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Mastocytosis

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487: 39: 518:. 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. 126: 137:, 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 369:
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)
459:(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 3446: 2634: 269:
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
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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:
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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).
942:"Depression in Patients with Mastocytosis: Prevalence, Features and Effects of Masitinib Therapy" 335: 3897: 3854: 3674: 3639: 3562: 3356: 3344: 3194: 3163: 3037: 2990: 2966: 2562: 2348: 2232: 2197: 2115: 2048: 1971: 1609:"Systemic mastocytosis in adults: 2017 update on diagnosis, risk stratification and management" 752:
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).
681:. Can have effect in rare cases without mutation in KIT(D816V) 3521: 2337: 1842: 1653:"Novel approaches to treating advanced systemic mastocytosis" 1459:(2). Archives of Pathology and Laboratory Medicine: 225–232. 503: 453:(UP), more common in children, although also seen in adults. 331: 300: 262:(due to inactivation of pancreatic enzymes by increased acid) 106: 2507:
Adult type of generalized eruption of cutaneous mastocytosis
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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).
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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
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Andrews' Diseases of the Skin: Clinical Dermatology
1246: 224:Decreased bone density or increased bone density ( 3079:Diffuse nonepidermolytic palmoplantar keratoderma 809: 3879: 2803:Nonbullous congenital ichthyosiform erythroderma 2428:Refractory cytopenia with multilineage dysplasia 1657:Clinical Pharmacology: Advances and Applications 1295:National Organization for Rare Disorders (NORD) 3268:Palmoplantar keratoderma and spastic paraplegia 1646: 1644: 1602: 1600: 1453:Archives of Pathology & Laboratory Medicine 521:There are five types of systemic mastocytosis: 3074:Diffuse epidermolytic palmoplantar keratoderma 2091:Accelerated phase chronic myelogenous leukemia 1253:The Journal of Allergy and Clinical Immunology 1201: 1199: 934: 932: 575:blockers may be helpful, often in combination. 443: 440:Mastocytosis can occur in a variety of forms: 399:Aberrant mast cell morphology (spindle-shaped) 292:, mast cells attract other key players of the 3605:Congenital malformations of the dermatoglyphs 2635: 1909: 1766: 1511: 854:. Mastocytosis Society Canada. Archived from 481: 1867:The American Journal of the Medical Sciences 1824:] (in German). Verlag von Leopold Voss. 1641: 1597: 296:to areas of the body where they are needed. 1196: 1147: 1090: 929: 803: 409: 3630:Melanotic neuroectodermal tumor of infancy 3367:Keratosis follicularis spinulosa decalvans 2798:Ichthyosis–sclerosing cholangitis syndrome 2642: 2628: 1916: 1902: 687:. Is being tested in trials. Not approved. 