Multicentric reticulohistiocytosis

Ali, Sayed; Huebner, Stephen

doi:10.1007/s00256-012-1508-0

Cited by 3 publications

(3 citation statements)

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“…The benign cephalic histiocytosis (BCH) is characterized by multiple cutaneous lesions which usually occur in very young children and are restricted to head and neck [8,110,111,162,221,223]. The lesions regress spontaneously in most patients.…”

Section: Benign Cephalic Histiocytosis General Remarks and Clinical Pmentioning

confidence: 99%

“…Pathognomonic lesions are ''coral bead'' periungual papules, papules on the pinna and vermicular lesions bordering the nostrils [223]. These may become disfiguring in some cases [8]. Both progredient and selflimited courses have been described, with very variable outcome, sometimes leading to permanent joint destructions [8,90,95,103,178,223].…”

Section: Multicentric Reticulohistiocytosis General Remarks and Clinimentioning

confidence: 99%

“…These may become disfiguring in some cases [8]. Both progredient and selflimited courses have been described, with very variable outcome, sometimes leading to permanent joint destructions [8,90,95,103,178,223]. Importantly, in a high proportion of patients, a malignant disease is pre-existent, such as hepatocellular carcinoma; occasionally hyperlipidemia or autoimmune diseases, as well as an association with M. Paget have been described [223].…”

Section: Multicentric Reticulohistiocytosis General Remarks and Clinimentioning

confidence: 99%

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The Non-Langerhans Cell Histiocytoses (Rare Histiocytoses) – Clinical Aspects and Therapeutic Approaches

2016

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Rare histiocytoses, also called non-Langerhans cell histiocytoses, include all proliferative disorders of histiocytes, macrophages and dendritic cells that are not classified as Langerhans cell histiocytosis (LCH) and do not belong to the hemophagocytic lymphohistiocytosis (HLH) group of diseases. Thus, the term includes numerous benign or malignant, localized or systemic, adult or pediatric diseases. The classification of the histiocytic disorders has been revised several times. Here, we follow the classification recently published by Jean Francois Emile and an international expert panel, defining subgroups of histiocytoses described as L-Group, C-Group, M-Group, R-Group, and H-Group, which stands for LCH-like, cutaneous or mucocutaneous, malignant, Rosai-Dorfman-Disease like and HLH like. Some of the diseases have an excellent prognosis after resection or even disappear spontanously, others progress rapidly, requiring intensive systemic therapies. The malignant non-Langerhans cell histiocytoses in general have a poor prognosis, here, complex chemotherapy protocols are usually applied, with inconsistant results. An interesting perspective in non-malignant rare histiocytoses might be small molecular inhibitors, in particular BRAF inhibitors, since BRAF mutations have been found in some subtypes of non-Langerhans cell histiocytoses. By prospective and retrospective collection of experiences in a new registry (the "International Rare Histiocytic Disorders Registry", IRHDR), knowledge about these rare diseases might hopefully be improved.

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Section: Benign Cephalic Histiocytosis General Remarks and Clinical Pmentioning

confidence: 99%