Comorbid diseases of vitiligo: A 10-year cross-sectional retrospective study of an urban US population.

BACKGROUND: Vitiligo is associated with medical conditions, primarily autoimmune disorders; however, only a few studies in the United States have investigated these associations. OBJECTIVE: Our purpose was to investigate the diseases associated with vitiligo in the New York, New York, population and evaluate if these associations differ by race/ethnicity and sex. METHODS: In this retrospective study, we analyzed data collected from the medical records of 1487 vitiligo patients seen at New York University during a 10-year period. RESULTS: Vitiligo patients had a statistically significant higher prevalence of hypothyroidism, multiple sclerosis, rheumatoid arthritis, idiopathic thrombocytopenic purpura, seronegative arthritis, pernicious anemia, myasthenia gravis, inflammatory bowel disease, lymphoma, and systemic lupus erythematosus. Rates of comorbid autoimmune diseases varied by race and sex. LIMITATIONS: Medical charts did not consistently report race/ethnicity, type of vitiligo, and total body surface area affected. Information from nondermatology medical visits was also included. CONCLUSION: This study revealed multiple new disease associations for vitiligo, including multiple sclerosis, idiopathic thrombocytopenic purpura, and lymphoma, as well as confirmed previously reported associations with other autoimmune diseases, the most common being hypothyroidism followed by rheumatoid arthritis. Associations did vary by race/ethnicity and sex.

Calciphylaxis with pseudoxanthoma elasticum-like changes: A case series.

BACKGROUND: Calciphylaxis and pseudoxanthoma elasticum (PXE) are rare, clinically distinct disorders that share a common feature of cutaneous calcification and that vary widely in their cutaneous presentation. METHODS: We conducted a descriptive, retrospective review of biopsy specimens collected over a 2-year period. Only specimens with a histologic and clinical diagnosis of calciphylaxis were included in the review. Specimens were then histologically examined for features of PXE in the dermis and/or subcutaneous fat, utilizing hematoxylin and eosin staining. Von Kossa and Verhoeff-Van Gieson special stains were also performed to examine calcification and elastic fibers, respectively. RESULTS: We reviewed 13 biopsy specimens from 9 patients with known clinical and histologic evidence of calciphylaxis, both uremic and non-uremic types. Upon re-examination, we found that 46.2% (n = 6/13) of the specimens showed concomitant PXE-like changes uniquely localized to the subcutaneous fat. CONCLUSION: The presence of PXE-like changes in the subcutis may heighten suspicion for a diagnosis of calciphylaxis in the appropriate clinical setting and be helpful when classic histologic features are subtle or absent.

Generalized essential telangiectasia.

The pathophysiology of generalized essential telangiectasia is not well understood. Generalized essential telangiectasia is an uncommon disorder in which widespread telangiectasias of unknown cause develop without associated systemic or antecedent dermatologic disease. We report a case of generalized essential telangiectasia in an otherwise healthy 49-year-old man.

Acquired immune deficiency syndrome-related epidemic Kaposi sarcoma.

Kaposi sarcoma (KS) is a vascular neoplasm that is one of the most common human immunodeficiency virus (HIV)-related malignancies. We present the case of a 42-year-old man with a new diagnosis of HIV and acquired immune deficiency syndrome (AIDS)-related epidemic KS.

Cutaneous Crohn disease without gastrointestinal involvement in a 9-year-old boy.

Cutaneous Crohn disease (CCD) is a rare dermatologic manifestation of Crohn disease and is defined as noncaseating, granulomatous skin lesions noncontiguous with the gastrointestinal tract. It most commonly affects the skin of the legs, although genital CCD is the most common presentation in children. Diagnosis of CCD is made by a combination of clinical and histopathological findings. Therapeutic options include topical, intralesional, and systemic corticosteroids as well as topical and systemic immunosuppressants and immunomodulators. Surgical excision may be considered for refractory cases. We report CCD in a 9-year old boy with penile swelling, granulomatous cheilitis-like lesions, and perianal plaques.

De Novo vs Nevus-Associated Melanomas: Differences in Associations With Prognostic Indicators and Survival.

