ABSTRACT
We report a case of congenital multisystem Langerhans cell histiocytosis with cutaneous and hematopoietic involvement. After the failure of first-line (vinblastine and prednisolone) and second-line (vincristine and cytarabine) therapies, treatment with cobimetinib, a mitogen-activated protein kinase (MEK) inhibitor, led to the remission of disease and a sustained response after 11 months of ongoing treatment. Protein kinase inhibitors targeting BRAF or MEK could represent a promising future therapeutic option, also in children with LCH.
Subject(s)
Azetidines , Histiocytosis, Langerhans-Cell , Piperidines , Humans , Histiocytosis, Langerhans-Cell/drug therapy , Histiocytosis, Langerhans-Cell/congenital , Azetidines/therapeutic use , Piperidines/therapeutic use , Protein Kinase Inhibitors/therapeutic use , Male , Female , InfantABSTRACT
Epithelioid fibrous histiocytoma (EFH) is a type of uncommon skin tumor mostly harboring Anaplastic Lymphoma Kinase (ALK) gene rearrangement, with different fusion partners reported. Whether this tumor is a separate entity or has a relationship with conventional fibrous histiocytomas is still a matter of debate. Benign course is the rule after complete surgical excision. A rare subtype of EFH with fusiform cells has been described, with specific fusion partners. Inflammatory myofibroblastic tumor (IMT) is a type of soft tissue tumor rarer than EFH, and it can display distant metastases. Some cases of primary cutaneous IMT included two with Cysteinyl-tRNA Synthetase 1 (CARS)-ALK rearrangement. IMT can have the same fusion partners as EFH, such as DCTN1, TMP3 or EML4 genes. We report the case of a 42-year-old woman presenting EFH with fusiform morphology harboring CARS-ALK fusion and discuss similarities and differences with IMT.
Subject(s)
COVID-19 , Chilblains , Interferon Type I , Humans , Chilblains/diagnosis , Chilblains/etiology , Chilblains/epidemiology , SARS-CoV-2 , PandemicsABSTRACT
Granulomatous dermatitis following the administration of various vaccines has previously been reported. However, cases of cutaneous granulomatosis following the measles, mumps, and rubella (MMR) vaccine have not yet been reported. We report the case of a 3-year-old boy with a granuloma annulare-like reaction following MMR vaccination.