ABSTRACT
Zinc deficiency has long been known as acrodermatitis enteric dermatitis (congenital zinc deficiency). On the other hand, acquired zinc deficiency has attracted attention as a familiar disease in recent years. Epidemiological studies in Japan have shown that acquired zinc deficiency is more common than expected. It is also known that serum zinc levels fall markedly with age. In this report, several cases of acquired zinc deficiency that caused cheilitis are described. In all cases, the only symptom was cheilitis, the serum zinc level was low, and all cases were relieved by zinc supplementation. Zinc deficiency is associated with a range of pathological conditions, including mucocutaneous symptoms, delayed wound healing, dysgeusia, anemia, impaired immunity, and retarded growth development disorders. However, zinc deficiency may be overlooked even in cases of cheilitis alone. Especially in intractable cases, it is important to suspect zinc deficiency as one at the differential diagnoses.
Subject(s)
Acrodermatitis , Cheilitis , Humans , Cheilitis/etiology , Cheilitis/complications , Acrodermatitis/diagnosis , Acrodermatitis/etiology , Zinc , Intestine, Small , JapanABSTRACT
The neonatal Fc receptor (FcRn) plays a role in trafficking IgG and albumin and is thought to mediate intravenous immunoglobulin (IVIG) therapy for certain diseases. IVIG can be used for the treatment of human Langerhans cell histiocytosis (LCH); however, the mechanism remains unclear. The expression and function of FcRn protein have not been studied in LCH, though the expression of FcRn messenger RNA (mRNA) have been reported. In this report, we confirmed the expression of FcRn in 26 of 30 pathological cases (86.7%) diagnosed immunohistochemically as LCH. The expression was independent of age, gender, location, multi- or single-system, and the status of BRAFV600E immunostaining. We also confirmed the expression of FcRn mRNA and protein in the human LCH-like cell line, ELD-1. FcRn suppressed albumin consumption and growth of IVIG preparation-treated ELD-1 cells, but not of IVIG preparation-untreated or FcRn-knockdown ELD-1 cells. In addition, FITC-conjugated albumin was taken into Rab11-positive recycle vesicles in mock ELD-1 cells but not in FcRn-knockdown ELD-1 cells. IVIG preparation prolonged this status in mock ELD-1 cells. Therefore, ELD-1 recycled albumin via FcRn and albumin was not used for metabolism. Our results increase our understanding of the molecular mechanism of IVIG treatment of LCH.
Subject(s)
Histiocytosis, Langerhans-Cell , Histocompatibility Antigens Class I/metabolism , Immunoglobulins, Intravenous , Receptors, Fc/metabolism , Adolescent , Adult , Albumins/metabolism , Cell Line , Child , Child, Preschool , Female , Fluorescein-5-isothiocyanate/analogs & derivatives , Fluorescein-5-isothiocyanate/metabolism , Histiocytosis, Langerhans-Cell/drug therapy , Histiocytosis, Langerhans-Cell/metabolism , Humans , Immunoglobulins, Intravenous/metabolism , Immunoglobulins, Intravenous/pharmacology , Infant , Infant, Newborn , Male , Serum Albumin/metabolismABSTRACT
Signaling lymphocytic activation molecule family member 8 (SLAMF8) / B-lymphocyte activator macrophage expressed/CD353 is a member of the CD2 family. SLAMF8 suppresses macrophage function but enhances the growth of neoplastic mast cells via SHP-2. In this study, we found that some anaplastic large cell lymphoma (ALCL) samples were immunohistochemically positive for SLAMF8. However, we found no significant differences between SLAMF8-positive and SLAMF8-negative ALCL samples with respect to age, gender, site, or prognosis. We also identified SLAMF8 expression in ALCL cell lines, Karpas299, and SU-DHL-1. SLAMF8 knockdown decreased the activation of SHP-2 and the growth of these cell lines, and increased the apoptosis of these cell lines. In addition, we observed the interaction between SLAMF8 and SHP-2 in these cell lines using the DuoLink in situ kit. Taken together, these results suggest that SLAMF8 may enhance the growth of ALCL via SHP-2 interaction.
