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Histopathologic and ultrastructural study of lupus‐like skin lesions in a patient with Bloom syndrome
Author(s) -
Grob Martin,
Wyss Myriam,
Spycher Max A.,
Dommann Stefan,
Schinzel Albert,
Burg Günter,
Trüeb Ralph M.
Publication year - 1998
Publication title -
journal of cutaneous pathology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.597
H-Index - 75
eISSN - 1600-0560
pISSN - 0303-6987
DOI - 10.1111/j.1600-0560.1998.tb01733.x
Subject(s) - pathology , dermoepidermal junction , dermis , basement membrane , discoid lupus erythematosus , histopathology , medicine , skin biopsy , systemic lupus erythematosus , lupus erythematosus , papillary dermis , biopsy , antibody , immunology , disease
The histopathology of the lupus‐like skin lesions associated with Bloom syndrome has been sporadically described. Skin biopsies from a 2‐year‐old boy with the classical features of Bloom syndrome, including lupus‐like skin lesions, demonstrated marked interface changes with basal liquefaction degeneration, a moderate superficial mononuclear infiltrate, pigmentary incontinence, and capillary dilation in the papillary dermis. Immunophenotyping of the dermal infiltrate revealed predominance of T‐cells. Basement membrane thickening on periodic acid‐Schiff examination was not seen. Direct immunofluorescence failed to demonstrate deposits of immunoglobulin other than nonspecific IgM deposition along the basement membrane zone of lesional skin. Ultrastructurally, the most striking findings were disintegration of basal cell cytoplasm and tubuloreticular inclusions in vascular endothelia. Taken together, the histologic and ultrastructural features of lipus‐like lesions associated with Bloom syndrome mimic those of cutaneous lupus erythematosus, with the exception of paucity of immune deposits at the dermoepidermal Junction.