| DISEASE ASSOCIATIONS |
CHARACTERIZATION |
| Paraprotein |
Up to 80% of cases |
| Malignant lymphoma |
|
|
The evolution of Hodgkin's disease and necrobiotic
xanthogranuloma syndrome.
Reeder CB, Connolly SM, Winkelmann RK.
Division of Hematology/Oncology, Mayo Clinic Scottsdale,
AZ 85259.
|
Mayo Clin Proc 1991 Dec;66(12):1222-4 Abstract quote
We describe a woman in whom hypogammaglobulinemia and severe granulomatous
cutaneous lesions had developed during childhood; subsequently, Hodgkin's
disease and necrobiotic xanthogranuloma were diagnosed.
This case illustrates an apparent association with disease activity
and raises the question of a direct relationship of necrobiotic xanthogranuloma
with lymphoproliferative disease.
|
| Myeloma |
|
|
Necrobiotic xanthogranuloma with myeloma. A case report.
Venencie PY, Puissant A, Verola O, Kerneis Y, Marchat
C, Le Bras P, D'Agay MF, Danon F, Valensi F, Turpin G.
|
Cancer 1987 Feb 1;59(3):588-92 Abstract quote
Necrobiotic xanthogranuloma is a newly recognized cutaneous manifestation
associated with paraproteinemia. Necrobiotic xanthogranuloma is distinctive
clinically by yellowish-red nodular lesions, often ulcerated, with predilection
for the periorbital area and ocular involvement. Histologically, it
is characterized by the combination of inflammatory xanthogranulomas
together with areas of necrobiosis. These skin lesions are associated
with a dysglobulinemia and preceded the development of an IgG lambda
myeloma in a previous patient. Hyperlipidemia may be found in some patients.
A case of necrobiotic xanthogranuloma is reported, occurring in a 46-year-old
man and associated with an IgG kappa myeloma.
|
|
Necrobiotic xanthogranuloma with IgA multiple myeloma:
a case report and literature review.
Valentine EA, Friedman HD, Zamkoff KW, Streeten BW.
Department of Medicine, Veterans Administration Hospital,
Syracuse, New York.
|
Am J Hematol 1990 Dec;35(4):283-5 Abstract quote
The association between necrobiotic xanthogranuloma of the dermis and
paraproteinemia and/or B-cell malignancy is best described in the ophthalmologic
literature.
We report a case which occurred in the eyelid and orbit of a 64 year
old man that led to the diagnosis of an IgA multiple myeloma.
To our knowledge, this is the first report of an IgA type paraproteinemia
and IgA type multiple myeloma associated with necrobiotic xanthogranuloma.
|
|
Periorbital necrobiotic xanthogranuloma and stage
I multiple myeloma. Ultrastructure and response to pulsed dexamethasone
documented by magnetic resonance imaging.
Plotnick H, Taniguchi Y, Hashimoto K, Negendank W,
Tranchida L.
Department of Dermatology, Wayne State University
School of Medicine, Detroit, MI 48201.
|
J Am Acad Dermatol 1991 Aug;25(2 Pt 2):373-7 Abstract quote
We observed a 40-year-old woman with necrobiotic xanthogranuloma from
the inception of indurated eyelid and periorbital infiltrates and concurrent
stage I multiple myeloma to resolution of infiltrates in skin and bone
marrow after pulsed high-dose oral dexamethasone therapy.
Ultrastructural studies revealed lipid vacuoles in epidermal keratinocytes,
in dermal histiocytic macrophages, and in vascular and lymphatic endothelial
cells. The presence of lipid vacuoles in epidermal keratinocytes has
not been reported previously in xanthogranuloma.
|
|
Necrobiotic xanthogranuloma in a human T-lymphotropic virus type
1 carrier.
Nishimura M, Takano-Nishimura Y, Yano I, Hayashi N, Toshitani S.
Department of Clinical Genetics, Kyushu University, Beppu, Japan.
|
J Am Acad Dermatol 1992 Nov;27(5 Pt 2):886-9 Abstract quote
A 67-year-old man who had necrobiotic xanthogranuloma associated with
paraproteinemia is described. He was a human T-lymphotropic virus type
1 (HTLV-1) carrier who had a high titer of circulating anti-HTLV-1 antibodies
and neurologic abnormalities that suggested HTLV-1-associated myelopathy.
