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The Mastocytosis Syndrome: Clinical and Biological Studies
165
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46
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1963
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Autoimmune DiseaseMast Cell DisorderSurgical PathologyHistopathologyMast CellsPathologyClinical DermatologyUrticaria PigmentosaAutoimmunityMastocytosis SyndromeGeneral PathologyDermatologyDermatopathologyExperimental DermatologyMedicineEndocrine DiseaseConnective Tissue Disease
Article1 August 1963The Mastocytosis Syndrome: Clinical and Biological StudiesD. JOSEPH DEMIS, PH.D., M.D.D. JOSEPH DEMIS, PH.D., M.D.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-59-2-194 SectionsAboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail ExcerptMastocytosis, an abnormal proliferation of tissue mast cells, is being recognized with increasing frequency as a systemic disease. This disorder was originally described by Nettleship in 1869 (1) as its cutaneous manifestation, urticaria pigmentosa. It was not until 1949 that Ellis (2) reported autopsy findings which conclusively demonstrated generalized systemic involvement with infiltration of skin, liver, spleen, bone marrow, lymph nodes, pancreas, and thymus. In the past decade, reports of symptoms associated with the typical clinical eruption have been steadily accumulating (3).Over the past 4 years, 31 patients with mastocytosis have been evaluated. Of these, 12 were noted to...References1. NETTLESHIP E: Rare forms of urticaria. Brit. Med. J. 2: 323, 1869. MedlineGoogle Scholar2. ELLIS JM: Urticaria pigmentosa: Report of a case with autopsy. Arch. Path. 48: 426, 1949. MedlineGoogle Scholar3. DEMISWALTONHIGDON DJMDRS: Histaminuria in urticaria pigmentosa and the mastocytosis syndrome: A clinical study and review of the recent literature with definition of the mastocytosis syndrome. Arch. Derm. (Chicago) 83: 127, 1961. CrossrefMedlineGoogle Scholar4. GROSS P: Urticaria pigmentosa (solitary lesion). Arch. Derm. Syph. 29: 451, 1934. Google Scholar5. SAGHERSCHORR FS: Bone lesions in urticaria pigmentosa. J. Invest. Derm. 26: 431, 1956. CrossrefMedlineGoogle Scholar6. LEESSTROUD MHCE: Bone lesions of urticaria pigmentosa in childhood. Arch. Dis. Child. 34: 205, 1959. CrossrefMedlineGoogle Scholar7. BROGRENDUNERHAMRINPERNOWTHEANDERWALDENSTROM NHBBGJ: Urticaria pigmentosa (mastocytosis). Acta Med. Scand. 163: 223, 1959. CrossrefMedlineGoogle Scholar8. HAVARDSCOTT CWRB: Urticaria pigmentosa with visceral and skeletal lesions. Quart. J. Med. 28: 459, 470, 1959. MedlineGoogle Scholar9. POPPELGRUBERSILBERHOLDERCHRISTMAN MWRAR: The roentgen manifestations of urticaria pigmentosa (mastocytosis). Amer. J. Roentgen. 82: 239, 1959. Google Scholar10. SAGHERLIBANUNGARSCHORR FEHS: Urticaria pigmentosa with bone involvement: Mast cell aggregates in bones and myelosclerosis found at autopsy in a case dying of monocytic leukemia. J. Invest. Derm. 27: 355, 1956. CrossrefMedlineGoogle Scholar11. STARKVAN BUSKIRKDALY EFWJF: Radiologic and pathologic bone changes associated with urticaria pigmentosa: Report of a case. Arch. Path. 62: 143, 1956. MedlineGoogle Scholar12. ZAKCOVEYSNODGRASS FGJAJJ: Osseous lesions in urticaria