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Objective:To investigate clinicopathological features and prognosis of transformed mycosis fungoides (TMF) .Methods:A retrospective analysis was performed on clinicopathological data collected from 24 patients with TMF, as well as on flow cytometry results of 16 peripheral blood samples obtained from 11 of the 24 patients, who visited Hospital of Dermatology, Chinese Academy of Medical Sciences between 2014 and 2020.Results:Among the 24 patients, 11 were males and 13 were females. Their average age at diagnosis of TMF was 50.0 years (range: 18 - 77 years), and patients with early-stage TMF (9 cases) and tumor-stage TMF (15 cases) were aged 44.8 and 52.6 years on average, respectively. The average time interval from diagnosis of MF to large cell transformation was 3.7 years, and 8 patients were diagnosed with TMF at the initial visit. Histopathologically, large cells infiltrated in a diffuse pattern in 20 cases, as well as in a multifocal pattern in 4, and the proportion of large cells in 7 cases was greater than 75%. Immunohistochemically, 18 patients showed positive staining for CD30, and the proportion of CD30-positive large cells was greater than 75% in 9; negative staining for CD30 was observed in 6. Flow cytometry of 16 peripheral blood samples showed the presence of cell subsets expressing clonal T cell receptor (TCR) -vβ in 2 of 4 patients with early-stage TMF and 10 of 12 with tumor-stage TMF, and tumor cells with higher forward scatter than normal lymphocytes were detected in 16 samples. During the follow-up, among the patients with early-stage TMF, 3 progressed to tumor-stage TMF 3.3 years on average after large cell transformation, 1 progressed to erythrodermic MF in stage IIIA, and the other 4 still showed an indolent course; among the patients with tumor-stage TMF, 1 progressed to stage-IV TMF, and 5 died 3.3 (1.5 - 6) years after large cell transformation.Conclusion:Large cell transformation may occur in patients with MF in any stage, some patients have poor prognosis, so close follow-up is needed for patients with TMF.
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Objective@#To analyze clinical and pathological features of papular elastorrhexis.@*Methods@#Clinical data were collected from 22 patients with confirmed papular elastorrhexis in Hospital for Skin Diseases, Chinese Academy of Medical Sciences and Pekin Union Medical College from September 2006 to May 2018. Clinical manifestations, pathological findings and follow-up results were retrospectively analyzed.@*Results@#The average age of onset of the 22 patients was 5.7 years (range: 1 - 10 years) , and the male to female ratio was 4.5∶1. The average duration from the occurrence of disease to the confirmation of diagnosis was 1.5 years, and no definite etiology was found. The patients had no itching or pain sensation. Skin lesions were soft, slightly elevated, well-circumscribed, round, oval or polygonal-shaped, white papules with diameters of 1 - 10 mm, and wrinkles appeared on the surface of the papule when the papule was pushed towards its center. Among the 22 patients, 16 (73%) presented with scattered lesions, 13 (59%) had less than 5 papules, and lesions were located in the trunk in 21 (95%) . Histopathological examination of skin lesions in 8 patients showed no obvious increase of collagen fibers in the superficial and middle dermis, which were normally arranged with slightly widened spaces between them. Elastic fiber staining showed that elastic fibers disappeared or were dissociated focally in the superficial and middle dermis. After confirmed diagnosis, the 22 patients received no treatment. In 18 patients, skin lesions did not continue to expand after onset, and no new skin lesions occurred. Skin lesions were slightly enlarged, but remained steady thereafter in 4 patients. Sixteen patients achieved partial remission.@*Conclusions@#Papular elastorrhexis is a rare skin disorder of elastic fibers that occurs predominantly during childhood and adolescence, and its diagnosis relies on clinical manifestations combined with histopathological findings. No special treatment is needed and the prognosis is good.
