Atrophic Papulosis

BACKGROUNDAtrophic papulosis is a rare occlusive vasculopathy that can present with isolated cutaneous disease or with systemic involvement. Pediatric cases are uncommon, and existing reports are heterogeneous, limiting understanding of disease patterns, timing of organ involvement, and outcomes.

METHODSA systematic literature search identified pediatric patients (< 18 years at symptom onset) diagnosed with atrophic papulosis. Case-level data were extracted where available. Patients were classified as benign atrophic papulosis (BAP) or malignant atrophic papulosis (MAP). Descriptive analyses were performed to summarize organ involvement, timing relative to cutaneous onset, multisystem disease patterns, treatments, and outcomes.

RESULTSThirty-six reports describing 54 pediatric patients were identified. At the most recent follow-up, 8 patients (14.8%) were classified as BAP and 46 (85.2%) as MAP. Among MAP patients, central nervous system (CNS) involvement was most common (71.7%), followed by gastrointestinal involvement (67.4%). Multisystem extracutaneous disease occurred frequently (78.3%). Systemic involvement typically developed within the first year after the onset of cutaneous disease, although the interval varied substantially among patients. Treatment outcomes ranged from clinical stability to progressive multisystem disease and death.

CONCLUSIONSIn pediatric patients, atrophic papulosis is often classified as malignant, with frequent CNS and gastrointestinal involvement. These findings underscore the need for early recognition and longitudinal follow-up.

Abstract from PubMed