The patient's necrobiotic xanthogranuloma and neurologic symptoms improved
after he received four 5-day courses of melphalan, 2 mg/day, and prednisolone,
20 mg/day, at 4-week intervals.
|
| PATHOGENESIS |
CHARACTERIZATION |
|
Necrobiotic xanthogranuloma with paraproteinemia. Case report and
a pathogenetic theory.
Bullock JD, Bartley GB, Campbell RJ, Yanes B, Connelly PJ, Funkhouser
JW.
|
Ophthalmology 1986 Sep;93(9):1233-6 Abstract quote
Necrobiotic xanthogranuloma with paraproteinemia is a clinical and
histopathological entity characterized by xanthelasma-like lesions in
the periorbital region and elsewhere, paraproteinemia, leukopenia, and
an elevated erythrocyte sedimentation rate. Multiple myeloma has been
reported as an accompanying feature in several cases.
We examined a patient with necrobiotic xanthogranuloma and multiple
myeloma in whom an IgG kappa monoclonal protein was identified in serum,
urine, bone marrow, and bilateral periorbital lesions.
We speculate that increased serum immunoglobulins complexed with lipid
may be deposited in the skin, leading to a foreign body giant cell reaction
and the subsequent characteristic histopathologic features of necrobiotic
xanthogranuloma.
|
|
Activation of monocytes in vivo causes intracellular accumulation
of lipoprotein-derived lipids and marked hypocholesterolemia--a possible
pathogenesis of necrobiotic xanthogranuloma.
Matsura F, Yamashita S, Hirano K, Ishigami M, Hiraoka H, Tamura
R, Nakagawa T, Nishida M, Sakai N, Nakamura T, Nozaki S, Funahashi T,
Matsumoto C, Higashiyama M, Yoshikawa K, Matsuzawa Y.
Second Department of Internal Medicine, Osaka University Medical
School, Suita, Japan.
|
Atherosclerosis 1999 Feb;142(2):355-65 Abstract quote
Necrobiotic xanthogranuloma (NXG) is a rare histiocytic disease with
generalized xanthomatosis. However, most cases with NXG are normolipidemic
or hypolipidemic. The mechanism for the formation of xanthoma in NXG
has not yet been clarified.
We observed a case of NXG with severe hypocholesterolemia (total cholesterol:
1.69 mmol/l) and analyzed the function of monocytes in this case. Histological
examinations by light microscopy revealed a large amount of lipid deposition
in the patient's freshly isolated monocytes. The patient's monocytes
showed a 3-fold increase in cholesteryl ester content and a 3-fold enhancement
of acetyl low density lipoprotein (LDL) uptake compared with the control
monocytes. However, no significant difference was noted in the expression
of CD36 protein and the mRNA levels of scavenger receptor-class A (SR-A)
between the monocytes of the patient and the control. The phagocytotic
ability of the patient's monocytes was enhanced 1.5-fold compared with
that of the control monocytes.
These findings suggest that the activated monocytes may have degraded
the modified LDL via a pathway other than CD36 or SR-A, and accumulated
a great amount of lipids in vivo. In conclusion, the present study has
demonstrated a possible pathogenesis of NXG that the activation of monocytes
in vivo may contribute to the intracellular accumulation of lipoprotein-derived
lipids leading to non-inherited xanthomatosis and the marked hypocholesterolemia.
|
GROSS APPEARANCE/
CLINICAL VARIANTS |
CHARACTERIZATION |
| GENERAL |
|
|
Necrobiotic xanthogranuloma with paraproteinemia. A review of 22
cases.
Finan MC, Winkelmann RK.
|
Medicine (Baltimore) 1986 Nov;65(6):376-88 Abstract quote
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.
|
|
Necrobiotic xanthogranuloma.
Mehregan DA, Winkelmann RK.
Department of Dermatology, Mayo Clinic, Rochester, Minn.
|
Arch Dermatol 1992 Jan;128(1):94-100 Abstract quote
We reviewed 32 cases of necrobiotic xanthogranuloma and 16 cases from
the world literature. Necrobiotic xanthogranuloma is a destructive dermal
and subcutaneous xanthogranuloma that most frequently involves the face
(periorbital region) and trunk. Monoclonal gammopathy is common (IgG-kappa
in 23 cases and IgG-lambda in nine cases), and cryoglobulins have also
been found. Low complement levels, anemia, and leukopenia are frequently
present. Bone marrow examination shows plasma cell proliferation and,
rarely, true myeloma.
