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Dermatopathological aspects of restrictive dermopathy
Author(s) -
Piérardfranchimont Claudine,
Piérard GéRald E.,
Estrada Trinh HermannsLě Jorge Arrese,
Verloes Alain,
Mulliez N.
Publication year - 1992
Publication title -
the journal of pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 2.964
H-Index - 184
eISSN - 1096-9896
pISSN - 0022-3417
DOI - 10.1002/path.1711670211
Subject(s) - factor xiiia , dermis , epidermis (zoology) , pathology , medicine , anatomy , dermatology , immunohistochemistry
We present an immunopathological and electronmicroscopic study of the skin of two newborns affected by restrictive dermopathy. Evidence of abnormal maturation was found in the epidermis, cutaneous appendages, dermis, and hypodermis. Our observations confirm two previous descriptions. We emphasize some unreported data concerning the L1 antigen and Factor XIIIa in the skin. The L1 antigen is expressed in the interadnexal epidermis, but not in hair follicles. This is the reverse pattern compared with normal skin. Factor XIIIa is poorly expressed in dermal dendrocytes, which appear rare compared with controls. The multiple defects in maturation found in all cutaneous tissues suggest a qualitative or quantitative aberration in control mechanisms of tissue interactions.