Natural history and extracutaneous involvement of congenital morphea: Multicenter retrospective cohort study and literature review
Why this work is in the frame
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Bibliographic record
Abstract
BACKGROUND: Congenital morphea is a form of localized scleroderma that presents at birth. There is limited information on its presentation and progression. METHODS: Patients with congenital morphea were identified from five pediatric dermatology and rheumatology tertiary care centers in Canada, the United States, and Italy from 2001 to 2016. Cases from the literature were identified by searching Ovid (EMBASE and MEDLINE) from inception to June 30, 2017. Disease characteristics and prevalence of extracutaneous involvement were analyzed. RESULTS: Thirteen patients were identified from the five centers, and 13 cases were described in the literature, representing 25 patients, with one duplication. Fourteen patients (56%) were female. Median age at diagnosis was 2.9 years (interquartile range 1.2-5.1 years). Linear morphea, including en coup de sabre and Parry-Romberg syndrome, was the most common subtype observed (n = 19, 76%), followed by circumscribed (n = 5, 20%), generalized (n = 2, 8%), and mixed (n = 2, 8%). The face (n = 14, 56%), scalp (n = 8, 32%), and trunk (n = 6, 24%) were the most common locations affected. Most lesions were active at diagnosis (n = 19, 76%), but all patients with follow-up later became inactive. Extracutaneous involvement was seen in 12 (48%) patients, all of whom had linear morphea. Musculoskeletal sequelae were seen in those with linear morphea of the extremities (4/5, 80%), and neurologic involvement was seen in those with linear morphea of the head (8/13, 62%). CONCLUSION: Congenital morphea is associated with extracutaneous manifestations and delayed diagnosis. More research is needed to determine whether early recognition, monitoring, and treatment can alter the disease course.
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Full frame distilled prediction
Teacher imitationNot calibrated prevalence, not ground truth. Human validation pending. Learned from the 10,348 direct Codex labels and 10,348 direct Gemma labels. Candidate is the union of thresholded teacher heads; consensus is their intersection. These outputs are machine_predicted_unvalidated and are not human labels or direct frontier model labels.
Codex and Gemma teacher scores by category
| Category | Codex | Gemma |
|---|---|---|
| Metaresearch | 0.000 | 0.000 |
| Meta-epidemiology (narrow) | 0.000 | 0.000 |
| Meta-epidemiology (broad) | 0.003 | 0.000 |
| Bibliometrics | 0.000 | 0.000 |
| Science and technology studies | 0.000 | 0.000 |
| Scholarly communication | 0.000 | 0.000 |
| Open science | 0.000 | 0.000 |
| Research integrity | 0.000 | 0.001 |
| Insufficient payload (model declined to judge) | 0.000 | 0.000 |
Machine scores (provisional)
The two teacher heads of the student model, read on this work. A score orders the frame for review; it never asserts a category, and the validation status ships verbatim with every row.
Baseline scores from an immature model (maturity gate not passed, 7 training rounds). Scores rank; they never assert a category.
score_only:v0-immature-baseline · verbatim from the scoring run: score_only means the number may rank works, and no category label ships from it