Objective:To investigate the immunological characteristics, particularly the alterations in peripheral blood lymphocyte subsets, including Th17 and Treg cells, cytokine dysregulation, and their clinical significance in patients with SAPHO syndrome.Methods:Fifty-three patients with SAPHO syndrome admitted to the Second Hospital of Shanxi Medical University from January 2012 to December 2023 in the department of dermatology and venereology and rheumatology with complete data were retrospectively analyzed as the study objects. At the same time, 55 healthy subjects matched by age and sex were included as healthy control group. General clinical data such as age, sex, clinical manifestations and results of laboratory tests were collected. We employed flow cytometry to assess the absolute counts of peripheral blood lymphocyte subsets and utilized cytokines detected by the flow cytometry-based multiplex protein quantification technique (CBA) to measure serum cytokine levels. We compared the differences in peripheral blood lymphocyte subsets and cytokine levels between the two groups using the rank-sum test and Spearman correlation analysis.Results:① Patients with SAPHO syndrome exhibited significantly elevated absolute counts of total B cells [240.77(180.65, 303.87)/μl vs. 165.00(132.00, 223.00)/μl, Z=-3.25, P<0.001], CD8 + T cells[504.6(381.43, 735.36)/μl vs. 429.00(357.00, 579.00)/μl, Z=-2.71, P=0.007], and CD4 + T cells 898.47(755.61, 1 019.68) vs. 637.00(544.00, 819.00), Z=-3.94, P<0.001], along with reduced NK cells[212.59(123.02, 307.72) vs. 283.00(189.00, 406.00), Z=2.95, P=0.003]. Compared with healthy controls, both Th1 [159.56(105.01, 233.09)/μl vs. 47.18(9.73, 99.12)/μl, Z=-6.52, P<0.001] and Th17 cells[17.88(12.97, 23.69)个/μl vs. 5.38(4.06, 7.42)/μl, Z=-7.11, P<0.001] and the Th17/Treg ratio[0.59(0.38, 0.84) vs. 0.17(0.13, 0.29), Z=-6.85, P<0.001] were significantly higher in the CD4 + T subset, with statistical significant difference; however, no significant differences was observed in Th2[13.09(7.98, 20.60)/μl vs. 10.22 (5.36, 15.60)/μl, Z=-1.73, P=0.084] and Treg cell [30.08(22.14, 45.16)/μl vs. 33.58(22.15, 42.13)/μl, Z=0.07, P=0.985] levels between the two groups. ② Subgroup analyses based on the presence of peripheral joint involvement and skin manifestations revealed no significant differences in lymphocyte subsets among the groups ( P>0.05). ③ No significant correlation was found between Th17, Treg cells, Th17/Treg ratio, and clinical data (ESR, CRP, skin manifestations, joint symptoms) in patients with SAPHO syndrome patients( P>0.05). ④ The serum IL-2 level in patients with SAPHO syndrome was significantly lower than in healthy controls [1.74 (1.18, 2.36)pg/ml vs. 2.73(1.76, 3.49)pg/ml, Z=4.00, P<0.001], while levels of IL-6[5.72(4.63, 7.75)pg/ml vs. 3.17(2.67, 4.06)pg/ml, Z=-7.13, P<0.001], IL-10[3.15(2.29, 4.15) pg/ml vs. 2.02(1.68, 3.13)pg/ml, Z=-0.40, P<0.001]、IL-17[8.11(4.31, 11.2)pg/ml vs. 1.47(1.15, 2.88)pg/ml, Z=-5.51, P<0.001]、IFN-γ[3.79(2.93, 5.05)pg/ml vs. 1.50(1.31, 2.09)pg/ml, Z=-7.12, P<0.001]、TNF-α[2.14 (1.56, 3.11)pg/ml vs. 0.27(0.00,1.43)pg/ml, Z=-6.84, P<0.001] were markedly elevated. ⑤Correlation analysis revealed a positive relationship between IL-17 and Th17 cells ( r=0.49, P<0.001) as well as between Th17/Treg ( r=0.37, P=0.006). Conclusion:Patients with SAPHO syndrome exhibit an increased ratio of proinflammatory Th17 cells leading to immune imbalance and disturbances in proinflammatory and anti-inflammatory cytokine levels, which may contribute to disease development. The reduction in IL-2 levels indicates a deficiency in IL-2 and decreased inhibition of Th17 cells, resulting in Th17/Treg immune imbalance, suggesting that low-dose IL-2 therapy could be beneficial to patients with SAPHO.

