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Mastocytosis

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498: 50: 529:. However, normal ranges for mast cell counts in the gastrointestinal tract mucosa are not well established in the literature, and depend upon the exact location (e.g. right versus left colon), gender, and patient populations (such as asymptomatic patients versus patients with chronic diarrhea of unknown etiology). Quantitative mast cell stains may yield little diagnostic information, and further research studies are warranted to determine whether "mastocytic enterocolitis" truly represents a distinct entity. 137: 148:, the substances that are released can cause a number of symptoms that can vary over time and can range in intensity from mild to severe. Because mast cells play a role in allergic reactions, the symptoms of mastocytosis often are similar to the symptoms of an allergic reaction. They may include, but are not limited to 380:
In case of suspicion of systemic disease the level of serum tryptase in the blood can be of help. If the base level of s-tryptase is elevated, this implies that the mastocytosis can be systemic. In cases of suspicion of SM help can also be drawn from analysis of mutation in KIT(D816V) in peripheral
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Some of the most important research advances for this rare disorder include improved diagnosis of mast cell disease and identification of growth factors and genetic mechanisms responsible for increased mast cell production. Researchers are currently evaluating approaches to improve ways to treat
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Hermine, Olivier; Lortholary, Olivier; Leventhal, Phillip S.; Catteau, Adeline; Soppelsa, Frédérique; Baude, Cedric; Cohen-Akenine, Annick; Palmérini, Fabienne; Hanssens, Katia; Yang, Ying; Sobol, Hagay; Fraytag, Sylvie; Ghez, David; Suarez, Felipe; Barete, Stéphane; Casassus, Philippe; Sans,
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Generalized eruption of cutaneous mastocytosis (adult type) is the most common pattern of mastocytosis presenting to the dermatologist, with the most common lesions being macules, papules, or nodules that are disseminated over most of the body but especially on the upper arms, legs, and
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Systemic mastocytosis involves the bone marrow in the majority of cases and in some cases other internal organs, usually in addition to involving the skin. Mast cells collect in various tissues and can affect organs where mast cells do not normally inhabit such as the
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Noack, F.; Escribano, L.; Sotlar, K.; Nunez, R.; Schuetze, K.; Valent, P.; Horny, H.-P. (July 2009). "Evolution of Urticaria Pigmentosa into Indolent Systemic Mastocytosis: Abnormal Immunophenotype of Mast Cells without Evidence of c-kit Mutation ASP-816-VAL".
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Broesby-Olsen, Sigurd; Farkas, Dóra Körmendiné; Vestergaard, Hanne; Hermann, Anne Pernille; Møller, Michael Boe; Mortz, Charlotte Gotthard; Kristensen, Thomas Kielsgaard; Bindslev-Jensen, Carsten; Sørensen, Henrik Toft; Frederiksen, Henrik (November 2016).
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Sethi, Aisha; Jain, Dhanpat; Roland, Bani Chander; Kinzel, Jason; Gibson, Joanna; Schrader, Ronald; Hanson, Joshua Anspach (2015-02-01). "Performing Colonic Mast Cell Counts in Patients With Chronic Diarrhea of Unknown Etiology Has Limited Diagnostic Use".
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Scientists also are focusing on identifying disease-associated mutations (changes in genes). NIH scientists have identified some mutations, which may help researchers understand the causes of mastocytosis, improve diagnosis, and develop better treatments.
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Moura, Daniela Silva; Sultan, Serge; Georgin-Lavialle, Sophie; Pillet, Nathalie; Montestruc, François; Gineste, Paul; Barete, Stéphane; Damaj, Gandhi; Moussy, Alain; Lortholary, Olivier; Hermine, Olivier; Hashimoto, Kenji (21 October 2011).
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Patients with indolent systemic mastocytosis have a normal life expectancy. The prognosis for patients with advanced systemic mastocytosis differs depending on type of disease with MCL being the most serious form with short survival.
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Monoclonal mast cell activation, defined by the World Health Organization definitions 2010, also has increased mast cells but insufficient to be systemic mastocytosis (in World Health Organization Definitions)
470:(TMEP) is a much rarer form of cutaneous mastocytosis that affects adults. MPCM and TMEP can be a part of indolent systemic mastocytosis. This should be considered if patients develop any systemic symptoms 2638: 2517: 280:
There are few qualitative studies about the effects of mastocytosis on daily life. However, a Danish study from 2018 describes the multidimensional impact of the disease on everyday life.
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Arber, Daniel A.; Orazi, Attilio; Hasserjian, Robert; Thiele, JĂĽrgen; Borowitz, Michael J.; Le Beau, Michelle M.; Bloomfield, Clara D.; Cazzola, Mario; Vardiman, James W. (19 May 2016).
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are an important and often overlooked tool in the treatment of mastocytosis. Depression and other neurological symptoms have been noted in mastocytosis. Some antidepressants, such as
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help reduce production of gastric acid, which is often increased in patients with mastocytosis. Excess gastric acid can harm the stomach, esophagus, and small intestine.
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Rogers, M P; Bloomingdale, K; Murawski, B J; Soter, N A; Reich, P; Austen, K F (July 1986). "Mixed organic brain syndrome as a manifestation of systemic mastocytosis".
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In cases of advanced systemic mastocytosis or rare cases with indolent systemic mastocytosis with very troublesome symptoms, cytoreductive therapy can be indicated.
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receptor (mutation KIT(D816V)), leading to constitutive signalling through the receptor is found in >90% of patients with systemic mastocytosis.
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was first described in 1869. The first report of a primary mast cell disorder is attributed to Unna, who in 1887 reported that skin lesions of
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constricts blood vessels and opens airways to maintain adequate circulation and ventilation when excessive mast cell degranulation has caused
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Cutaneous mastocytosis in children usually presents in the first year after birth and in most cases vanishes during adolescence.
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In Europe the European Competence Network on Mastocytosis (ECNM) coordinates studies, registries and education on mastocytosis.
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or no mast cells has been found, which indicates to some scientists we may not be able to survive with too few mast cells.
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Ellis DL (1996). "Treatment of telangiectasia macularis eruptiva perstans with the 585-nm flashlamp-pumped dye laser".