456:Telangiectasia macularis eruptiva perstans 414:Other types of mast cell disease include: 377:three minor criteria has to be fulfilled: 244:Increased stomach acid production causing 37: 3590:Congenital cartilaginous rest of the neck 3185:Focal palmoplantar and gingival keratosis 1864: 1792: 1720: 1678: 1668: 1624: 1606: 1537: 1426: 1272: 1223: 1179: 1114: 1026: 1016: 975: 965: 912: 902: 827: 3655:Rapidly involuting congenital hemangioma 3245:Keratosis punctata of the palmar creases 3240:Keratosis punctata palmaris et plantaris 3111:Clouston's hidrotic ectodermal dysplasia 1815: 1411:"Mast Cells in Gastrointestinal Disease" 1308: 852:"Mastocytosis & mast cell disorders" 644: 558: 485: 124: 2408:Refractory anemia with excess of blasts 1381: 528:Smouldering systemic mastocytosis (SSM) 3880: 1247:Akin C, Valent P, Metcalfe DD (2010). 534:Aggressive systemic mastocytosis (ASM) 2716:Congenital ichthyosiform erythroderma 2623: 1897: 810:Horny HP, Sotlar K, Valent P (2007). 370:blood using sensitive PCR-technology 120: 3377:Keratosis pilaris atrophicans faciei 2962:Dermatopathia pigmentosa reticularis 2573:Acute panmyelosis with myelofibrosis 1607:Pardanani, Animesh (November 2016). 706:Allogeneic stem cell transplantation 252:and direct histamine stimulation on 2423:Paroxysmal nocturnal hemoglobinuria 13: 3670:Superficial lymphatic malformation 3615:Congenital smooth muscle hamartoma 3084:Palmoplantar keratoderma of Sybert 2653:malformations and deformations of 2194: 1858: 1495:"Mastocytosis - Treatment Options" 380: 366:) may help confirm the diagnosis. 272: 14: 3909: 3142:Scleroatrophic syndrome of Huriez 2972:Hypohidrotic ectodermal dysplasia 1518:Expert Opinion on Pharmacotherapy 1415:Gastroenterology & Hepatology 1005:World Journal of Gastroenterology 435: 402:Finding of mutation in KIT(D816V) 390: 3255:Porokeratosis plantaris discreta 3212:Striate palmoplantar keratoderma 2148:Juvenile myelomonocytic leukemia 1879:10.1097/00000441-199506000-00007 1816:Unna, Paul Gerson; Unna (1887). 1575:10.1097/00006842-198607000-00006 1116:10.1111/j.1365-2141.2007.06619.x 812:"Mastocytosis: state of the art" 3888:Dermal and subcutaneous growths 3680:Verrucous vascular malformation 3610:Congenital preauricular fistula 3580:Accessory nail of the fifth toe 3457:Template:Pigmentation disorders 3250:Schöpf–Schulz–Passarge syndrome 3230:Acrokeratoelastoidosis of Costa 2935:Laryngoonychocutaneous syndrome 2808:Ichthyosis linearis circumflexa 2793:Ichthyosis prematurity syndrome 2413:Chromosome 5q deletion syndrome 2324:Acute megakaryoblastic leukemia 2153:Chronic myelomonocytic leukemia 2064:Myeloid dendritic cell leukemia 1836: 1809: 1760: 1745: 1695: 1554: 1505: 1487: 1402: 1337: 1302: 1289: 732: 202:from allergic or immune causes) 64:Clinical Immunology and Allergy 3294:Erythrokeratodermia variabilis 3205:Pachyonychia congenita type II 2497:Diffuse cutaneous mastocytosis 1709:American Journal of Hematology 1613:American Journal of Hematology 1043: 992: 869: 844: 49:of mastocytosis. Skin biopsy. 1: 3620:Cystic lymphatic malformation 3573:Cavernous venous malformation 3200:Pachyonychia congenita type I 3123:Corneodermatoosseous syndrome 2783:Ichthyosis bullosa of Siemens 2265:Chronic eosinophilic leukemia 2024:Chronic neutrophilic leukemia 1172:10.1158/0008-5472.CAN-16-2234 797: 754:National Institutes of Health 422:Mast cell activation syndrome 3028:Popliteal pterygium syndrome 2720:Epidermolytic hyperkeratosis 2605:Biphenotypic acute leukaemia 2540:Xanthelasmoidal mastocytosis 1767:Nettleship E, Tay W (1869). 