BACKGROUND: Although 20% to 30% of melanomas are histopathologically 'nevus associated,' the majority of melanomas arise de novo, ie, in clinically normal skin with no associated nevus. We examined whether these forms of melanoma differed in their associations with clinical and histopathologic features and patient survival. METHODS: We analyzed two prospective cohorts from our institution with protocol-driven follow-up information (NYU1, n = 1024; NYU2, n = 1125). We used univariate and multivariable analyses to examine associations between de novo vs nevus-associated melanoma classification and age, anatomic site, tumor thickness, tumor ulceration, mitotic index, histological subtype, clinical stage, and survival. We tested the associations identified in NYU1 using NYU2 as a replication cohort. All tests of statistical significance were two-sided. RESULTS: In NYU1, de novo melanomas were associated with tumor thickness greater than 1.0 mm (odds ratio [OR] = 1.96, 95% confidence interval [CI] = 1.43 to 2.70, P < .001), ulceration (OR = 1.65, 95% CI = 1.10 to 2.54, P = .02), nodular subtype (OR = 3.26, 95% CI = 1.70 to 7.11, P = .001), greater than stage I (OR = 2.35, 95% CI = 1.65 to 3.40, P < .001), older age (OR = 1.64, 95% CI = 1.18 to 2.30, P = .004), and shorter overall survival (HR = 1.63, 95% CI = 1.22 to 2.18, P < .001). In NYU2, de novo melanoma was again statistically significantly associated with thickness greater than 1.0 mm (OR = 2.24, 95% CI = 1.72 to 2.93, P < .001), ulceration (OR = 2.88, 95% CI = 1.95 to 4.37, P < .001), nodular subtype (OR = 2.41, 95% CI = 1.75 to 3.37, P < .001), greater than stage I (OR = 2.42, 95% CI = 1.80 to 3.29, P < .001), older age (OR = 1.68, 95% CI = 1.31 to 2.17, P < .001), and shorter overall survival (HR = 2.52, 95% CI = 1.78 to 3.56, P < .001). In multivariable analysis, de novo classification was an independent, poor prognostic indicator in NYU2 (HR = 1.70, 95% CI = 1.19 to 2.44, P = .004). Male patients had a statistically significantly worse survival than female patients if their melanoma was de novo (NYU1, P < .001; NYU2, P < .001); unexpectedly, there was no sex difference in survival among patients with nevus-associated tumors. CONCLUSIONS: These data suggest that de novo melanomas are more aggressive than nevus-associated melanomas. This classification scheme may also provide a useful framework for investigations into sex differences in melanoma outcomes.

Vulvar nevi, melanosis, and melanoma: an epidemiologic, clinical, and histopathologic review.

Pigmented vulvar lesions are present in approximately 1 in 10 women and include melanocytic and nonmelanocytic proliferations. Vulvar nevi, melanosis, and melanoma are particularly challenging because of the similarity of their clinical and/or histopathological presentation. As a result, they are often difficult to diagnose, may result in patient and physician anxiety, and can lead to unneeded, potentially disfiguring surgical procedures. Because it is often detected late, vulvar melanoma carries a poor prognosis with associated significant morbidity and mortality, underscoring the importance of prompt recognition and treatment. In this review, we analyze the distinct epidemiologic, clinical, and histopathologic characteristics of vulvar nevi, melanosis, and melanoma, discuss treatment options, and propose a practical, systematic approach to facilitate formulation of a differential diagnosis and initiation of appropriate management.

Development of a melanoma risk prediction model incorporating MC1R genotype and indoor tanning exposure: impact of mole phenotype on model performance.

BACKGROUND: Identifying individuals at increased risk for melanoma could potentially improve public health through targeted surveillance and early detection. Studies have separately demonstrated significant associations between melanoma risk, melanocortin receptor (MC1R) polymorphisms, and indoor ultraviolet light (UV) exposure. Existing melanoma risk prediction models do not include these factors; therefore, we investigated their potential to improve the performance of a risk model. METHODS: Using 875 melanoma cases and 765 controls from the population-based Minnesota Skin Health Study we compared the predictive ability of a clinical melanoma risk model (Model A) to an enhanced model (Model F) using receiver operating characteristic (ROC) curves. Model A used self-reported conventional risk factors including mole phenotype categorized as "none", "few", "some" or "many" moles. Model F added MC1R genotype and measures of indoor and outdoor UV exposure to Model A. We also assessed the predictive ability of these models in subgroups stratified by mole phenotype (e.g. nevus-resistant ("none" and "few" moles) and nevus-prone ("some" and "many" moles)). RESULTS: Model A (the reference model) yielded an area under the ROC curve (AUC) of 0.72 (95% CI = 0.69, 0.74). Model F was improved with an AUC = 0.74 (95% CI = 0.71-0.76, p<0.01). We also observed substantial variations in the AUCs of Models A & F when examined in the nevus-prone and nevus-resistant subgroups. CONCLUSIONS: These results demonstrate that adding genotypic information and environmental exposure data can increase the predictive ability of a clinical melanoma risk model, especially among nevus-prone individuals.