“…The immunophenotypic profile is identical to JXG. 406–411 Figure 20.Benign cephalic histiocytosis. This is an uncommon histiocytosis in the family of xanthogranulomas.…”
This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Because lymphoblastic leukemias and lymphomas are more frequent in the pediatric setting, cutaneous leukemic infiltrates are relatively common in this age-group. Similarly, histiocytic disorders are more common in children, particularly Langerhans cell histiocytosis and juvenile xanthogranuloma. Notably, the histiocytic disorders have undergone significant modifications on their nomenclature in the basis of the molecular characteristics that are present in them. A summary of the most frequent cutaneous hematopoietic disorders in children will be discussed further in this review.
“…The immunophenotypic profile is identical to JXG. 406–411 Figure 20.Benign cephalic histiocytosis. This is an uncommon histiocytosis in the family of xanthogranulomas.…”
This article focuses on cutaneous hematopoietic neoplasms that are more likely to be encountered in the pediatric age-group and includes both lymphoproliferative and histiocytic disorders. The cutaneous hematologic disorders in children have a different epidemiologic profile to what is seen during adulthood. Although mycosis fungoides is the most frequent form of cutaneous lymphoma in adults, it is very rare in children. Because lymphoblastic leukemias and lymphomas are more frequent in the pediatric setting, cutaneous leukemic infiltrates are relatively common in this age-group. Similarly, histiocytic disorders are more common in children, particularly Langerhans cell histiocytosis and juvenile xanthogranuloma. Notably, the histiocytic disorders have undergone significant modifications on their nomenclature in the basis of the molecular characteristics that are present in them. A summary of the most frequent cutaneous hematopoietic disorders in children will be discussed further in this review.
“…In the case of a benign cephalic histiocytosis, the lesions are usually smaller and localized (2-5 mm papules on the face and neck with residual hyperpigmentation). 6,7 Revista SPDV 73 (4)…”
Introdução: As histiocitoses são um grupo heterogéneo de doenças caracterizadas pela proliferação e infiltração dos tecidos por histiocitos. Durante muito tempo as histiocitoses foram conhecidas por diferentes nomes, reflectindo a falta de conhecimentos em relação à sua origem.Caso clínico: Uma criança aparentemente saudável de 4 meses de idade foi observada por lesão cutânea de crescimento progressivo com 1 mês de evolução. Mãe com diagnóstico de síndrome de Sjögren. Observava-se uma placa bem definida, firme, eritematosa, com 30mm diâmetro, de bordos elevados, no dorso do nariz, além de pequenas pápulas eritematosas, com 5 dias de evolução, na região malar esquerda, região torácica esquerda e braço direito. O estudo analítico mostrou anticorpos antinucleares elevados e anti-SS-A positivo. O exame histopatológico confirmou o diagnóstico de histiocitose de células não Langerhans. Surgiram progressivamente novas pápulas e placas anulares, eritematosas, dispersas pela face, tronco e membros, com resolução espontânea da lesão inicial do dorso do nariz. Cerca de 2 meses após a observação inicial, todas as lesões regrediram espontaneamente, com normalização laboratorial ao 1 ano de idade.Conclusão: Muitos autores acreditam que os diferentes subtipos de histiocitose representam apenas diferentes estadios da mesma doença. A imunofenotipagem da histiocitose nem sempre permite identificar o subtipo envolvido, pelo que são frequentes os casos atípicos.
“…[4] Electron microscopy shows that 5–30% of infiltrating cells have cytoplasm rich in comma-shaped bodies and coated vesicles. [5] Dense bodies may be present, and worm-like bodies have been described. [4]…”
A one and a half year old girl born of a non-consanguineous marriage presented with multiple asymptomatic erythematous to hyperpigmented and skin colored papules on both cheeks slowly increasing in number of 1 year duration. On the basis of clinical, histopathological, and immunohistochemistry findings, a diagnosis of benign cephalic histiocytosis was made.
scite is a Brooklyn-based organization that helps researchers better discover and understand research articles through Smart Citations–citations that display the context of the citation and describe whether the article provides supporting or contrasting evidence. scite is used by students and researchers from around the world and is funded in part by the National Science Foundation and the National Institute on Drug Abuse of the National Institutes of Health.