We describe mucosal, muscle, and systemic lesions, including xanthogranulomatous
myocardial lesions. The clinical course is chronic and often progressive.
Low-dose chlorambucil treatment is safe and effective, but individual
patients have responded to treatment with corticosteroids, melphalan,
local radiation, and plasma exchange.
|
| Systemic involvment |
Lungs
Airways
Heart
Ovary
Kidney
Liver
Spleen |
| VARIANTS |
|
|
Ophthalmic features of necrobiotic xanthogranuloma with paraproteinemia.
Robertson DM, Winkelmann RK.
|
Am J Ophthalmol 1984 Feb;97(2):173-83 Abstract quote
Necrobiotic xanthogranuloma of the skin is associated with paraproteinemia
and, often, with plasma proliferative disorders, including multiple
myeloma. Other commonly recognized systemic abnormalities include hepatosplenomegaly,
a highly increased erythrocyte sedimentation rate, and leukopenia.
Fifteen of 16 patients (seven men and nine women with a mean age of
54 years) with this condition had ophthalmic manifestations. Thirteen
patients had lesions affecting the skin of the eyelids and periorbital
tissue; on casual examination these lesions resembled plane xanthoma.
Unlike plane xanthoma, however, the lesions of necrobiotic xanthogranuloma
were almost always indurated. Further, the lesions frequently became
inflamed, leading to superficial ulceration. Deeper lesions occasionally
involved the orbit. Yellow lesions were sometimes visible in the episcleral
tissues where they were associated with recurrent symptoms of scleritis
and episcleritis. Biopsy specimens of the skin lesions disclosed a distinctive
pattern of subepidermal granulomatous masses with focal aggregates of
histiocytes and giant cells surrounded by hyaline necrobiosis.
Surgical excision of the eyelid lesions was often followed by recurrence
and increased activity of the lesions. Low-dose chemotherapy is likely
to produce a favorable response, with regard to both the skin lesions
and the paraprotein abnormalities.
|
|
Necrobiotic xanthogranuloma with IgA paraproteinemia and extracutaneous
involvement.
Fortson JS, Schroeter AL.
Department of Dermatology, Wright State University, Dayton, Ohio.
|
Am J Dermatopathol 1990 Dec;12(6):579-84 Abstract quote
Numerous nodular xanthomatous lesions arose, gradually increasing in
number on the back, chest, lips, tongue, and exposed "V" area of the
chest on a 78-year-old man.
Painful oral nodules and hoarseness developed subsequently. Histologic
examination of the skin revealed a suppurative and granulomatous inflammation
with an accumulation of acute polymorphonuclear neutrophils, nuclear
dust, and xanthomatous histiocytes. Giant cells were rare and cholesterol
clefts were absent. Biopsy of the oral mucosa and the larynx showed
a similar process. Direct immunofluorescence was positive for vascular
immune reactants at the basement membrane and fibrin in between collagen
bundles. The patient had a serum IgA level of 432 mg/dl (normal level,
up to 380), with normal IgG and IgM serum concentrations. A Raji cell
assay was 240 micrograms/AHGEQ/ml (normal less than 5). CH-50 was decreased
and C2, C4, and C3 were within normal limits. The dermatopathology was
initially thought to be consistent with erythema elevatum diutinum,
and the patient was treated with dapsone and prednisone, with a marginal
response. There were no dermatopathologic findings of vasculitis. On
review, the patient's dermatopathology was more consistent with necrobiotic
xanthogranuloma (NXG).
To our knowledge, this is the first report of an association of NXG
with an IgA gammopathy and the second reported case of extracutaneous
involvement with NXG.
|
|
Varied clinical spectrum of necrobiotic xanthogranuloma.
Cornblath WT, Dotan SA, Trobe JD, Headington JT. W. K.
Kellogg Eye Center, Department of Ophthalmology, University of Michigan,
Ann Arbor 48105.
|
Ophthalmology 1992 Jan;99(1):103-7 Abstract quote
Four cases are presented that illustrate a wide spectrum of ophthalmologic
and systemic features of necrobiotic xanthogranuloma (NXG).
Case 1 initially had signs of Cogan syndrome, and then developed chronic
lymphocytic leukemia. Case 2, the first case of NXG to undergo autopsy,
had progressive cicatricial lid retraction and corneal perforation.
