Page last updated: 2024-09-27

Necrobiotic Xanthogranuloma

A cutaneous necrobiotic disorder characterized by firm, yellow plaques or nodules, often in a periorbital distribution. It is often accompanied by an elevated ERYTHROCYTE SEDIMENTATION RATE; LEUKOPENIA; and MONOCLONAL GAMMOPATHY (IgG-kappa type) and systemic involvement.

Synonyms(1)

Synonym
Necrobiotic Xanthogranuloma

Research Excerpts

Overview

ExcerptReference
"Necrobiotic xanthogranuloma is a non-Langerhans histiocytic disorder characterized by chronic and progressively enlarging cutaneous plaques and nodules predominantly at the head and facial regions."( Nguyen, BD, 2014)
"Necrobiotic xanthogranuloma is a rare non-Langerhans-cell histiocytosis."( Hansel, G; Klingner, M; Schönlebe, J; Wollina, U, 2016)
"Necrobiotic xanthogranuloma is a rare chronic condition, belonging to the group C non-Langerhans cell histiocytoses, which is relevant due to the possibility of extracutaneous involvement and association with systemic diseases, particularly hematologic malignancies."( Mello, RB; Vale, ECSD, 2019)
"Necrobiotic xanthogranuloma is a rare form of non-Langerhans histiocytosis characterized by development of chronic cutaneous nodules that enlarge over time to form plaques."( Eltilib, M; Fenner, J; Oldan, J; Saramago, I, 2020)
"Necrobiotic xanthogranuloma is a rare disease that is part of the non-Langerhans cell histiocytoses."( Abeck, F; Booken, N; Ghandili, S; Hansen, I; Schneider, SW, 2023)

Context

ExcerptReference
"Necrobiotic xanthogranulomas have a predilection for the bilateral periorbital region and often present with consequential ophthalmic findings."( Bookout, A; Hogan, DJ; Seastrom, S, 2014)

Research

Studies (36)

TimeframeStudies, This Condition (%)All Conditions %
pre-19900 (0.00)23.3326
1990's0 (0.00)12.5806
2000's0 (0.00)18.1394
2010's26 (72.22)28.8240
2020's10 (27.78)9.53
DrugIndicatedRelationship StrengthStudiesTrials
salicylic acid0low10
chlorambucil0low20
isoniazid0low10
mechlorethamine0low10
thalidomide0low60
prednisolone0low20
prednisone0low20
triamcinolone acetonide0low10
methylprednisolone0low20
cladribine0low10
vidarabine0low10
propiconazole0low10
fluorodeoxyglucose f180low40
methotrexate0low20
lenalidomide0low50
bortezomib0low20
tacrolimus0low10
fludarabine0low10
bilirubin0low10
acitretin0low10
clobetasol0low10
an27280low10
cyclosporine0low10
orabase0low10
rifampin0low10
eye0low10

Protein Targets (455)

