Comorbid autoimmune diseases in patients with vitiligo: A cross-sectional study.

BACKGROUND: Few large-scale studies have quantified the burden of comorbid autoimmune diseases in patients with vitiligo. OBJECTIVE: We sought to determine the prevalence of comorbid autoimmune diseases in patients with vitiligo. METHODS: We conducted a manual chart review on a cohort of 1873 patients with vitiligo seen between January 2002 and October 2012 at the Henry Ford Health System in Detroit, MI. Patients were excluded if they had fewer than 2 dermatology notes (N = 595) or if they were never given a diagnosis of vitiligo by a dermatologist (N = 180). RESULTS: Of 1098 patients with vitiligo, nearly 20% had at least 1 comorbid autoimmune disease. Compared with the general US population, we found a higher prevalence of thyroid disease (12.9%, P < .001), alopecia areata (3.8%, P < .001), inflammatory bowel disease (0.9%, P = .046), pernicious anemia (0.5%, P = .007), systemic lupus erythematosus (0.3%, P = .048), Guillain-Barre syndrome (0.3%, P < .001), discoid lupus (0.2%, P = .003), linear morphea (0.2%, P < .001), myasthenia gravis (0.2%, P = .002), and Sjögren syndrome (0.2%, P = .011). LIMITATIONS: The study lacked a control group. This was a single-institution study with possible selection bias, and thus the findings may not be representative of the overall population of patients with vitiligo. CONCLUSIONS: We observed a high prevalence of comorbid autoimmune diseases in patients with vitiligo and report several new associations.

Gender-based variability in disease presentation in pemphigus vulgaris.

Pemphigus vulgaris is a chronic autoimmune blistering disorder of the skin. As with many autoimmune diseases, a female predominance in pemphigus vulgaris is well established. The genetic and physiological basis for this gender bias is not well understood. Moreover, it is unclear whether the affect of gender extends beyond disease susceptibility to influence disease presentation. To address this issue, we performed a comprehensive analysis of 72 male and 125 female pemphigus vulgaris patients across a set of defined demographic (HLA type, ethnicity) and clinical (age at disease onset, anti-desmoglein antibody levels, site of lesions, and history of autoimmune disease) factors. We find that male patients are more likely to present with disease onset before age 40 than females. Additionally, we find that males have increased cutaneous involvement and display greater co-expression of anti-Dsg1 and anti-Dsg3 antibodies, while females tend to have mucosal predominance and stronger personal and family histories of autoimmunity. We do not find any differences in the distribution of HLA type or ethnicity between male and female pemphigus vulgaris patients. Our findings establish that gender does influence disease presentation in pemphigus vulgaris, supporting a role for genetic and hormonal factors in immune dysregulation and perpetuation of the autoimmune phenotype.

Update on hidradenitis suppurativa: connecting the tracts.

Hidradenitis suppurativa (HS) is a debilitating skin disease characterized by recurrent abscesses, sinus tract formation, and scarring. Prevalence estimates range from 0.053% to 4.1%, although HS is likely an underdiagnosed disease. Although the first reports of HS date back to the mid-19th century, the disease continues to plague patients and physicians desperate for a definitive treatment. Advances in the understanding of the disease process include the possibility of a defective basement membrane at the sebofollicular junction of the folliculopilosebaceous unit (FPSU; that is, where the sebaceous gland empties into the hair follicle) as an initiating event followed by secondary bacterial colonization. New evidence suggests that bacteria living in a community, known as a biofilm, rather than single planktonic bacteria in HS lesions may explain why HS can be resistant to current antibiotic treatment regimens. Available treatment options have expanded to include triple-antibiotic therapy, tumor necrosis factor (TNF-α) and interleukin-1 (IL-1) inhibitors (biologics), laser therapy, and surgical excision, including the skin tissue-sparing excision with electrosurgical peeling procedure. Despite the array of treatments available, many patients continue to struggle with the embarrassment, pain, odor, and frustration that accompany this often isolating disease. Physicians should address comorbidities in HS, including the psychosocial issues patients with HS frequently encounter. Patients can be directed to HS support groups, where they can openly discuss their frustrations, share their experiences in dealing with HS, and band together to advocate for themselves. HS is misunderstood by both patients and physicians, often resulting in a delay in clinical presentation and diagnosis. Patients and physicians across multiple specialties must work together to expand awareness of and interest in HS, so that one day, individuals with HS can be freed from this crippling disease.