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The true incidence and prevalence of mastocytosis is unknown, but mastocytosis generally has been considered to be an "
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Drugs to prevent/treat osteoporosis include Calcium-Vitamine D, bisphosphonates and in rare cases inhibitors of RANK-L
435:– has normal number of mast cells, but all the symptoms and in some cases the genetic markers of systemic mastocytosis 3116: 3023: 674:(CdA). Chemotherapy which is given as subcutaneous injections. Side effects include immunodeficiency and infections. 384:
To set the diagnosis of systemic mastocytosis, certain criteria must be met. Either one major + one minor criterion
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has been used in rare cases with aggressive systemic mastocytosis in patients deemed to be fit for the procedure.
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affecting both children and adults caused by the accumulation of functionally defective mast cells (also called
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Beatrice; Arock, Michel; Kinet, Jean Pierre; Dubreuil, Patrice; Moussy, Alain; Soyer, H. Peter (28 May 2008).
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may be thickened and infiltrated with mast cells to produce a peculiar orange color, giving rise to the term
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is available in Canada and Europe and more recently in the U.S. It is also available as eyedrops (Zaditor).
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To quell unpredictable allergic reactions, an experimental drug takes aim at a genetic cause, not symptoms
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The most common cutaneous mastocytosis is maculopapular cutaneous mastocytosis, previously named papular
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Ramsay, David B.; Stephen, Sindu; Borum, Marie; Voltaggio, Lysandra; Doman, David B. (December 2010).
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contained numerous mast cells. Systemic mastocytosis was first reported by French scientists in 1936.
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can be used topically, inhaled, or systemically to reduce inflammation associated with mastocytosis.
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There is no cure for mastocytosis, but there are a number of medicines to help treat the symptoms:
210: 74: 686:. TKI acting on many different tyrosine kinases, approved by FDA and EMA for advanced mastocytosis 357:
appears to be important for the proliferation of mast cells. Mutations of the gene coding for the
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Jensen, Britt; Broesby-Olsen, Sigurd; Bindslev-Jensen, Carsten; Nielsen, Dorthe S. (April 2019).
953:"Depression in Patients with Mastocytosis: Prevalence, Features and Effects of Masitinib Therapy" 548: 221: 3865: 1620:"Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and management" 763:
scientists have been studying and treating patients with mastocytosis for several years at the
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released by mast cells. Researchers also think mast cells may have a role in the growth of
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is the only medicine specifically approved by the FDA for the treatment of mastocytosis.
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Gruchalla, Rebecca S. (June 1995). "Mastocytosis: Developments During the Past Decade".
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Valent P, Akin C, Hartmann K, Nilsson G, Reiter A, Hermine O, et al. (March 2017).
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and other beta-2 agonists open airways that can constrict in the presence of histamine.
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in trials; currently being tested but showing promise in reduction of tryptase levels.
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Dense infiltrates of >15 mast cells in the bone marrow or an extracutaneous organ
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People affected by mastocytosis are susceptible to a variety of symptoms, including
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SĂ©zary A, Levy-Coblentz G, Chauvillon P (1936). "Dermographisme et mastocytose".
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Contributions to the anatomy and pathogenesis of urticaria simplex and pigmentosa
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Mast cells seem to have other roles as well. Because they gather together around
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Keratosis linearis with ichthyosis congenita and sclerosing keratoderma syndrome
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antagonists block receptors targeted by leukotrienes released from mast cells.
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highlights mast cells (darkly stained) in the mucosa of the small intestine.
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Diffuse cutaneous mastocytosis has diffuse involvement in which the entire
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Beiträge zur Anatomie und Pathogenese der Urticaria simplex und Pigmentosa
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block receptors targeted by histamine released from mast cells. Both H
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Template:Congenital malformations and deformations of skin appendages
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Bulletin de la Société Française de Dermatologie et de Syphiligraphie
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can relieve skin symptoms, but may increase the risk of skin cancer.
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Systemic mastocytosis with associated hematological neoplasm (SM-AHN)
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Indolent systemic mastocytosis (ISM). The most common SM (>90%)
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help prevent mast cells from releasing their chemical contents.
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Focal palmoplantar keratoderma with oral mucosal hyperkeratosis
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Aberrant phenotype on the mast cells (pos. for CD2 and/or CD25)
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Cardet, Juan Carlos; Akin, Cem; Lee, Min Jung (October 2013).
692:. Can have effect in rare cases without mutation in KIT(D816V) 1664:"Novel approaches to treating advanced systemic mastocytosis" 1470:(2). Archives of Pathology and Laboratory Medicine: 225–232. 464:(UP), more common in children, although also seen in adults. 273:(due to inactivation of pancreatic enzymes by increased acid) 2518:
Adult type of generalized eruption of cutaneous mastocytosis
178:, a reaction to stroking or scratching of urticaria lesions. 1108:
Orfao A, Garcia-Montero AC, Sanchez L, Escribano L (2007).
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Lee, JK; Whittaker, SJ; Enns, RA; Zetler, P (Dec 7, 2008).
1012:"Gastrointestinal manifestations of systemic mastocytosis" 438:
Another known but rare mast cell proliferation disease is
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Further information on the investigational new drug:
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Congenital hypertrophy of the lateral fold of the hallux
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Gilreath, JA; Tchertanov, L; Deininger, MW (July 2019).
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Template:DNA replication and repair-deficiency disorder
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and other pro-inflammatory substances from mast cells.
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James, William; Berger, Timothy; Elston, Dirk (2005).
