Clinical Features of Patients with Morphea and the Pansclerotic Subtype: A Cross-sectional Study from the Morphea in Adults and Children Cohort

Abstract

Introduction Pansclerotic morphea is a poorly described form of morphea with little information on prevalence, demographics, and clinical features. Classification criteria for this subtype varies and the distinction from other forms of morphea such as extensive generalized morphea and pansclerotic morphea is not always clear. The purpose of this study was to clarify classification criteria for pansclerotic morphea by identifying its prevalence in the morphea in adults and children (MAC) cohort and describing its demographic and clinical features as compared with generalized morphea. Methods Patients who met predefined criteria for generalized and pansclerotic morphea were identified using a modified Laxer and Zulian classification system. Baseline demographic and clinical features of the patients were compiled and then analyzed for traits characteristic of pansclerotic morphea versus those of generalized morphea. 113 met criteria for inclusion – 13 pansclerotic and 100 generalized morphea type. Results Pansclerotic patients were more frequently male (46.2% vs. 6%, p<0.0001); had a shorter time to diagnosis (mean difference of 10.4 months [95% CI: 0.8-19.9 months], p=0.0332); higher rates of functional impairment (61.5% vs. 16%, p=0.0046); higher rates of deep involvement (61.5% vs. 17%, p=0.004); and higher average mRSS (mean difference of 10.8 points [95% CI: 5-16.6], p=0.0018), LoSDI (mean difference 28.3 [95% CI: 9-47.6], p=0.009), and PGA-D scores (mean difference 25.1 [95% CI: 0.3-50], p=0.048). Conclusions Our results suggest demographic and clinical features are sufficient to define the pansclerotic subtype as they represent a distinct clinical phenotype with a more rapidly progressive and severe course commonly accompanied by disability. Presence of features of the pansclerotic phenotype should alert practitioners to the possibility of significant morbidity and the need for early aggressive treatment.

Morphea in Adults and Children Cohort III

“…Nonetheless, our own cross-sectional survey of dermatologists and rheumatologists practicing in the United States revealed that 15% to 47% order ANA testing in the evaluation of their patients with morphea. 8 …”

Systemic involvement in localized scleroderma/morphea

“…Restrictive defects in these patients is attributed to circumferential involvement of the chest. 35 In a large study among children with LoSc, less than 1% had respiratory involvement consisting of restrictive changes presented as moderate dyspnea and/or persistent cough and pulmonary insufficiency. In this study, results of pulmonary function tests showed a mild decrease in respiratory volume in some patients and abnormalities in diffusing capacity carbon monoxide (DLCO) in other.…”

“…34 Circumferential full thickness sclerosis of the forearm can cause hand edema with necrosis of the fingertips and autoamputation. 35 Bone involvement, which is always secondary, consists of osteoporosis and retardation of growth. The vertebral column is involved rarely, often secondary to linear subset affecting lower limbs.…”

Scleroderma Mimickers

“…Patients may develop dysphagia secondary to external compression (76). In the setting of normal CT scans, restrictive patterns have been observed on pulmonary function tests; attributed to external limitations of rib cage mobility (77). Other extra-dermal manifestations are rare.…”