Case 3 had a more typical presentation of diplopia and blepharoptosis
caused by orbital and periorbital infiltrative masses. Case 4 had nondeforming
periocular skin lesions over a 6-year period.
In all four cases, the diagnosis was made on the basis of characteristic
histopathologic and laboratory findings. Although the cause of NXG is
still obscure, in many cases it appears to be a forerunner of lymphoproliferative
diseases.
|
|
Necrobiotic xanthogranuloma with myocardial lesions and nodular
transformation of the liver.
Novak PM, Robbins TO, Winkelmann RK.
Department of Anatomic Pathology, William Beaumont Hospital, Royal
Oak, MI 48073-6769.
|
Hum Pathol 1992 Feb;23(2):195-6 Abstract quote
A 73-year-old woman with a history of necrobiotic xanthogranuloma (NXG)
with paraproteinemia died suddenly while hospitalized for bronchitis
and congestive heart failure. At postmortem examination, myocardial
lesions histologically typical of NXG were found.
This is the fifth reported autopsy of a patient with NXG. In all five
autopsied cases, and in two additional surgical cases, necrobiotic granulomas
of internal viscera were present, and in three cases the myocardium
was involved. In addition, our patient had nodular transformation of
the liver, a rare lesion that was also reported in one of the previous
autopsy cases with NXG.
|
|
Necrobiotic xanthogranuloma without periorbital lesions.
McGregor JM, Miller J, Smith NP, Hay RJ.
Department of Dermatology, Guy's Hospital, London, United Kingdom.
|
J Am Acad Dermatol 1993 Sep;29(3):466-9 Abstract quote
Cutaneous necrobiotic xanthogranuloma is rare and closely resembles
widespread necrobiosis lipoidica. It is important to recognize this
skin disorder because of its strong association with paraproteinemia
and, in some cases, with a hematologic malignancy.
We describe two patients with necrobiotic xanthogranuloma who are unusual
in that they had no periorbital involvement, a feature previously believed
to be diagnostic of this condition.
|
|
Necrobiotic xanthogranuloma with cardiac involvement.
Umbert I, Winkelmann RK.
Mayo Clinic, Scottsdale, AZ 85259, USA.
|
Br J Dermatol 1995 Sep;133(3):438-43 Abstract quote
A patient with necrobiotic xanthogranuloma (NXG) and paraproteinaemia,
who was followed-up for several years, and treated with low-dose chlorambucil,
died as a result of a respiratory illness. The significant findings
at autopsy were a xanthogranuloma of the spleen and giant cell myocarditis.
The myocardial lesions were composed of macrophages, giant cells and
lymphocytes.
This finding is important because four of five known autopsied patients
with NXG have had giant cell myocardial disease, and an effort at antemortem
diagnosis should be made.
|
|
Giant cell granulomatous pulmonary and myocardial lesions in necrobiotic
xanthogranuloma with paraproteinemia.
Winkelmann RK, Litzow MR, Umbert IJ, Lie JT.
Department of Dermatology, Mayo Clinic Scottsdale, Arizona, USA.
|
Mayo Clin Proc 1997 Nov;72(11):1028-33 Abstract quote
OBJECTIVE: To report three cases of pulmonary or myocardial disease
(or both) and necrobiotic xanthogranuloma.
MATERIAL AND METHODS: Giant cell granulomas of the lung and myocardium
were demonstrated in three patients who had pulmonary and myocardial
lesions of necrobiotic xanthogranuloma in conjunction with skin lesions,
leukopenia, paraproteinemia, and complement deficiencies. The patients
were two men who were 47 and 64 years of age and a 39-year-old woman.
RESULTS: Biopsies of skin and visceral lesions showed asteroid and
cytoplasmic inclusions. B-cell lymphoid nodules were found. In one of
the male patients, a major clonal T-cell receptor gene rearrangement
was detected in the peripheral blood. Prednisone was ineffective in
two of the patients. The other patient experienced regression of skin
lesions and diminishment of a chest nodule after receiving alkylating
agent therapy.
CONCLUSION: Establishing the correct diagnosis is important, and apparently
it is possible to establish the nature of the myocardial and pulmonary
lesions with use of appropriate scans and by biopsy. Successful treatment
of necrobiotic xanthogranuloma skin lesions with corticosteroids or
alkylating agents (or both) implies that evolution of serious disease
that compromises the heart and lungs could be controlled.
|
|
Necrobiotic xanthogranuloma presenting as a solitary tumor.
