Connective Tissue Diseases



Connective Tissue Diseases


Karin Eshagh

Basil M. Hantash



Connective tissues diseases (CTDs), such as cutaneous lupus erythematosus (CLE), dermatomyositis (DM), and scleroderma (Scl), are systemic inflammatory diseases that may present initially with significant cutaneous manifestations that frequently establish diagnosis. Women are more commonly affected than men, with estimates ranging from 2:1 up to 15:1 female predominance.1,2,3,4 This chapter will review the etiology and pathogenesis of 3 common CTDs (lupus erythematosus [LE], DM, and Scl) and will aim to address the current cosmetic treatments available to reduce the disfigurement triggered by the multisystem diseases.


LUPUS


BACKGROUND

About 14 to 122 per 100,000 people are affected by LE, which is a chronic autoimmune disorder that can involve multiple organs and lead to anemia, nephritis,
serositis, arthritis, and/or cardiac conduction defects.5 Skin involvement, known as CLE, is very common and occurs at some point in the course of the disease in about 75% patients with LE.1


PRESENTATION

The most common dermatologic manifestations are malar rash (40%), alopecia (24%), and oral ulcers (19%).1



PATHOGENESIS

Genetic predisposition is the highest risk factor associated with developing CLE. Different HLA genes have been associated with ACLE, such as HLA-DR2 and HLA-DR3, and with SCLE, such as HLA-A1, HLA-B8, HLA-DR2, HLA-DR3, HLA-DRw52, HLA-DRw6, HLA-DQ1, and HLA-DQ2.5,6 Environmental factors also can play a role via photosensitivity because UVB radiation leads to proinflammatory cytokines, such as tumor necrosis factor-alpha, interleukin (IL)-6, IL-10.7,8 Thus, the patient’s medication list should be reviewed for any photosensitizing medications, such as ACE inhibitors, calcium channel blockers, or sulfonylureas.9

In a study by Kuhn (2008), the authors examined skin samples from patients with various subtypes of CLE and found a deficient number of CD4+CD25+ regulatory T cells (Treg), which are important for suppression of the immune response to self-antigens.10 Abnormal cytokine levels, such as increased type I interferons, have also been observed in patients with CLE.


Jun 29, 2020 | Posted by in Dermatology | Comments Off on Connective Tissue Diseases
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