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National Institute of Allergy and Infectious Diseases
3710: 1260:"Mast cell activation: proposed diagnostic criteria" 3826: 3714: 3561: 3531: 3495: 3486: 3405: 3324: 3071: 3057: 3010: 2959: 2836: 2786: 2768: 2717: 2699: 2685: 2676: 2597: 2570: 2563: 2541: 2496: 2464: 2455: 2400: 2378: 2356: 2347: 2314: 2305: 2296: 2262: 2240: 2231: 2205: 2196: 2176: 2145: 2123: 2114: 2083: 2056: 2047: 2021: 1979: 1970: 1961: 1946: 1400:
Andrews' Diseases of the Skin: Clinical Dermatology
68: 37: 32: 235:Decreased bone density or increased bone density ( 3090:Diffuse nonepidermolytic palmoplantar keratoderma 2814:Nonbullous congenital ichthyosiform erythroderma 2439:Refractory cytopenia with multilineage dysplasia 1668:Clinical Pharmacology: Advances and Applications 1306:National Organization for Rare Disorders (NORD) 3279:Palmoplantar keratoderma and spastic paraplegia 1657: 1655: 1613: 1611: 1464:Archives of Pathology & Laboratory Medicine 532:There are five types of systemic mastocytosis: 3085:Diffuse epidermolytic palmoplantar keratoderma 2102:Accelerated phase chronic myelogenous leukemia 1264:The Journal of Allergy and Clinical Immunology 1212: 1210: 945: 943: 586:blockers may be helpful, often in combination. 451:Mastocytosis can occur in a variety of forms: 410:Aberrant mast cell morphology (spindle-shaped) 303:, mast cells attract other key players of the 3616:Congenital malformations of the dermatoglyphs 2646: 1920: 865:. Mastocytosis Society Canada. Archived from 8: 1878:The American Journal of the Medical Sciences 1835:] (in German). Verlag von Leopold Voss. 307:to areas of the body where they are needed. 3711: 3641:Melanotic neuroectodermal tumor of infancy 3492: 3378:Keratosis follicularis spinulosa decalvans 3068: 2809:Ichthyosis–sclerosing cholangitis syndrome 2696: 2682: 2653: 2639: 2631: 2567: 2461: 2353: 2311: 2302: 2237: 2202: 2120: 2053: 1976: 1967: 1958: 1927: 1913: 1905: 698:. Is being tested in trials. Not approved. 467:Telangiectasia macularis eruptiva perstans 425:Other types of mast cell disease include: 388:three minor criteria has to be fulfilled: 255:Increased stomach acid production causing 48: 29: 3601:Congenital cartilaginous rest of the neck 3196:Focal palmoplantar and gingival keratosis 1803: 1731: 1689: 1679: 1635: 1548: 1437: 1283: 1234: 1190: 1125: 1037: 1027: 986: 976: 923: 913: 838: 3666:Rapidly involuting congenital hemangioma 3256:Keratosis punctata of the palmar creases 3251:Keratosis punctata palmaris et plantaris 3122:Clouston's hidrotic ectodermal dysplasia 1422:"Mast Cells in Gastrointestinal Disease" 863:"Mastocytosis & mast cell disorders" 2419:Refractory anemia with excess of blasts 813: 539:Smouldering systemic mastocytosis (SSM) 1258:Akin C, Valent P, Metcalfe DD (2010). 545:Aggressive systemic mastocytosis (ASM) 2727:Congenital ichthyosiform erythroderma 821:Horny HP, Sotlar K, Valent P (2007). 381:blood using sensitive PCR-technology 7: 3388:Keratosis pilaris atrophicans faciei 2973:Dermatopathia pigmentosa reticularis 2584:Acute panmyelosis with myelofibrosis 1618:Pardanani, Animesh (November 2016). 717:Allogeneic stem cell transplantation 263:and direct histamine stimulation on 2434:Paroxysmal nocturnal hemoglobinuria 3681:Superficial lymphatic malformation 3626:Congenital smooth muscle hamartoma 3095:Palmoplantar keratoderma of Sybert 2664:malformations and deformations of 1506:"Mastocytosis - Treatment Options" 377:) may help confirm the diagnosis. 25: 3153:Scleroatrophic syndrome of Huriez 2983:Hypohidrotic ectodermal dysplasia 1529:Expert Opinion on Pharmacotherapy 1426:Gastroenterology & Hepatology 1016:World Journal of Gastroenterology 413:Finding of mutation in KIT(D816V) 3266:Porokeratosis plantaris discreta 3223:Striate palmoplantar keratoderma 2159:Juvenile myelomonocytic leukemia 1890:10.1097/00000441-199506000-00007 1827:Unna, Paul Gerson; Unna (1887). 1586:10.1097/00006842-198607000-00006 1127:10.1111/j.1365-2141.2007.06619.x 823:"Mastocytosis: state of the art" 3899:Dermal and subcutaneous growths 3691:Verrucous vascular malformation 3621:Congenital preauricular fistula 3591:Accessory nail of the fifth toe 3468:Template:Pigmentation disorders 3261:Schöpf–Schulz–Passarge syndrome 3241:Acrokeratoelastoidosis of Costa 2946:Laryngoonychocutaneous syndrome 2819:Ichthyosis linearis circumflexa 2804:Ichthyosis prematurity syndrome 2424:Chromosome 5q deletion syndrome 2335:Acute megakaryoblastic leukemia 2164:Chronic myelomonocytic leukemia 2075:Myeloid dendritic cell leukemia 213:from allergic or immune causes) 75:Clinical Immunology and Allergy 3305:Erythrokeratodermia variabilis 3216:Pachyonychia congenita type II 2508:Diffuse cutaneous mastocytosis 1720:American Journal of Hematology 1624:American Journal of Hematology 60:of mastocytosis. Skin biopsy. 