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Granulomatous Diseases
Published in Ayşe Serap Karadağ, Lawrence Charles Parish, Jordan V. Wang, Roxburgh's Common Skin Diseases, 2022
Albert Alhatem, Robert A. Schwartz, Muriel W. Lambert, W. Clark Lambert
Mastocytoma(s) of the skin affects infants and young children. It resolves spontaneously by the teenage years. Their tendency to suddenly undergo release of vasoactive secretions, predominately serotonin, can produce flushing reactions and dangerous drops in blood pressure.
Skin
Published in A. Sahib El-Radhi, Paediatric Symptom and Sign Sorter, 2019
Mastocytosis is a group of disorders that are characterised by an accumulation of mast cells in the skin and other organs. Mastocytoma: a benign cutaneous tumour occurring exclusively in infancy; urticaria pigmentosa: multiple small salmon-coloured or red, cutaneous papules; systemic mastocytosis: mast cell infiltrates in the skin, lymph nodes, liver, spleen, bone and GI tract.
Skin
Published in A Sahib El-Radhi, James Carroll, Paediatric Symptom Sorter, 2017
A Sahib El-Radhi, James Carroll
Mastocytosis is a group of disorders that are characterised by accumulation of mast cells in the skin and other organs. Mastocytoma: a benign cutaneous tumour that occurs exclusively in infancy; urticaria pigmentosa: multiple small salmon-coloured or red cutaneous papules; systemic mastocytosis: mast cell infiltrates in the skin, lymph nodes, liver, spleen, bone, gastrointestinal tract.
Mastocytosis and related entities: a practical roadmap
Published in Acta Clinica Belgica, 2023
Michiel Beyens, Jessy Elst, Marie-Line van der Poorten, Athina Van Gasse, Alessandro Toscano, Anke Verlinden, Katrien Vermeulen, Marie-Berthe Maes, J. N. G. Hanneke Oude Elberink, Didier Ebo, Vito Sabato
As mentioned in the introduction, mastocytosis comprises multiple variants as defined by the WHO [13]. As shown in Table 1, mastocytosis is classified into four main categories: cutaneous mastocytosis (CM), a generally benign skin-limited disease, MC sarcoma, extracutaneous mastocytoma and systemic mastocytosis (SM) with distinct grades of aggressiveness, viz. bone marrow mastocytosis (BMM), indolent systemic mastocytosis (ISM), smouldering systemic mastocytosis (SSM), aggressive systemic mastocytosis (ASM) and mast cell leukemia (MCL) all of which might be accompanied with another hematologic non-mast cell lineage neoplasm which results in another distinctive subclassification. In CM, systemic involvement is absent. CM is further subdivided into three variants based on clinical findings. If systemic involvement is confirmed, SM is further subdivided into five different variants based on clinical and biochemical findings. An overview of all variants is presented in Table 1.
Lipotoxicity-associated inflammation is prevented by guarana (Paullinia cupana) in a model of hyperlipidemia
Published in Drug and Chemical Toxicology, 2021
Jader B. Ruchel, Viviane M. Bernardes, Josiane B. S. Braun, Alessandra G. Manzoni, Daniela F. Passos, Lívia G. Castilhos, Fátima H. Abdalla, Juliana S. de Oliveira, Cinthia M. de Andrade, Emerson A. Casali, Ivana B. M. da Cruz, Daniela B. R. Leal
Cytokine release appears to be mediated primarily by A2B receptor activation. A2B receptor stimulation in human mastocytoma HMC-1 cells induces secretion of Th2 cytokines, such as IL-4 as well as a number of other pro-inflammatory cytokines. Although it is not clear whether adenosine receptor activation stimulates the degranulation of human mast cells, increased IL-4 and IL-13 production, following adenosine receptor activation, strongly implicates human mast-cell adenosine receptors as important players in the pathophysiology of human disease (Hua et al.2007). On the other hand, methylxanthines raise intracellular cAMP concentrations. Elevated levels of intracellular cAMP suppress inflammatory and cell function, which may underlie the anti-inflammatory effects of methylxanthines (Escofier et al.1999).
Recent advances in our understanding of mast cell activation – or should it be mast cell mediator disorders?
Published in Expert Review of Clinical Immunology, 2019
Theoharis C. Theoharides, Irene Tsilioni, Huali Ren
In this subcategory, affected skin most commonly presents as urticaria pigmentosa (UP) or diffuse CM, and less frequently as bullous CM or solitary mastocytoma [96,116]. Different from adults with CM, bone marrow biopsy is not recommended for children [117,118]. It is generally considered that pediatric CM regresses by puberty in most children [116], but there have not been any systematic longitudinal studies to prove this point. However, increased serum baseline total tryptase identifies children at risk for SM [119], as does the presence of maculopapular cutaneous mastocytosis [120] [121] or detection of KIT D816V in peripheral blood [122]. It is interesting that KIT D816V is not present in most skin lesions suggesting that there may be committed stem cells outside the bone marrow. In this case, the molecules triggering mast cell proliferation in the skin may be NGF [123], RANTES [124], or even CRH acting on precursors in the hair follicle mesenchyme [125]. It is interesting that new UP lesions in children often occur in areas of trauma.