The Experts below are selected from a list of 258 Experts worldwide ranked by ideXlab platform

D D Metcalfe - One of the best experts on this subject based on the ideXlab platform.

  • Pediatric mastocytosis.
    Annals of allergy, 1994
    Co-Authors: B V Kettelhut, D D Metcalfe
    Abstract:

    The information presented will aid the practicing allergist in the recognition and management of pediatric-onset mastocytosis. Index Medicus from 1985 to present with keywords: mastocytosis; pediatrics; cutaneous. Limited to English language and to human disease. Information relative to mastocytosis in the pediatric age group to adulthood was reviewed. Mastocytosis in children is an uncommon disease and is characterized by mast cell hyperplasia and release of mast cell mediators, particularly in the skin. It generally presents during the first 2 years of life. The most common manifestation is a Solitary Mastocytoma, with urticaria pigmentosa being the next most frequent manifestation. The most common initial presenting symptom of pediatric mastocytosis is pruritus. Complications of severe mastocytosis include formation of bullae and gastrointestinal bleeding attributed to high levels of circulating plasma histamine, which in turn stimulates gastric acid secretion. Treatment of pediatric mastocytosis is largely symptomatic. Prognosis seems to be somewhat related to the severity of the disease, with children with less extensive skin involvement tending to have the best chance to have resolution of the disease by adulthood.

  • Pediatric mastocytosis.
    The Journal of investigative dermatology, 1991
    Co-Authors: B V Kettelhut, D D Metcalfe
    Abstract:

    The onset of mastocytosis occurs between birth and 2 years of age in approximately 55% of all cases; an additional 10% develop the disease before the age of 15 years. Mastocytosis in these age groups differs in many respects from mastocytosis that has its onset in adulthood. The typical presentation of pediatric-onset mastocytosis consists of cutaneous manifestations: either a Solitary Mastocytoma, urticaria pigmentosa, or, less commonly, diffuse cutaneous mastocytosis. Particularly in infants, bullous eruptions may occur. Mastocytosis in infants and children may involve internal organs, including the bone marrow and the gastrointestinal tract, although such manifestations appear to be less common in children than in adults. Plasma histamine levels may be elevated in pediatric-onset mastocytosis. Treatment usually involves the use of H1 and H2 antihistamines to control itching and to control the hypersecretion of gastric acid that may occur. The prognosis for children with mast cell disease is variable; approximately half of the children with urticaria pigmentosa may experience resolution of lesions and symptoms by adolescence.

B V Kettelhut - One of the best experts on this subject based on the ideXlab platform.

  • Pediatric mastocytosis.
    Annals of allergy, 1994
    Co-Authors: B V Kettelhut, D D Metcalfe
    Abstract:

    The information presented will aid the practicing allergist in the recognition and management of pediatric-onset mastocytosis. Index Medicus from 1985 to present with keywords: mastocytosis; pediatrics; cutaneous. Limited to English language and to human disease. Information relative to mastocytosis in the pediatric age group to adulthood was reviewed. Mastocytosis in children is an uncommon disease and is characterized by mast cell hyperplasia and release of mast cell mediators, particularly in the skin. It generally presents during the first 2 years of life. The most common manifestation is a Solitary Mastocytoma, with urticaria pigmentosa being the next most frequent manifestation. The most common initial presenting symptom of pediatric mastocytosis is pruritus. Complications of severe mastocytosis include formation of bullae and gastrointestinal bleeding attributed to high levels of circulating plasma histamine, which in turn stimulates gastric acid secretion. Treatment of pediatric mastocytosis is largely symptomatic. Prognosis seems to be somewhat related to the severity of the disease, with children with less extensive skin involvement tending to have the best chance to have resolution of the disease by adulthood.

  • Pediatric mastocytosis.
    The Journal of investigative dermatology, 1991
    Co-Authors: B V Kettelhut, D D Metcalfe
    Abstract:

    The onset of mastocytosis occurs between birth and 2 years of age in approximately 55% of all cases; an additional 10% develop the disease before the age of 15 years. Mastocytosis in these age groups differs in many respects from mastocytosis that has its onset in adulthood. The typical presentation of pediatric-onset mastocytosis consists of cutaneous manifestations: either a Solitary Mastocytoma, urticaria pigmentosa, or, less commonly, diffuse cutaneous mastocytosis. Particularly in infants, bullous eruptions may occur. Mastocytosis in infants and children may involve internal organs, including the bone marrow and the gastrointestinal tract, although such manifestations appear to be less common in children than in adults. Plasma histamine levels may be elevated in pediatric-onset mastocytosis. Treatment usually involves the use of H1 and H2 antihistamines to control itching and to control the hypersecretion of gastric acid that may occur. The prognosis for children with mast cell disease is variable; approximately half of the children with urticaria pigmentosa may experience resolution of lesions and symptoms by adolescence.

Smita Ghate - One of the best experts on this subject based on the ideXlab platform.