1: 3631:Cystic lymphatic malformation 3584:Cavernous venous malformation 3211:Pachyonychia congenita type I 3134:Corneodermatoosseous syndrome 2794:Ichthyosis bullosa of Siemens 2276:Chronic eosinophilic leukemia 2035:Chronic neutrophilic leukemia 1183:10.1158/0008-5472.CAN-16-2234 765:National Institutes of Health 433:Mast cell activation syndrome 3039:Popliteal pterygium syndrome 2731:Epidermolytic hyperkeratosis 2616:Biphenotypic acute leukaemia 2551:Xanthelasmoidal mastocytosis 1778:Nettleship E, Tay W (1869). 1541:10.1517/14656566.2013.824424 1334:10.1016/1076-0512(95)00388-6 1236:10.1182/blood-2016-03-643544 978:10.1371/journal.pone.0026375 915:10.1371/journal.pone.0002266 416:S-tryptase >20 ng/ml 203:Ear/nose/throat inflammation 3671:Rosenthal–Kloepfer syndrome 3656:Omphalomesenteric duct cyst 3373:Keratolytic winter erythema 3033:Gerodermia osteodysplastica 1993:Acute myeloblastic leukemia 1370:10.1080/1042819021000037967 1067:Journal of Clinical Nursing 455:Cutaneous mastocytosis (CM) 124:, caused by the release of 42:Clonal bone marrow disorder 3925: 3246:Focal acral hyperkeratosis 2993:Ellis–van Creveld syndrome 2513:Erythrodermic mastocytosis 2280:Hypereosinophilic syndrome 1276:10.1016/j.jaci.2010.08.035 678:Tyrosine kinase inhibitors 554: 493:Systemic mastocytosis (SM) 353:). In laboratory studies, 259:(increased stimulation of 3170:Papillon–Lefèvre syndrome 2741:Harlequin-type ichthyosis 2328:Essential thrombocythemia 1476:10.5858/arpa.2013-0594-oa 56: 47: 3044:Pseudoxanthoma elasticum 2760:Sjögren–Larsson syndrome 1940:hematological malignancy 1402:. (10th ed.). Saunders. 421:Other mast cell diseases 144:When mast cells undergo 109:+ mast cell precursors. 3904:Immune system disorders 3569:Aplasia cutis congenita 2988:Focal dermal hypoplasia 2951:Skin fragility syndrome 2799:Ichthyosis follicularis 2097:Philadelphia chromosome 1358:Leukemia & Lymphoma 767:(NIH) Clinical Center. 218:very low blood pressure 3686:Thyroglossal duct cyst 3651:Nasolacrimal duct cyst 3574:Amniotic band syndrome 3368:Dyskeratosis congenita 3356:Dyskeratosis congenita 3206:Pachyonychia congenita 3117:Bart–Pumphrey syndrome 3049:Van der Woude syndrome 3024:Ehlers–Danlos syndrome 1768:, Stat, March 16, 2020 1574:Psychosomatic Medicine 610:Proton-pump inhibitors 509: 349:for stem cell factor ( 220:(including shock) and 141: 18:Mastocytosis, systemic 3551:Nevus flammeus nuchae 3462:Template:Phakomatoses 3434:Hereditary lymphedema 2998:Rapp–Hodgkin syndrome 2687:Congenital ichthyosis 2611:Primary myelofibrosis 2543:Systemic mastocytosis 2488:Systemic mastocytosis 1796:10.1136/bmj.2.455.323 656:Cytoreductive therapy 596:Mast cell stabilizers 570:Anti-mediator therapy 500: 335:cell surface receptor 333:Mast cells express a 305:immune defense system 261:enterochromaffin cell 200:Olfactive intolerance 139: 3541:Capillary hemangioma 3443:Urticaria pigmentosa 3201:Howel–Evans syndrome 2961:Ectodermal dysplasia 2902:Generalized atrophic 2533:Solitary mastocytoma 2523:Urticaria pigmentosa 2429:Sideroblastic anemia 1681:10.2147/CPAA.S206615 791:urticaria pigmentosa 787:Urticaria pigmentosa 502:Immunohistochemistry 462:urticaria pigmentosa 371:urticaria pigmentosa 182:Abdominal discomfort 158:urticaria pigmentosa 3228:Tyrosinemia type II 2778:X-linked ichthyosis 2736:Lamellar ichthyosis 2709:Ichthyosis vulgaris 2692:erythrokeratodermia 1029:10.3748/wjg.14.7005 969:2011PLoSO...626375M 906:2008PLoSO...3.2266H 3827:External resources 3611:Congenital lip pit 3175:Haim–Munk syndrome 3127:Vohwinkel syndrome 3002:Hay–Wells syndrome 2978:Hay–Wells syndrome 2824:Ichthyosis hystrix 2750:Netherton syndrome 2478:Mast cell leukemia 2370:Erythroleukemia/M6 2254:Acute eosinophilic 1079:10.1111/jocn.14676 1073:(7–8): 1114–1124. 