ProteinPotency MeasurementsInhibition MeasurementsActivation MeasurementsDrugs
Chain A, JmjC domain-containing histone demethylation protein 3A1001
Luciferase3003
USP1 protein, partial4004
TDP1 protein150015
Microtubule-associated protein tau6006
AR protein170017
aldehyde dehydrogenase 1 family, member A18008
glucocorticoid receptor [Homo sapiens]110011
estrogen nuclear receptor alpha170017
peroxisome proliferator activated receptor gamma8008
euchromatic histone-lysine N-methyltransferase 27007
cytochrome P450, family 19, subfamily A, polypeptide 1, isoform CRA_a100010
vitamin D3 receptor isoform VDRA6006
thyroid hormone receptor beta isoform 2110011
flap endonuclease 12002
serine/threonine-protein kinase PLK11001
eyes absent homolog 2 isoform a1001
DNA polymerase eta isoform 12002
geminin130013
peripheral myelin protein 224004
survival motor neuron protein isoform d5005
histone acetyltransferase KAT2A isoform 14004
ATP-binding cassette sub-family C member 3013013
Multidrug resistance-associated protein 4013014
Bile salt export pump0707
Bile salt export pump015015
Rap guanine nucleotide exchange factor 31001
Cellular tumor antigen p53120012
Rap guanine nucleotide exchange factor 42002
Fumarate hydratase5005
EWS/FLI fusion protein110011
polyprotein5005
cGMP-dependent 3',5'-cyclic phosphodiesterase0202
cGMP-specific 3',5'-cyclic phosphodiesterase0101
cAMP-specific 3',5'-cyclic phosphodiesterase 4A0202
Calcium/calmodulin-dependent 3',5'-cyclic nucleotide phosphodiesterase 1A0101
Spike glycoprotein4004
Calcium/calmodulin-dependent 3',5'-cyclic nucleotide phosphodiesterase 1B0101
cAMP-specific 3',5'-cyclic phosphodiesterase 4B0202
cAMP-specific 3',5'-cyclic phosphodiesterase 4C0202
cAMP-specific 3',5'-cyclic phosphodiesterase 4D0202
cGMP-inhibited 3',5'-cyclic phosphodiesterase B0101
High affinity cAMP-specific 3',5'-cyclic phosphodiesterase 7A0101
Calcium/calmodulin-dependent 3',5'-cyclic nucleotide phosphodiesterase 1C0101
cGMP-inhibited 3',5'-cyclic phosphodiesterase A0101
Beta-lactamase 0101
cAMP and cAMP-inhibited cGMP 3',5'-cyclic phosphodiesterase 10A0101
Chain A, TYROSYL-DNA PHOSPHODIESTERASE3003
Chain A, Putative fructose-1,6-bisphosphate aldolase1001
Chain A, HADH2 protein3003
Chain B, HADH2 protein3003
Chain A, 2-oxoglutarate Oxygenase4004
Chain A, Cruzipain2002
phosphopantetheinyl transferase4004
DNA polymerase kappa isoform 13003
caspase-1 isoform alpha precursor2002
lamin isoform A-delta107007
Caspase-72002
Inositol monophosphatase 11001
Solute carrier organic anion transporter family member 1B30609
Solute carrier organic anion transporter family member 1B10507
Chain H, Proteasome component PUP10101
Chain I, Proteasome component PUP30101
Chain K, Proteasome component PRE20101
Chain L, Proteasome component C50101
Chain K, Proteasome component PRE20101
Chain L, Proteasome component C50101
Proteasome subunit beta type-110101
RAR-related orphan receptor gamma6006
PPM1D protein3003
caspase 7, apoptosis-related cysteine protease2002
estrogen receptor 2 (ER beta)4004
nuclear receptor subfamily 1, group I, member 38008
progesterone receptor7007
cytochrome P450 family 3 subfamily A polypeptide 46006
retinoic acid nuclear receptor alpha variant 18008
retinoid X nuclear receptor alpha100010
estrogen-related nuclear receptor alpha100010
farnesoid X nuclear receptor5005
pregnane X nuclear receptor9009
peroxisome proliferator-activated receptor delta8008
vitamin D (1,25- dihydroxyvitamin D3) receptor8008
