Background: NF-B regulation of COL1A1 in physiopathological situations is largely unknown. Results: NF-B down-regulates COL1A1 in normal and scleroderma fibroblasts, through its recruitment on COL1A1 by protein interactions with the Sp1/Sp3/c-Krox trans-activators, which are different in fibrotic fibroblasts. Conclusion: Our findings highlight a new mechanism for COL1A1 regulation. Significance: These data could allow the development of new antifibrotic strategies.
Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder of unknown origin, which occurs in children in good health. It is characterized by the multiplication of transient cutaneous papules and nodules, mainly located on the head and periarticular areas that spontaneously resolve. Histological features of SHJCM have been well described; therefore, the diagnosis is usually made easily when papules are biopsied. We report a series of 3 new cases of SHJCM presenting mainly with nodular lesions. Histological examination of these nodules showed either lesions consistent with nodular or proliferative fasciitis or nonspecific panniculitis. Mucinous deposits were present but often inconspicuous, so could be disregarded. We wanted to emphasize this misleading presentation because a biopsy for histological examination is always mandatory in cases of proliferating nodules to rule out malignant tumors. Therefore, the diagnosis always requires discussion between pathologists and clinicians to rapidly reassure the parents and avoid inappropriate therapy.
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