pigmentosa. New Eng. J. Med. 256: 56, 1957. CrossrefMedlineGoogle Scholar13. SZWEDAABRAHAMFINENIXONRUPE JAJPGRKCE: Systemic mast cell disease. Amer. J. Med. 32: 227, 1962. CrossrefGoogle Scholar14. ENDECHERNISS NEI: Splenic mastocytosis. Blood 13: 631, 1958. CrossrefMedlineGoogle Scholar15. EFRATIKLAJMANSPITZ PAHL: Mast cell leukemia-malignant mastocytosis with leukemia-like manifestations. Blood 12: 869, 1957. CrossrefMedlineGoogle Scholar16. WATERSLACSON WJPS: Mast cell leukemia presenting as urticaria pigmentosa. Pediatrics 19: 1033, 1957. MedlineGoogle Scholar17. LENNERTKOSTERMARTIN KEH: Uber die mastzellenleukämie. Acta Haemat. (Basel) 16: 255, 1956. CrossrefMedlineGoogle Scholar18. FRIEDMANWILLFREIMANBRAUNSTEIN BIJJDGH: Tissue mast cell leukemia. Blood 13: 70, 1958. CrossrefMedlineGoogle Scholar19. HANNAY MG: Case for diagnosis (urticaria pigmentosa). Proc. Roy. Soc. Med. 17: 63, 1924. CrossrefMedlineGoogle Scholar20. DICKISON : Discussion. Arch. Derm. (Chicago) 75: 141, 1957. Google Scholar21. KLAUSWINKLEMANN SNRK: Course of urticaria pigmentosa in children. Arch. Derm. (Chicago) 83: 68, 1962. CrossrefGoogle Scholar22. WINKLEMANN RK: Personal communication. Google Scholar23. CAPLAN RM: The natural course of urticaria pigmentosa. Arch. Derm. (Chicago) 87: 146, 1963. CrossrefMedlineGoogle Scholar24. RILEY JF: Histamine in tissue mast cells. Science 118: 332, 1953. CrossrefMedlineGoogle Scholar25. LINDELLRORSMANWESTLING SEHH: Formation of histamine in a canine mastocytoma. Experientia 15: 31, 1959. CrossrefMedlineGoogle Scholar26. SCHAYER RW: Formation and binding of histamine by free mast cells of rat peritoneal fluid. Amer. J. Physiol. 186: 199, 1956. CrossrefMedlineGoogle Scholar27. HAGENLEE PFL: Amino acid decarboxylases of mouse mast cells. J. Physiol. 143: 7P, 1958. Google Scholar28. DEMISBROWN DJDD: Histidine metabolism in urticaria pigmentosa. JAMA 172: 182, 1960. Google Scholar29. DEMISBROWN DJDD: Histidine metabolism in urticaria pigmentosa. J. Invest. Derm. 36: 253, 1961. CrossrefMedlineGoogle Scholar30. LINDELLRORSMANWESTLING SEHH: Histamine formation in urticaria pigmentosa. Acta Dermatovener. (Stockholm) 41: 277, 1961. MedlineGoogle Scholar31. BIRTHAGENZEBROWSKI ARPE: Amino acid decarboxylases of urticaria pigmentosa mast cells. J. Invest. Derm. 37: 273, 1961. CrossrefMedlineGoogle Scholar32. BIRTNICKERSON ARM: Generalized flushing of the skin with urticaria pigmentosa. Arch. Derm. 80: 311, 1959. CrossrefMedlineGoogle Scholar33. DAYGREEN MJP: The uptake of amino acids and the synthesis of amines by neoplastic mast cells in culture. J. Physiol. 164: 210, 1962. CrossrefMedlineGoogle Scholar34. DEMISWALTONWOOLEYWILNERMCNEIL DJMDDNG: Further studies of histidine and histamine metabolism in urticaria pigmentosa. J. Invest. Derm. 37: 513, 1961. CrossrefMedlineGoogle Scholar35. UDENFRIENDLOVENBERGWEISSBACH SWMH: L-amino acid decarboxylase activity in mammalian tissues and its inhibition by a-methyl-dopa. Fed. Proc. 19: 7, 1960. Google Scholar36. SCHULTZCODEBRUNSTING HJCFLA: Blood histamine and basophil-eosinophil counts in skin diseases. Arch. Derm. 80: 44, 1959. CrossrefGoogle Scholar37. ROBERTSADAM MHM: New methods for the quantitative estimation of free and conjugated histamine in body fluids. Brit. J. Pharmacol. 