Disease relevance of Mastocytosis, Cutaneous

• Soluble mast-cell growth factor may cause the characteristic accumulation of mast cells and the hyperpigmentation of skin found in cutaneous mastocytosis [1].
• MBP was localized by immunofluorescence to mast cells in 6 of 7 nasal polyps, 4 of 4 ileal tissue specimens, and 12 of 14 cutaneous mastocytosis specimens [2].
• EXPERIMENTAL DESIGN: We used nucleic acid in situ hybridization to detect bFGF-specific mRNA transcripts within MCs in formalin-fixed, paraffin-embedded specimens of nonlesional human skin and skin containing increased numbers of MCs, including granulation tissues, benign cutaneous mastocytosis, and solitary neurofibromas [3].
• CONCLUSION: Elevated histamine excretion in biopsy-proven cutaneous mastocytosis may be associated with preterm labor [4].
• BACKGROUND: Telangiectasia macularis eruptiva perstans, a rare form of cutaneous mastocytosis associated with elevated histamine excretion, has not previously been reported to complicate pregnancy [4].

High impact information on Mastocytosis, Cutaneous

• Altered metabolism of mast-cell growth factor (c-kit ligand) in cutaneous mastocytosis [1].
• Abnormalities of the SCF-kit signaling pathway, with increased local concentrations of soluble SCF, have been implicated in the pathogenesis of the human disease cutaneous mastocytosis, but have not yet been shown to play a causal role [5].
• In addition, reverse transcription-polymerase chain reaction was used to investigate levels of bcl-2 and bcl-xL mRNA in cutaneous mastocytosis lesions [6].
• In the present study, we have assessed the BM microvessel density (MVD) by CD34 immunohistochemistry in 21 patients with SM, 5 with cutaneous mastocytosis (no BM infiltrates), and 5 control cases (normal BM) [7].
• Furthermore, by immunoelectron microscopy MBP was localized to mast cell granules in cutaneous mastocytosis lesions [2].

Chemical compound and disease context of Mastocytosis, Cutaneous

• Treatment of bullous mastocytosis with disodium cromoglycate [8].
• Symptomatic therapy of cutaneous mastocytosis involves agents that inhibit the release of mediators or antagonize H1 and H2 receptors such as antihistamines ketodifen and Aspirin [9].
• The excretion of MH but not MIMA increased in four patients (ages 37, 45, 61 and 65 years) and decreased in two patients (ages 26 and 48 years), including the only patient with paediatric-onset cutaneous mastocytosis [10].
• Serine proteinases in human cutaneous mastocytosis [11].
• Bullous mastocytosis treated with oral betamethasone therapy [12].

Gene context of Mastocytosis, Cutaneous

• Neoplastic mast cells in all cases of cutaneous mastocytosis retained their VMAT2 positivity [13].
• Congenital bullous mastocytosis with myeloproliferative disorder and c-kit mutation [14].
• Our case presented the triad clue for the diagnosis, characterized by follicular hyperkeratosis, prematurity and perinatal complications, but the clinical diagnosis was further complicated by hypereosinophilia and a strongly positive Darier's sign suggesting diffuse cutaneous mastocytosis [15].
• She has short stature, microcephaly, conductive hearing loss, skin mastocytosis and microtia [16].
• We performed polymerase chain reaction and direct sequencing using genomic DNA samples from 16 nonfamilial Japanese patients with indolent cutaneous mastocytosis (12 with childhood-onset disease and 4 with adult-onset disease) to look for the most common c-kit mutations at codons 816, 560, 820, and 839 [17].

References