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 Dr Sampurna Roy MD

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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.

Abstracts:

Necrobiotic xanthogranuloma associated with lymphoplasmacytic lymphoma. Palliative treatment with carbon dioxide laser.Eur J Dermatol. 2005;15(3):182-5.

Necrobiotic xanthogranuloma-rapid progression under treatment with melphalan.Eur J Dermatol. 2005;15(5):363-5.

Long-term course of necrobiotic xanthogranuloma with ocular involvement. Hautarzt. 2005 Feb 12;

Case of recalcitrant necrobiotic xanthogranuloma.Australas J Dermatol. 2004;45(4):213-5.

Systemic necrobiotic xanthogranuloma with initial pericardial and pulmonary involvement.Ann Dermatol Venereol. 2003 Mar;130(3):341-4.

Necrobiotic xanthogranuloma associated with a benign monoclonal gammopathy.Cutis. 2003;72(1):47-50.

Bilateral necrobiotic xanthogranuloma of the eyelids with associated paraproteinemia: Case report and review of literature.Klin Monatsbl Augenheilkd. 2002;219(1-2):55-8.

Necrobiotic xanthogranuloma , a cutaneous disorder associated with monoclonal gammopathy.Rev Med Interne. 2002 May;23(5):460-4.

Necrobiotic xanthogranulom presenting as a solitary tumor. Am J Dermatopathol.2000;22(5): 453-6.

Necrobiotic xanthogranuloma: long-term outcome of ocular and systemic involvement. Am J Ophthalmol 2000;129:651-7

Lipid and giant cell poor necrobiotic xanthogranuloma. J Cutan Pathol 2000;27:374-378.

Necrobiotic xanthogranuloma with paraproteinemia: an evolving presentation. Cutis 1997;59:333-36.

Necrobiotic xanthogranuloma with eye involvement. Overview and case report.Hautarzt. 1995 May;46(5):330-4.

Histopathology of necrobiotic xanthogranuloma with paraproteinemia. J Cutan Pathol 1987;14:92-99

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