Objective: Relative to studies of systemic lupus erythematosus (SLE), epidemiologic studies of chronic cutaneous lupus erythematosus (CCLE) are rare and are limited to populations with no racial diversity. We sought to provide minimum estimates of the incidence of primary CCLE (CCLE in the absence of SLE) in a population comprised predominantly of white individuals and black individuals in the southeastern region of the US.
Methods: The Georgia Lupus Registry allowed for the use of multiple sources for case-finding, including dermatology and rheumatology practices, multispecialty health care facilities, and dermatopathology reports. Cases with a clinical or clinical/histologic diagnosis of CCLE were classified as definite. Cases ascertained exclusively from dermatopathology reports were categorized as probable. Age-standardized incidence rates stratified by sex and race were calculated for discoid lupus erythematosus (DLE) in particular and for CCLE in general.
Results: The overall age-adjusted estimates for combined (definite and probable) CCLE were 3.9 per 100,000 person-years (95% confidence interval [95% CI] 3.4–4.5). The overall age-adjusted incidences of definite and combined DLE were 2.9 (95% CI 2.4–3.4) and 3.7 (95% CI 3.2–4.3) per 100,000 person-years, respectively. When capture–recapture methods were used, the age-adjusted incidence of definite DLE increased to 4.0 (95% CI 3.2–4.3). The black:white and female:male incidence ratios for definite DLE were 5.4 and 3.1, respectively.
Conclusion: Our findings underscore the striking racial disparities in susceptibility to primary CCLE, with black individuals having a 3-fold to 5-fold increased incidence of CCLE in general, and DLE in particular, compared with white individuals. The observed sex differences were consistent with those reported previously, with a 3 times higher risk of DLE in women compared with men.
Objective:
Depression may occur in up to 30% of individuals with cutaneous lupus erythematosus (CLE), many of whom may also have systemic manifestations. Compared to acute and subacute, chronic cutaneous lupus erythematosus (CCLE) conditions are less likely to present systemic involvement, but more often cause permanent scarirng and dyspigmentation. Little is known, however, about depression in those who have CCLE confined to the skin (primary CCLE). As African Americans are at high risk for primary CCLE and depression, we aimed to investigate the prevalence and explore risk factors of depression in a predominantly Black population-based cohort of patients with primary CCLE.
Methods:
Cross-sectional analysis of a cohort of individuals with a documented diagnosis of primary CCLE, which is established in the metropolitan Atlanta. Participants were recruited from the Centers for Disease Control and Prevention (CDC) population-based Georgia Lupus Registry, multi-center dermatology clinics, community practices, and self-referrals. The Patient Reported Outcomes Measurement Information System (PROMIS) was used to measure the primary outcome: depressive symptoms. Stand-alone questions were used to assess sociodemographics and healthcare utilization. Emotional, informational, and instrumental support were measured with PROMIS short forms, interpersonal processes of care with the IPC-29 Survey, and skin-related quality of life with the Skindex-29+ tool.
Results:
Of 106 patients, 92 (86.8%) were female, 91 (85.8%) Black, and 45 (42.9%) unemployed or disabled. Twenty-eight (26.4%) reported moderate to severe depressive symptoms. Depression severity was lower in patients aged ≥60, married, or college-graduated. Univariate analysis showed that being employed (OR=0.24, 95% Confidence Interval (CI)=0.10-0.61), insured (OR=0.23, 95%CI=0.09-0.60), reporting higher instrumental, informational, and emotional support (OR=0.94, 95%CI=0.90-0.99; OR=0.91, 95%CI=0.87-0.95; and OR=0.86, 95%CI=0.81-0.92, respectively), visiting a primary care physician in the last year (OR=0.16, 95%CI=0.04-0.61) and reporting better physician-patient interactions (OR=0.56, 95%CI=0.37-0.87) were negatively associated with depression. Patient’s perceptions of staff disrespect (OR=2.30, 95%CI=1.19-4.47) and worse skin-related quality of life (OR=1.04, 95%CI=1.02-1.06) rendered higher risk. In multivariate analysis, only perception of staff disrespect (OR=2.35, 95%CI=1.06-5.17) and lower emotional support (OR=0.48, 95%CI=0.35-0.66) remained associated with depression.
Conclusions:
Over one quarter of a predominantly Black population-based cohort of individuals with primary CCLE reported moderate to severe depression, a rate 3 to 5 times higher than those described previously in the general population from the same metropolitan Atlanta area. Our findings suggest that while patient’ perceptions of discrimination in the healthcare setting may play a role as determinant of depression, social support may be protective. In addition to routine mental health screening and depression treatment, patients with CCLE and depression may benefit from interventions directed to provide emotional support and improve office staff interpersonal interactions.
