The Bone Marrow in Urticaria Pigmentosa and Systemic Mastocytosis

Ridell, Børje

doi:10.1001/archderm.1986.01660160078023

Cited by 34 publications

(10 citation statements)

References 20 publications

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“…Elevated levels of histamine and its metabolites in 24-h urine samples may be indicative of mastocytosis. 23,24 The quantity of the metabolite N-methylimidazole acetic acid excreted by the kidneys reflects the body's total mature mast cell load 25 and may be elevated in patients with systemic mastocytosis, but also in cases restricted to the skin. 2 Cutaneous mastocytosis of the xanthelasmoid type is clinically characterized by the typical yellowish hue and is accompanied histologically by mast cells infiltrating far into the lower dermis.…”

Section: Discussionmentioning

confidence: 99%

Nodular and bullous cutaneous mastocytosis of the xanthelasmoid type: case report

et al. 2001

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Severe generalized nodular and bullous mastocytosis of the xanthelasmoid type is described in a 7-month-old boy. Reddish to yellowish-brown xanthelasmoid papules and nodules first developed in the inguinal region a few weeks after birth and then progressively spread to cover nearly the entire body surface. There was severe pruritus and recurrent episodes of blistering. The diagnosis of cutaneous mastocytosis of the xanthelasmoid type with subepidermal bullae was confirmed by skin biopsies showing solid and deeply penetrating infiltrates of metachromatic mast cells under light and electron microscopy. Systemic involvement of other organs, however, was excluded by bone scintigraphy, abdominal ultrasound, bone marrow aspiration and echocardiography. The extensive skin involvement was reflected in highly elevated urinary levels of histamine (263.4 microg L(-1)) and its metabolite N-methylimidazole acetic acid (20.8 mg L(-1)). The patient was systematically well and received only symptomatic treatment. Over a period of 1 year, the condition gradually improved, and the skin lesions began to flatten and regress.

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Section: Discussionmentioning

confidence: 99%

Nodular and bullous cutaneous mastocytosis of the xanthelasmoid type: case report

et al. 2001

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“…The bone marrow histopathology of adult-onset disease has been described extensively (8,27,28,34,35), but only one study investigated such findings in children (36). Most adults with systemic disease have normocellular to hypercellular bone marrow, with focal mast cell lesions or increased numbers of mast cells in the absence of focal lesions (8,27,28,34,35). Typical bone marrow lesions are foci of mast cells within a fibrotic background, usually with an admixture of eosinophils and lymphocytes, the socalled MEL lesions (33).…”

Section: Discussionmentioning

confidence: 99%

Urticaria Pigmentosa: A Review of 67 Pediatric Cases

Defez

Torrelo²,

Mediero

et al. 1994

Pediatric Dermatology

134

139

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Mastocytosis is a disorder of mast cell proliferation that may appear during infancy, childhood, or adulthood. We studied 67 consecutive patients (33 males, 34 females) with urticaria pigmentosa and assessed them fully to determine the presence of systemic involvement. Ages at onset of lesions ranged from birth to 11 years, with most developing in the first year of life. Pruritus was the primary symptom. Hematologic and serum chemistry profile, radiologic skeletal surveys, and bone marrow aspirations were performed. Slight anemia was present in three patients. Radiologic bone lesions were observed in eight. Bone marrow aspirates showed slight changes in six patients, with only an increased number of mast cells in an additional patient. The disease tended to resolve spontaneously. This prospective study emphasizes the benign nature of pediatric urticaria pigmentosa.

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“…The clinical signs of systemic mastocytosis in this case were pruritic red-brown pigmented skin lesions, an increased number of mast cells in the skin and bone biopsy specimens, increased mineral bone density, osteosclerosis, focal accumulation of mast cells in the bone marrow, and increased excretion of MeImAA, the main metabolite of histamine. An increased excretion of MeImAA has been found to positively correlate to an increased number of mast cells in the bone marrow 16 and could also be an indicator of osteosclerosis in mastocytosis. 17 The patient described in this article is a smoker, which could be one factor causing her periodontal disease.…”

Section: Discussionmentioning

confidence: 99%