caspase-32002
nuclear receptor subfamily 1, group I, member 24004
Histone H2A.x4004
Caspase-71001
caspase-31001
heat shock protein beta-12002
nuclear factor erythroid 2-related factor 2 isoform 19009
26S proteasome non-ATPase regulatory subunit 110101
26S proteasome non-ATPase regulatory subunit 120101
26S proteasome non-ATPase regulatory subunit 140101
Proteasome subunit alpha type-70101
Histone deacetylase 30101
26S proteasome non-ATPase regulatory subunit 30101
Carbonic anhydrase 120303
Voltage-dependent calcium channel gamma-2 subunit4004
Prothrombin0101
Chymotrypsinogen A0101
Carbonic anhydrase 10404
Carbonic anhydrase 20415
Interferon beta6006
Carbonic anhydrase 30202
Cathepsin B0101
Neutrophil elastase0101
Cathepsin G0202
Lysosomal protective protein0101
Chymotrypsinogen B0101
26S proteasome regulatory subunit 6A0101
Glutamate receptor 24004
Nuclear factor NF-kappa-B p105 subunit0101
Proteasome subunit beta type-10102
Carbonic anhydrase 40202
Carbonic anhydrase 60202
Chymase0101
Proteasome subunit alpha type-10101
Proteasome subunit alpha type-20101
Proteasome subunit alpha type-30101
Proteasome subunit alpha type-40101
Proteasome subunit beta type-80101
Proteasome subunit beta type-80101
Proteasome subunit beta type-90101
Proteasome subunit alpha type-50101
Proteasome subunit beta type-40101
Proteasome subunit beta type-60101
Proteasome subunit beta type-50203
Carbonic anhydrase 5A, mitochondrial0202
26S proteasome regulatory subunit 70101
Lon protease homolog, mitochondrial0101
Proteasome subunit beta type-100101
Carbonic anhydrase 70202
26S proteasome regulatory subunit 6B0101
26S proteasome non-ATPase regulatory subunit 80101
Proteasome subunit beta type-30101
Proteasome subunit beta type-20102
26S proteasome non-ATPase regulatory subunit 70101
26S proteasome non-ATPase regulatory subunit 40101
26S proteasome complex subunit SEM10101
Proteasome subunit alpha type-60101
26S proteasome regulatory subunit 40101
26S proteasome regulatory subunit 80101
26S proteasome regulatory subunit 10B0101
Beta-carbonic anhydrase 10101
Nuclear factor NF-kappa-B p100 subunit 0101
Transcription factor p650101
26S proteasome non-ATPase regulatory subunit 20101
Histone deacetylase 10101
26S proteasome non-ATPase regulatory subunit 60101
Proteasomal ubiquitin receptor ADRM10101
Carbonic anhydrase 90303
ATP-dependent Clp protease proteolytic subunit0112
Carbonic anhydrase 130101
NACHT, LRR and PYD domains-containing protein 3 0101
Proteasome subunit alpha-type 80101
Histone deacetylase 20101
Canalicular multispecific organic anion transporter 1012012
ATPase family AAA domain-containing protein 59009
Proteasome subunit beta type-70101
26S proteasome non-ATPase regulatory subunit 10101
Ataxin-29009
Carbonic anhydrase 150202
Histone deacetylase 80101
Histone deacetylase 60101
Carbonic anhydrase 140202
26S proteasome non-ATPase regulatory subunit 130101
Carbonic anhydrase 5B, mitochondrial0202
interleukin 83003
thioredoxin reductase4004
NFKB1 protein, partial1001
GALC protein1001
GLS protein2002
GLI family zinc finger 39009
Thrombopoietin1001
thioredoxin glutathione reductase2002
hypoxia-inducible factor 1, alpha subunit (basic helix-loop-helix transcription factor)5005
thyroid stimulating hormone receptor3003
bromodomain adjacent to zinc finger domain 2B4004
activating transcription factor 62002
v-jun sarcoma virus 17 oncogene homolog (avian)3003
Bloom syndrome protein isoform 13003
