Development and Validation of the Morphea Activity Measure in Patients With Pediatric Morphea.

Importance: Morphea is an insidious inflammatory disorder of the skin and deeper tissues. Determining disease activity is challenging yet important to medical decision-making and patient outcomes. Objective: To develop and validate a scoring tool, the Morphea Activity Measure (MAM), to evaluate morphea disease activity of any type or severity that is easy to use in clinical and research settings. Design, Setting, and Participants: This pilot diagnostic study was conducted from September 9, 2019, to March 6, 2020, in 2 phases: development and validation. During the development phase, 14 morphea experts (dermatologists and pediatric dermatologists) used a Delphi consensus method to determine items that would be included in the MAM. The validation phase included 8 investigators who evaluated the tool in collaboration with 14 patients with pediatric morphea (recruited from a referral center [Medical College of Wisconsin]) during a 1-day in-person meeting on March 6, 2020. Main Outcomes and Measures: During the development phase, online survey items were evaluated by experts in morphea using a Likert scale (score range, 0-10, with 0 indicating not important and 10 indicating very important); agreement was defined as a median score of 7.0 or higher, disagreement as a median score of 3.9 or lower, and no consensus as a median score of 4.0 to 6.9. During the validation phase, reliability (interrater and intrarater agreement using intraclass correlation coefficients), validity (using the content validity index and κ statistics as well as correlations with the modified Localized Scleroderma Severity Index and the Physician Global Assessment of Activity using Spearman ρ coefficients), and viability (using qualitative interviews of investigators who used the MAM tool) were evaluated. Descriptive statistics were used for quantitative variables. Data on race and ethnicity categories were collected but not analyzed because skin color was more relevant for the purposes of this study. Results: Among 14 survey respondents during the development phase, 9 (64.3%) were pediatric dermatologists and 5 (35.7%) were dermatologists. After 2 rounds, a final tool was developed comprising 10 items that experts agreed were indicative of morphea activity (new lesion in the past 3 months, enlarging lesion in the past 3 months, linear lesion developing progressive atrophy in the past 3 months, erythema, violaceous rim or color, warmth to the touch, induration, white-yellow or waxy appearance, shiny white wrinkling, and body surface area). The validation phase was conducted with 14 patients (median age, 14.5 years [range, 8.0-18.0 years]; 8 [57.1%] female), 2 dermatologists, and 6 pediatric dermatologists. Interrater and intrarater agreement for MAM total scores was good, with intraclass correlation coefficients of 0.844 (95% CI, 0.681-0.942) for interrater agreement and 0.856 (95% CI, 0.791-0.901) for intrarater agreement. Correlations between the MAM and the modified Localized Scleroderma Severity Index (Spearman ρ = 0.747; P < .001) and the MAM and the Physician Global Assessment of Activity (Spearman ρ = 0.729; P < .001) were moderately strong. In qualitative interviews, evaluators agreed that the tool was easy to use, measured morphea disease activity at a single time point, and should be responsive to changes in morphea disease activity over multiple time points. Conclusions and Relevance: In this study, the MAM was found to be a reliable, valid, and viable tool to measure pediatric morphea activity. Further testing to assess validity in adults and responsiveness to change is needed.

The safety and efficacy of autologous adipose-derived stromal vascular fraction for nonscarring alopecia: A systematic review.

IMPORTANCE: Nonscarring alopecia, including androgenetic alopecia and alopecia areata, are common and can negatively impact quality of life. Recent clinical studies have investigated autologous, adipose-derived stromal vascular fraction (SVF) as a potentially beneficial treatment option. OBJECTIVE: To assess the available evidence on the utility and safety of SVF for nonscarring alopecia. EVIDENCE REVIEW: A systematic review of the literature was performed using MEDLINE (PubMed), Embase, and CENTRAL from inception to November 2020. Included articles were prospective, observational or interventional studies of SVF for nonscarring alopecia in humans. FINDINGS: Six studies of 188 patients were identified, including three randomized controlled trials. There were no reported severe adverse events. All studies found improved hair density with SVF compared to control or pre-treatment baseline. One study reported that improvement in hair density varied based on time for follow-up, severity of hair loss, and concentration of adipose-derived stem cells (ADSCs) within the SVF. Two studies reported an increase in hair diameter from baseline, and two studies reported an improvement in hair pull test outcomes. CONCLUSIONS AND RELEVANCE: SVF may be safe and effective for nonscarring alopecia in the appropriate patients. Hair loss severity, method of SVF preparation and frequency of treatment, and adjunctive therapies may be important considerations for treatment success. Additional studies evaluating appropriate patient selection and treatment methods are needed.

Metastatic calcinosis cutis in a patient with Hodgkin's lymphoma.

Calcinosis cutis results from the deposition of insoluble calcium salts in the skin and subcutaneous tissue. Herein, we report a case of extensive metastatic calcinosis cutis in an 18-year-old woman with stage IV Hodgkin lymphoma with skeletal involvement. With combination therapy including radiation directed at her lymphoma and diltiazem, her lesions improved dramatically. This case demonstrates the previously unreported association between calcinosis cutis and Hodgkin lymphoma.

Inter-rater reliability of cutaneous sarcoidosis assessment tools via remote photographic assessment.

Background: Recently two outcome instruments have been developed and validated for assessing cutaneous sarcoidosis in a live, in-person setting. Teledermatology is a rapidly growing field; yet, to date, no instrument has been validated for use in a remote setting, which could ultimately impact clinical trial design. Objective: To assess the interrater reliability of these outcome instruments for store-and-forward teledermatology. Methods: Seven sarcoidosis experts, including both pulmonologists and dermatologists, scored photographs of cutaneous sarcoidosis lesions in 13 patients utilizing the Cutaneous Sarcoidosis Activity and Morphology Index (CSAMI), the Sarcoidosis Activity and Severity Index (SASI) and the Physician Global Assessment (PGA). Interrater reliability was assessed for each instrument and was compared to results obtained from a prior study involving sarcoidosis experts evaluating the same patient population in an in-person setting. Results: Interrater reliability (presented as ICC [95%CI]) was poor for the CSAMI Activity scale (0.36 [0.16 - 0.65]) and the CSAMI Damage scale (0.17 [0.04 - 0.43]) and was fair for the Modified Facial SASI (0.59 [0.36 - 0.82]) and the PGA (0.47 [0.23 - 0.74]). All results were inferior to those obtained from the prior studies validating these instruments for in-person use. Conclusions: Given the superiority of these instruments when utilized in person, it is recommended to have an on-site sarcoidosis expert evaluate cutaneous sarcoidosis lesions whenever possible. (Sarcoidosis Vasc Diffuse Lung Dis 2017; 34: 165-169).

Granuloma Annulare.

Granuloma annulare (GA) is a noninfectious granulomatous skin condition that can present with a variety of cutaneous morphologies. It is characterized by collagen degeneration, mucin deposition, and palisaded or interstitial histiocytes. Although the mechanism underlying development of GA is unknown, studies point to a cell-mediated hypersensitivity reaction to an as-yet undetermined antigen. Systemic associations with diabetes, thyroid disorders, lipid abnormalities, malignancy, and infection are described in atypical GA. Treatment is divided into localized skin-directed therapies and systemic immunomodulatory or immunosuppressive therapies. The selected treatment modality should be based on disease severity, comorbid conditions, consideration of potential side effects, and patient preference.