Stork J, Kodetova D, Vosmik F, Krejca M.
Department of Dermatology, General Faculty Hospital, Charles University,
Praha, Czech Republic.
|
Am J Dermatopathol 2000 Oct;22(5):453-6 Abstract quote
We report on a 60-year-old overweight white woman who presented with
an asymptomatic flat, hard, yellow-brown subcutaneous plaque on her
right hip.
A total excision was performed. Histopathologic examination showed
all the major features of a necrobiotic xanthogranuloma (NX) involving
the entire dermis and subcutis, including areas of necrobiosis with
cholesterol clefts, granulomatous infiltrate with some bizarre giant
cells, numerous Touton cells, foamy cells, lymphoid follicles with germinal
centers, foci of plasma cells, and "Touton cell panniculitis." A laboratory
investigation revealed only slightly increased titers of cholesterol,
strong positivity of anti-Borrelia antibodies, and diffuse skeletal
osteoporosis with fractures of seven vertebrae. After 4 years of close
follow-up, the osteoporosis was improved, and there were no signs of
paraproteinemia, malignancy, or new skin lesions.
The authors suggest that this case could represent a solitary clinical
variant of NX without paraproteinemia.
|
|
Necrobiotic xanthogranuloma with two monoclonal paraproteins and
no periorbital involvement at presentation.
Chave TA, Hutchinson PE.
Department of Dermatology, Leicester Royal Infirmary, Leicester,
UK.
|
Clin Exp Dermatol 2001 Sep;26(6):493-6 Abstract quote
A patient with necrobiotic xanthogranuloma is presented to highlight
the clinico-pathological features of this rare condition which must
be differentiated from atypical necrobiosis lipoidica.
The patient is unusual in that he has two associated monoclonal paraproteins
and did not have periorbital involvement at presentation.
|
| HISTOLOGICAL TYPES |
CHARACTERIZATION |
| GENERAL |
Necrobiotic granulomas with histiocytes, foam cells, and giant cells.
Occasionally, there are cholesterol clefts with cellular debris
Touton type giant cells may be present and sometimes extensive in the
subcutaneous fat (Touton-cell panniculitis)
|
|
Histopathology of necrobiotic xanthogranuloma with paraproteinemia.
Finan MC, Winkelmann RK.
|
J Cutan Pathol 1987 Apr;14(2):92-9 Abstract quote
Fifty-three cutaneous biopsies from 22 patients with necrobiotic xanthogranuloma
(NXG) were reviewed.
One or more biopsies from each patient displayed a typical pattern
of palisading histiocytic xanthogranuloma with bands of hyaline necrobiosis.
Multiple foam cells and Touton giant cells were present, and atypical,
bizarre-appearing foreign body giant cells were characteristic. Cholesterol
clefts were found in 18 specimens, and lymphoid nodules were found in
24, 8 of which had germinal center formation. Twenty-one specimens contained
foci of plasma cells, and these were located perivascularly, at the
periphery of lymphoid nodules, and, as infiltrates, between dermal collagen
bundles.
Unique features were xanthogranulomatous panniculitis, often appearing
as Touton cell panniculitis, and a rare but distinctive palisading cholesterol
cleft granuloma. The cytoplasm of giant cells and histiocytes contained
PAS-positive, diastase-resistant polysaccharide. Examination of 3 cases
by electron microscopy revealed dendritic cells in the epidermis and
vacuolated dermal histiocytes.
S-100 antibody was negative in 3 cases. Leukocyte monoclonal antibody
studies in 6 patients showed predominantly T-helper lymphocytes within
the granuloma. NXG is a T-helper cell, non-X histiocytic granuloma with
a distinctive histopathology.
|
| VARIANTS |
|
|
Necrobiotic xanthogranuloma: a case without paraproteinemia but
with transepithelial elimination.
Dupre A, Viraben R.
Dermatology Service, Purpan Hopital, Toulouse, France
|
J Cutan Pathol 1988 Apr;15(2):116-9 Abstract quote
We report a female patient, now aged 60, followed for 20 years for
lesions originally diagnosed as necrobiosis lipoidica diabeticorum.