1530:10.1517/14656566.2013.824424 1323:10.1016/1076-0512(95)00388-6 1225:10.1182/blood-2016-03-643544 967:10.1371/journal.pone.0026375 904:10.1371/journal.pone.0002266 723: 550: 405:S-tryptase >20 ng/ml 353: 192:Ear/nose/throat inflammation 7: 3660:Rosenthal–Kloepfer syndrome 3645:Omphalomesenteric duct cyst 3362:Keratolytic winter erythema 3022:Gerodermia osteodysplastica 1982:Acute myeloblastic leukemia 1359:10.1080/1042819021000037967 1056:Journal of Clinical Nursing 785: 744: 444:Cutaneous mastocytosis (CM) 113:, caused by the release of 31:Clonal bone marrow disorder 10: 3914: 3235:Focal acral hyperkeratosis 2982:Ellis–van Creveld syndrome 2502:Erythrodermic mastocytosis 2269:Hypereosinophilic syndrome 1265:10.1016/j.jaci.2010.08.035 770: 667:Tyrosine kinase inhibitors 543: 482:Systemic mastocytosis (SM) 342:). In laboratory studies, 248:(increased stimulation of 3815: 3703: 3550: 3520: 3484: 3475: 3394: 3313: 3159:Papillon–Lefèvre syndrome 3060: 3046: 2999: 2948: 2825: 2775: 2757: 2730:Harlequin-type ichthyosis 2706: 2688: 2665: 2586: 2559: 2552: 2530: 2485: 2453: 2444: 2389: 2367: 2345: 2336: 2317:Essential thrombocythemia 2303: 2294: 2285: 2251: 2229: 2220: 2185: 2165: 2134: 2112: 2103: 2072: 2045: 2036: 2010: 1968: 1959: 1950: 1935: 1465:10.5858/arpa.2013-0594-oa 57: 45: 36: 26: 21: 3033:Pseudoxanthoma elasticum 2749:Sjögren–Larsson syndrome 1929:hematological malignancy 1391:. (10th ed.). Saunders. 711: 410:Other mast cell diseases 133:When mast cells undergo 98:+ mast cell precursors. 3893:Immune system disorders 3558:Aplasia cutis congenita 2977:Focal dermal hypoplasia 2940:Skin fragility syndrome 2788:Ichthyosis follicularis 2086:Philadelphia chromosome 1347:Leukemia & Lymphoma 756:(NIH) Clinical Center. 207:very low blood pressure 3675:Thyroglossal duct cyst 3640:Nasolacrimal duct cyst 3563:Amniotic band syndrome 3357:Dyskeratosis congenita 3345:Dyskeratosis congenita 3195:Pachyonychia congenita 3106:Bart–Pumphrey syndrome 3038:Van der Woude syndrome 3013:Ehlers–Danlos syndrome 1757:, Stat, March 16, 2020 1563:Psychosomatic Medicine 599:Proton-pump inhibitors 498: 338:for stem cell factor ( 209:(including shock) and 130: 3540:Nevus flammeus nuchae 3451:Template:Phakomatoses 3423:Hereditary lymphedema 2987:Rapp–Hodgkin syndrome 2676:Congenital ichthyosis 2600:Primary myelofibrosis 2532:Systemic mastocytosis 2477:Systemic mastocytosis 1785:10.1136/bmj.2.455.323 645:Cytoreductive therapy 585:Mast cell stabilizers 559:Anti-mediator therapy 489: 324:cell surface receptor 322:Mast cells express a 294:immune defense system 250:enterochromaffin cell 189:Olfactive intolerance 128: 3530:Capillary hemangioma 3432:Urticaria pigmentosa 3190:Howel–Evans syndrome 2950:Ectodermal dysplasia 2891:Generalized atrophic 2522:Solitary mastocytoma 2512:Urticaria pigmentosa 2418:Sideroblastic anemia 1670:10.2147/CPAA.S206615 780:urticaria pigmentosa 776:Urticaria pigmentosa 491:Immunohistochemistry 451:urticaria pigmentosa 360:urticaria pigmentosa 171:Abdominal discomfort 147:urticaria pigmentosa 3217:Tyrosinemia type II 2767:X-linked ichthyosis 2725:Lamellar ichthyosis 2698:Ichthyosis vulgaris 2681:erythrokeratodermia 1018:10.3748/wjg.14.7005 958:2011PLoSO...626375M 895:2008PLoSO...3.2266H 3816:External resources 3600:Congenital lip pit 3164:Haim–Munk syndrome 3116:Vohwinkel syndrome 2991:Hay–Wells syndrome 2967:Hay–Wells syndrome 2813:Ichthyosis hystrix 2739:Netherton syndrome 2467:Mast cell leukemia 2359:Erythroleukemia/M6 2243:Acute eosinophilic 1068:10.1111/jocn.14676 1062:(7–8): 1114–1124. 