549:Mast cell leukemia 510: 276:Hepatosplenomegaly 142: 132:Signs and symptoms 122:anaphylactic shock 3886: 3885: 3708: 3707: 3704: 3703: 3636:Median raphe cyst 3596:Bronchogenic cyst 3518:PHACE association 3482: 3481: 3401: 3400: 3393:Keratosis pilaris 3337:Keratosis pilaris 3291:Carvajal syndrome 3271:Spiny keratoderma 2936:Costello syndrome 2832: 2831: 2628: 2627: 2624: 2623: 2559: 2558: 2528:Mast cell sarcoma 2483:Mast cell sarcoma 2451: 2450: 2447: 2446: 2414:Refractory anemia 2392:Polycythemia vera 2343: 2342: 2292: 2291: 2288: 2287: 2227: 2226: 2192: 2191: 2172: 2171: 2110: 2109: 2043: 2042: 1733:10.1002/ajh.24490 1726:(11): 1069–1075. 1637:10.1002/ajh.24553 1630:(11): 1146–1159. 1535:(15): 2033–2045. 1229:(20): 2391–2405. 840:10.1159/000101711 729:ultraviolet light 440:mast cell sarcoma 293:connective tissue 252:Ocular discomfort 88: 87: 27:Medical condition 16:(Redirected from 3916: 3712: 3523:Sinus pericranii 3493: 3349:Darier's disease 3158:Olmsted syndrome 3069: 2969:Naegeli syndrome 2941:Kindler syndrome 2697: 2683: 2655: 2648: 2641: 2632: 2604: 2577: 2568: 2564:Multiple/unknown 2544: 2501: 2471: 2462: 2407: 2385: 2363: 2354: 2321: 2312: 2303: 2269: 2247: 2238: 2219:Acute basophilic 2212: 2203: 2152: 2130: 2121: 2090: 2063: 2054: 2028: 1986: 1977: 1968: 1959: 1929: 1922: 1915: 1906: 1901: 1864: 1863: 1851: 1845: 1844: 1824: 1818: 1817: 1807: 1775: 1769: 1760: 1754: 1753: 1735: 1710: 1704: 1703: 1693: 1683: 1659: 1650: 1649: 1639: 1615: 1606: 1605: 1569: 1563: 1562: 1552: 1520: 1514: 1513: 1502: 1496: 1495: 1458: 1452: 1451: 1441: 1417: 1411: 1396: 1390: 1389: 1352: 1346: 1345: 1317: 1311: 1304: 1298: 1297: 1287: 1255: 1249: 1248: 1238: 1214: 1205: 1204: 1194: 1177:(6): 1261–1270. 1162: 1156: 1155: 1129: 1105: 1099: 1098: 1058: 1052: 1051: 1041: 1031: 1007: 1001: 1000: 990: 980: 947: 938: 937: 927: 917: 884: 878: 877: 875: 874: 859: 853: 852: 842: 818: 600:Cromoglicic acid 345:), which is the 295:, including the 52: 30: 21: 3924: 3923: 3919: 3918: 3917: 3915: 3914: 3913: 3889: 3888: 3887: 3882: 3881: 3822: 3821: 3723: 3709: 3700: 3562:Other/ungrouped 3557: 3546:Port-wine stain 3527: 3488: 3478: 3397: 3320: 3062: 3053: 3006: 2955: 2840: 2828: 2782: 2764: 2713: 2690: 2672: 2659: 2629: 2620: 2598: 2593: 2589:Myeloid sarcoma 2571: 2555: 2542: 2537: 2497: 2492: 2465: 2443: 2401: 2396: 2379: 2374: 2357: 2339: 2315: 2284: 2263: 2258: 2241: 2223: 2206: 2188: 2168: 2146: 2141: 2124: 2106: 2084: 2079: 2057: 2039: 2022: 2017: 1980: 1951: 1942: 1933: 1875: 1872: 1870:Further reading 1867: 1853: 1852: 1848: 1826: 1825: 1821: 1777: 1776: 1772: 1762:Garde, Damian, 1761: 1757: 1712: 1711: 1707: 1661: 1660: 1653: 1617: 1616: 1609: 1571: 1570: 1566: 1522: 1521: 1517: 1512:. 25 June 2012. 1504: 1503: 1499: 1460: 1459: 1455: 1432:(12): 772–777. 1419: 1418: 1414: 1397: 1393: 1354: 1353: 1349: 1319: 1318: 1314: 1305: 1301: 1270:(6): 1099–104. 1257: 1256: 1252: 1216: 1215: 1208: 1171:Cancer Research 1164: 1163: 1159: 1114:Br. J. Haematol 1107: 1106: 1102: 1060: 1059: 1055: 1009: 1008: 1004: 949: 948: 941: 886: 885: 881: 872: 870: 861: 860: 856: 820: 819: 815: 811: 799: 784: 758: 746: 737: 727:Treatment with 725: 658: 642:Antidepressants 632:Corticosteroids 585: 581: 572: 564: 559: 495: 457: 449: 423: 404: 394: 392:Major criterion 367: 326:). No one with 291:are located in 286: 284:Pathophysiology 176:"Darier's Sign" 167:dermatographic 134: 28: 23: 22: 15: 12: 11: 5: 3922: 3920: 3912: 3911: 3906: 3901: 3891: 3890: 3884: 3883: 3880: 3879: 3868: 3857: 3846: 3831: 3830: 3828: 3824: 3823: 3820: 3819: 3808: 3797: 3786: 3778: 3759: 3740: 3724: 3719: 3718: 3716: 3715:Classification 3706: 3705: 3702: 3701: 3699: 3698: 3693: 3688: 3683: 3678: 3673: 3668: 3663: 3661:Poland anomaly 3658: 3653: 3648: 3646:Mongolian spot 3643: 3638: 3633: 3628: 3623: 3618: 3613: 3608: 3603: 3598: 3593: 3587: 3586: 3581: 3579:Branchial cyst 3576: 3571: 3565: 3563: 3559: 3558: 3556: 3555: 3554: 3553: 3543: 3537: 3535: 3529: 3528: 3526: 3525: 3520: 3515: 3510: 3505: 3499: 3497: 3490: 3484: 3483: 3480: 3479: 3453: 3452: 3447: 3446: 3445: 3436: 3424: 3423: 3422: 3409: 3407: 3403: 3402: 3399: 3398: 3396: 3395: 3390: 3385: 3380: 3375: 3370: 3364: 3363: 3361:Lelis syndrome 3358: 3353: 3352: 3351: 3339: 3334: 3332:Meleda disease 3328: 3326: 3322: 3321: 3319: 3318: 3317: 3316: 3307: 3295: 3294: 3293: 3281: 3274: 3273: 3268: 3263: 3258: 3253: 3248: 3243: 3233: 3232: 3231: 3230: 3225: 3220: 3219: 3218: 3213: 3203: 3198: 3193: 3184: 3182:Camisa disease 3179: 3178: 3177: 3172: 3160: 3155: 3150: 3149: 3148: 3146:Naxos syndrome 3136: 3131: 3130: 3129: 3124: 