Necrobiosis
lipoidica (NL), originally known as necrobiosis lipoidica diabeticorum,
is a disorder of collagen degeneration with a granulomatous response and
thickening of blood vessels.
Diabetes mellitus is present in more than half the patients with necrobiosis lipoidica.
Age & sex:
Average age of onset is 30 years (may occur at any age) and females are
commonly affected.
Site:
Most cases are located on the leg specially above the tibiae, but may
also occur on the face, scalp, forearm and trunk.
Clinical presentation:
Lesions may be single but multiple lesions are more common. NL may present as red papules which may enlarge to form patches or
plaques with an atrophic yellowish-brown and slightly depressed center.
The lesions may resolve spontaneously or become persistent chronic
lesions which may ulcerate.
Histopathological
features:
Diagram: click here
In a fully developed
case the characteristic features are present at the edge of the
lesion.
Necrobiotic granuloma and
inflammatory infiltrate:
Full thickness of the
dermis is involved with extension into the subcutis.
The inflammatory cells
are composed of histiocytes, lymphocytes, plasma
cells and occasional eosinophils are arranged in two or three tiers.
These are aligned parallel to the skin surface.
There are several layers of necrobiosis within the reticular dermis.
Necrobiotic areas are rimmed by histiocytes and multinucleate Langhans
or foreign body giant cells.
The necrobiosis is irregular and less complete than in granuloma
annulare.
( Note:
Palisaded granuloma
in necrobiosis lipoidica- Early lesions show prominent collagen
degeneration.
Late lesions show crowded and thickened collagen bundles. )
The intervening areas of the dermis are also abnormal.
Lymphoid cell aggregates with germinal centers may be present.
Abnormalities present in the reticular dermis are also present in the
septa of the subcutaneous tissue (septal panniculitis with granulomatous
inflammation).
Vascular changes:
Vascular changes are
more prominent in diabetic patients.
Superficial and deep perivascular inflammatory infiltrate.
Plasma cells are conspicuous.
Superficiall vessels are telangiectatic & increased in number.
Deeper vessels may show endothelial swelling.
Lymphocytic vasculitis may
be present.
Epithelioid granulomas within or adjacent to the vessel
wall.
Other features:
Intradermal nerves
are reduced in number.
Old and atrophic lesions show dermal fibrosis and thickened septa of the
subcutaneous fat .
Lipid in the upper part of the dermis can be demonstrated by Sudan black
and oil red O stain.
Stains for mucin (colloidal iron or alcian blue) are usually negative.
Differential diagnosis:
Granuloma annulare: click
Rheumatoid nodule: click
Necrobiosis
Xanthogranuloma: click
Interstitial Granulomatous Dermatitis: click
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