Clinical History
A 70 year old man has a history of gradually expanding and ulcerating nodules of the neck areas.
Case 10 - Figure 1 - There are confluent nodular histiocytic infiltrates in the mid and deep reticular dermis
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Case 10 - Figure 2 - There is an irregular palisading granulomatous process involving the deep dermis; granulomas include giant cells with Touton forms as well as foam cells. Cholesterol clefts completed the picture (not illustrated).
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Histology
 | near normal epidermis and papillary dermis |
| irregular palisading granulomatous process involving the deep dermis and subcutaneous fat |
| see necrotizing and sclerotic foci within the involved zones with cholesterol clefts |
| granulomas principally of the giant cell type with bizarre features, touton-forms as well as foam cells |
| variable vascular changes? |
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Paraneoplastic syndrome of indurated cutaneous plaques and nodules associated with paraproteinemia
and hematopoetic malignancy.
| Also associated with scleritis and keratitis |
| Normolipemic plane xanthoma |
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Pathogenesis is unknown.
Dermatologic findings consist of yellow-to-erythematous nodules and plaques that preferentially
involve the face and particularly the periorbital area. Well-developed lesions show central atrophy with
ulceration.
Laboratory findings include paraproteinemia (invariable), leukopenia, plasmacytosis,
hypocomplementemia, hyperlipidemia.
Differential diagnosis includes
| other palisading granulomatous processes (granuloma annulare, rheumatoid nodule, necrobiosis lipoidica) |
| ruptured epidermal inclusion cyst reaction |
| phaeomycotic 'cyst' |
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References
- Finan M. Necrobiotic xanthogranuloma with paraproteinemia. A review of 22 cases. Medicine 1986; 65:376-88.
- McGregor J. Necrobiotic xanthogranuloma without periorbital lesions. J Am Acad Dermatol 1993; 29: 466-69.
- Macfarlane A. Necrobiotic xanthogranuloma with paraproteinemia. Br J Dermatol 113: 339-43.
- Mehregan D. Necrobiotic xanthogranuloma. Arch Dermatol 1992; 128: 94-100.
