Gangrene as a Manifestation of Systemic Lupus Erythematosus

Abstract

Five cases of gangrene as a manifestation of systemic lupus erythematosus (SLE) are described. Only one previous case of this syndrome has been reported. The occurrence of this complication was unrelated to either steroid or antimalarial therapy. Treatment with large doses of corticoids was helpful in 4 of the patients in relieving pain and limiting the extension of the gangrene. The close relationship of this type of vasculitis to endarteritis occurring in the retina ("chloroquine retinopathy"), aseptic necrosis of bone, and "malignant rheumatoid arthritis" is discussed. A plea is made for cessation of the current tendency to ascribe the vascular complications of multisystemic disorders to the therapy employed and for a trial of hi gh\ x=req-\ dosage steroid therapy in the treatment of severe vasculitis due to both SLE and "malignant rheumatoid arthritis." DURING the past 8 months, 4 cases of periph¬ eral gangrene as a presenting manifestation of systemic lupus erythematosus (SLE) have been observed. Review of our case records added a fifth patient with similar changes, giving a 1% in¬ cidence of this finding in our series of approxi¬ mately 500 cases collected over an 11-year period.One previous, well-documented case whose initial finding was a rheumatoid-like arthritis has been described by Rywaters.1 (See addendum.) Prior to the onset of symptoms, 3 of our patients had never received any corticoids and 4 had never taken antimalarial drugs. The purposes of this report are not only to present these 5 cases which illustrate the extensive disease of small blood vessels which may occur in SLE, but also to emphasize the fact that this type of vasculitis is due entirely to the under¬ lying disorder and is not a result of therapy. The relationship of this lesion to aseptic necrosis of bone and retinopathy attributed to chloroquine, both recently described as vascular lesions caused by therapy, will be discussed. The syndrome re¬ ported is another common bond between SLE and rheumatoid arthritis. A plea is made for cessation of the current tendency to blame vascular compli¬ cations of Case 1.-A 48-year-old white housewife was admitted to the Los Angeles County General Hospital on fan. 13, 1962, complaining of pain and blueness of 2 months' duration in her fingers. Present Illness.-The patient apparently was in good health until approximately age 31, when spontaneous thrombophlebitis developed in the right leg and gradually subsided. She again felt well until age 40, 8 years prior to admission, when a transitory right hemiparesis occurred,lasting approximately one month and followed by almost full recovery. At age 45, 3 years prior to admission, throm¬ bophlebitis occurred in the left leg. There were several re¬ currences of phlebitis in the left leg, and in April, 1961, a series of pulmonary emboli occurred, apparently origi¬ nating from this site. The patient was treated with anti¬ coagulants, and several of the affected veins were stripped. From this date until the time of admission, she was treated with warfarin so...

Envolvimento mucocutâneo no lúpus eritematoso sistêmico e sua associação com auto-anticorpos

“…O eritema malar é achado muito sugestivo da forma sistêmica da doença, e o anticorpo antiDNA é considerado um dos mais específicos para o lúpus sistêmico, sugerindo, aliás, atividade renal. 1 Outro dado relevante foi a alta prevalência do fenômeno de Raynaud na população estudada, que apresentou associação com a presença do anticorpo anti-Sm. Embora a constatação do fenômeno de Raynaud seja achado comum a todas as colagenoses, sua presença sugere o diagnóstico de esclerodermia, a qual ocorre em até 95% dos casos.…”

“…12 No lúpus sistêmi-co, a literatura registra prevalência entre 18 e 45%. 1 Por último, é necessário observar que, nessa população, a idade ao diagnóstico não influiu no aparecimento de nenhuma das lesões cutâneas observadas. Como a amostra analisada compunha-se de pacientes com ampla variação de idade ao diagnóstico (dos seis aos 73 anos), pode-se inferir que o perfil dessa forma de manifestação não difere entre crianças, adultos e idosos.…”

“…1 Esses pacientes foram submetidos à anamnese e examinados ectoscopicamente por dermatologista, que preencheu protocolo preestabelecido em que constavam dados demográficos, as principais lesões mucocutâneas possíveis de ser encontradas, assim como as manifestações presentes no momento do diagnóstico. Seus prontuários foram revisados para dados demográficos (idade, tempo de diagnóstico, sexo), manifestação inicial da doença, achados mucocutâneos anteriores e auto-anticorpos (antiRo/SS-A, antiLa/SS-B, anti-RNP, anti-Sm e antiDNA).…”

“…1 O envolvimento mucocutâneo aparece em torno de 70% dos pacientes, sendo seu achado extremamente útil para o diagnóstico dessa entidade. 2 Em cerca de um quarto dos pacientes a manifestação de pele está presente ao diagnóstico, sendo a segunda mais freqüente, só superada pelas manifestações articulares.…”

“…3 Sua importância fica bastante explícita ao se observar que quatro dos 11 critérios da classificação para essa doença proposta pelo Colégio Americano de Reumatologia são dermatológicos: fotossensibilidade, eritema malar, úlceras orais e lesões discóides. 1 Em certos casos, a lesão cutânea oferece, também, ajuda no prognóstico e na previsão de envolvimento de determinados órgãos internos. 4,5 As manifestações cutâneas podem ser divididas em lesões específicas e inespecíficas.…”