A 20-year-old male patient presented to the Department of Dermatology of Peking Union Medical College Hospital with complaints of an 8-year history of facial scarring, swelling of the lower limbs, and a 4-year history of scalp thickening. Physical examination showed thickening furrowing wrinkling of the skin on the face and behind the ears, ciliary body hirsutism, blepharoptosis, and cutis verticis gyrate. Both lower limbs were swollen, especially the knees and ankles. The skin of the palms and soles of the feet was keratinized and thickened. Laboratory examination using bone and joint X-ray showed periostosis of the proximal middle phalanges and metacarpals of both hands, distal ulna and radius, tibia and fibula, distal femurs, and metatarsals.Genetic testing revealed two variants in SLCO2A1, c.1658T > A and c.96+4A > C.Through multidisciplinary consultation, we confirmed the diagnosis of pachydermoperiostosis.

Epitope spreading often occurs in patients with autoimmune bullous diseases (ABDs), resulting in exposure of more antigenic epitopes, aggravation or transformation of pre-existing diseases, or concurrence of other diseases. With the increase in the immunological evidence for epitope spreading, more and more scholars have realized that epitope spreading plays an important role in the development of ABDs. This review introduces the phenomenon of epitope spreading in ABDs from 4 aspects, including the concurrence of or transformation between different types of pemphigoid, different types of pemphigus, pemphigus and pemphigoid, as well as between ABDs and other skin diseases.

Nonbullous pemphigoid (NBP) , which is related to bullous pemphigoid, has various clinical manifestations, and is frequently accompanied by itching. Typical clinical manifestations of bullous pemphigoid (BP) , such as tense blisters or bullae, are absent in NBP cases. It is easy to misdiagnose. Histopathological findings are not specific, and its diagnosis should be confirmed by direct immunofluo-rescence, indirect immunofluorescence or salt-split indirect immunofluorescence. NBP may develop into BP in some cases, and the prognosis of NBP is better than that of BP. However, delayed diagnosis usually leads to a relatively high dosage of drugs for disease control, and a high rate of adverse reactions.

A middle-aged male patient initially appeared scattered erythema with pruritus all over the body without obvious cause. According to the skin manifestations of the patient, combined with pathological diagnosis, direct immunofluorescence examination, and different serum autoantibody spectrum, the diagnosis and differential diagnosis of the patient was made by clinicians. The diagnosis of dermatitis herpetiformis was confirmed by the use of autoantibodies in the absence of any apparent history of pasta discomfort. With targeted treatment, the patient′s symptoms and laboratory indicators improved significantly.

There are overlaps in the pathogenesis of autoimmune bullous diseases (AIBDs) and psoriasis. In recent years, there have been many reports on correlations between psoriasis and AIBDs. Bullous pemphigoid is the most common AIBD associated with psoriasis, followed by pemphigus vulgaris, pemphigus erythematosus and linear IgA bullous dermatosis. AIBDs occur after the onset of psoriasis in most cases, but simultaneously with or before the onset of psoriasis in some patients. This review summarizes correlations between AIBDs and psoriasis, as well as possible mechanisms.

Thymic stromal lymphopoietin (TSLP) , a cytokine similar to interleukin-7 (IL-7) , can promote the differentiation and proliferation of a variety of cells, promote the secretion of Th2 cytokines by these cells, and plays an important role in the immune system. In recent years, abnormal expression of TSLP has been found in many skin diseases, and its level is also related to the severity of some skin diseases, suggesting that TSLP may be a potential target for the treatment of various skin diseases. This review summarizes recent research progress in the role of TSLP in the occurrence and development of various skin diseases, including inflammatory diseases, immune diseases, genetic diseases and tumors, and provides a basis and some ideas for the diagnosis and treatment of related diseases.

Objective:To investigate the relationship of eosinophil cationic protein (ECP) levels in sera and blister fluids with bullous pemphigoid (BP) .Methods:From January 2012 to October 2019, 40 patients with newly diagnosed BP and 40 healthy individuals were enrolled from Department of Dermatology, Peking Union Medical College Hospital, and serum ECP levels were detected by enzyme-linked immunosorbent assay. Meanwhile, another 33 patients with newly diagnosed BP and 41 patients with non-autoimmune bullous diseases were enrolled, and the ECP level was detected in blister fluids by enzyme-linked immunosorbent assay. Pathological sections of skin lesions of 1 patient with BP and 1 with contact dermatitis were subjected to immunohistochemical staining for ECP. Normally distributed data were compared between 2 groups by using t test or t′ test, while enumeration data were compared by using chi-square test. Pearson′s correlation coefficient was used to analyze the relationship between serum levels of ECP and proportions of peripheral blood eosinophils in patients with BP. Results:The serum level of ECP was significantly higher in the BP group (116.9 ± 19.3 ng/L) than in the healthy control group (93.3 ± 15.9 ng/L, t = 5.96, P<0.001) , and the blister fluid level of ECP was also significantly higher in the BP group (665.8 ± 189.0 ng/L) than in the non-autoimmune bullous disease group (547.5 ± 240.6 ng/L, t = 2.31, P = 0.02) . Immunohistochemical study showed more brown-yellow particles in the cytoplasm of ECP-positive cells in the BP group than in the contact dermatitis group. There was no significant correlation between the serum level of ECP and proportion of peripheral blood eosinophils in the BP patients ( r = -0.15, P = 0.35) . Conclusion:The levels of ECP in the sera and blister fluids markedly increased in the patients with BP, and blister fluid levels of ECP were much higher than serum levels of ECP, suggesting that ECP may be involved in the occurrence of BP.