peripheral myelin protein 22 isoform 11001
chromobox protein homolog 15005
potassium voltage-gated channel subfamily H member 2 isoform d2002
FAD-linked sulfhydryl oxidase ALR0001
urokinase-type plasminogen activator precursor4004
plasminogen precursor4004
urokinase plasminogen activator surface receptor precursor4004
muscleblind-like protein 1 isoform 15005
muscarinic acetylcholine receptor M11001
Cytochrome P450 2D60101
Nuclear receptor ROR-gamma3003
ATP-dependent phosphofructokinase3003
Chain A, BCL-2-RELATED PROTEIN A10011
glp-1 receptor, partial2002
Smad33003
G3003
67.9K protein6006
IDH15005
cellular tumor antigen p53 isoform a2002
nuclear factor erythroid 2-related factor 2 isoform 27007
huntingtin isoform 21001
tyrosine-protein kinase Yes1001
nuclear receptor ROR-gamma isoform 17007
relaxin receptor 1 isoform 11001
bcl-2-like protein 11 isoform 10011
HLA class I histocompatibility antigen, B alpha chain 3003
Integrin beta-32002
Integrin alpha-IIb2002
Deoxycytidine kinase0001
Adenosine receptor A30202
Inositol hexakisphosphate kinase 13003
cytochrome P450 2C9, partial3003
aryl hydrocarbon receptor5005
Chain A, MAJOR APURINIC/APYRIMIDINIC ENDONUCLEASE4004
6-hydroxymethyl-7,8-dihydropterin pyrophosphokinase 0101
cytochrome P450 2C9 precursor1001
cytochrome P450 3A4 isoform 12002
Gamma-aminobutyric acid receptor subunit pi2002
Myeloperoxidase0202
Gamma-aminobutyric acid receptor subunit beta-12002
Dihydrofolate reductase0303
Gamma-aminobutyric acid receptor subunit delta2002
Gamma-aminobutyric acid receptor subunit gamma-22002
Gamma-aminobutyric acid receptor subunit alpha-52002
Gamma-aminobutyric acid receptor subunit alpha-32002
Gamma-aminobutyric acid receptor subunit gamma-12002
Gamma-aminobutyric acid receptor subunit alpha-22002
Gamma-aminobutyric acid receptor subunit alpha-42002
Gamma-aminobutyric acid receptor subunit gamma-32002
Gamma-aminobutyric acid receptor subunit alpha-62002
Histamine H2 receptor3003
5-hydroxytryptamine receptor 60101
Gamma-aminobutyric acid receptor subunit alpha-12002
Gamma-aminobutyric acid receptor subunit beta-32002
Gamma-aminobutyric acid receptor subunit beta-22002
GABA theta subunit2002
Gamma-aminobutyric acid receptor subunit epsilon2002
Chain A, Beta-lactamase3003
corticotropin-releasing hormone receptor 20112
corticotropin releasing factor-binding protein0112
glycogen synthase kinase-3 beta isoform 10022
5'-nucleotidase0101
Adenosine deaminase 0002
Glycogen phosphorylase, muscle form0101
Asialoglycoprotein receptor 10112
3-oxoacyl-[acyl-carrier-protein] synthase 3 0101
cytochrome P450 2D6 isoform 11001
N-glycosylase/DNA lyase0101
Polyphenol oxidase 20203
Cytochrome P450 1A20101
Formamidopyrimidine-DNA glycosylase0101
Cytochrome P450 3A40303
DNA gyrase subunit A0101
DNA gyrase subunit B0101
Cytochrome P450 2C80101
Cytochrome P450 2A60101
Macrophage migration inhibitory factor0101
Cytochrome P450 2C190101
Endonuclease III-like protein 10101
DNA gyrase subunit B0202
DNA gyrase subunit A0202
Enoyl-[acyl-carrier-protein] reductase [NADH]0203
Pantothenate synthetase0101
Isocitrate lyase0101
Putative FAD-containing monooxygenase MymA0101
Dihydrofolate reductase0202
Endonuclease 8-like 10101
Cereblon isoform 40303
Bromodomain-containing protein 40101
Tyrosine-protein kinase ABL10012
Breakpoint cluster region protein0001
DNA-binding protein Ikaros0014
DNA damage-binding protein 10213
Protein cereblon0429
Zinc finger protein Aiolos0011