538:Mast cell leukemia 499: 265:Hepatosplenomegaly 131: 121:Signs and symptoms 111:anaphylactic shock 3875: 3874: 3697: 3696: 3693: 3692: 3625:Median raphe cyst 3585:Bronchogenic cyst 3507:PHACE association 3471: 3470: 3390: 3389: 3382:Keratosis pilaris 3326:Keratosis pilaris 3280:Carvajal syndrome 3260:Spiny keratoderma 2925:Costello syndrome 2821: 2820: 2617: 2616: 2613: 2612: 2548: 2547: 2517:Mast cell sarcoma 2472:Mast cell sarcoma 2440: 2439: 2436: 2435: 2403:Refractory anemia 2381:Polycythemia vera 2332: 2331: 2281: 2280: 2277: 2276: 2216: 2215: 2181: 2180: 2161: 2160: 2099: 2098: 2032: 2031: 1722:10.1002/ajh.24490 1715:(11): 1069–1075. 1626:10.1002/ajh.24553 1619:(11): 1146–1159. 1524:(15): 2033–2045. 1218:(20): 2391–2405. 829:10.1159/000101711 718:ultraviolet light 429:mast cell sarcoma 282:connective tissue 241:Ocular discomfort 77: 76: 16:Medical condition 3905: 3701: 3700: 3512:Sinus pericranii 3482: 3481: 3338:Darier's disease 3147:Olmsted syndrome 3058: 3057: 2958:Naegeli syndrome 2930:Kindler syndrome 2686: 2685: 2672: 2671: 2644: 2637: 2630: 2621: 2620: 2593: 2566: 2557: 2556: 2553:Multiple/unknown 2533: 2490: 2460: 2451: 2450: 2396: 2374: 2352: 2343: 2342: 2310: 2301: 2300: 2292: 2291: 2258: 2236: 2227: 2226: 2208:Acute basophilic 2201: 2192: 2191: 2141: 2119: 2110: 2109: 2079: 2052: 2043: 2042: 2017: 1975: 1966: 1965: 1957: 1956: 1948: 1947: 1918: 1911: 1904: 1895: 1894: 1890: 1853: 1852: 1840: 1834: 1833: 1813: 1807: 1806: 1796: 1764: 1758: 1749: 1743: 1742: 1724: 1699: 1693: 1692: 1682: 1672: 1648: 1639: 1638: 1628: 1604: 1595: 1594: 1558: 1552: 1551: 1541: 1509: 1503: 1502: 1491: 1485: 1484: 1447: 1441: 1440: 1430: 1406: 1400: 1385: 1379: 1378: 1341: 1335: 1334: 1306: 1300: 1293: 1287: 1286: 1276: 1244: 1238: 1237: 1227: 1203: 1194: 1193: 1183: 1166:(6): 1261–1270. 1151: 1145: 1144: 1118: 1094: 1088: 1087: 1047: 1041: 1040: 1030: 1020: 996: 990: 989: 979: 969: 936: 927: 926: 916: 906: 873: 867: 866: 864: 863: 848: 842: 841: 831: 807: 589:Cromoglicic acid 334:), which is the 284:, including the 41: 19: 18: 3913: 3912: 3908: 3907: 3906: 3904: 3903: 3902: 3878: 3877: 3876: 3871: 3870: 3811: 3810: 3712: 3698: 3689: 3551:Other/ungrouped 3546: 3535:Port-wine stain 3516: 3477: 3467: 3386: 3309: 3051: 3042: 2995: 2944: 2829: 2817: 2771: 2753: 2702: 2679: 2661: 2648: 2618: 2609: 2587: 2582: 2578:Myeloid sarcoma 2560: 2544: 2531: 2526: 2486: 2481: 2454: 2432: 2390: 2385: 2368: 2363: 2346: 2328: 2304: 2273: 2252: 2247: 2230: 2212: 2195: 2177: 2157: 2135: 2130: 2113: 2095: 2073: 2068: 2046: 2028: 2011: 2006: 1969: 1940: 1931: 1922: 1861: 1859:Further reading 1856: 1841: 1837: 1814: 1810: 1765: 1761: 1751:Garde, Damian, 1750: 1746: 1700: 1696: 1649: 1642: 1605: 1598: 1559: 1555: 1510: 1506: 1501:. 25 June 2012. 1493: 1492: 1488: 1448: 1444: 1421:(12): 772–777. 1407: 1403: 1386: 1382: 1342: 1338: 1307: 1303: 1294: 1290: 1259:(6): 1099–104. 1245: 1241: 1204: 1197: 1160:Cancer Research 1152: 1148: 1103:Br. J. Haematol 1095: 1091: 1048: 1044: 997: 993: 937: 930: 874: 870: 861: 859: 850: 849: 845: 808: 804: 800: 788: 773: 747: 735: 726: 716:Treatment with 714: 647: 631:Antidepressants 621:Corticosteroids 574: 570: 561: 553: 548: 484: 446: 438: 412: 393: 383: 381:Major criterion 356: 315:). No one with 280:are located in 275: 273:Pathophysiology 165:"Darier's Sign" 156:dermatographic 123: 17: 12: 11: 5: 3911: 3901: 3900: 3895: 3890: 3873: 3872: 3869: 3868: 3857: 3846: 3835: 3820: 3819: 3817: 3813: 3812: 3809: 3808: 3797: 3786: 3775: 3767: 3748: 3729: 3713: 3708: 3707: 3705: 3704:Classification 3695: 3694: 3691: 3690: 3688: 3687: 3682: 3677: 3672: 3667: 3662: 3657: 3652: 3650:Poland anomaly 3647: 3642: 3637: 3635:Mongolian spot 3632: 3627: 3622: 3617: 3612: 3607: 3602: 3597: 3592: 3587: 3582: 3576: 3575: 3570: 3568:Branchial cyst 3565: 3560: 3554: 3552: 3548: 3547: 3545: 3544: 3543: 3542: 3532: 3526: 3524: 3518: 