In fact, the anatomical and clinical features of her disorder correspond
to the new entity described as necrobiotic xanthogranuloma.
Two elements distinguish this from earlier cases: 1) two examinations
failed to reveal paraproteinemia; 2) there was transepithelial elimination
of cholesterol crystals and degenerated xanthomatous cell debris via
hair follicles.
This demonstrates the characteristic histology of the disorder and
indicates that the diagnosis of necrobiotic xanthogranuloma should be
considered even in the absence of paraproteinemia.
|
|
Asteroid bodies and other cytoplasmic inclusions in necrobiotic
xanthogranuloma with paraproteinemia.
Winkelmann RK, Dahl PR, Perniciaro C, Dahl PM.
Mayo Clinic Scottsdale, Ariz, USA.
|
J Am Acad Dermatol 1998 Jun;38(6 Pt 1):967-70 Abstract quote
BACKGROUND: Necrobiotic xanthogranuloma (NXG) with paraproteinemia
is a distinctive palisading granuloma of the skin. Extracutaneous lesions
are rarely present.
OBJECTIVE: The purpose of this study was to confirm the presence and
significance of giant cell asteroid bodies and other cytoplasmic inclusions
in NXG with paraproteinemia.
METHODS: Skin biopsy specimens from 24 patients with NXG with paraproteinemia
were reviewed; autopsy and lung biopsy specimens from two patients were
stained for iron, calcium, and polysaccharide.
RESULTS: Giant cell asteroid bodies were observed in skin biopsy specimens
of 8 (33%) of the 24 patients. In addition, large acidophilic polygonal
cytoplasmic inclusions were observed in myocardial tissue of one of
the autopsy cases. Iron and calcium were not found.
CONCLUSION: Asteroid bodies and other inclusions can be present in
the giant cells of NXG with paraproteinemia. They are as frequent as,
or more frequent than, in other granulomatous diseases and should be
considered in the diagnosis of NXG with paraproteinemia.
|
|
Necrobiosis lipoidica: a case with prominent cholesterol clefting
and transepithelial elimination.
De la Torre C, Losada A, Cruces MJ.
Service of Dermatology, Hospital Provincial, Pontevedra, Spain.
|
Am J Dermatopathol 1999 Dec;21(6):575-7 Abstract quote
Transepithelial elimination of degenerated collagen through the hair
follicle in necrobiosis lipoidica is rare, clinically manifesting as
comedo-like plugs. Also unusual in necrobiosis lipoidica is the finding
of cholesterol cleft formation.
We report a case of necrobiosis lipoidica with transepithelial elimination
of cholesterol crystals through hair follicles. This has been described
in necrobiotic xanthogranuloma, demonstrating some overlap in the histopathologic
findings in both necrobiotic granulomas. Additional criteria should
be used to establish the diagnosis.
|
|
Lipid and giant cell poor necrobiotic xanthogranuloma.
Kossard S, Chow E, Wilkinson B, Killingsworth M.
Skin and Cancer Foundation Australia, Darlinghurst, New South Wales.
|
J Cutan Pathol 2000 Aug;27(7):374-8 Abstract quote
An 88-year-old man over a 7-month period developed multiple yellow
firm focally ulcerative papules and nodules over his face, neck and
forearms.
Seven skin biopsies showed a diffuse infiltrate of epithelioid histiocytes
associated with areas of necrosis with neutrophilia. Two biopsies showed
xanthogranulomatous foci, but cholesterol clefts, prominent giant cells
or lymphoid aggregates were not evident. Necrosis with leukocytoclastic
debris overshadowed the presence of hyaline necrobiosis. Ultrastructural
examination and oil red-o stains on frozen sections revealed focal lipid
vacuoles within histiocytes. A paraprotein was detected in the patient's
serum.
This presentation may represent a lipid and giant cell poor variant
of necrobiotic xanthogranuloma (NXG) and may potentially lead to a delay
in diagnosis.
|
| PROGNOSIS AND TREATMENT |
CHARACTERIZATION |
| PROGNOSIS |
|
|
Necrobiotic xanthogranuloma: long-term outcome of
ocular and systemic involvement.
Ugurlu S, Bartley GB, Gibson LE.
