Morphea in adults and children cohort II: patients with morphea experience delay in diagnosis and large variation in treatment

Abstract

Background: Little is known about the diagnosis, evaluation, and therapy of morphea (localized scleroderma) in the United States. Delays in diagnosis and initiation of appropriate therapy, if present, may negatively affect patient care. Further, this gap in knowledge hinders planning for clinical trials and therapeutic guidelines. The morphea in adults and children (MAC) cohort is designed to address this gap.

Objective: We sought to determine the duration between morphea onset and diagnosis, specialty of the diagnosing provider, and initial evaluation and therapy in the MAC cohort.

Methods: This was a cross-sectional survey of the inception cohort of the MAC study.

Results: In all, 63% (n = 141 of 224) of patients were given the diagnosis more than 6 months after onset. Dermatologists diagnosed and treated the majority of patients (83.5%, n = 187). Rheumatologists diagnosed and treated the more severe forms of morphea (linear and generalized). The most commonly prescribed therapy was topical corticosteroids (63%). Dermatologists predominantly prescribed topical treatments or phototherapy (P < .0001, P = .0018, respectively), even to patients with linear and generalized morphea. In contrast, rheumatologists predominantly prescribed systemic immunosuppressives and physical therapy (P < .0001, P = .0021, respectively).

Limitations: Referral bias and recall bias may affect patterns of evaluation/therapy and ascertainment of disease duration before diagnosis.

Conclusions: Patients with morphea experience delay in diagnosis, which likely impacts outcome. Therapeutic decision making is largely determined by the specialty of the provider rather than disease characteristics and many treatments with little or no proven efficacy are used, whereas others with proven efficacy are underused. This underscores the need for a collaborative, multispecialty approach in designing therapeutic trials and guidelines.