Caracterización clínica de las morfeas en pacientes pediátricos chilenos

Abstract

Introduction

Morphea is an uncommon autoimmune disease, characterized by thickening and induration of the skin. Thirty-four percent of the cases affected pediatric patients, generating significant morbidity and disability. The literature of this pathology in the pediatric Latino population is very scarce.

Objective

To characterize clinical features of Chilean pediatric patients with a diagnosis of morphea.

Methodology

Retrospective study, based on the review of clinical records of patients aged 0–15 years with a diagnosis of morphea, between 2009 and 2019, in three pediatric Chilean hospitals. Demographic variables (age and sex), clinical variables (diagnosis time, subtype, location, laterality, comorbidities and treatment), diagnostic confirmation method (skin biopsy or skin ultrasound) and laboratory tests (hemogram and antinuclear antibodies) were analyzed.

Results

Forty-four patients, 32 female (72.7%). Average age 6.3 (± 4.2) years. Clinical subtypes: 19 cases (43.2%) lineal morphea (ML), 4 of them subtype “in coup de sabre”; 17 (38.6%) circumscribed morphea (MC), 4 (9.1%) panesclerosing morphea (MP); 3 (6.8%) mixed morphea and 1 case (2.3%) generalized morphea. The most frequent locations were lower limbs (29.5%), trunk (20.5%) and head (13.6%). A total of 14/44 patients (35.9%) presented positive antinuclear antibodies and 7/44 (17.9%) eosinophilia in blood count.

Conclusions

This is the first study on Chilean pediatric patients with morphea. Like international literature, it was more frequent in female and the predominant subtypes were ML and MC. The higher number MP cases stands out, exceeding several international series; this fact is very relevant, since this subtype generates the greatest disability, and its therapy is more complex.