Objective:To investigate the immunological characteristics, particularly the alterations in peripheral blood lymphocyte subsets, including Th17 and Treg cells, cytokine dysregulation, and their clinical significance in patients with SAPHO syndrome.Methods:Fifty-three patients with SAPHO syndrome admitted to the Second Hospital of Shanxi Medical University from January 2012 to December 2023 in the department of dermatology and venereology and rheumatology with complete data were retrospectively analyzed as the study objects. At the same time, 55 healthy subjects matched by age and sex were included as healthy control group. General clinical data such as age, sex, clinical manifestations and results of laboratory tests were collected. We employed flow cytometry to assess the absolute counts of peripheral blood lymphocyte subsets and utilized cytokines detected by the flow cytometry-based multiplex protein quantification technique (CBA) to measure serum cytokine levels. We compared the differences in peripheral blood lymphocyte subsets and cytokine levels between the two groups using the rank-sum test and Spearman correlation analysis.Results:① Patients with SAPHO syndrome exhibited significantly elevated absolute counts of total B cells [240.77(180.65, 303.87)/μl vs. 165.00(132.00, 223.00)/μl, Z=-3.25, P<0.001], CD8 + T cells[504.6(381.43, 735.36)/μl vs. 429.00(357.00, 579.00)/μl, Z=-2.71, P=0.007], and CD4 + T cells 898.47(755.61, 1 019.68) vs. 637.00(544.00, 819.00), Z=-3.94, P<0.001], along with reduced NK cells[212.59(123.02, 307.72) vs. 283.00(189.00, 406.00), Z=2.95, P=0.003]. Compared with healthy controls, both Th1 [159.56(105.01, 233.09)/μl vs. 47.18(9.73, 99.12)/μl, Z=-6.52, P<0.001] and Th17 cells[17.88(12.97, 23.69)个/μl vs. 5.38(4.06, 7.42)/μl, Z=-7.11, P<0.001] and the Th17/Treg ratio[0.59(0.38, 0.84) vs. 0.17(0.13, 0.29), Z=-6.85, P<0.001] were significantly higher in the CD4 + T subset, with statistical significant difference; however, no significant differences was observed in Th2[13.09(7.98, 20.60)/μl vs. 10.22 (5.36, 15.60)/μl, Z=-1.73, P=0.084] and Treg cell [30.08(22.14, 45.16)/μl vs. 33.58(22.15, 42.13)/μl, Z=0.07, P=0.985] levels between the two groups. ② Subgroup analyses based on the presence of peripheral joint involvement and skin manifestations revealed no significant differences in lymphocyte subsets among the groups ( P>0.05). ③ No significant correlation was found between Th17, Treg cells, Th17/Treg ratio, and clinical data (ESR, CRP, skin manifestations, joint symptoms) in patients with SAPHO syndrome patients( P>0.05). ④ The serum IL-2 level in patients with SAPHO syndrome was significantly lower than in healthy controls [1.74 (1.18, 2.36)pg/ml vs. 2.73(1.76, 3.49)pg/ml, Z=4.00, P<0.001], while levels of IL-6[5.72(4.63, 7.75)pg/ml vs. 3.17(2.67, 4.06)pg/ml, Z=-7.13, P<0.001], IL-10[3.15(2.29, 4.15) pg/ml vs. 2.02(1.68, 3.13)pg/ml, Z=-0.40, P<0.001]、IL-17[8.11(4.31, 11.2)pg/ml vs. 1.47(1.15, 2.88)pg/ml, Z=-5.51, P<0.001]、IFN-γ[3.79(2.93, 5.05)pg/ml vs. 1.50(1.31, 2.09)pg/ml, Z=-7.12, P<0.001]、TNF-α[2.14 (1.56, 3.11)pg/ml vs. 0.27(0.00,1.43)pg/ml, Z=-6.84, P<0.001] were markedly elevated. ⑤Correlation analysis revealed a positive relationship between IL-17 and Th17 cells ( r=0.49, P<0.001) as well as between Th17/Treg ( r=0.37, P=0.006). Conclusion:Patients with SAPHO syndrome exhibit an increased ratio of proinflammatory Th17 cells leading to immune imbalance and disturbances in proinflammatory and anti-inflammatory cytokine levels, which may contribute to disease development. The reduction in IL-2 levels indicates a deficiency in IL-2 and decreased inhibition of Th17 cells, resulting in Th17/Treg immune imbalance, suggesting that low-dose IL-2 therapy could be beneficial to patients with SAPHO.

Kaposi varicelliform eruption(KVE)refers to an infectious skin disease that occurs after in-fection with herpes simplex virus,Coxsackie virus,or other viruses on the basis of preexisting skin diseases,commonly observed in the patients with eczema.It is rare for the patients with erythroderma to be complicated with KVE,and symptoms are often atypical,which pose a challenge to the diagnosis and treatment.This article reports a case of erythroderma complicated with KVE,aiming to raise awareness of clinicians in treating this disease.

Panniculitis is a group of skin diseases involving subcutaneous adipose tissues, including primary and secondary panniculitis associated with tumors and inflammations. According to patterns of inflammation and types of fat necrosis, and combined with the learning experience at Ackerman Academy of Dermatopathology, the authors summarize pathological changes in various types of primary panniculitis, and propose some diagnostic clues, hoping to provide some ideas for the pathological diagnosis of panniculitis.

Objective To report a case of X-linked ichthyosis complicated by Mal de Meleda,and to identify the gene mutations.Methods Clinical data were collected from the patient,and peripheral blood samples were obtained from the patient,his parents and 100 unrelated healthy people who served as controls.Genomic DNA was extracted from these blood samples,and PCR was performed to amplify all the exons and their flanking sequences of the SLURP-1 and STS genes.All the amplification products were analyzed by agarose gel electrophoresis,and amplification products of the SLURP-1 gene were analyzed by DNA sequencing.Results The patient presented with regularly-arranged polygonal brown or black scales all over the trunk and limbs,erythematous hyperkeratotic lesions on the palms and soles,elbows and knees,inguinal and perianal regions,which extended to the dorsa of the hands and feet.Then,the patient was diagnosed with X-linked ichthyosis complicated by Mal de Meleda.Genetic testing showed complete deletion of the STS gene,and a homozygous mutation (c.286C ＞ T) at position 286 in exon 3 of the SLURP-1 gene,which led to the formation of a premature termination codon at amino acid position 96 (p.R96*).His parents were heterozygous carriers of the mutation (c.286C ＞ T).No mutation was found in the unrelated healthy controls.Conclusion The complete deletion of the STS gene and the homozygous nonsense mutation in the SLURP-1 gene may be the reason for X-linked ichthyosis complicated by Mal de Meleda in the patient.