Chain B, DIHYDROFOLATE REDUCTASE0011
Chain B, DIHYDROFOLATE REDUCTASE0011
Chain B, DIHYDROFOLATE REDUCTASE0011
Chain A, Ferritin light chain3003
acid sphingomyelinase1001
dopamine D1 receptor1001
ClpP1001
15-lipoxygenase, partial2002
ATAD5 protein, partial2002
Thymidylate synthase 0101
alpha-galactosidase1001
Thymidylate synthase 0101
polyunsaturated fatty acid lipoxygenase ALOX121001
15-hydroxyprostaglandin dehydrogenase [NAD(+)] isoform 12002
transcriptional regulator ERG isoform 31001
importin subunit beta-1 isoform 11001
DNA polymerase beta1001
nuclear receptor coactivator 1 isoform 1 [Homo sapiens]0101
snurportin-11001
peptidyl-prolyl cis-trans isomerase NIMA-interacting 11001
DNA polymerase iota isoform a (long)2002
transactivating tegument protein VP16 [Human herpesvirus 1]0101
M-phase phosphoprotein 81001
nuclear receptor coactivator 3 isoform a0101
Toll-like receptor 40101
Fatty-acid amide hydrolase 10101
ATP-binding cassette sub-family C member 30102
Dihydrofolate reductase0203
Dihydrofolate reductase0203
Dihydrofolate reductase 0101
Dihydrofolate reductase0101
Dihydrofolate reductase0101
Dihydrofolate reductase0203
Thymidylate synthase0101
Thymidylate synthase0202
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Thymidylate synthase0202
5-hydroxytryptamine receptor 2C0102
High mobility group protein B10022
Dihydrofolate reductase0101
Thymidylate synthase0101
Dihydrofolate reductase0224
Glucose-6-phosphate 1-dehydrogenase0101
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Folate receptor beta0101
5-hydroxytryptamine receptor 2A0101
Cytochrome P450 11B1, mitochondrial 0101
Folate receptor alpha0101
Histidine decarboxylase0101
Trifunctional purine biosynthetic protein adenosine-30101
Cyclin-dependent kinase 20011
Bifunctional purine biosynthesis protein ATIC0202
Multidrug resistance-associated protein 1 0001
Delta-type opioid receptor0101
Mu-type opioid receptor0101
Kappa-type opioid receptor0101
Reduced folate transporter0202
Folylpolyglutamate synthase, mitochondrial0103
6-phosphogluconate dehydrogenase, decarboxylating0101
Solute carrier organic anion transporter family member 1A30001
Pteridine reductase 10101
Folylpolyglutamate synthase, mitochondrial0103
Bifunctional dihydrofolate reductase-thymidylate synthase0101
TAR DNA-binding protein 433003
Bifunctional dihydrofolate reductase-thymidylate synthase0101
Solute carrier family 22 member 60102
Canalicular multispecific organic anion transporter 10102
Trifunctional purine biosynthetic protein adenosine-30101
Solute carrier family 22 member 200202
Dihydrofolate reductase 0101
Solute carrier family 22 member 80102
Solute carrier family 22 member 60202
Dihydrofolate reductase0101
ATP-binding cassette sub-family C member 110001
Proton-coupled folate transporter0101
Multidrug resistance associated protein0102
Solute carrier family 22 member 110001
Protein-arginine deiminase type-40101
Broad substrate specificity ATP-binding cassette transporter ABCG20203
acetylcholinesterase1001
hypoxia-inducible factor 1 alpha subunit3003
nonstructural protein 12002
Nuclear receptor subfamily 1 group I member 20033
Glucocorticoid receptor08215
Glycine receptor subunit alpha-10808
Androgen receptor0303
Glycine receptor subunit beta0808
Glycine receptor subunit alpha-20808
Glycine receptor subunit alpha-30808
Nuclear receptor subfamily 1 group I member 20011
luciferase0001
Fatty acid-binding protein, liver0101
Estrogen receptor0202
