| Necrobiotic xanthogranuloma with paraproteinemia. A review of 22 cases. | |
| | |
MedLine Citation:
|
PMID: 3097454 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
|
Cutaneous biopsy specimens from 22 patients showed the distinctive histopathologic pattern of necrobiotic xanthogranuloma within the dermis or subcutaneous tissue (or both). Twenty of the 22 patients had 1 or more serum protein abnormalities, consisting of an IgG monoclonal protein in 16, multiple myeloma in 3, cryoglobulinemia in 3, and an abnormal serum protein electrophoresis in 1. Cutaneous lesions were seen as discrete, slowly developing red nodules and plaques with a xanthomatized hue and a predilection for the face (periorbital region in particular), trunk, and extremities. Ulceration was a notable finding in 10 patients. Histologically, the dermis and lobules of subcutaneous tissue were involved with a granulomatous infiltrate containing bands of hyaline necrobiosis and bizarre foreign body, as well as Touton giant cells. Cholesterol clefts, lymphoid nodules with or without germinal centers, and foci of plasma cells were variable but significant features. Leukocyte monoclonal antibody studies in 6 patients demonstrated helper T cells within the granulomas. Electron microscopy in 3 cases showed lipid vacuoles in macrophages in the dermis and dendritic cells in the epidermis, and study confirmed this entity as a non-X histiocytosis. Pertinent laboratory findings, in addition to the serum protein abnormalities, included elevation of the erythrocyte sedimentation rate, leukopenia with absolute neutropenia, and decreased serum complement levels, as well as decreased levels of C1-esterase inhibitor in some patients. Thirteen of the 22 patients have survived, the mean duration being 9.5 years after the onset of cutaneous disease. While given to only a few patients in the current series, low-dose chemotherapy seems to induce a favorable response in both the cutaneous and the hematologic disease. |
| | |
Authors:
|
M C Finan; R K Winkelmann |
Related Documents
:
|
20599414 - Elevated serum levels of a proliferation-inducing ligand in patients with systemic scle... 20094744 - Serum hyaluronic acid levels do not explain morning stiffness in patients with fibromya... 19756734 - Identification of potential serum markers for endometrial cancer using protein expressi... 20202824 - Serum biomarkers of cell death for monitoring therapy response of gastrointestinal carc... 6783904 - Endorphins and the control of breathing. ability of naloxone to restore flow-resistive ... 8839214 - Elevated amylase is related to the development of respiratory failure in organophosphat... |
Publication Detail:
|
Type: Journal Article |
Journal Detail:
|
Title: Medicine Volume: 65 ISSN: 0025-7974 ISO Abbreviation: Medicine (Baltimore) Publication Date: 1986 Nov |
Date Detail:
|
Created Date: 1986-12-29 Completed Date: 1986-12-29 Revised Date: 2005-11-17 |
Medline Journal Info:
|
Nlm Unique ID: 2985248R Medline TA: Medicine (Baltimore) Country: UNITED STATES |
Other Details:
|
Languages: eng Pagination: 376-88 Citation Subset: AIM; IM |
Export Citation:
|
APA/MLA Format Download EndNote Download BibTex |
| MeSH Terms | |
Descriptor/Qualifier:
|
Adult Aged Female Granuloma / complications*, drug therapy, pathology Humans Hypergammaglobulinemia / complications*, pathology Immunoglobulin G* Immunoglobulin Light Chains* Male Middle Aged Orbital Diseases / pathology Osteonecrosis / pathology Xanthomatosis / complications*, drug therapy, pathology |
| Chemical | |
Reg. No./Substance:
|
0/Immunoglobulin G; 0/Immunoglobulin Light Chains |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
Previous Document: Acute effects of marihuana smoking on maximal exercise performance.
Next Document: The interaction of 2 diseases: diabetes mellitus and cystic fibrosis.