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Objective To analyze clinical and pathological features of papular elastorrhexis.Methods Clinical data were collected from 22 patients with confirmed papular elastorrhexis in Hospital for Skin Diseases,Chinese Academy of Medical Sciences and Pekin Union Medical College from September 2006 to May 2018.Clinical manifestations,pathological findings and follow-up results were retrospectively analyzed.Results The average age of onset of the 22 patients was 5.7 years (range:1-10 years),and the male to female ratio was 4.5:1.The average duration from the occurrence of disease to the confirmation of diagnosis was 1.5 years,and no definite etiology was found.The patients had no itching or pain sensation.Skin lesions were soft,slightly elevated,well-circumscribed,round,oval or polygonal-shaped,white papules with diameters of 1-10 mm,and wrinkles appeared on the surface of the papule when the papule was pushed towards its center.Among the 22 patients,16 (73％) presented with scattered lesions,13 (59％)had less than 5 papules,and lesions were located in the trunk in 21 (95％).Histopathological examination of skin lesions in 8 patients showed no obvious increase of collagen fibers in the superficial and middle dermis,which were normally arranged with slightly widened spaces between them.Elastic fiber staining showed that elastic fibers disappeared or were dissociated focally in the superficial and middle dermis.After confirmed diagnosis,the 22 patients received no treatment.In 18 patients,skin lesions did not continue to expand after onset,and no new skin lesions occurred.Skin lesions were slightly enlarged,but remained steady thereafter in 4 patients.Sixteen patients achieved partial remission.Conclusions Papular elastorrhexis is a rare skin disorder of elastic fibers that occurs predominantly during childhood and adolescence,and its diagnosis relies on clinical manifestations combined with histopathological findings.No special treatment is needed and the prognosis is good.
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Objective To discuss the diagnosis and differential diagnosis of microcystic adnexal carcinoma (MAC).Methods Totally,10 patients with MAC were enrolled from Hospital for Skin Diseases,Chinese Academy of Medical Sciences from 2003 to 2017.Their clinical manifestations,histopathological and immunohistochemical features,treatment and prognosis were retrospectively analyzed.Results Of the 10 patients,3 were males and 7 were females.Their average age at the onset of MAC was 51.65 years.Skin lesions all occurred on the face,and on the upper lip in 6 cases.The lesions usually presented as solitary plaque or nodule,and ulceration occurred in 4 cases.Histopathologically,skin lesions consisted of epithelial cords with different numbers of keratinous cysts and tubular structures,and neural involvement occurred in 6 cases.However,mitotic figures were rare.Immunohistochemical staining showed epithelial cells and keratinous cysts stained positive for cytokeratin,as well as tubular structures and glandular cavities stained positive for carcinoembryonic antigen and epithelial membrane antigen.All the patients received surgical excision,and one patient experienced in situ recurrence 13 years later.No distant metastasis occurred in these patients.Conclusions MAC mainly presents as red plaques with occasional ulceration on the upper lip.Its definite diagnosis depends on characteristic histopathological changes in bidirectional differentiation into hair follicles and sweat glands,and immunohistochemical features are helpful to distinguish MAC from other adnexal tumors.