5: 526, 1960. Google Scholar38. PERNOWWALDENSTROM BJ: Determination of 5-hydroxytryptamine, 5-hydroxyindole acetic acid and histamine in thirty-three cases of carcinoid tumor (Argentaffinoma) Amer. J. Med. 23: 16, 1957. CrossrefMedlineGoogle Scholar39. SUTTERBEAULIEUBIRT MCGAR: Histamine liberation by codeine and polymyxin B in urticaria pigmentosa. Arch. Derm. (Chicago) 86: 135, 1962. CrossrefGoogle Scholar40. OATESMARSHSJOERDSMA JAEA: Studies on histamine in human urine using a fluorometric method of assay. Clin. Chim. Acta 7: 488, 1962. CrossrefMedlineGoogle Scholar41. WESTLING H: Personal communication. Google Scholar42. ROWLEYBENDITT DAEP: 5-Hydroxytryptamine and histamine as mediators of the vascular injury produced by agents which damage mast cells in rats. J. Exp. Med. 103: 399, 1956. CrossrefMedlineGoogle Scholar43. SJOERDSMAWAALKESWEISSBACH ATPH: Serotonin and histamine in mast cells. Science 125: 1202, 1957. CrossrefMedlineGoogle Scholar44. PARRATTWEST JRGB: 5-hydroxytryptamine and tissue mast cells. J. Physiol. 137: 169, 1957. CrossrefMedlineGoogle Scholar45. HARBERWITTEN LCV: Editors note, Yearbook of Dermatology, Year Book Medical Publishers, Chicago, p. 302, 1959-1960. Google Scholar46. DEMIS DJ: Abnormalities of histamine metabolism in mastocytosis with particular reference to increased urinary excretion of 1,4-methylimidazole acetic acid. 12th Int. Cong. Derm. Transactions (In press). Google Scholar47. RILEY JF: The Mast Cells, E. & S. Livingstone Ltd., Edinburgh and London, 1959. Google Scholar48. WALDENSTRÖM J: The production of substances with specific activity (hormones) by some tumour cells. Acta. Endocr. (Kobenhavn) 17: 432, 1954. MedlineGoogle Scholar49. DEMISDAVISLAWLER DJMJJC: A study of the cutaneous effects of serotonin. J. Invest. Derm. 34: 43, 1960. CrossrefMedlineGoogle Scholar50. DEMISZIMMER DJJG: Carcinoid flush. Lancet 2: 360, 1962. CrossrefGoogle Scholar51. SCHORRSAGHERLIBAN SFE: Generalized osteosclerosis in urticaria pigmentosa: The radiologic aspect. Acta Radiol. 46: 575, 1956. CrossrefMedlineGoogle Scholar52. THORSONBIÖRCKBJORKMANWALDENSTROM AGGJ: Malignant carcinoid of the small intestine with metastases to the liver, valvular disease of the right side of the heart (pulmonary stenosis and tricuspid regurgitation after regurgitation without septal defects), peripheral vasomotor symptoms, bronchoconstriction, and an unusual type of cyanosis. A clinical and pathological syndrome. Amer. Heart J. 47: 795, 1954. CrossrefMedlineGoogle Scholar53. CURRY JJ: The action of histamine on the respiratory tract in normal and asthmatic subjects. J. Clin. Invest. 25: 785, 1946. CrossrefMedlineGoogle Scholar54. DEMISZIMMERVERHONICKCATALANO DJJGPJPM: The pharmacology of human skin. I. Epinephrine and norepinephrine; catecholamine-serotonin combinations. J. Invest. Derm. 39: 419, 1962. CrossrefMedlineGoogle Scholar55. ROBERTSONPEARTANDREWS JIWSTM: The mechanism of facial flushes in the carcinoid syndrome. Quart. J. Med. 31: 103, 1962. MedlineGoogle Scholar56. SJOERDSMAWEISSBACHUDENFRIEND AHS: A clinical, physiologic and biochemical study of patients with malignant carcinoid (Argentaffinoma). Amer. J. Med., 20: 520, 1956. CrossrefMedlineGoogle Scholar57. OATESSJOERDSMA JAA: A unique syndrome associated with secretion of 5-hydroxytryptophan by metastatic gastric carcinoids. Amer. J. Med. 32: 333, 1961. CrossrefGoogle Scholar58. HEDINGER C: The carcinoid syndrome. Schweiz. Med. Wschr. 