Systemic Disease and the Skin

Abstract

This chapter begins with a review of the various autoinflammatory disorders which result from immune dysregulation and have been traced to a variety of gene mutations, with the exception of Schnitzler’s and SAPHO syndromes. A wide variety of cutaneous diseases are linked to cardiovascular abnormalities, ranging from dermal deposits (amyloidosis) to eosinophilic infiltrates to infectious disease (bacterial endocarditis). Skin lesions may provide an early clue to neurological disease, but in addition, management of the protean cutaneous manifestations of neurofibromatosis and tuberous sclerosis can provide its own significant challenges. Conditions of the pilosebaceous apparatus and pigment anomalies accompany many of the endocrinologic disorders, and a textbook could be devoted to the manifestations of diabetes mellitus alone. Gastrointestinal disorders are linked with numerous skin conditions, and this chapter devotes particular attention to Cowden’s disease, Crohn’s disease, and pyoderma gangrenosum. Hepatic disorders must be suspected in patients with forms of porphyria, Wilson’s disease, and necrolytic acral erythema. Much of the chapter deals with malignancy and the skin; the general areas are hormone-secreting syndromes, inherited cancer syndromes, proliferative and inflammatory dermatoses, and complications of leukemias and lymphomas. Finally, among the important cutaneous manifestations of renal disease are calciphylaxis and a rather unique condition – nephrogenic systemic fibrosis – that for a time was a major focus of investigation but now appears to be uncommon since the discovery of its linkage to gadolinium-based contrast agents.