Isabel Betlloch - One of the best experts on this subject based on the ideXlab platform.

  • Presentation of Telangiectasia Macularis Eruptiva Perstans as a Long-Standing Solitary Plaque Associated with Renal Carcinoma
    Journal of Cutaneous Medicine and Surgery: Incorporating Medical and Surgical Dermatology, 2003
    Co-Authors: José Carlos Pascual, José Bañuls, M. P. Albares, Gloria Vergara, Isabel Belinchón, Juan Francisco Silvestre, Isabel Betlloch
    Abstract:

    Background: Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues. The skin is the most frequently affected organ. Cutaneous mastocytosis, including urticaria pigmentosa, Solitary Mastocytoma, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptiva perstans (TMEP), is usually distinguished from systemic mastocytosis. TMEP is characterized mainly by telangiectatic macules. Objective and Methods: We report a case of TMEP with an unusual clinical presentation as a Solitary plaque of telangiectatic macules. A renal clear cell carcinoma was detected in a workup for systemic mastocytosis. We discuss the clinical and histological findings and treatment of TMEP. Conclusions: TMEP is a rare form of mastocytosis, which occurs mainly in adults, generally has a good prognosis, and little tendency to urticate or show constitutional symptoms. Clinicians should consider this disorder when confronted with a progressive atypical telangiectatic lesion. However, the malignant neoplasm also found in this patient is of uncertain significance. On appelle mastocytose tout groupe de syndromes cliniques dont les signes et les symptômes sont dus à la prolifération de mastocytes dans divers tissus et organes. La peau est l’organe le plus fréquemment touché. La mastocyte cutanée se présente sous quatre types, le plus commun étant l’urticaire pigmentaire. Les autres formes sont le mastocytome solitaire, la telangiectasia macularis eruptiva perstans (TMEP) et la mastocytose diffuse. La TMEP se caractérise principalement par des macules télangiectasiques, un pronostic favorable et peu de tendance à l’urticaire et aux symptômes constitutionnels. Nous décrivons un cas de TMEP présentant une manifestation clinique inhabituelle de macules télangiectasiques individuelles. Un hypernéphrome à cellules claires a été détecté lors de l’élaboration d’un diagnostic de mastocytose systémique.

  • Presentation of telangiectasia macularis eruptiva perstans as a long-standing Solitary plaque associated with renal carcinoma.
    Journal of cutaneous medicine and surgery, 2003
    Co-Authors: José C. Pascual, José Bañuls, M. P. Albares, Gloria Vergara, Isabel Belinchón, Juan Francisco Silvestre, Isabel Betlloch
    Abstract:

    Background: Mastocytosis is a rare disease characterized by a primary pathological increase in mast cells in different tissues. The skin is the most frequently affected organ. Cutaneous mastocytosis, including urticaria pigmentosa, Solitary Mastocytoma, diffuse cutaneous mastocytosis, and telangiectasia macularis eruptiva perstans (TMEP), is usually distinguished from systemic mastocytosis. TMEP is characterized mainly by telangiectatic macules. Objective and Methods: We report a case of TMEP with an unusual clinical presentation as a Solitary plaque of telangiectatic macules. A renal clear cell carcinoma was detected in a workup for systemic mastocytosis. We discuss the clinical and histological findings and treatment of TMEP. Conclusions: TMEP is a rare form of mastocytosis, which occurs mainly in adults, generally has a good prognosis, and little tendency to urticate or show constitutional symptoms. Clinicians should consider this disorder when confronted with a progressive atypical telangiectatic lesion. However, the malignant neoplasm also found in this patient is of uncertain significance.

Guillermo Velázquez-sámano - One of the best experts on this subject based on the ideXlab platform.

  • 499 Mastocytosis: Importance as Differential Diagnosis in Skin Diseases. Report of Two Cases.
    World Allergy Organization Journal, 2012
    Co-Authors: Aida Gonzalez-carsolio, Adriana Barreto-sosa, Andres-leonardo Burbano-ceron, Andrea Aida Velasco-medina, Guillermo Velázquez-sámano
    Abstract:

    Background Is a heterogeneous disorder characterized by clonal proliferation of mast cells (MCs) leading accumulation in different organs. Pathologic activation of KIT due to a mutation in codon 816 replacing aspartic acid for valine: KIT-D816V (>93%) has been identified. Cutaneous Mastocytosis (CM), Classified in Urticaria Pigmentosa (UP), Solitary Mastocytoma, diffuse, and telangiectasia macularis eruptiva perstans (TMEP). The most common is the Urticaria Pigmentosa as fixed, reddish brown macular or papular, urticate in physical irritation (Darier's sign). WHO Diagnostic Criteria for cutaneous Mastocytosis: Presence of at least 1 of skin lesions with Focal dense MC infiltrates (>15 MCs per cluster) or diffuse (>20 cells per high-power field).