89: 1362, 1959. MedlineGoogle Scholar59. CAMPBELLGOWENLOCKPLATTSNOW ACAHDSPJ: A 5-hydroxytryptophan-secreting carcinoid tumor. Gut 4: 61, 1963. CrossrefMedlineGoogle Scholar60. SJOERDSMAOATESZALTZMANUDENFRIEND AJAPS: Serotonin synthesis in carcinoid patients: its inhibition by α-methyl-dopa, with measurement of associated increased in urinary 5-hydroxytryptophan. New Eng. J. Med. 263: 585, 1960. CrossrefMedlineGoogle Scholar61. DEMISZIMMER DJJG: Histaminuria in mastocytosis; the effect of alpha methyl-dopa on urinary excretion of free histamine. Arch. Intern. Med. (Chicago) 111: 309, 1963. CrossrefGoogle Scholar This content is PDF only. To continue reading please click on the PDF icon. Author, Article, and Disclosure InformationAffiliations: Washington, D. C.From the Department of Dermatology, Walter Reed Army Institute of Research, Walter Reed Army Medical Center, Washington 12, D. C.Presented at the Forty-third Annual Session of the American College of Physicians, Philadelphia, Pennsylvania, April 9-13, 1962.Requests for reprints should be addressed to D. Joseph Demis, Ph.D., M.D., Chief, Department of Dermatology, Walter Reed Army Institute of Research, Walter Reed Army Medical Center, Washington 12, D. C. PreviousarticleNextarticle Advertisement FiguresReferencesRelatedDetails Metrics Cited byThe role of mast cells in human skin cancersGastrointestinal Involvement in Mast Cell Activation DisordersIndolent Systemic Mastocytosis – a Case ReportSystemic Mastocytosis and Mast Cell TumorsA CASE REPORT OF INDOLENT SYSTEMIC MASTOCYTOSISMastocytosis: the puzzling clinical spectrum and challenging diagnostic aspects of an enigmatic diseaseSystemic Mastocytosis and Mast Cell TumorsCarcinoid SyndromeGastrointestinal manifestations in mastocytosis: A study of 83 patientsPolycythemia From Mast Cell Activation Syndrome: Lessons LearnedGastrointestinal involvement and manifestations in systemic mastocytosisCarcinoid SyndromeMastocytose : manifestations digestivesCurrent approaches to the diagnosis and treatment of systemic mastocytosisSystemic Mastocytosis: Clinical Manifestations and Differential DiagnosisBone Marrow Angiogenesis in Systemic Mast Cell DiseaseImmunohistochemical studies of c-kit, transforming growth factor-β, and basic fibroblast growth factor in mast cell diseaseMastocytoma: topical corticosteroid treatmentDiagnosis of mastocytosis: value of cytochemistry and immunohistochemistryMASTOCYTOSIS AND THE SKINGASTROINTESTINAL ABNORMALITIES AND INVOLVEMENT IN SYSTEMIC MASTOCYTOSISDiffuse Cutaneous Mastocytosis with Bone Marrow Infiltration in a Child: A Case ReportSystemic mastocytosis presenting as profound cardiovascular collapse during anaesthesiaEndogenous histamine stimulates ischemically sensitive abdominal visceral afferents through H 1 receptorsDifferential diagnosis of hot flashesMastocytosisTreatment of Telangiectasia Macularis Eruptiva Perstans with the 585-nm Flashlamp-pumped Dye LaserClinical advances in mastocytosisHepatic involvement in mastocytosis: Clinicopathologic correlations in 41 casesMastocytosis: Developments During the Past DecadeMast Cell Disease and Its DiagnosisThe mast cell and mast cell diseaseEvaluation of anorectal physiology in patients with increased mast cellsThe Differential Diagnosis of Arthritis in ChildhoodGeneralized mastocytosis and neurological complications in a 71-year-old patientMastocytosisRecurrent gastrointestinal bleeding associated with myelofibrosis and diffuse intestinal telangiectasiasSkin and bones. 