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Objective To analyze differentially expressed proteins in A375 melanoma cells before and after short hairpin RNA (shRNA)-mediated Cbl-b gene silencing.Methods The label-free quantitative proteomics approach was performed to identify differentially expressed proteins between A375 cells transfected with lentiviral vectors containing Cbl-b shRNA (Cbl-b shRNA group) and those with control lentiviral vectors (control group).Then,the properties of differentially expressed proteins were analyzed by gene ontology (GO) and Kyoto Encyclopedia of Genes and Genome (KEGG) enrichment analysis.Western blot analysis was conducted to determine the expression of differential proteins (EphA2 and GSK3β) and phosphorylated protein kinase (p-AKT) after shRNA-mediated Cbl-b gene silencing.Statistical analysis was carried out by t test of two independent-samples for comparison of protein expression abundance between the two groups with SPSS 23.0 software.Additionally,the results of GO and KEGG enrichment analysis were analyzed by Fisher's exact test.Results A total of 3 449 proteins were identified and quantified,and 74 of them were differentially expressed between the Cbl-b shRNA group and control group.Compared with the control group,52 proteins were up-regulated and 22 were down-regulated in the Cbl-b shRNA group.GO enrichment analysis of differential proteins revealed that the top five significantly enriched biological processes were integrin-mediated cell adhesion,single-organism metabolic process,regulation of integrin-mediated cell adhesion,regulation of protein-targeting mitochondria and nucleic acid metabolic process.The top five significantly enriched molecular functions included DNA binding,2-iron,2-sulfur cluster binding,signaling receptor activity,cadherin binding and cell adhesion molecule binding.The top five significantly enriched cell components included nucleosome,DNA packaging complex,photoreceptor connecting cilium,DNA-protein complex and extracellular region part.KEGG enrichment analysis demonstrated that the top five significantly enriched melanoma-related signaling pathways were folate biosynthesis,axon guidance,extracellular matrix-receptor interaction,adherens junction and Wnt signaling pathways.As Western blot analysis revealed,the Cbl-b shRNA group showed lower protein expression of EphA2 (0.369),but higher protein expression of GSK3β (3.524) compared with the control group (1),which were consistent with the results of proteomics analysis.Additionally,the protein expression of p-AKT was down-regulated in Cbl-b shRNA group (0.453) compared with the control group (1).Conclusion Cbl-b may be involved in the occurrence of melanoma through a variety of biological pathways,and the EphA2/PI3K/AKT signaling pathway may be one important pathway.
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Objective To investigate clinical and pathological features of 6 cases of disabling pansclerotic morphea (DPM).Methods Clinical and pathological manifestations of and follow-up results in 6 patients,who were clinically and histopathologically diagnosed with DPM in the Department of Pathology,Hospital for Skin Diseases,Chinese Academy of Medical Sciences and Peking Union Medical College from 2007 to 2017,were retrospectively analyzed.Results Among the 6 patients,4 were male and 2 were female.The age of onset ranged from 3 to 10 years,with an average age of 6.5 years.The average duration from the occurrence to the confirmation of the diagnosis was 6.2 years (range,2-10 years).At all the lesional sites,skin atrophy,thining and tightness occurred,and the limbs became thin.Additionally,there were muscular atrophy and visible deep thick veins on the surface of the limbs.The contracture,deformity and dysfunction of the adjacent joints occurred in 4 cases,and the lower limbs were obviously shortened in 2 cases.Peripheral blood examination showed no increase of eosinophils or hypergammaglobulinemia.Imaging examination revealed smooth cortical bone and clear trabecular bone,and no osseous abnormality was observed.Histopathological examination of contracted skin lesions of the lower limbs revealed atrophic and thinned epidermis,hyperpigmentation in the basal layer,hyperplastic,thickened,hardened and partly homogenized collagen fibers in the middle to deep dermis,subcutaneous adipose tissue region and deep tissue of the skin.Conclusions DPM usually does not affect viscera,but often involves deep tissue of the limbs.Histopathologically,DPM is mainly characterized by obviously hyperplastic and hardened collagen fibers in the dermis and subcutaneous tissue.
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A 55-year-old male patient presented with plaques on the face for more than 20 years,and no immunodeficiency diseases were diagnosed.Skin examination showed large areas of pink plaques on the nose,bilateral cheeks and upper oral lips with slight desquamation,verrucous hyperplasia on the dorsal area of the nose,and a bean-sized verrucous protuberance on the tip of the nose.Histopathological examination of the skin lesions revealed pseudoepitheliomatous hyperplasia in the epidermis and hyphae-like structures in the stratum corneum.Moreover,there was diffuse infiltration of inflammatory cells in the dermis,which mainly included neutrophils,lymphocytes,histiocytes and multinucleated giant cells.Periodic acid-Schiff (PAS)-positive spore-like structures were observed in the multinucleated giant cells.Culture of the lesional tissues on Sabouraud dextrose agar (SDA) medium showed grey-brown villous colonies.Microculture on the potato dextrose agar (PDA) medium yielded dark septate hyphae and pycnidia filled with a large number of spores.Microsphaeropsis arundinis was identified by fungal molecular biological techniques.The patient was diagnosed with cutaneous phaeohyphomycosis caused by Microsphaeropsis arundinis.The patient was treated with CO2 laser for the removal of verrucous protuberance on the tip of the nose,and oral itraconazole capsules at a dose of 200 mg twice a day.After 3-month treatment,the skin lesions subsided and the drug was withdrew.During 6-month follow-up,no relapse occurred.
