Diffuse Cutaneous Mastocytosis with Bone Marrow Infiltration in a Child: A Case Report

Waxtein, Leon; Vega-Memije, Ma Elisa; Cortés‐Franco, Roberto; Domínguez‐Soto, Luciano

doi:10.1046/j.1525-1470.2000.01751.x

Cited by 24 publications

(24 citation statements)

References 31 publications

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“…There is limited information on the prognosis of DCM, but it seems to be similar to that in other forms of CM in childhood [24,25]. Three of our patients with a follow-up of more than 10 years were completely cured.…”

Section: Discussionsupporting

confidence: 54%

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Clinical Aspects of Diffuse Cutaneous Mastocytosis in Children: Two Variants

Heide¹,

Zuidema²,

Beishuizen

et al. 2009

Dermatology

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Objective: This paper describes two different clinical presentations of diffuse cutaneous mastocytosis (DCM), based on the largest series published to date. As far as we are aware, these two variants of clinical presentations have not yet been reported. Design: We undertook a case controlled analysis of 8 children with DCM. Results of laboratory testing including mast cell mediator levels, and clinical symptoms on presentation and during follow-up were analyzed. Results: The levels of relevant mast cell mediators were initially high in all cases but declined sharply later on. There was a reduction of 20% in 2 of the 7 cases, whereas there was a reduction of 80% in the remaining 5. No reduction occurred in 1 case. Clinical improvement followed the same pattern. Conclusions: DCM is a rare variant of cutaneous childhood onset mastocytosis. Various forms show the same or overlapping features at various times. It appears to follow a course similar to that in other types of childhood onset mastocytosis, taking into account the decreased symptoms and the levels of mast cell mediators during follow-up. Obtaining a bone marrow biopsy should be considered only in those cases where there is no improvement or even worsening of signs or symptoms and persistent elevated levels of mast cell mediators.

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Section: Discussionsupporting

confidence: 54%

“…Arguably, bone marrow analysis is important for the choice of therapy, especially in the case of persistent rising serum tryptase levels, c- kit abnormalities, and suspicion of systemic and myeloproliferative disease. DCM was shown to be a part of systemic cases of mastocytosis [24,25]. …”

Section: Discussionmentioning

confidence: 99%

Clinical Aspects of Diffuse Cutaneous Mastocytosis in Children: Two Variants

Heide¹,

Zuidema²,

Beishuizen

et al. 2009

Dermatology

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“…Systemic involvement is found in about 10% of patients but more often in adults and older children [4]. Bone marrow infiltration occurs in 15% of children.…”

Section: Discussionmentioning

confidence: 99%

Neonatal Mastocytosis with Pachydermic Bullous Skin without c-Kit 816 Mutation

Walker

Komorowski²,

Scheurlen³

et al. 2006

Dermatology

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Background: Bullous mastocytosis is an unusual variant of mast cell disease with widespread bullae as the main cutaneous feature induced by mast cell proteases that cause dermoepidermal separation. Case Report: A rare case of diffuse cutaneous bullous mastocytosis with pachydermia and unusually extensive skin folding is described in a 3-week-old girl. The diagnosis was confirmed by immunohistochemistry with Giemsa stain, the naphthol ASD chloroacetate esterase reaction and elevated blood levels for tryptase, histamine in serum and histamine and 1.4-methylimidazol acetic acid in the 24-hour urine. Blood cell count was normal, as were thrombocytosis and leukocytosis. FACS analysis of the bone marrow aspiration material showed 1% mast cells. No c-Kit 816 [Asp→Val] somatic mutation was found. Systemic involvement of other organs was excluded. Discussion: The prognosis of c-Kit-negative diffuse bullous mastocytosis is not known. Regular blood controls are mandatory, and screening for germ cell ovarian cancer and bone marrow controls should be performed as well.

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“…Diffuse cutaneous mastocytosis is one of the rare benign forms of mastocytosis with varied clinical presentations. The skin may become diffusely infiltrated (grained leather appearance) (2) with a pebbly feel, develop a doughy consistency, or develop bullae on a normal looking skin. The pseudoxanthomatous variant is an extremely rare multinodular nonpigmented form, termed earlier as xanthelasmoidae, with lesions resembling both xanthoma and pseudoxanthoma elasticum (1).…”

Section: Discussionmentioning

confidence: 99%

Diffuse Cutaneous Mastocytosis: Pseudoxanthomatous Variant

Jain¹,

Dogra²,

Verma³

et al. 2002

The Journal of Dermatology

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Diffuse cutaneous mastocytosis is one of the extremely rare benign forms of mastocytosis that have varied clinical presentations. The pseudoxanthomatous variant is an extremely rare multinodular nonpigmented entity. Only two cases have been reported so far in the literature. We report a 21 year old female patient who had diffusely infiltrated skin with multiple papulo‐nodular lesions resembling xanthomas and the classical histopathological features of mastocytosis.

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Diffuse Cutaneous Mastocytosis with Bone Marrow Infiltration in a Child: A Case Report

Cited by 24 publications

References 31 publications

Clinical Aspects of Diffuse Cutaneous Mastocytosis in Children: Two Variants

Clinical Aspects of Diffuse Cutaneous Mastocytosis in Children: Two Variants

Neonatal Mastocytosis with Pachydermic Bullous Skin without c-Kit 816 Mutation

Diffuse Cutaneous Mastocytosis: Pseudoxanthomatous Variant

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