3517: 3515: 3514: 3509: 3504: 3499: 3494: 3488: 3486: 3479: 3473: 3472: 3469: 3468: 3442: 3441: 3436: 3435: 3434: 3425: 3413: 3412: 3411: 3398: 3396: 3392: 3391: 3388: 3387: 3385: 3384: 3379: 3374: 3369: 3364: 3359: 3353: 3352: 3350:Lelis syndrome 3347: 3342: 3341: 3340: 3328: 3323: 3321:Meleda disease 3317: 3315: 3311: 3310: 3308: 3307: 3306: 3305: 3296: 3284: 3283: 3282: 3270: 3263: 3262: 3257: 3252: 3247: 3242: 3237: 3232: 3222: 3221: 3220: 3219: 3214: 3209: 3208: 3207: 3202: 3192: 3187: 3182: 3173: 3171:Camisa disease 3168: 3167: 3166: 3161: 3149: 3144: 3139: 3138: 3137: 3135:Naxos syndrome 3125: 3120: 3119: 3118: 3113: 3108: 3092: 3091: 3089:Meleda disease 3086: 3081: 3076: 3066: 3064: 3055: 3048:Hyperkeratosis 3044: 3043: 3041: 3040: 3035: 3030: 3025: 3015: 3009: 3007: 2997: 2996: 2994: 2993: 2984: 2979: 2974: 2969: 2964: 2954: 2952: 2946: 2945: 2943: 2942: 2937: 2932: 2927: 2920: 2919: 2918: 2917: 2912: 2901: 2900: 2899: 2898: 2893: 2888: 2883: 2872: 2871: 2870: 2869: 2864: 2859: 2854: 2849: 2844: 2833: 2831: 2823: 2822: 2819: 2818: 2816: 2815: 2810: 2805: 2800: 2795: 2790: 2785: 2779: 2777: 2773: 2772: 2770: 2769: 2763: 2761: 2755: 2754: 2752: 2751: 2746: 2744:CHIME syndrome 2741: 2735: 2734: 2733: 2732: 2722: 2712: 2710: 2704: 2703: 2701: 2700: 2694: 2692: 2683: 2669: 2667:Genodermatosis 2663: 2662: 2647: 2646: 2639: 2632: 2624: 2615: 2614: 2611: 2610: 2608: 2607: 2602: 2596: 2594: 2584: 2583: 2581: 2580: 2575: 2569: 2567: 2554: 2550: 2549: 2546: 2545: 2543: 2542: 2536: 2534: 2528: 2527: 2525: 2524: 2519: 2514: 2509: 2504: 2499: 2493: 2491: 2483: 2482: 2480: 2479: 2474: 2469: 2463: 2461: 2448: 2442: 2441: 2438: 2437: 2434: 2433: 2431: 2430: 2425: 2420: 2415: 2410: 2405: 2399: 2397: 2387: 2386: 2384: 2383: 2377: 2375: 2365: 2364: 2362: 2361: 2355: 2353: 2340: 2334: 2333: 2330: 2329: 2327: 2326: 2320: 2319: 2313: 2311: 2298: 2289: 2283: 2282: 2279: 2278: 2275: 2274: 2272: 2271: 2261: 2259: 2249: 2248: 2246: 2245: 2239: 2237: 2224: 2218: 2217: 2214: 2213: 2211: 2210: 2204: 2202: 2189: 2183: 2182: 2179: 2178: 2176: 2175: 2169: 2167: 2163: 2162: 2159: 2158: 2156: 2155: 2150: 2144: 2142: 2132: 2131: 2129: 2128: 2122: 2120: 2107: 2101: 2100: 2097: 2096: 2094: 2093: 2088: 2082: 2080: 2070: 2069: 2067: 2066: 2061: 2055: 2053: 2040: 2034: 2033: 2030: 2029: 2027: 2026: 2020: 2018: 2008: 2007: 2005: 2004: 1999: 1994: 1989: 1984: 1978: 1976: 1963: 1954: 1945: 1933: 1932: 1921: 1920: 1913: 1906: 1898: 1892: 1891: 1873:(6): 328–338. 1860: 1857: 1855: 1854: 1835: 1808: 1779:(455): 323–4. 1759: 1744: 1694: 1640: 1596: 1569:(6): 437–447. 1553: 1504: 1486: 1442: 1401: 1380: 1353:(2): 313–319. 1336: 1301: 1297:"Mastocytosis" 1288: 1239: 1195: 1146: 1089: 1042: 1011:(45): 7005–8. 991: 952:(10): e26375. 928: 868: 843: 801: 799: 796: 795: 794: 787: 784: 772: 769: 760:mastocytosis. 746: 743: 739:orphan disease 734: 731: 725: 722: 713: 710: 703: 702: 701: 700: 694: 693:- 60% respond. 688: 682: 676: 664: 658: 646: 643: 628: 627: 624: 618: 612: 602: 596: 582: 576: 572: 568: 565:Antihistamines 560: 557: 552: 549: 542: 541: 535: 532: 529: 526: 483: 480: 476: 475: 464: 460: 445: 442: 437: 436:Classification 434: 433: 432: 425: 419: 411: 408: 407: 406: 403: 400: 397: 392: 391:Minor criteria 389: 388: 387: 382: 379: 355: 352: 274: 271: 267: 266: 263: 257: 242: 239: 236: 233: 230:osteosclerosis 222: 213: 203: 193: 190: 187: 182: 173: 168: 162: 161:(skin writing) 144:Skin lesions ( 142: 122: 119: 86:disease, is a 75: 74: 61: 55: 54: 43: 42: 34: 33: 28: 24: 23: 15: 9: 6: 4: 3: 2: 3910: 3899: 3898:Rare diseases 3896: 