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Part IIStudies on Histamine Metabolism in MastocytosisMastocytosis and the mast cellSystemic mastocytosis, myelofibrosis and portal hypertension.Systemic mast cell disease: A clinical and hematopathologic study of 26 casesThe Differential Diagnosis of Arthritis in ChildhoodThe Clinical Significance of Arachidonic Acid MetabolitesUrticaria pigmentosa in a case of vitiligoIncreased Production of Prostaglandin D2 in Patients with Systemic MastocytosisThe effect of cimetidine and propantheline on the symptoms of a patient with systemic mastocytosisMastocytosisOral Disodium Cromoglycate in the Treatment of Systemic MastocytosisThe Mast Cell — A Fascinating RiddleEffect of Cimetidine on Gastric Hypersecretion and Diarrhea in Systemic MastocytosisB. I. HIRSCHOWITZ, M.D., J. F. GROARKE, M.B, B.Ch.Systemic Mastocytosis and the Mastocytosis SyndromeRespiratory Distress and Hypoxemia in Systemic MastocytosisBasophilic leukemia presenting as gastroduodenal ulcerationPortal hypertension in systemic mastocytosis"Allergic" cryptococcal pneumoniaSystemic mastocytosisGastrointestinal involvement in systemic mastocytosis.IMIDAZOLESPseudoxanthomatous mastocytosisWatery diarrhoea and hypokalaemia associated with a phaeochromocytomaMast cells: Extended lifespan and lack of granule turnover under normal in vivo conditionsSemimaligne Primärtumoren der Bronchien und LungenBullous urticaria pigmentosa with bleedingSOLITARY MASTOCYTOMAMastocytosis and intestinal malabsorptionMastocytoma and Gastroduodenal Ulceration: Gastric and Duodenal Ulcers in Dogs with MastocytomaRetikulosenMastzellenretikulose (Mastocytose)Systemic mast cell disease in a patient with unusual gastrointestinal and pulmonary abnormalitiesUrticaria and AngioedemaJOHN S. THOMPSON, M.D., F.A.C.P.MASTOCYTOSIS TREATED WITH L-HYOSCYAMINE (EGAZIL®)Chronic urticariaCaseUrticaria as a Sign of Internal DiseaseBiologically active substances of the mast cellSome studies on the fluorescence of mast cells after paraformaldehyde treatmentSYNTHESIS, UPTAKE AND BINDING OF HISTAMINE AND 5-HYDROXYTRYPTAMINE IN MAST CELLSSolitary mast cell granuloma (histiocytoma) of the lung.A Histopathologic, Tissue Culture and Time-Lapse Cinematographic StudyPerspectives in DermatopathologySome Disorders of Amino Acid Metabolism in Man"Abnormal Metabolites" of Amino Acid OriginMastocytosis Manifested by HepatosplenomegalyClinical, Cytologic, and Biochemical Studies in Systemic Mast Cell DiseaseJ. E. ULTMANN, M.D., F.A.C.P., R. D. MUTTER, M.D., M. TANNENBAUM, M.D., R. R. P. WARNER, M.D., F.A.C.P. 1 August 1963Volume 59, Issue 2Page: 194-206KeywordsExcretionLymph nodesMast cellsMastocytosisPancreasSpleenSystemic diseasesTachycardiaThymusUrticaria Issue Published: 1 August 1963 PDF downloadLoading ...
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