3119: 3103: 3102: 3100:Meleda disease 3097: 3092: 3087: 3077: 3075: 3066: 3059:Hyperkeratosis 3055: 3054: 3052: 3051: 3046: 3041: 3036: 3026: 3020: 3018: 3008: 3007: 3005: 3004: 2995: 2990: 2985: 2980: 2975: 2965: 2963: 2957: 2956: 2954: 2953: 2948: 2943: 2938: 2931: 2930: 2929: 2928: 2923: 2912: 2911: 2910: 2909: 2904: 2899: 2894: 2883: 2882: 2881: 2880: 2875: 2870: 2865: 2860: 2855: 2844: 2842: 2834: 2833: 2830: 2829: 2827: 2826: 2821: 2816: 2811: 2806: 2801: 2796: 2790: 2788: 2784: 2783: 2781: 2780: 2774: 2772: 2766: 2765: 2763: 2762: 2757: 2755:CHIME syndrome 2752: 2746: 2745: 2744: 2743: 2733: 2723: 2721: 2715: 2714: 2712: 2711: 2705: 2703: 2694: 2680: 2678:Genodermatosis 2674: 2673: 2660: 2658: 2657: 2650: 2643: 2635: 2626: 2625: 2622: 2621: 2619: 2618: 2613: 2607: 2605: 2595: 2594: 2592: 2591: 2586: 2580: 2578: 2565: 2561: 2560: 2557: 2556: 2554: 2553: 2547: 2545: 2539: 2538: 2536: 2535: 2530: 2525: 2520: 2515: 2510: 2504: 2502: 2494: 2493: 2491: 2490: 2485: 2480: 2474: 2472: 2459: 2453: 2452: 2449: 2448: 2445: 2444: 2442: 2441: 2436: 2431: 2426: 2421: 2416: 2410: 2408: 2398: 2397: 2395: 2394: 2388: 2386: 2376: 2375: 2373: 2372: 2366: 2364: 2351: 2345: 2344: 2341: 2340: 2338: 2337: 2331: 2330: 2324: 2322: 2309: 2300: 2294: 2293: 2290: 2289: 2286: 2285: 2283: 2282: 2272: 2270: 2260: 2259: 2257: 2256: 2250: 2248: 2235: 2229: 2228: 2225: 2224: 2222: 2221: 2215: 2213: 2200: 2194: 2193: 2190: 2189: 2187: 2186: 2180: 2178: 2174: 2173: 2170: 2169: 2167: 2166: 2161: 2155: 2153: 2143: 2142: 2140: 2139: 2133: 2131: 2118: 2112: 2111: 2108: 2107: 2105: 2104: 2099: 2093: 2091: 2081: 2080: 2078: 2077: 2072: 2066: 2064: 2051: 2045: 2044: 2041: 2040: 2038: 2037: 2031: 2029: 2019: 2018: 2016: 2015: 2010: 2005: 2000: 1995: 1989: 1987: 1974: 1965: 1956: 1944: 1943: 1934: 1932: 1931: 1924: 1917: 1909: 1903: 1902: 1884:(6): 328–338. 1871: 1868: 1866: 1865: 1846: 1819: 1790:(455): 323–4. 1770: 1755: 1705: 1651: 1607: 1580:(6): 437–447. 1564: 1515: 1497: 1453: 1412: 1391: 1364:(2): 313–319. 1347: 1312: 1308:"Mastocytosis" 1299: 1250: 1206: 1157: 1100: 1053: 1022:(45): 7005–8. 1002: 963:(10): e26375. 939: 879: 854: 812: 810: 807: 806: 805: 798: 795: 783: 780: 771:mastocytosis. 757: 754: 750:orphan disease 745: 742: 736: 733: 724: 721: 714: 713: 712: 711: 705: 704:- 60% respond. 699: 693: 687: 675: 669: 657: 654: 639: 638: 635: 629: 623: 613: 607: 593: 587: 583: 579: 576:Antihistamines 571: 568: 563: 560: 553: 552: 546: 543: 540: 537: 494: 491: 487: 486: 475: 471: 456: 453: 448: 447:Classification 445: 444: 443: 436: 430: 422: 419: 418: 417: 414: 411: 408: 403: 402:Minor criteria 400: 399: 398: 393: 390: 366: 363: 285: 282: 278: 277: 274: 268: 253: 250: 247: 244: 241:osteosclerosis 233: 224: 214: 204: 201: 198: 193: 184: 179: 173: 172:(skin writing) 155:Skin lesions ( 153: 133: 130: 97:disease, is a 86: 85: 72: 66: 65: 54: 53: 45: 44: 39: 35: 34: 26: 24: 14: 13: 10: 9: 6: 4: 3: 2: 3921: 3910: 3909:Rare diseases 3907: 3905: 3902: 3900: 3897: 3896: 3894: 3878: 3874: 3873: 3869: 3867: 3863: 3862: 3858: 3856: 3852: 3851: 3847: 3845: 3842: 3838: 3837: 3833: 3832: 3829: 3825: 3818: 3814: 3813: 3809: 3807: 3803: 3802: 3798: 3796: 3792: 3791: 3787: 3784: 3783: 3779: 3777: 3773: 3769: 3768: 3764: 3760: 3758: 3754: 3750: 3749: 3745: 3741: 3739: 3735: 3734: 3730: 3726: 3725: 3722: 3717: 3713: 3697: 3694: 3692: 3689: 3687: 3684: 3682: 3679: 3677: 3674: 3672: 3669: 3667: 3664: 3662: 3659: 3657: 3654: 3652: 3649: 3647: 3644: 3642: 3639: 3637: 3634: 3632: 3629: 3627: 3624: 3622: 3619: 3617: 3614: 3612: 3609: 3607: 3604: 3602: 3599: 3597: 3594: 3592: 3589: 3588: 3585: 3582: 3580: 3577: 3575: 3572: 3570: 3567: 3566: 3564: 3560: 3552: 3549: 3548: 3547: 3544: 3542: 3539: 3538: 3536: 3534: 3530: 3524: 3521: 3519: 3516: 3514: 3511: 3509: 3508:Encephalocele 3506: 3504: 3501: 3500: 3498: 3494: 3491: 3487:Developmental 3485: 3477: 3476: 3475: 3470: 3469: 3464: 3463: 3459: 3451: 3450:Hailey–Hailey 3448: 3444: 3440: 3437: 3435: 3432: 3431: 3430: 3429: 3428:immune system 3425: 3421: 3418: 3417: 3416: 3415: 3411: 3410: 3408: 3404: 3394: 3391: 3389: 3386: 3384: 3381: 3379: 3376: 3374: 3371: 3369: 3366: 3365: 3362: 3359: 3357: 3354: 3350: 3347: 3346: 3345: 3344: 3340: 3338: 3335: 3333: 3330: 3329: 3327: 3323: 3315: 3311: 3308: 3306: 3303: 3302: 3301: 3300: 3296: 3292: 3289: 3288: 3287: 3286: 3282: 3280: 3276: 3275: 3272: 3269: 3267: 3264: 3262: 3259: 3257: 3254: 