Sjögren's syndrome with specific cutaneous manifestations and multifocal clonal T-cell populations progressing to a cutaneous pleomorphic T-cell lymphoma.
A case is presented of primary Sjögren's syndrome of the parotid gland with specific skin manifestations, consisting of diffuse lymphoid infiltrates with proliferative and metaplastic changes of sweat glands, a histologic picture similar to that seen in the parotid gland. Material of the parotid gland, a submandibular lymph node showing expanded T-cell areas, and several skin biopsies were studied immunohistologically for the presence of clonal B-cell populations. Because of a polyclonal kappa/lambda pattern of the B-lymphocytes, the possibility of a B-cell non-Hodgkin's lymphoma (NHL) was excluded. The initially morphologically benign lymphocytic skin infiltrates (1985) changed into a pleomorphic T-cell lymphoma (1987). Southern blot analysis of the atypical skin infiltrates and of the original material of the parotid gland and submandibular lymph node revealed identical clonal rearrangements of the T-cell receptor B-chain. Rearrangement of Ig genes was not found.
van der Valk
P G
PG
Department of Dermatology, University Hospital, Groningen, The Netherlands.
Hollema
H
H
van Voorst Vander
P C
PC
Brinker
M G
MG
Poppema
S
S
D000368:Aged; D015139:Blotting, Southern; D002999:Clone Cells; D004247:DNA; D005260:Female; D015321:Gene Rearrangement; D006801:Humans; D007150:Immunohistochemistry; D008223:Lymphoma / Q000235:genetics / Q000473:pathology*; D009693:Nucleic Acid Hybridization; D010306:Parotid Gland / Q000473:pathology; D012859:Sjogren's Syndrome / Q000150:complications*; D012867:Skin / Q000473:pathology; D012871:Skin Diseases / Q000150:complications* / Q000473:pathology; D012878:Skin Neoplasms / Q000235:genetics / Q000473:pathology*; D013601:T-Lymphocytes / Q000473:pathology* / Q000502:physiology
D004247:DNA