Progesterone receptor0203
Glucocorticoid receptor0112
Glucocorticoid receptor0011
Corticosteroid-binding globulin0101
Mineralocorticoid receptor 0213
Androgen receptor0202
Cytochrome P450 2C9 0101
Glutamine synthetase0011
Histamine H1 receptor0022
Prostaglandin G/H synthase 20202
Nociceptin receptor0011
Cytochrome P450 2J20101
Estrogen receptor beta0202
pregnane X receptor2002
SMAD family member 22002
SMAD family member 32002
heat shock 70kDa protein 5 (glucose-regulated protein, 78kDa)1001
thyroid hormone receptor beta isoform a2002
heat shock protein HSP 90-alpha isoform 20001
Polyunsaturated fatty acid lipoxygenase ALOX15B2002
Guanine nucleotide-binding protein G1001
heat shock protein 90, putative0001
DNA-directed RNA polymerase subunit beta0101
Solute carrier organic anion transporter family member 1A40101
Solute carrier organic anion transporter family member 2B1 0202
Albumin0112
NH(3)-dependent NAD(+) synthetase0101
60 kDa heat shock protein, mitochondrial0101
Polyunsaturated fatty acid lipoxygenase ALOX150101
Cytochrome P450 2B60011
Arylacetamide deacetylase0102
Caspase-10101
Alpha-synuclein0101
Solute carrier organic anion transporter family member 1A10101
Solute carrier organic anion transporter family member 1A20101
10 kDa heat shock protein, mitochondrial0101
Glutathione S-transferase omega-10101
DNA-directed RNA polymerase subunit beta0101
Thiosulfate sulfurtransferase0101
Lanosterol 14-alpha demethylase0101
60 kDa chaperonin 0101
10 kDa chaperonin 0101
chaperonin-containing TCP-1 beta subunit homolog1001
Estrogen receptor beta0001
Inhibitor of nuclear factor kappa-B kinase subunit beta0001
Solute carrier family 22 member 60101
5'-AMP-activated protein kinase subunit beta-20001
Polymerase acidic protein0011
Stromelysin-10011
Tyrosine-protein phosphatase YopH0101
Aldo-keto reductase family 1 member B10101
Prolyl 4-hydroxylase subunit alpha-10101
Tyrosine-protein phosphatase non-receptor type 10103
Estrogen receptor0001
Acetylcholinesterase0101
Prostaglandin G/H synthase 10101
Cystathionine gamma-lyase0101
5'-AMP-activated protein kinase subunit gamma-10001
5'-AMP-activated protein kinase catalytic subunit alpha-20001
Xanthine dehydrogenase/oxidase0101
5'-AMP-activated protein kinase catalytic subunit alpha-10001
Carbonic anhydrase0101
Solute carrier family 22 member 70001
Nicotinate phosphoribosyltransferase0101
Ubiquitin-like domain-containing CTD phosphatase 10101
Carbonic anhydrase 130101
5'-AMP-activated protein kinase subunit gamma-30001
5'-AMP-activated protein kinase subunit gamma-20001
5'-AMP-activated protein kinase subunit beta-10001
UDP-glucuronosyltransferase 2B70001
Cytochrome P450 3A50101
UDP-glucuronosyltransferase 1A40001
Serine/threonine-protein kinase mTOR0101
Peptidyl-prolyl cis-trans isomerase FKBP1A0213
Peptidyl-prolyl cis-trans isomerase FKBP1B0011
Serine/threonine-protein phosphatase 2B catalytic subunit alpha isoform0101
Peptidyl-prolyl cis-trans isomerase FKBP50101
Splicing factor 3B subunit 30101
cytochrome P450 2D62002
arylsulfatase A1001
Prostaglandin G/H synthase 10101
Prostaglandin G/H synthase 20101
nuclear factor of kappa light polypeptide gene enhancer in B-cells 1 (p105), isoform CRA_a1001
Thymidine kinase, cytosolic0001
Dipeptidyl peptidase 40101
Adenosylhomocysteinase0101
RNA-directed RNA polymerase 0112
[prepared from compound, protein, and bioassay information from National Library of Medicine (NLM), extracted Dec-2023]