. Many of the therapeutic agents used to treat RA can also result in cutaneous adverse effects, which pose their own diagnostic and therapeutic challenges. Anti-TNFα agents, in particular, have a wide variety of adverse effects including psoraisiform eruptions, granulomatous conditions, and cutaneous connective tissue disorders. Herein we provide an update on the clinical presentations and management of RA-associated cutaneous findings as well as drug-induced cutaneous effects, with particular attention to the adverse effects of biologic disease-modifying agents.

Neutrophilic dermatoses: a review of current treatment options.

Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis are neutrophilic dermatoses - conditions that have an inflammatory infiltrate consisting of mature polymorphonuclear leukocytes. The neutrophils are usually located within the dermis in Sweet syndrome and pyoderma gangrenosum; however, in subcorneal pustular dermatosis, they are found in the upper layers of the epidermis. Sweet syndrome, also referred to as acute febrile neutrophilic dermatosis, is characterized by pyrexia, elevated neutrophil count, painful erythematous cutaneous lesions that have an infiltrate of mature neutrophils typically located in the upper dermis, and prompt clinical improvement following the initiation of systemic corticosteroid therapy. Classical, malignancy-associated, and drug-induced variants of Sweet syndrome exist. Pyoderma gangrenosum is characterized by painful, enlarging necrotic ulcers with bluish undermined borders surrounded by advancing zones of erythema; its clinical variants include: ulcerative or classic, pustular, bullous or atypical, vegetative, peristomal, and drug-induced. Subcorneal pustular dermatosis is an uncommon relapsing symmetric pustular eruption that involves flexural and intertriginous areas; it can be idiopathic or associated with cancer, infections, medications, and systemic diseases. Since Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis share not only the same inflammatory cell but also similar associated systemic diseases, it is not surprising that the concurrent or sequential development of these neutrophilic dermatoses has been observed in the same individual. Also, it is not unexpected that several of the effective therapeutic interventions - including systemic drugs, topical agents, and other treatment modalities - for the management of these dermatoses are the same. The treatment of choice for Sweet syndrome and idiopathic pyoderma gangrenosum is systemic corticosteroids; however, for subcorneal pustular dermatosis, dapsone is the drug of choice. Yet, tumor necrosis factor-alpha antagonists are becoming the preferred choice when pyoderma gangrenosum is accompanied by inflammatory bowel disease or rheumatoid arthritis. Potassium iodide and colchicine are alternative first-line therapies for Sweet syndrome and indomethacin (indometacin), clofazimine, cyclosporine (ciclosporin), and dapsone are second-line treatments. Cyclosporine is effective in the acute management of pyoderma gangrenosum; however, when tapering the drug, additional systemic agents are necessary for maintaining the clinical response. In some patients with subcorneal pustular dermatosis, systemic corticosteroids may be effective; yet, systemic retinoids (such as etretinate and acitretin) have effectively been used for treating this neutrophilic dermatosis - either as monotherapy or in combination with dapsone or as a component of phototherapy with psoralen and UVA radiation. Topical agents can have an adjuvant role in the management of these neutrophilic dermatoses; however, high-potency topical corticosteroids may successfully treat localized manifestations of Sweet syndrome, pyoderma gangrenosum, and subcorneal pustular dermatosis. Intralesional corticosteroid therapy for patients with Sweet syndrome and pyoderma gangrenosum, hyperbaric oxygen and plasmapheresis for patients with pyoderma grangrenosum, and phototherapy for patients with subcorneal pustular dermatosis are other modalities that have been used effectively for treating individuals with these neutrophilic dermatoses.

Síndrome de Sweet: revisión bibliográfica / Sweets syndrome: a review

El síndrome de Sweet es una dermatosis caracterizada por la aparición brusca de placas eritematosas, dolorosas, predominantemente en cara, cuello y extremidades, acompañada de fiebre, leucocitosis a predominio neutrofílico y denso infiltrado dérmico leucocitario polimorfonuclear. Es bien conocida su relación con procesos neoplásicos malignos, motivo por el cuál es necesario el estudio minucioso y seguimiento del paciente. Presentamos un caso de leucemia mielomonocítica aguda asociada a ésta dermatosis, realizando una revisión bibliográfica del tema (AU)

Síndrome de Sweet: revisión bibliográfica / Sweet's syndrome: a review

El síndrome de Sweet es una dermatosis caracterizada por la aparición brusca de placas eritematosas, dolorosas, predominantemente en cara, cuello y extremidades, acompañada de fiebre, leucocitosis a predominio neutrofílico y denso infiltrado dérmico leucocitario polimorfonuclear. Es bien conocida su relación con procesos neoplásicos malignos, motivo por el cuál es necesario el estudio minucioso y seguimiento del paciente. Presentamos un caso de leucemia mielomonocítica aguda asociada a ésta dermatosis, realizando una revisión bibliográfica del tema

Sweet's syndrome during treatment with all-trans retinoic acid in a patient with acute promyelocytic leukemia.

A 46 year old male with acute promyelocytic leukemia treated with all-trans retinoic acid (ATRA), developed fever, bilateral erythematous nodules in his axillary area, lower abdomen and inguinal region. Histopathologic examination of the skin biopsy revealed dense neutrophil infiltration in the dermis without vasculitis. The diagnosis of Sweet's syndrome was made. High dose methylprednisolone was administered and the lesions started to improve within 24 hours.

Sweet's syndrome with an exogenous cause.

A sixty-six-year-old white man presented with a five-day history of painful erythematous papules, plaques, pustules, and hemorrhagic bullae on both hands. His history was remarkable for having prepared and pickled fifteen quarts of home-grown jalapeño peppers several days before the eruption occurred. The light microscopic examination of sections of lesional skin stained with hematoxylin and eosin revealed pathologic findings characteristic of Sweet's syndrome. We report this case because of its unusual presentation and apparent exogenous cause.