Department of Ophthalmology, Mayo Clinic and Mayo
Foundation, Rochester, Minnesota 55905, USA.
|
Am J Ophthalmol 2000 May;129(5):651-7 Abstract quote
PURPOSE: To report long-term outcomes of patients with necrobiotic
xanthogranuloma, to investigate the propriety of therapeutic surgical
excision or debulking, and to study tissue specimens by immunoperoxidase
staining and in situ hybridization.
METHODS: Medical records of all patients at the Mayo Clinic, Rochester,
Minnesota, with necrobiotic xanthogranuloma between 1980 and 1997 were
reviewed. A follow-up letter was sent to each patient inquiring about
the current status of the lesions, the treatment regimen, and associated
systemic diseases.
RESULTS: The average age (+/- standard deviation) of the 15 men and
11 women was 56.8 +/- 14.8 years. Of the 26 patients, 21 (81%) had lesions
of the ocular adnexa. Ulceration of the lesions occurred in 11 patients
(42%). The lesions recurred after surgical removal in 11 patients (42%)
and on prior incision sites from unrelated operations in three patients
(12%). The average duration of follow-up from the appearance of characteristic
skin lesions was 10 +/- 6.1 years. Four patients had multiple myeloma,
five had a plasma cell dyscrasia, and one had a lymphoproliferative
disorder during this period. Time to development of associated malignancy
ranged from 8 years before the skin lesions to 11 years after the skin
lesions. Overall survival was 100% at 10 years and 90% at 15 years (95%
confidence limit, 0.73 to 1.00). Immunoperoxidase stains demonstrated
that most histiocytes are not of Langerhans cell lineage. Monoclonal
immune globulins were not identified in tissue specimens.
CONCLUSION: Care of patients with necrobiotic xanthogranuloma should
include avoidance of surgical removal, if possible, and lifelong follow-up
to detect the development of associated malignancy.
|
| TREATMENT |
|
|
Plasmapheresis, a treatment modality for necrobiotic
xanthogranuloma.
Finelli LG, Ratz JL.
|
J Am Acad Dermatol 1987 Aug;17(2 Pt 2):351-4 Abstract quote
A case of necrobiotic xanthogranuloma treated with plasmapheresis is
reported.
This entity is characterized by multiple inflammatory, ulcerative nodules
and atrophic plaques with a xanthomatous quality that develop in conjunction
with a paraproteinemia.
Successful healing of ulcerated lesions and lowering of the paraproteinemia
was achieved by plasmapheresis in our patient with necrobiotic xanthogranuloma
when cytotoxic therapy failed.
|
|
Recombinant interferon alfa-2b treatment of necrobiotic
xanthogranuloma with paraproteinemia.
Venencie PY, Le Bras P, Toan ND, Tchernia G, Delfraissy
JF.
Department of Dermatology, CHU Bicetre, Le Kremlin
Bicetre, France.
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J Am Acad Dermatol 1995 Apr;32(4):666-7
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Cutaneous necrobiotic xanthogranuloma (NXG)--successfully
treated with low dose chlorambucil.
Machado S, Alves R, Lima M, Leal I, Massa A.
Service of Dermatology, Hospital Geral Santo Antonio,
Rua D. Manuel II, Edificio ex: Cicap, 4099-001 Porto, Portugal.
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Eur J Dermatol 2001 Sep-Oct;11(5):458-62 Abstract quote
We report a case of necrobiotic xanthogranuloma in a 51 year-old white
male patient presenting with a 6-year history of multiple indurated
violaceous nodules and plaques involving the eyelids, trunk and extremities.
He had an associated paraproteinemia (Ig G lambda), elevated sedimentation
rate, cryoglobulinemia and hypocomplementemia. No extracutaneous involvement
was detected.
He was successfully treated with chlorambucil (2 mg/d for 7 months),
leading to disappearance of all skin lesions.
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Recalcitrant necrobiotic xanthogranuloma responding
to pulsed high-dose oral dexamethasone plus maintenance therapy with
oral prednisolone.
Chave TA, Chowdhury MM, Holt PJ.
Department of Dermatology, University Hospital of
Wales, Cardiff CF14 4XN, UK.
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Br J Dermatol 2001 Jan;144(1):158-61 Abstract quote
A patient with the rare condition necrobiotic xanthogranuloma was treated
from 1992 to 1999 with multiple therapies, none of which was effective.
Her disease was progressive, causing significant disability.
In September 1999 a trial of high-dose pulsed oral dexamethasone with
maintenance corticosteroids led to a substantial improvement in her
condition.
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