Primary paediatric cutaneous mucinoses

Abstract

Summary Background Primary cutaneous mucinoses are a heterogeneous group of diseases characterized by the abnormal deposition of glycosaminoglycans (mucin) in the skin. This is the main histopathological clue resulting in distinctive clinical presentations. They are usually diseases of adult age and are uncommon in children, in whom diagnosis and management are difficult tasks. In particular, the classification of primary cutaneous mucinoses occurring in childhood is a difficult and confusing challenge because of the very limited number of described cases and overlaps in their clinicopathological features in the absence of reports of homogeneous case series. Objectives The aim of this review is to help dermatologists and paediatricians to identify patients with cutaneous mucinoses at a paediatric age and to enable institution of appropriate management. A classification of primary paediatric cutaneous mucinoses is also proposed. Results New investigations have yielded significant insights into a classification of paediatric cutaneous mucinoses. Conclusions The current update tries to offer a more meaningful guide to the diagnosis and treatment of these rare diseases in paediatric patients. What's already known about this topic? The nosography of primary cutaneous mucinoses occurring in childhood is confusing because of the very limited number of cases and overlaps in their clinicopathological features in the absence of homogeneous case series. The diagnosis and management of paediatric patients with cutaneous mucinoses are also difficult as these diseases are rarely seen in children. What does this study add? New investigations have yielded significant insights into a classification of paediatric cutaneous mucinoses. The current update tries to offer a more meaningful guide to diagnosis and treatment of these rare diseases in patients of paediatric age.

Long‐term follow‐up of a patient with congenital cutaneous mucinosis of infancy and description of a new case

“…In our report (case 1), with a follow‐up of 6 years, we observed an increase in the number of skin lesions in the first years of life, followed by almost complete resolution in the following years, without symptoms or signs of associated systemic disease. The first case highlights the benign clinical course of CMI, while case 2 presented with infrequent histologic findings, although previously reported, such as mucin deposition in the reticular dermis, together with a slight proliferation of fibroblasts.…”

“…Primary (idiopathic) pediatric cutaneous mucinoses (PPCM) are a group of rare disorders characterized by mucin deposition as the main histologic feature . The nosography of PPCM can be confusing, and recently a new classification has been proposed by Rongioletti . PPCM can be divided into degenerative/inflammatory and hamartomatous/neoplastic (including mucinous nevus, mucinous eccrine nevus, superficial angiomyxoma, and nevoid follicular mucinosis).…”

“…Spontaneous resolution is observed within weeks or months (most frequently 2–8 months). On histopathology, SHCM may reveal mucin deposition in the dermis in the papular lesions and fibroblast proliferation, fasciitis‐like features, or lobar panniculitis with a plump spindle‐cell infiltrate in the nodules . Differential diagnosis also includes atypical or intermediate forms of papular mucinosis of infancy (CMI/SHCM overlap), exceedingly rare conditions with only a few cases reported …”

Cutaneous mucinosis of infancy: a rare entity in pediatrics

“…Cutaneous mucinoses usually affect adults and are uncommon in children. Primary pediatric cutaneous mucinoses have been classified into degenerative/inflammatory and hamartomatous/neoplastic 3 . CMI is an uncommon subtype of papular mucinosis; it can be present at birth or start in early childhood 4‐5 .…”

Classifying primary paediatric cutaneous mucinoses

“…In this issue of the BJD , Rongioletti reviews and classifies paediatric cutaneous mucinoses and to my knowledge, this is the first review specifically on cutaneous mucinoses in the paediatric age group . Primary paediatric cutaneous mucinoses are even rarer than the adult conditions and this review is therefore a useful addition to the literature.…”