Cryptococcoid Sweet syndrome is a rare histologic variant of the neutrophilic dermatosis presenting clinically with skin lesions typical of classical Sweet syndrome but with yeast-like structures suggestive of Cryptococcus on histopathology. Histochemical stains for fungus and cultures are negative whereas staining for myeloperoxidase is positive. We present 2 cases of cryptococcoid Sweet syndrome with atypical skin manifestations, including hemorrhagic bullae and plaques, and provide a brief review of the literature. Clinicians should be aware that this variant of Sweet syndrome can present with uncommon clinical findings and has histopathologic findings suggestive of Cryptococcus species.
Objective Depression is common in individuals with chronic cutaneous lupus erythematosus (CCLE). However, how CCLE may impact patients' psychological well-being is poorly understood, particularly among disproportionally affected populations. We examined the relationships between depression and psychosocial factors in a cohort of predominantly Black patients with primary CCLE (CCLE without systemic manifestations). Methods Cross-sectional assessment of individuals with dermatologist-validated diagnosis of primary CCLE. NIH-PROMIS short-forms were used to measure depression, disease-related stigma, social isolation and emotional support. Linear regression analyses (I'=0.05) were used to test an a priori conceptual model of the relationship between stigma and depression and the effect of social isolation and emotional support on that association. Results Among 121 participants (87.6% women; 85.1% Black), 37 (30.6%) reported moderate to severe depression. Distributions of examined variables divided equally among those which did (eg, work status, stigma (more), social isolation (more), emotional support (less)) and did not (eg, age, sex, race, marital status) significantly differ by depression. Stigma was significantly associated with depression (b=0.77; 95% CI0.65 to 0.90), whereas social isolation was associated with both stigma (b=0.85; 95% CI 0.72 to 0.97) and depression (b=0.70; 95% CI0.58 to 0.92). After controlling for confounders, stigma remained associated with depression (b=0.44; 95% CI0.23 to 0.66) but lost significance (b=0.12; 95% CI -0.14 to 0.39) when social isolation (b=0.40; 95% CI 0.19 to 0.62) was added to the model. Social isolation explained 72% of the total effect of stigma on depression. Emotional support was inversely associated with depression in the univariate analysis; however, no buffer effect was found when it was added to the multivariate model. Conclusion Our findings emphasise the psychosocial challenges faced by individuals living with primary CCLE. The path analysis suggests that stigmatisation and social isolation might lead to depressive symptoms. Early clinical identification of social isolation and public education demystifying CCLE could help reduce depression in patients with CCLE.
While the contribution of B-cells to SLE is well established, its role in chronic cutaneous lupus erythematosus (CCLE) remains unclear. Here, we compare B-cell and serum auto-antibody profiles between patients with systemic lupus erythematosus (SLE), CCLE, and overlap conditions. B-cells were compared by flow cytometry amongst healthy controls, CCLE without systemic lupus (CCLE+/SLE−) and SLE patients with (SLE+/CCLE+) or without CCLE (SLE+/CCLE−). Serum was analyed for autoreactive 9G4+, anti-double-stranded DNA, anti-chromatin and anti-RNA antibodies by ELISA and for anti-RNA binding proteins (RBP) by luciferase immunoprecipitation. Patients with CCLE+/SLE− share B-cell abnormalities with SLE including decreased unswitched memory and increased effector B-cells albeit at a lower level than SLE patients. Similarly, both SLE and CCLE+/SLE- patients have elevated 9G4+ IgG autoantibodies despite lower levels of anti-nucleic acid and anti-RBP antibodies in CCLE+/SLE−. CCLE+/SLE− patients could be stratified into those with SLE-like B-cell profiles and a separate group with normal B-cell profiles. The former group was more serologically active and more likely to have disseminated skin lesions. CCLE displays perturbations in B-cell homeostasis and partial B-cell tolerance breakdown. Our study demonstrates that this entity is immunologically heterogeneous and includes a disease segment whose B-cell compartment resembles SLE and is clinically associated with enhanced serological activity and more extensive skin disease. This picture suggests that SLE-like B-cell changes in primary CCLE may help identify patients at risk for subsequent development of SLE. B-cell profiling in CCLE might also indentify candidates who would benefit from B-cell targeted therapies.
To the editor: We enjoyed reading the case series on pernio-like eruptions in skin of color and appreciate the awareness the authors brought to this COVID-19–associated manifestation, previously largely documented in Fitzpatrick skin types I and II and darker skin types.1 Pityriasis rosea-like manifestations have been reported in COVID-19–positive patients,2, 3, 4 though similar published images largely show presentations in lighter skin types.5 Here, we present 2 cases of pityriasis rosea in Fitzpatrick types III and IV skin in otherwise asymptomatic COVID-19–positive patients. We hope that they contribute to an accurate diagnosis of COVID-19 manifestations in darker skin types.