Necrobiotic Xanthogranuloma

A rare systemic disease primarily associated with cutaneous plaques.

Plaques can manifest at any site but most commonly on the face and periorbital regions.

Associated with extensive necrobiosis and infiltration of inflammatory cells.

Patients often have an associated paraproteinemia, or hematologic disorder such as multiple myeloma or a lymphoproliferative disease.

Because of associations with hematologic malignancies and MGUS evaluation of serum immunoglobulins and free light chains are necessary.

Most common presentation with yellow plaques, and nodules occurring anywhere on the body with lesions 0.3-25 cm in diameter.

Skin lesions are usually asymptomatic, but can be associated with pruritus, burning and tenderness.

Usually a slowly progessive process.

Ulceration of lesions can occur.

Frequently associated with ocular complaints with unilateral or bilateral findings including: scleritis, diplopia, proptosis, blepharoptosis, impaired vision and decreased ocular motility.

Systemic involvement include the entire respiratory tract, epiglottis, larynx, pharynx, heart, spleen, lacrimal glands, brain, and skeletal muscle.

Biopsy is required for diagnosis and histological findings consist granulomatous inflammation with histiocytes, lymphocytes, giant cells, and foreign body giant cells extending from the middle dermis throgh the panniculus.

Majority of cases have cholesterol clefts within necrobiotic foci.

Differential diagnosis is necrobiosis lipodica.

Up to 80% of patients have a monoclonal gammopathy.

No specific treatment exists.

Management ranges from observation in asymptomatic patients to surgical resection, radiation, plasmapheresis, intro lesion oh corticosteroids, and cytotoxic agents, and high-dose steroids.

Treatment of underlying plasma cell dyscrasias may result in resolution of the lesions-responses to thalidomide, lenalidomide, and other agents may be efficacious.

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