Calcinose cútis distrófica universal associada a lúpus eritematoso sistêmico: um caso exuberante

Abstract

Calcinosis cutis is an uncommon disease of unclear pathophysiology that is often disabling. It is characterized by the formation of calcium deposits in the skin or subcutaneous tissue. It is classified into four subtypes: dystrophic, metastatic, idiopathic or iatrogenic. It may be seen in a variety of systemic diseases such as hyperparathyroidism and hypervitaminosis D, but is most commonly found in dermatomyositis, scleroderma and overlap syndromes and is a rare complication of systemic lupus erythematosus. The management of secondary complications and the success of therapy are constant challenges in the follow-up of these cases. Keywords: Calcinosis; Systemic lupus erythematosus; rheumatic diseases Resumo: Calcinose cutânea é uma doença incomum, de fisiopatologia incerta e, muitas vezes, incapacitante. Caracteriza-se pela deposição de sais de cálcio na pele ou tecido subcutâneo. É classificada em quatro subtipos: metastática, distrófica, idiopática e iatrogênica. Pode ser vista em várias doenças sistêmicas como hiperparatireoidismo e hipervitaminose D, ocorrendo com maior frequência na dermatomiosite, esclerodermia e síndromes overlap, sendo uma complicação infrequente no lúpus eritematoso sistêmico. O manejo das complicações secundárias, assim como o sucesso terapêutico, constituem desafios constantes no seguimento destes casos.

Calcinosis cutis: A rare cause for difficult airway

“…In calcinosis circumscipta the deposition is in a well-demarcated area and in calcinosis universalis there is widespread deposition of calcium in the skin. [ 4 5 6 ]…”

“…Calcinosis cutis may be found in various overlapping connective tissue disorders like scleroderma, rheumatoid arthritis, and dermatomyositis and rarely in SLE. [ 6 ]…”

“…CREST syndrome may be associated with difficult airway as microstomia, stiff temporomandibular joint and ineffective BURP maneuver. [ 6 7 ] Other problems in CREST syndrome are related to thick skin, cutaneous bleeding and stiff joints, which pose problems for securing arterial, venous lines and for positioning. [ 7 ]…”