3252: 3249: 3247: 3244: 3242: 3238: 3235: 3234: 3229: 3226: 3224: 3221: 3217: 3214: 3212: 3209: 3208: 3207: 3204: 3202: 3199: 3197: 3194: 3192: 3188: 3185: 3183: 3180: 3176: 3173: 3171: 3168: 3167: 3166: 3165: 3161: 3159: 3156: 3154: 3151: 3147: 3144: 3143: 3142: 3141: 3137: 3135: 3132: 3128: 3125: 3123: 3120: 3118: 3115: 3114: 3113: 3112: 3108: 3107: 3105: 3104: 3101: 3098: 3096: 3093: 3091: 3088: 3086: 3082: 3079: 3078: 3076: 3074: 3070: 3067: 3065: 3064:keratinopathy 3060: 3056: 3050: 3047: 3045: 3042: 3040: 3037: 3034: 3030: 3027: 3025: 3022: 3021: 3019: 3017: 3013: 3009: 3003: 2999: 2996: 2994: 2991: 2989: 2986: 2984: 2981: 2979: 2976: 2974: 2970: 2967: 2966: 2964: 2962: 2958: 2952: 2949: 2947: 2944: 2942: 2939: 2937: 2933: 2932: 2927: 2924: 2922: 2919: 2918: 2917: 2914: 2913: 2908: 2905: 2903: 2900: 2898: 2895: 2893: 2890: 2889: 2888: 2885: 2884: 2879: 2876: 2874: 2871: 2869: 2866: 2864: 2861: 2859: 2856: 2854: 2851: 2850: 2849: 2846: 2845: 2843: 2839: 2835: 2825: 2822: 2820: 2817: 2815: 2812: 2810: 2807: 2805: 2802: 2800: 2797: 2795: 2792: 2791: 2789: 2785: 2779: 2776: 2775: 2773: 2771: 2767: 2761: 2758: 2756: 2753: 2751: 2748: 2747: 2742: 2739: 2738: 2737: 2734: 2732: 2728: 2725: 2724: 2722: 2720: 2716: 2710: 2707: 2706: 2704: 2702: 2698: 2695: 2693: 2688: 2684: 2681: 2679: 2675: 2671: 2667: 2663: 2656: 2651: 2649: 2644: 2642: 2637: 2636: 2633: 2617: 2614: 2612: 2609: 2608: 2606: 2603: 2602: 2596: 2590: 2587: 2585: 2582: 2581: 2579: 2576: 2575: 2569: 2566: 2562: 2552: 2549: 2548: 2546: 2540: 2534: 2531: 2529: 2526: 2524: 2521: 2519: 2516: 2514: 2511: 2509: 2506: 2505: 2503: 2500: 2495: 2489: 2486: 2484: 2481: 2479: 2476: 2475: 2473: 2470: 2469: 2463: 2460: 2458: 2454: 2440: 2437: 2435: 2432: 2430: 2427: 2425: 2422: 2420: 2417: 2415: 2412: 2411: 2409: 2406: 2405: 2399: 2393: 2390: 2389: 2387: 2384: 2383: 2377: 2371: 2368: 2367: 2365: 2362: 2361: 2355: 2352: 2350: 2346: 2336: 2333: 2332: 2329: 2326: 2325: 2323: 2320: 2319: 2313: 2310: 2308: 2304: 2301: 2299: 2295: 2281: 2277: 2274: 2273: 2271: 2268: 2267: 2261: 2255: 2252: 2251: 2249: 2246: 2245: 2239: 2236: 2234: 2230: 2220: 2217: 2216: 2214: 2211: 2210: 2204: 2201: 2199: 2195: 2185: 2184:Histiocytosis 2182: 2181: 2179: 2175: 2165: 2162: 2160: 2157: 2156: 2154: 2151: 2150: 2144: 2138: 2135: 2134: 2132: 2129: 2128: 2122: 2119: 2117: 2116:Myelomonocyte 2113: 2103: 2100: 2098: 2095: 2094: 2092: 2089: 2088: 2082: 2076: 2073: 2071: 2068: 2067: 2065: 2062: 2061: 2055: 2052: 2050: 2046: 2036: 2033: 2032: 2030: 2027: 2026: 2020: 2014: 2011: 2009: 2006: 2004: 2001: 1999: 1996: 1994: 1991: 1990: 1988: 1985: 1984: 1978: 1975: 1973: 1969: 1966: 1964: 1960: 1957: 1955: 1949: 1945: 1941: 1937: 1930: 1925: 1923: 1918: 1916: 1911: 1910: 1907: 1899: 1895: 1891: 1887: 1883: 1879: 1874: 1873: 1869: 1861: 1857: 1850: 1847: 1842: 1838: 1834: 1830: 1823: 1820: 1815: 1811: 1806: 1801: 1797: 1793: 1789: 1785: 1781: 1774: 1771: 1767: 1766: 1759: 1756: 1751: 1747: 1743: 1739: 1734: 1729: 1725: 1721: 1717: 1709: 1706: 1701: 1697: 1692: 1687: 1682: 1677: 1673: 1669: 1665: 1658: 1656: 1652: 1647: 1643: 1638: 1633: 1629: 1625: 1621: 1614: 1612: 1608: 1603: 1599: 1595: 1591: 1587: 1583: 1579: 1575: 1568: 1565: 1560: 1556: 1551: 1546: 1542: 1538: 1534: 1530: 1526: 1519: 1516: 1511: 1507: 1501: 1498: 1493: 1489: 1485: 1481: 1477: 1473: 1469: 1465: 1457: 1454: 1449: 1445: 1440: 1435: 1431: 1427: 1423: 1416: 1413: 1409: 1408:0-7216-2921-0 1405: 1401: 1395: 1392: 1387: 1383: 1379: 1375: 1371: 1367: 1363: 1359: 1351: 1348: 1343: 1339: 1335: 1331: 1327: 1323: 1322:Dermatol Surg 1316: 1313: 1309: 1303: 1300: 1295: 1291: 1286: 1281: 1277: 1273: 1269: 1265: 1261: 1254: 1251: 1246: 1242: 1237: 1232: 1228: 1224: 1220: 1213: 1211: 1207: 1202: 1198: 1193: 1188: 1184: 1180: 1176: 1172: 1168: 1161: 1158: 1153: 1149: 1145: 1141: 1137: 1133: 1128: 1123: 1119: 1115: 1111: 1104: 1101: 1096: 1092: 1088: 1084: 1080: 1076: 1072: 1068: 1064: 1057: 1054: 1049: 1045: 1040: 1035: 1030: 1025: 1021: 1017: 1013: 1006: 1003: 998: 994: 989: 984: 979: 974: 970: 966: 962: 958: 954: 946: 944: 940: 935: 931: 926: 921: 916: 911: 907: 903: 899: 895: 891: 883: 880: 869:on 2013-12-28 868: 864: 858: 855: 850: 846: 841: 836: 833:(2): 121–32. 