Hereditary Progressive Mucinous Histiocytosis: New Insights Into a Rare Disease.

Abstract:

:Hereditary progressive mucinous histiocytosis is a rare, benign, skin-limited form of non-Langerhans cell histiocytosis. We report on a 5-year-old boy who presented in infancy with self-resolving dermal nodules but later developed persistent and progressive erythematous papules on the face and scalp. Histologic evaluation revealed dermal aggregates of S-100/CD1a-negative histiocytes with abundant mucin. We present this case to highlight the evolution of the lesional morphology in infancy and early childhood and to stress the importance of histology in confirming this rare disorder.

journal_name

Pediatr Dermatol

journal_title

Pediatric dermatology

authors

Nguyen NV,Prok L,Burgos A,Bruckner AL

doi

10.1111/pde.12694

subject

Has Abstract

pub_date

2015-11-01 00:00:00

pages

e273-6

issue

6

eissn

0736-8046

issn

1525-1470

journal_volume

32

pub_type

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