Generalized Flushing of the Skin with Urticaria Pigmentosa

Birt, Arthur R.; Nickerson, Mark

doi:10.1001/archderm.1959.01560210053010

Cited by 43 publications

(8 citation statements)

References 13 publications

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“…Similar to the successful treatment of infant-onset solitary mastocytoma [23], an excisional biopsy or subsequent excision of a biopsy-confirmed cutaneous mastocytoma was performed in 75% (9/12) of the patients with adult onset solitary mastocytomas. Some symptomatic relief was provided with oral antihistamines, topical corticosteroids or intralesional corticosteroids; however, these interventions were only performed in a small number of patients.…”

Section: Discussionmentioning

confidence: 99%

Solitary mastocytoma presenting in an adult: report and literature review of adult-onset solitary cutaneous mastocytoma with recommendations for evaluation and treatment

Cohen

2016

Dermatol Pract Concept

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Background:Mastocytosis is either cutaneous (with skin-limited proliferation of mast cells) or systemic (with mast cells in extracutaneous sites). The onset of solitary mastocytoma in an adult is rare.Purpose:A woman with the new onset of solitary mastocytoma is described. The clinical features of patients with adult-onset solitary mastocytoma are summarized. Recommendations for the evaluation and treatment of individuals with adult-onset solitary mastocytoma are proposed.Methods:PubMed was searched with the key words: adult, CD2, CD25, cell, cutaneous, disease, KIT, KIT D816V, mast, mastocytoma, mutation, pigmentosa, solitary, tryptase, and urticarial. The papers generated by the search, and their references, were reviewed.Results:A 38-year-old Taiwanese woman presented with an asymptomatic brown patch, which morphologically mimicked a dysplastic nevus, on her right abdomen; biopsy demonstrated a solitary mastocytoma. Comprehensive evaluation (including serologic and bone marrow examination) excluded systemic mastocytosis and her residual mastocytoma is being monitored. Adult-onset solitary mastocytoma has been described in 16 patients. Lesions were either on the head and neck (5/14), torso (5/14) or extremities (4/14). Urtication following lesion rubbing was noted in 79% (11/14) of patients. Excision of the mastocytoma [75% (9/12)] was the most common treatment. Other management approaches included corticosteroids (topical or intralesional), antihistamines (systemic) or observation. Systemic symptoms were noted in 5 patients: flushing (3 women) and pruritus (3 women); gastrointestinal symptoms and headaches, flushing and/or anaphylaxis were each noted in one woman. None of the patients with adult-onset solitary mastocytoma had systemic mastocytosis; however, only 3 women were evaluated for systemic mastocytosis.Conclusions:Systemic mastocytosis is common in adults with new onset cutaneous mastocytosis. Therefore, a conservative work up for new onset solitary mastocytosis in adults is proposed to include complete blood cell counts, serum chemistries (including liver function tests), and serum tryptase level and bone marrow biopsy to evaluate for mast cell clusters, morphology and immunophenotype and KIT gene mutation in codon 816. Similar serologic testing should be considered annually for adult-onset solitary mastocytosis patients without systemic disease.

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Section: Discussionmentioning

confidence: 99%

Solitary mastocytoma presenting in an adult: report and literature review of adult-onset solitary cutaneous mastocytoma with recommendations for evaluation and treatment

Cohen

2016

Dermatol Pract Concept

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“…Analogous to therapeutic considerations in idiopathic histiocyto sis [46], cytostatic medication can also be considered in generalized mas tocytosis, even before any sign of malignant degeneration is evident. Surgical treatment of mastocytosis seems indicated only in cases which in volve an isolated mastocytoma [3,7] with systemic symptoms. Splenecto my is to be considered in the case of hypersplenism.…”

Section: Discussionmentioning

confidence: 99%

Generalized Mastocytosis

Kammen¹

1974

Acta Haematol

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The case histories of three patients with generalized mastocytosis are presented and discussed. The first patient had extensive urticaria pigmentosa, hepatosplenomegaly, polylymphadenopathy and myelofibrosis. The best therapeutic results were obtained with prednisone in combination with an antihistamine. The second patient showed virtually no skin lesions but had splenomegaly and upper abdominal pains. The latter disappeared after splenectomy. The spleen disclosed a greatly increased histamine concentration. The third patient started with thrombocytopenic purpura that was cured by splenectomy. Seven years later, urticaria pigmentosa developed with mast cell proliferation in skin and bone marrow and a low IgM level. In addition to skin biopsies, determination of histamine in blood and urine can supply valuable diagnostic information, especially in the more advanced cases. In systemic mastocytosis immune deficiency may occur.

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“…This patient had an increased urinary excretion of histamine. General flushing is characteristic of urticaria pigmentosa and also of the solitary variant (2,3,7,12,23,25). The flush is generally believed to be induced by the release of histamine into the circulation from the tumour.…”

Section: Discussionmentioning

confidence: 99%

Solitary Mastocytoma

Holmberg

1970

Acta Paediatrica

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Generalized Flushing of the Skin with Urticaria Pigmentosa

Cited by 43 publications

References 13 publications

Solitary mastocytoma presenting in an adult: report and literature review of adult-onset solitary cutaneous mastocytoma with recommendations for evaluation and treatment

Solitary mastocytoma presenting in an adult: report and literature review of adult-onset solitary cutaneous mastocytoma with recommendations for evaluation and treatment

Generalized Mastocytosis

Solitary Mastocytoma

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