Adult Still's disease (ASD) is a rare disorder of unknown aetiology, characterized by an evanescent, erythematous, maculopapular rash, fever, arthralgia, and a variety of systemic features. We report a case which illustrates the typical features of ASD, and manifests the hitherto unreported complication of diffuse cutaneous mucinosis.
SUMMARYThe IL-I cytokine network in epidermal cells was studied in vitro, using the spontaneously transformed HaCAT human keratinocyte line. Intracellular (ic) IL-la and IL-1 receptor antagonist protein (IL-lRa) following cell lysis were readily identified assayed using a capture ELISA; whereas in culture supematants IL-lRa was not detected, and IL-Uv was present at only very low levels. Confiuent cultures of HaCAT cells were shown to provide optimal conditions for the study, since confluence increased the icIL-lRa: IL-la ratio to a level as seen in vivo, which was independent of Ca^ ^ concentration in the culture medium. The IL-lRa extracted from HaCAT cell lysates was functionally active, as demonstrated in the mouse thymocyte co-proliferation assay which could be blocked using a rabbit anti-IL-lRa antibody. Transforming growth factor-beta (TGF-/?1) stimulated a dose-dependent increase in HaCAT cell 1L-I(v without changing lL-lRa concentration, with a resultant reduction in the iclL-lRa: IL-IQ ratio from 320:1 to 100:1. Similarly. TGF-Q. interferon-gamma (IFN-7). IL-6, and tumour necrosis factor-alpha (TNF-a) substantially increased HaCAT cell IL-IQ, but had no efFect on the IL-IRa. with a concomitant reduction in theicIL-lRa: IL-la ratio. In contrast to their effects on monocytes, IL-4 and IL-IO at biologically active levels had no effect on IL-1«, IL-lRa or the icIL-lRa : IL-la ratio in confiuent HaCAT cells. Hydrocortisone reduced IL-ln to below the limit of sensitivity of the ELISA, and induced a small increase in IL-lRa of questionable biological significance. Thus, regulation ofthe IL-1 cytokine network in keratinocytes involves modulation of icIL lu rather than of icIL-lRa levels, and is markedly different from that noted in monocytes.
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