3894: 3891: 3889: 3886: 3885: 3883: 3867: 3863: 3862: 3858: 3856: 3852: 3851: 3847: 3845: 3841: 3840: 3836: 3834: 3831: 3827: 3826: 3822: 3821: 3818: 3814: 3807: 3803: 3802: 3798: 3796: 3792: 3791: 3787: 3785: 3781: 3780: 3776: 3773: 3772: 3768: 3766: 3762: 3758: 3757: 3753: 3749: 3747: 3743: 3739: 3738: 3734: 3730: 3728: 3724: 3723: 3719: 3715: 3714: 3711: 3706: 3702: 3686: 3683: 3681: 3678: 3676: 3673: 3671: 3668: 3666: 3663: 3661: 3658: 3656: 3653: 3651: 3648: 3646: 3643: 3641: 3638: 3636: 3633: 3631: 3628: 3626: 3623: 3621: 3618: 3616: 3613: 3611: 3608: 3606: 3603: 3601: 3598: 3596: 3593: 3591: 3588: 3586: 3583: 3581: 3578: 3577: 3574: 3571: 3569: 3566: 3564: 3561: 3559: 3556: 3555: 3553: 3549: 3541: 3538: 3537: 3536: 3533: 3531: 3528: 3527: 3525: 3523: 3519: 3513: 3510: 3508: 3505: 3503: 3500: 3498: 3497:Encephalocele 3495: 3493: 3490: 3489: 3487: 3483: 3480: 3476:Developmental 3474: 3466: 3465: 3464: 3459: 3458: 3453: 3452: 3448: 3440: 3439:Hailey–Hailey 3437: 3433: 3429: 3426: 3424: 3421: 3420: 3419: 3418: 3417:immune system 3414: 3410: 3407: 3406: 3405: 3404: 3400: 3399: 3397: 3393: 3383: 3380: 3378: 3375: 3373: 3370: 3368: 3365: 3363: 3360: 3358: 3355: 3354: 3351: 3348: 3346: 3343: 3339: 3336: 3335: 3334: 3333: 3329: 3327: 3324: 3322: 3319: 3318: 3316: 3312: 3304: 3300: 3297: 3295: 3292: 3291: 3290: 3289: 3285: 3281: 3278: 3277: 3276: 3275: 3271: 3269: 3265: 3264: 3261: 3258: 3256: 3253: 3251: 3248: 3246: 3243: 3241: 3238: 3236: 3233: 3231: 3227: 3224: 3223: 3218: 3215: 3213: 3210: 3206: 3203: 3201: 3198: 3197: 3196: 3193: 3191: 3188: 3186: 3183: 3181: 3177: 3174: 3172: 3169: 3165: 3162: 3160: 3157: 3156: 3155: 3154: 3150: 3148: 3145: 3143: 3140: 3136: 3133: 3132: 3131: 3130: 3126: 3124: 3121: 3117: 3114: 3112: 3109: 3107: 3104: 3103: 3102: 3101: 3097: 3096: 3094: 3093: 3090: 3087: 3085: 3082: 3080: 3077: 3075: 3071: 3068: 3067: 3065: 3063: 3059: 3056: 3054: 3053:keratinopathy 3049: 3045: 3039: 3036: 3034: 3031: 3029: 3026: 3023: 3019: 3016: 3014: 3011: 3010: 3008: 3006: 3002: 2998: 2992: 2988: 2985: 2983: 2980: 2978: 2975: 2973: 2970: 2968: 2965: 2963: 2959: 2956: 2955: 2953: 2951: 2947: 2941: 2938: 2936: 2933: 2931: 2928: 2926: 2922: 2921: 2916: 2913: 2911: 2908: 2907: 2906: 2903: 2902: 2897: 2894: 2892: 2889: 2887: 2884: 2882: 2879: 2878: 2877: 2874: 2873: 2868: 2865: 2863: 2860: 2858: 2855: 2853: 2850: 2848: 2845: 2843: 2840: 2839: 2838: 2835: 2834: 2832: 2828: 2824: 2814: 2811: 2809: 2806: 2804: 2801: 2799: 2796: 2794: 2791: 2789: 2786: 2784: 2781: 2780: 2778: 2774: 2768: 2765: 2764: 2762: 2760: 2756: 2750: 2747: 2745: 2742: 2740: 2737: 2736: 2731: 2728: 2727: 2726: 2723: 2721: 2717: 2714: 2713: 2711: 2709: 2705: 2699: 2696: 2695: 2693: 2691: 2687: 2684: 2682: 2677: 2673: 2670: 2668: 2664: 2660: 2656: 2652: 2645: 2640: 2638: 2633: 2631: 2626: 2625: 2622: 2606: 2603: 2601: 2598: 2597: 2595: 2592: 2591: 2585: 2579: 2576: 2574: 2571: 2570: 2568: 2565: 2564: 2558: 2555: 2551: 2541: 2538: 2537: 2535: 2529: 2523: 2520: 2518: 2515: 2513: 2510: 2508: 2505: 2503: 2500: 2498: 2495: 2494: 2492: 2489: 2484: 2478: 2475: 2473: 2470: 2468: 2465: 2464: 2462: 2459: 2458: 2452: 2449: 2447: 2443: 2429: 2426: 2424: 2421: 2419: 2416: 2414: 2411: 2409: 2406: 2404: 2401: 2400: 2398: 2395: 2394: 2388: 2382: 2379: 2378: 2376: 2373: 2372: 2366: 2360: 2357: 2356: 2354: 2351: 2350: 2344: 2341: 2339: 2335: 2325: 2322: 2321: 2318: 2315: 2314: 2312: 2309: 2308: 2302: 2299: 2297: 2293: 2290: 2288: 2284: 2270: 2266: 2263: 2262: 2260: 2257: 2256: 2250: 2244: 2241: 2240: 2238: 2235: 2234: 2228: 2225: 2223: 2219: 