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.
alterations resulting from symptomatology, such as decreased overall activity level, as well as increased systemic inflammation, similar to that which may be observed in other predominantly cutaneous diseases, like psoriasis.Limitations of the study include the retrospective methodology: only recorded comorbid diagnoses, BMI and laboratory data collected in the electronic medical record were available for review. We were unable to grade the severity of LS or associated comorbidities or assess the effect of LS-specific treatments on the risk of cardiovascular comorbidities. Our patient population came from a tertiary referral centre and was overwhelming composed of white women, which may limit generalizability. Additionally, the matched cohort was selected from dermatology and gynaecology outpatients who did not carry a diagnosis of LS, rather than the general population, although how these populations may differ, including rates of cardiovascular comorbidities, is uncertain. Further prospective investigation is warranted to more precisely determine the pathogenesis of these risks and the effect of intervention on risk reduction.
BP. 6,7 Our study showed a direct positive correlation between TARC levels and disease activity scores in BP patients for the first time. In particular, TARC levels correlated with urticaria/ erythema scores of the BPDAI score; this may reflect the inflammatory pathogenic response in BP similar to the Th2 response in atopic dermatitis. 8 Notably, a longitudinal analysis of our cases showed that TARC levels sensitively reflected skin flare-ups and were upregulated before the increase in anti-BP180 Ab levels in some cases. Serum TARC levels sharply reflect disease activity in BP with high sensitivity and across a wide range of changes. In conclusion, these results suggest that in clinical practice, TARC levels could usefully predict disease activity in patients with BP.
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