Gastrointestinal Lesions in Mastocytosis

Clemett, Arthur R.; Fishbone, Gerald; Levine, Robert J.; James, A. Everette; Janower, Murray L.

doi:10.2214/ajr.103.2.405

Cited by 42 publications

(10 citation statements)

References 7 publications

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“…Despite the malabsorptive state, the small bowel has a normal radiographic appearance except for mild dilatation lesions are present in approximately 10% of cases and have a variable radiographic appearance [6,8]. The latter stages of the disease are characterized by fibrosis of the involved organs [5].…”

Section: Discussionmentioning

confidence: 99%

“…It is thought that the thickened folds are secondary to mast cell proliferation in the lamina propria [5,6]. The typical 2-5-mm nodular defects may be secondary to urticarial changes but this is unproven [1, 5, 6].…”

Section: Discussionmentioning

confidence: 99%

“…In addition, there is an increased incidence of peptic ulcers [1,5,6] and malabsorption [1-3, 5, 6]. We report a case of systemic mastocytosis with gastrointestinal involvement that presented radiographically as large nodules in the duodenum and gastric antrum, with several bull's-eye lesions in the duodenum.…”

mentioning

confidence: 99%

“…The roentgenographic manifestations include thickening of bowel wall and mucosal nodularity [3][4][5]. In addition, there is an increased incidence of peptic ulcers [1,5,6] and malabsorption [1-3, 5, 6].…”

mentioning

confidence: 99%

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Bull's-eye lesions: A new gastrointestinal presentation of mastocytosis

Quinn

Shaffer

Willard

et al. 1984

Gastrointest Radiol

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Systemic mastocytosis is a rare disorder that infrequently affects the GI tract. Bowel involvement in mastocytosis is characterized by thickened folds and small mucosal nodules, and there is an increased incidence of peptic ulcer disease and malabsorption. This paper describes a new case of mastocytosis that presented radiographically as 1.0-1.5 cm gastric and duodenal nodules. Some of the duodenal nodules were bull's-eye lesions with central collections of barium. Mastocytosis, along with primary neoplasms, aberrant pancreas, eosinophilic granuloma, and metastases should be included in the differential for bull's-eye lesions of the GI tract.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

mentioning

confidence: 99%

mentioning

confidence: 99%

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Bull's-eye lesions: A new gastrointestinal presentation of mastocytosis

Quinn

Shaffer

Willard

et al. 1984

Gastrointest Radiol

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“…20,23,[26][27][28] Gastritis lesions with mucosal and submucosal inflammatory infiltrates associated with mast cell infiltrates were observed in several SM patients.…”

Section: Stomach: Other Lesionsmentioning

confidence: 99%

Gastrointestinal involvement and manifestations in systemic mastocytosis

Sokol¹,

Georgin‐Lavialle²,

Grandpeix-Guyodo³

et al. 2010

Inflammatory Bowel Diseases

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Mastocytosis is a rare and heterogeneous disease characterized by various biological and clinical features with different prognosis and treatments. The disease is usually divided into 2 categories: a pure cutaneous and a systemic disease. Clinical features can be related to mast cells' mediators release or to pathological mast cells infiltration. The diagnosis of mastocytosis is based on clinical, biological, histological, and molecular international criteria. Among all manifestations of the disease, gastrointestinal (GI) symptoms are common and can significantly impair the quality of life. The aim of this article is to review the data regarding GI involvement in mastocytosis. Articles dealing with clinical, pathophysiological, and therapeutic aspects of mastocytosis GI tract involvement were searched for using PubMed. GI manifestations in mastocytosis are reviewed. Pathogenesis of GI symptoms in systemic mastocytosis and their treatment are critically discussed. The most frequent GI symptoms are abdominal pain, diarrhea, nausea, and vomiting. GI lesions may involve all the digestive tract, from the esophagus to the rectum. The histological diagnosis of GI involvement is difficult. The treatment of GI symptoms aims to prevent and limit mast cells degranulation and/or its consequences and more rarely to control tumoral mast cells infiltration. The high prevalence of GI symptoms in mastocytosis and their important functional impact deserves better characterization and treatment in order to improve patients' quality of life. Diagnosis of mastocytosis GI manifestations should be evoked in the case of unexplained severe GI disorders.

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Urticaria Pigmentosa

et al. 1991

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Nine patients with adult-onset urticaria pigmentosa were studied for the incidence of extracutaneous mast cell involvement and the efficacy of potent topical corticosteroid therapy for cutaneous lesions. Seven of the nine patients had increased mast cells in the marrow biopsy specimens, and five patients had focal aggregates of mast cells. The bone scan was abnormal in one patient. Liver-spleen scans revealed a shift of colloid uptake from liver to spleen in four patients. No abnormal gastrointestinal tract roentgenograms were obtained. Urinary histamine metabolites correlated with nodular bone marrow involvement, but not with other parameters. Results of the psychoneurologic testing revealed significant deviation from the norm with a verbal memory deficit in all nine patients and abnormalities on the Minnesota Multiphasic Personality Inventory in four patients. All nine patients were treated with 0.05% betamethasone dipropionate ointment under occlusion over half of the body nightly for 6 weeks. Seven of nine patients treated responded with almost complete resolution of their lesions. Hypothalamic pituitary adrenal axis suppression was evaluated with intramuscular cosyntropin stimulation and metyrapone administration during treatment. Only two patients, both of whom used the medication improperly, developed transient abnormalities. Slow return of lesions was noted 6 months after completion of therapy. Remissions could be lengthened with single weekly applications of topical steroids. Systemic involvement is frequent in patients with cutaneous mast cell disease and it is best demonstrated by bone marrow biopsy. Mast cell lesions can be safely and effectively treated with topical steroids in motivated patients.

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Gastrointestinal Lesions in Mastocytosis

Cited by 42 publications

References 7 publications

Bull's-eye lesions: A new gastrointestinal presentation of mastocytosis

Bull's-eye lesions: A new gastrointestinal presentation of mastocytosis

Gastrointestinal involvement and manifestations in systemic mastocytosis

Urticaria Pigmentosa

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