2209: 2206: 2205: 2203: 2200: 2199: 2193: 2190: 2188: 2184: 2174: 2173:Histiocytosis 2171: 2170: 2168: 2164: 2154: 2151: 2149: 2146: 2145: 2143: 2140: 2139: 2133: 2127: 2124: 2123: 2121: 2118: 2117: 2111: 2108: 2106: 2105:Myelomonocyte 2102: 2092: 2089: 2087: 2084: 2083: 2081: 2078: 2077: 2071: 2065: 2062: 2060: 2057: 2056: 2054: 2051: 2050: 2044: 2041: 2039: 2035: 2025: 2022: 2021: 2019: 2016: 2015: 2009: 2003: 2000: 1998: 1995: 1993: 1990: 1988: 1985: 1983: 1980: 1979: 1977: 1974: 1973: 1967: 1964: 1962: 1958: 1955: 1953: 1949: 1946: 1944: 1938: 1934: 1930: 1926: 1919: 1914: 1912: 1907: 1905: 1900: 1899: 1896: 1888: 1884: 1880: 1876: 1872: 1868: 1863: 1862: 1850: 1846: 1839: 1831: 1827: 1823: 1819: 1812: 1804: 1800: 1795: 1790: 1786: 1782: 1778: 1774: 1770: 1763: 1756: 1755: 1748: 1740: 1736: 1732: 1728: 1723: 1718: 1714: 1710: 1706: 1698: 1690: 1686: 1681: 1676: 1671: 1666: 1662: 1658: 1654: 1647: 1645: 1636: 1632: 1627: 1622: 1618: 1614: 1610: 1603: 1601: 1592: 1588: 1584: 1580: 1576: 1572: 1568: 1564: 1557: 1549: 1545: 1540: 1535: 1531: 1527: 1523: 1519: 1515: 1508: 1500: 1496: 1490: 1482: 1478: 1474: 1470: 1466: 1462: 1458: 1454: 1446: 1438: 1434: 1429: 1424: 1420: 1416: 1412: 1405: 1398: 1397:0-7216-2921-0 1394: 1390: 1384: 1376: 1372: 1368: 1364: 1360: 1356: 1352: 1348: 1340: 1332: 1328: 1324: 1320: 1316: 1312: 1311:Dermatol Surg 1305: 1298: 1292: 1284: 1280: 1275: 1270: 1266: 1262: 1258: 1254: 1250: 1243: 1235: 1231: 1226: 1221: 1217: 1213: 1209: 1202: 1200: 1191: 1187: 1182: 1177: 1173: 1169: 1165: 1161: 1157: 1150: 1142: 1138: 1134: 1130: 1126: 1122: 1117: 1112: 1108: 1104: 1100: 1093: 1085: 1081: 1077: 1073: 1069: 1065: 1061: 1057: 1053: 1046: 1038: 1034: 1029: 1024: 1019: 1014: 1010: 1006: 1002: 995: 987: 983: 978: 973: 968: 963: 959: 955: 951: 947: 943: 935: 933: 924: 920: 915: 910: 905: 900: 896: 892: 888: 884: 880: 872: 858:on 2013-12-28 857: 853: 847: 839: 835: 830: 825: 822:(2): 121–32. 821: 817: 813: 806: 802: 793: 790: 789: 783: 781: 777: 768: 765: 761: 757: 755: 751: 742: 740: 730: 721: 719: 709: 707: 698: 695: 692: 689: 686: 683: 680: 677: 674: 671: 670: 668: 665: 662: 659: 656: 652: 651: 650: 642: 640: 636: 632: 625: 622: 619: 616: 613: 610: 606: 603: 600: 597: 594: 590: 586: 583: 580: 577: 566: 563: 562: 556: 547: 546:Bezuclastinib 539: 536: 533: 530: 527: 524: 523: 522: 519: 517: 516:enterocolitis 513: 509: 505: 496: 492: 488: 479: 473: 469: 465: 461: 458: 457: 452: 448: 447: 441: 430: 426: 423: 420: 417: 416: 415: 404: 401: 398: 395: 394: 385: 384: 378: 376: 371: 367: 365: 361: 358:Diagnosis of 351: 349: 345: 341: 337: 333: 329: 325: 320: 318: 314: 310: 309:blood vessels 306: 302: 297: 295: 291: 287: 283: 279: 270: 264: 261: 260:Malabsorption 258: 255: 254:parietal cell 251: 247: 246:peptic ulcers 243: 240: 237: 234: 231: 227: 223: 221: 217: 214: 212: 208: 204: 201: 197: 194: 191: 188: 186: 183: 181: 177: 174: 172: 169: 166: 163: 160: 159: 153: 149: 148: 143: 140: 139: 138: 136: 135:degranulation 127: 118: 116: 112: 108: 104: 99: 97: 93: 89: 88:rare disorder 85: 81: 73: 69: 65: 62: 60: 56: 52: 51:H&E stain 48: 44: 40: 35: 32: 29: 25: 20: 3859: 3855:mastocytosis 3848: 3844:mastocytosis 3837: 3823: 3799: 3788: 3777: 3769: 3750: 3731: 3716: 3502:Nasal glioma 3492:Dermoid cyst 3461: 3455: 3444: 3443: 3428:Mastocytosis 3427: 3415: 3409:EEM syndrome 3401: 3330: 3286: 3272: 3151: 3127: 3098: 2659:skin disease 2588: 2561: 2488:Mastocytosis 2487: 2476: 2455: 2391: 2369: 2347: 2305: 2253: 2231: 2196: 2136: 2114: 2074: 2047: 2012: 1970: 1943:granulocytes 1870: 1866: 1848: 1844: 1838: 1821: 1817: 1811: 1776: 1772: 1762: 1752: 1747: 1712: 1708: 1697: 1660: 1656: 1616: 1612: 1566: 1562: 1556: 1521: 1517: 1507: 1498: 1489: 1456: 1452: 1445: 1418: 1414: 1404: 1388: 1383: 1350: 1346: 1339: 1314: 1310: 1304: 1291: 1256: 1252: 1242: 1215: 1211: 1163: 1159: 1149: 1109:(1): 12–30. 