Histopathological changes in morphea and their clinical correlates: Results from the Morphea in Adults and Children Cohort V.

BACKGROUND: Histopathological features in morphea (localized scleroderma) and their clinical correlates are poorly described. OBJECTIVE: We sought to systematically describe histologic changes of morphea in a large, well-annotated cohort and determine the association between histopathology and clinical features. METHODS: This was a cross-sectional study of 83 patients enrolled in the Morphea in Adults and Children cohort. The main outcome measure was the association of microanatomical location and degree of sclerosis and inflammation seen on histologic samples with patient-reported symptoms and physician-based measures of severity. RESULTS: Pattern of sclerosis was associated with morphea subtype, the presence of patient-reported symptoms, and functional limitation. A bottom-heavy pattern of sclerosis was associated with pain and tightness (P = .0039 and .001, respectively). These symptoms were not associated with a top-heavy pattern. Severe inflammation may be associated with pain and functional limitation (P = .073 for both). LIMITATIONS: Small sample size limits ability to detect associations, particularly in subgroups. CONCLUSIONS: Histopathological examination of morphea may assist in identifying patients who may require additional monitoring and treatment. Features such as patterns of sclerosis and severity of inflammation should be included in pathology reports to help aid in clinical management.

Keratinocyte-Derived Chemokines Orchestrate T-Cell Positioning in the Epidermis during Vitiligo and May Serve as Biomarkers of Disease.

Vitiligo is an autoimmune disease of the skin that results in the destruction of melanocytes and the clinical appearance of white spots. Disease pathogenesis depends on IFN-γ and IFN-γ-induced chemokines to promote T-cell recruitment to the epidermis where melanocytes reside. The skin is a complex organ, with a variety of resident cell types. We sought to better define the microenvironment and distinct cellular contributions during autoimmunity in vitiligo, and we found that the epidermis is a chemokine-high niche in both a mouse model and human vitiligo. Analysis of chemokine expression in mouse skin showed that CXCL9 and CXCL10 expression strongly correlate with disease activity, whereas CXCL10 alone correlates with severity, supporting them as potential biomarkers for following disease progression. Further studies in both our mouse model and human patients showed that keratinocytes were the major chemokine producers throughout the course of disease, and functional studies using a conditional signal transducer and activator of transcription (STAT)-1 knockout mouse showed that IFN-γ signaling in keratinocytes was critical for disease progression and proper autoreactive T-cell homing to the epidermis. In contrast, epidermal immune cell populations including endogenous T cells, Langerhans cells, and γδ T cells were not required. These results have important clinical implications, because topical therapies that target IFN-γ signaling in keratinocytes could be safe and effective new treatments, and skin expression of these chemokines could be used to monitor disease activity and treatment responses.

Confetti-like depigmentation: A potential sign of rapidly progressing vitiligo.

BACKGROUND: Confetti-like depigmentation was noted in patients reporting recent worsening of vitiligo. OBJECTIVE: We sought to determine if confetti-like depigmentation is a marker of rapidly progressing vitiligo. METHODS: Review of patient records and images of patients from a vitiligo registry resulted in 7 patients with 12 images that fit inclusion criteria and were evaluated for percent depigmentation by 3 independent reviewers. The Vitiligo Disease Activity Score and the Koebner Phenomenon in Vitiligo Score in an additional cohort of patients with confetti-like lesions were compared with patients who had vitiligo without confetti-like lesions. RESULTS: The mean percentage of depigmentation at baseline was 19.2%, which increased to 43.9% in images obtained at a mean of 16 weeks of follow-up. Vitiligo Disease Activity Score and Koebner Phenomenon in Vitiligo Score were significantly higher in the patients with confetti-like lesions compared with those without confetti-like lesions. A skin biopsy specimen of a confetti-like lesion in 1 patient revealed an inflammatory infiltrate in the papillary dermis with CD8(+) T cells localized to the dermoepidermal junction. LIMITATIONS: Small, single-center retrospective review and lack of full-body photographs are limitations. CONCLUSIONS: A confetti-like pattern of depigmentation may be a negative prognostic indicator for patients with rapidly progressing vitiligo. Further, prospective studies to evaluate this physical finding should be performed.

Suction blister grafting for vitiligo: efficacy and clinical predictive factors.

BACKGROUND: Suction blister epidermal grafting (SBEG) is a well-established treatment modality for vitiligo, but predictive factors for outcomes are not well characterized. OBJECTIVE: To determine the efficacy and predictive variables for response to SBEG in patients with vitiligo. MATERIALS AND METHODS: A retrospective single-center review of all cases treated with SBEG was performed. Repigmentation was assessed by 2 independent reviewers by assessing pigment spread of grafts during the postoperative period. Repigmentation rates were then compared with patient demographics and transplant location. RESULTS: A total of 28 patients were enrolled in this study. The total number of grafts was 129, of which 86.8% (112/129) survived. Highest rate of graft survival was seen in patients younger than 20 years (100%) and the lowest in patients older than 40 years (75%-78%). Repigmentation was seen in 68% of patients. The highest degree of pigment spread was on the neck (283%) and face (231%), whereas the hands and feet had the least response (119%). CONCLUSION: Blister grafting is successful in most patients with vitiligo, with a high graft survival rate; however, the degree of pigment spread is variable and depends on clinical characteristics of the patient and graft site.