Objective SSc is a connective tissue disease with multisystem disorder induced by the inflammation and fibrosis following T and B cell abnormalities. Follicular helper CD4+ T (TFH) cells play a crucial role in the formation of germinal centres and specialize in interacting to aid B cell differentiation. We aimed to investigate TFH cells and their subsets to evaluate their involvement with B cell alteration in SSc. Method Circulating TFH cells (cTFH), B cells and their subsets were assessed by flow cytometry. The concentration of serum cytokines was measured by cytokine array assay. Immunohistochemistry and IF were performed to evaluate the migration of TFH cells in SSc skin lesions. Results The proportion of cTFH cells did not differ from controls, but their subsets were imbalanced in SSc patients. The frequency of TFH 1 was increased and correlated with ACA titre, serum IgM or CRP levels of patients, and cytokine concentrations of IL-21 and IL-6 that induce B cell differentiation in SSc. cTFH cells from SSc showed activated phenotype with expressing higher cytokine levels compared with controls. The frequency of TFH 17 was also increased, but was not correlated with a high level of Th17 cytokines in patients’ sera. Furthermore, infiltration of TFH cells was found in skin lesion of SSc patients. Conclusion We here describe an imbalance of cTFH toward TFH 1 that may induce B cell alteration through IL-21 and IL-6 pathways and promote inflammation, contributing to the pathogenesis of SSc disease.
Palisaded neutrophilic and granulomatous dermatitis (PNGD) is a skin disease characterized by a reactive granulomatous inflammatory pattern in the presence or absence of leukocytoclastic vasculitis. It has been associated with systemic immunological diseases, particularly rheumatoid arthritis and systemic lupus erythematosus (SLE). 1,2 The prototypical clinical lesion consists of 2-to 10-mm erythematous papules or plaques. Basil et al. 1 reported 92 cases of PNGD that had a rash with papules, plaques, and nodules, accounting for 53% of the clinical findings. In other publications, the clinical features of six cases of PNGD were described as plaques, macules, papules, and nodules, but no pustules or pustular eruptions were described. 3 Although one cannot completely exclude the possibility that pustules may have been included in the list of 'nonspecific findings' in the literature, there have been no case reports of pustules. | C A S E REP ORTA 60-year-old woman with fatigue, edema, and cough was found to have interstitial pneumonia, and treatment with prednisolone 40 mg/day was started about 1 year before the first dermatological examination. Initially, because of arthralgia, slight swelling of the fingers, and positivity of antibodies including antinuclear, anticardiolipins, and anti-U1RNP, SLE or mixed connective tissue disease was suspected. In addition, malar rash and anti-Sm antibody positivity appeared during the course of treatment. Anti-SS-A, anticentromere, anti-Topo1, anti-dsDNA, anti-MDA5 and anti-TIF1γ antibodies were all negative, and there was neither skin hardening nor muscular symptoms. Based on these findings, the patient was diagnosed with SLE. The prednisolone dosage was then gradually reduced, although skin lesions appeared when the dose was 5 mg/ day. Physical examination revealed erythema and pustules on the trunk, which later spread to the upper limbs and thighs. Suspecting folliculitis, the pustules were first treated with topical and oral antibiotics. However, since bacteriological culture of the pustules was negative, a topical steroid ointment was then applied, resulting in disappearance of the pustules, although with persistence of the erythema (Figure 1a). Histopathological examination of an area of erythema showed neutrophilic and histiocytic infiltration around degenerated collagen fibers in the upper dermis, and nuclear dust and red blood cell leakage around the blood vessels (Figure 1b,c).Immunohistochemical staining for CD68 was diffusely positive (Figure 1d). Additionally, deposits of IgM and C3 were observed by
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