Objective To investigate the correlations of the serum level of interleukin-21 (IL-21) with anti-BP 180 antibodies,peripheral eosinophil level,bullous pemphigoid disease area index (BPDAI)in patients with bullous pemphigoid (BP).Methods A total of 31 patients with bullous pemphigoid and 31 healthy controls were enrolled into this study.Blood samples were collected from all the subjects,and enzyme-linked immunosorbent assay (ELISA) was performed to detect the serum level of IL-21 and serum titer of anti-BP180 antibodies.BPDAI was assessed among 13 patients,and the correlation of the IL-21 level with anti-BP180 antibody titers,eosinophil count and BPDAI were analyzed.Results No significant difference in the serum level of IL-21 was observed between the patients (M [P25-P75]:255.28 [156.19-361.59] ng/L) and healthy controls (150.47 [72.4-268.73] ng/L,P =0.14).There was no correlation between the serum level of IL-21 and serum titer of anti-BP180 antibodies (82.98 [26.82-132.92] U/ml,rs =0.18,P =0.332).Among the 27 BP patients who underwent blood cell count,no correlation was found between the serum levels of IL-21 and whole-blood eosinophil counts (rs =0.104,P =0.606).Among the 13 BP patients who underwent assessment of BPDAI,there was no correlation between the serum levels of IL-21 and BPDAI scores (rs =0.233,P =0.464).Conclusion The IL-21 level is uncorrelated with anti-BP 180 antibody level,peripheral eosinophil level or BPDAI in patients with BP.

Objective: To establish a content determination method for ferric iron and ferrous iron in ferrous succinate tablets.Methods: Using a Dionex RFICTM protection column (4 mm×50 mm)and a Dionex RFICM Ion PacRCS5A analytical column (4 mm×250 mm), the eluent solution consisted of 7 mmol·L-1 dipicolinic acid, 66 mmol·L-1 potassium hydroxide, 5.6 mmol·L-1 potassium phosphate monobasic and 74 mmol·L-1 formic acid.The flow rate was 1.5 ml·min-1, the column temperature was 30.0℃ and the injection volume was 1.3 μl.Results: Fe3+ showed good linearity within the range of 0.5-15 μg·ml-1(r=1.000 0), Fe2+ showed good linearity within the range of 25-200 μg·ml-1(r=1.000 0), and the average recovery was 103.6%(RSD=2.7%, n=9)and 98.3%(RSD=1.9%, n=9), respectively.Conclusion: The method is simple, reliable and accurate, and can be used for the determination of Fe3+and Fe2+ in ferrous succinate tablets.

Objective To evaluate the prognostic accuracy of the score of toxic epidermal necrolysis (SCORTEN) scoring system in patients with toxic epidermal necrolysis (TEN) or Stevens-Johnson syndrome (SJS).Methods Clinical data were collected from 39 patients with SJS/TEN hospitalized in Peking Union Medical College Hospital during April 1992 and March 2014,and retrospectively analyzed.Among the 39 patients,13 had died,and the other 26 patients,who were matched to the dead patients in a ratio of 2:1 for age,all had a definite diagnosis and were discharged with improved conditions.The SCORTEN scoring system was used to evaluate the 39 patients with SJS/TEN and calculate expected mortality.The expected mortality and actual mortality were compared between different groups stratified by age in the 39 patients.The receiver operating characteristic (ROC) curve was drawn to assess the prognostic accuracy of the SCORTEN scoring system.Results According to the SCORTEN scoring system,15 out of the 39 patients scored 1 point,14 scored 2 points,6 scored 3 points,and 4 scored 4 points.The total number of expected deaths was 6.808,while that of actual deaths was 13.There was no significant difference between the expected mortality and actual mortality in every SCORTEN score-based group.The area under curve (AUC) was 0.832 8,indicating a good predictive ability of the SCORTEN scoring system.Conclusion The SCORTEN scoring system can predict mortality in TEN/SJS patients at early stage.