Ann Dermatol.  2000 Sep;12(3):193-196. 10.5021/ad.2000.12.3.193.

A Case of Congenital Self-Healing Reticulohistiocytosis

• Shin N
• Kang MJ
• Cho S
• Whang KK
• Hahm JH

• KMID: 2163979
• DOI: http://doi.org/10.5021/ad.2000.12.3.193

Abstract

Congenital self-healing reticulohistiocytosis (CSHRH) is a rare Langerhans cell disorder usually showing spontaneous resolution within 3-4 months. By electron microscopy, the identification of Birbeck granules and laminated dense bodies in the infiltrated cells is mandatory for the diagnosis of CSHRH. However, in some reported cases, Birbeck granules could not be demonstrated and only cytoplasmic dense bodies were seen. If the lesion is more advanced, Birbeck granules are transformed to lysosomes, i.e., 'unique phagosomes', in which they are degraded. A 2-month-old Korean girl presented with congenital, numerous red-brown pigmented papules on the left side of trunk and upper extremity without systemic symptoms. A biopsy specimen demonstrated papillary dermis containing epidermotropic infiltrates of histiocytes with abundant eosinophilic cytoplasm. Some had kidney-shaped nuclei and PAS-positive cytoplasmic inclusions. Immunohistochemically, infiltrating cells expressed S-100 protein and ultrastructurally, no Birbeck granules but many dense laminated bodies and unique phagosomes were found. It was ten months since the skin lesions developed that they have started resolving.

Keyword

Congenital self-healing reticulohistiocytosis; Laminated dense body; Unique phagosome

MeSH Terms

Biopsy
Cytoplasm
Dermis
Diagnosis
Eosinophils
Female
Histiocytes
Humans
Inclusion Bodies
Infant
Lysosomes
Microscopy, Electron
Phagosomes
S100 Proteins
Skin
Upper Extremity
S100 Proteins