Necrobiotic xanthogranuloma
is a rare disorder which is frequently associated with
paraproteinemia and lymphoproliferative diseases.
Age:
Middle-aged or elderly patients.
Clinical
presentation: Lesion presents as reddish, partly xanthomatous nodules
or plaques.
Site:
These are usually located around the periorbital area. Other sites
include extremities & trunk.
Histopathological features:
Image1
;
Image2
;
Image3
Extensive areas of
hyaline necrobiosis surrounded by a palisade of histiocytes &
multinucleated giant cells ;
Large numbers of necrotic inflammatory cells in the reticular dermis ;
Superficial and deep perivascular lymphoplasmacytic infiltrate ;
Presence of foam cells, multinucleated giant cells (Touton & foreign
body types), cholesterol clefts & extracellular lipid ;
Lymphoid follicles may be present ;
Extensive areas of fat necrosis in the subcutaneous tissue.
Differential diagnosis:
Necrobiosis Lipoidica: Much less collagen degeneration. Foam cells, large
numbers of necrotic inflammatory cells & cholesterol clefts are absent .
Atypical giant cells are not prominent. Panniculitis is localized to the
septa . In necrobiotic xanthogranuloma the lobules are extensively
involved. Necrobiosis
lipoidica diabeticorum with cholesterol clefts in the differential
diagnosis of necrobiotic xanthogranuloma.J
Cutan Pathol. 1988
Feb;15(1):18-21. |