Atrophic Papulosis With Gl or Neurological Symptoms More Common in Children

Unlike adults, many children with atrophic papulosis (AP) present with gastrointestinal and neurological symptoms, data from a study published in the Journal of the European Academy of Dermatology & Venerology found

Investigators from Dessau Medical Center in Germany pooled data from a cross-sectional study conducted at their center from 2000 through 2021 with data from the European Rare Disease Registry Infrastructure (ERDRI), European Platform on Rare Diseases (EU RD), and the REDCap platform. Trends in AP features and outcomes were compared between pediatric (0-17 years of age) and adult (older than 17 years of age) patients.

A total of 19 pediatric and 77 adult patients with AP were included in this study. The patient groups differed as to male:female ratios (1.7:1 vs 1:2.2; P =.01), age at onset (median, 5 vs 38 years; P <.001), rates of malignant AP (78.9% vs 53.3%; P =.042), central nervous system involvement (80% vs 53.6%; P =.005), gastrointestinal involvement (73.3% vs 58.5%; P =.03), and cardiac involvement (33.3% vs 2.4%; P <.001), respectively.

The mortality rate of patients with pediatric AP was 31.6% and only occurred in the setting of malignant AP. The median survival time from onset was 15 months, ranging from 1.7 months to 6 years. The causes of death were documented as involving the central nervous system (n=3), gastrointestinal system (n=2), and unknown (n=1).

Mortality was associated with other organ involvement (hazard ratio [HR], 14.01; 95% CI, 3.78-51.9; P <.0001) and central nervous system involvement (HR, 8.43; 95% CI, 2.12-33.48; P =.0024) among patients with malignant AP.

The limitations of this study included the small sample sizes and missing data.

Study authors concluded, “Our study provides evidence for the occurrence and severity of this rare but potentially life-threatening disease in pediatric patients. The fact that malignant AP is more common in children than in adults makes it essential to raise awareness on the disease among pediatricians, who may probably identify first the characteristic skin lesions.”