1106: 1102: 1092: 1059: 1055: 1045: 1008: 1004: 994: 949: 945: 889:(5): e2266. 886: 882: 871: 860:. Retrieved 856:the original 846: 819: 816:Pathobiology 815: 805: 779: 775: 774: 766: 762: 758: 748: 736: 733:Epidemiology 727: 715: 704: 648: 629: 554: 520: 500: 477: 472:homme orange 471: 454: 450: 439: 413: 374: 372: 368: 359: 357: 347: 343: 339: 327: 321: 316: 313:angiogenesis 298: 276: 268: 226:osteoporosis 205:Episodes of 157: 145: 132: 129:Mastocytosis 100: 91: 82:, a type of 80:Mastocytosis 79: 78: 30: 22:Mastocytosis 3665:Skin dimple 3274:desmoplakin 3266:ungrouped: 3153:Cathepsin C 3129:plakoglobin 2830:and related 2457:Mastocytoma 1317:(1): 33–7. 1125:10261/62846 792:Mastocytoma 697:Avapritinib 691:Midostaurin 673:Midostaurin 639:mirtazapine 609:anaphylaxis 605:Epinephrine 579:Leukotriene 512:lymph nodes 220:muscle pain 196:Anaphylaxis 27:Other names 3882:Categories 3801:DiseasesDB 3095:syndromic 3018:Cutis laxa 3005:Connective 2655:integument 2651:Congenital 1941:and other 1499:Cancer.Net 862:2017-11-02 798:References 661:Cladribine 655:interferon 615:Salbutamol 468:integument 278:Mast cells 238:Depression 92:mastocytes 72:hematology 47:Micrograph 3825:eMedicine 3685:Birthmark 3478:anomalies 3445:see also 2923:related: 2776:Ungrouped 1961:Myelocyte 1927:-related 1851:: 359–61. 1830:840287852 1663:: 77–92. 1473:1543-2165 724:Prognosis 685:Masitinib 593:Ketotifen 551:Treatment 354:Diagnosis 305:histamine 290:histamine 211:faintness 158:urticaria 115:histamine 84:mast cell 59:Specialty 3861:Orphanet 3833:med/1401 3830:derm/258 3774:: 9741/3 3403:cadherin 3288:connexin 3226:punctate 3100:connexin 2446:CFU-Mast 2187:CFU-Baso 2038:Monocyte 1803:20745623 1773:Br Med J 1739:24088671 1731:27428296 1689:31372066 1635:27762455 1591:37335288 1548:24044484 1481:25611105 1437:21301631 1375:30459720 1367:12688351 1283:21035176 1234:27069254 1190:28254862 1141:12120327 1133:17555444 1084:52298220 1076:30230078 1037:19058339 986:22031830 946:PLOS ONE 923:18509466 883:PLOS ONE 838:17587883 786:See also 745:Research 679:Imatinib 336:receptor 235:Headache 185:Diarrhea 180:vomiting 68:Oncology 3795:D008415 3485:Midline 3070:diffuse 3001:Elastic 2296:CFU-Meg 2222:CFU-Eos 2059:AMoL/M5 1925:Myeloid 1887:7771504 1794:2260962 1680:6630092 1583:3749421 1539:4362676 1428:3033552 1331:8556255 1274:3753019 1181:5354959 1028:2773867 977:3198767 954:Bibcode 914:2386235 891:Bibcode 771:History 635:doxepin 317:too few 152:itching 141:Fatigue 103:itching 3784:154800 3761:757.33 3332:ATP2A2 2896:JEB-PA 2867:EBS-MP 2862:EBS-MD 2857:EBS-OG 2852:EBS-DM 2847:EBS-WC 2002:APL/M3 1952:CFU-GM 1937:CFU-GM 1885:  1828:  1801:  1791:  1737:  1729:  1687:  1677:  1633:  1589:  1581:  1546:  1536:  1479:  1471:  1435:  1425:  1395:  1373:  1365:  1329:  1299:, 2017 1281:  1271:  1232:  1188:  1178:  1139:  1131:  1082:  1074:  1035:  1025:  984:  974:  921:  911:  836:  669:(TKI) 508:spleen 364:biopsy 301:wounds 176:Nausea 154:, and 109:, and 94:) and 3866:98292 3771:ICD-O 3765:202.6 3746:C96.2 3742:Q82.2 3522:Nevus 3395:Other 3314:Other 3176:focal 2886:Mitis 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Index


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
muscle pain

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