832: 828: 824: 817: 814: 808: 804: 801: 800: 796: 794: 792: 788: 781: 779: 776: 772: 768: 766: 762: 755: 753: 751: 743: 741: 734: 732: 730: 722: 720: 718: 709: 706: 703: 700: 697: 694: 691: 688: 685: 682: 681: 679: 676: 673: 670: 667: 663: 662: 661: 655: 653: 651: 647: 643: 636: 633: 630: 627: 624: 621: 617: 614: 611: 608: 605: 601: 597: 594: 591: 588: 577: 574: 573: 569: 567: 561: 558: 557:Bezuclastinib 550: 547: 544: 541: 538: 535: 534: 533: 530: 528: 527:enterocolitis 524: 520: 516: 507: 503: 499: 492: 490: 484: 480: 476: 472: 469: 468: 463: 459: 458: 454: 452: 446: 441: 437: 434: 431: 428: 427: 426: 420: 415: 412: 409: 406: 405: 401: 396: 395: 391: 389: 387: 382: 378: 376: 372: 369:Diagnosis of 364: 362: 360: 356: 352: 348: 344: 340: 336: 331: 329: 325: 321: 320:blood vessels 317: 313: 308: 306: 302: 298: 294: 290: 283: 281: 275: 272: 271:Malabsorption 269: 266: 265:parietal cell 262: 258: 257:peptic ulcers 254: 251: 248: 245: 242: 238: 234: 232: 228: 225: 223: 219: 215: 212: 208: 205: 202: 199: 197: 194: 192: 188: 185: 183: 180: 177: 174: 171: 170: 164: 160: 159: 154: 151: 150: 149: 147: 146:degranulation 138: 131: 129: 127: 123: 119: 115: 110: 108: 104: 100: 99:rare disorder 96: 92: 84: 80: 76: 73: 71: 67: 63: 62:H&E stain 59: 55: 51: 46: 43: 40: 36: 31: 19: 3870: 3866:mastocytosis 3859: 3855:mastocytosis 3848: 3834: 3810: 3799: 3788: 3780: 3761: 3742: 3727: 3513:Nasal glioma 3503:Dermoid cyst 3472: 3466: 3455: 3454: 3439:Mastocytosis 3438: 3426: 3420:EEM syndrome 3412: 3341: 3297: 3283: 3162: 3138: 3109: 2670:skin disease 2599: 2572: 2499:Mastocytosis 2498: 2487: 2466: 2402: 2380: 2358: 2316: 2264: 2242: 2207: 2147: 2125: 2085: 2058: 2023: 1981: 1954:granulocytes 1881: 1877: 1859: 1855: 1849: 1832: 1828: 1822: 1787: 1783: 1773: 1763: 1758: 1723: 1719: 1708: 1671: 1667: 1627: 1623: 1577: 1573: 1567: 1532: 1528: 1518: 1509: 1500: 1467: 1463: 1456: 1429: 1425: 1415: 1399: 1394: 1361: 1357: 1350: 1325: 1321: 1315: 1302: 1267: 1263: 1253: 1226: 1222: 1174: 1170: 1160: 1120:(1): 12–30. 1117: 1113: 1103: 1070: 1066: 1056: 1019: 1015: 1005: 960: 956: 900:(5): e2266. 897: 893: 882: 871:. Retrieved 867:the original 857: 830: 827:Pathobiology 826: 816: 790: 786: 785: 777: 773: 769: 759: 747: 744:Epidemiology 738: 726: 715: 659: 640: 565: 531: 511: 488: 483:homme orange 482: 465: 461: 450: 424: 385: 383: 379: 370: 368: 358: 354: 350: 338: 332: 327: 324:angiogenesis 309: 287: 279: 237:osteoporosis 216:Episodes of 168: 156: 143: 140:Mastocytosis 111: 102: 93:, a type of 91:Mastocytosis 90: 89: 41: 33:Mastocytosis 3676:Skin dimple 3285:desmoplakin 3277:ungrouped: 3164:Cathepsin C 3140:plakoglobin 2841:and related 2468:Mastocytoma 1328:(1): 33–7. 1136:10261/62846 803:Mastocytoma 708:Avapritinib 702:Midostaurin 684:Midostaurin 650:mirtazapine 620:anaphylaxis 616:Epinephrine 590:Leukotriene 523:lymph nodes 231:muscle pain 207:Anaphylaxis 38:Other names 3893:Categories 3812:DiseasesDB 3106:syndromic 3029:Cutis laxa 3016:Connective 2666:integument 2662:Congenital 1952:and other 1510:Cancer.Net 873:2017-11-02 809:References 672:Cladribine 666:interferon 626:Salbutamol 479:integument 289:Mast cells 249:Depression 103:mastocytes 83:hematology 58:Micrograph 3836:eMedicine 3696:Birthmark 3489:anomalies 3456:see also 2934:related: 2787:Ungrouped 1972:Myelocyte 1938:-related 1862:: 359–61. 1841:840287852 1674:: 77–92. 1484:1543-2165 735:Prognosis 696:Masitinib 604:Ketotifen 562:Treatment 365:Diagnosis 316:histamine 301:histamine 222:faintness 169:urticaria 126:histamine 95:mast cell 70:Specialty 3872:Orphanet 3844:med/1401 3841:derm/258 3785:: 9741/3 3414:cadherin 3299:connexin 3237:punctate 3111:connexin 2457:CFU-Mast 2198:CFU-Baso 2049:Monocyte 1814:20745623 1784:Br Med J 1750:24088671 1742:27428296 1700:31372066 1646:27762455 1602:37335288 1559:24044484 1492:25611105 1448:21301631 1386:30459720 1378:12688351 1294:21035176 1245:27069254 1201:28254862 1152:12120327 1144:17555444 1095:52298220 1087:30230078 1048:19058339 997:22031830 957:PLOS ONE 934:18509466 894:PLOS ONE 849:17587883 797:See also 756:Research 690:Imatinib 347:receptor 246:Headache 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Index

Mastocytosis, systemic

Micrograph
H&E stain
Specialty
Clinical Immunology and Allergy
Oncology
hematology
mast cell
rare disorder
CD34
itching
hives
anaphylactic shock
histamine

degranulation
urticaria pigmentosa
itching
dermatographic urticaria
"Darier's Sign"
Abdominal discomfort
Nausea
vomiting
Diarrhea
Anaphylaxis
shock
very low blood pressure
faintness
Bone

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