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Objective To construct a recombinant lentiviral vector carrying the casitas B-lineage lymphoma (Cbl)-b short hairpin RNA (shRNA),and to evaluate its effect on the biological behavior of A375 melanoma cells in vitro.Methods Three specific shRNAs targeting Cbl-b gene and a negative control shRNA were designed and synthesized,and recombinant lentiviral vectors were constructed.A375 cells were divided into 5 groups to be transfected with 3 kinds of lentiviral vector expressing Cbl-b genespecific shRNAs (CBLB-shRNA-1 group,CBLB-shRNA-2 group and CBLB-shRNA-3 group),a lentiviral vector containing negative control shRNA (negative control group),and an empty vector (blank control group).Real-time fluorescence-based quantitative PCR and Western blot analysis were performed to determine the silencing efficiency at 72 hours after transfection.Cell counting kit-8 (CCK-8)assay was conducted to evaluate cellular proliferative activity at 24,48,72 and 96 hours after transfection,flow cytometry to detect cell apoptosis and cell cycle at 72 hours after transfection,and Transwell invasion assay to assess cellular invasive activity at 72 hours after transfection.Results Three recombinant lentiviral vectors containing Cbl-b shRNA were constructed successfully.As Western blot analysis revealed,the CBLB-shRNA-3 showed the highest silencing efficiency.CCK-8 assay indicated that the proliferative activity of A375 cells was significantly lower in the CBLB-shRNA-3 group than in the negative control group and blank control group at 72 and 96 hours after transfection(all P ＜ 0.01).Flow cytometry showed that the apoptosis rate of A375 cells was significantly higher in the CBLB-shRNA-3 group (22.73％ ± 6.58％) than in the negative control group (6.08％ ± 1.35％,P ＜ 0.01) and blank control group (6.34％ ± 1.07％,P ＜ 0.01).The CBLB-shRNA-3 group showed a significantly higher proportion of A375 cells at G1 phase,but a significantly lower proportion of A375 cells at S phase compared with the negative control group and blank control group(all P ＜ 0.01).Transwell assay showed that there were significant differences in the number of A375 cells crossing the artificial basement membrane (matrigel) at 72 hours after transfection among the negative control group,blank control group and CBLB-shRNA-3 group (76.60 ± 1.82,73.20 ± 3.83,19.60 ± 1.14,respectively;F =794.50,P ＜ 0.01).Conclusions A recombinant CBLB-shRNA-3-expressing lentiviral vector which can efficiently silence Cbl-b gene has been successfully constructed.It can inhibit the proliferation,cell cycle progression and invasive activity of A375 cells,but promote the apoptosis of A375 cells.
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Objective To evaluate the effect ofT-cell immunoglobulin and mucin domain-3 (TIM-3) on TRP-2180-188 peptide-stimulated murine spleen lymphocytes co-cultured with B16F10 murine melanoma cells.Methods A recombinant plasmid pFUSE-TIM-3-mIgG2Aae1-Fc2 encoding TIM-3 was constructed.Then,the recombinant plasmid and an empty plasmid pFUSE-mIgG2Aae1-Fc2 were transfected into human 293T epithelial cells followed by 48-hour culture for the preparation of supernatants containing TIM-3 and Ig-tail respectively.C57BL/6 mice were immunized with the TRP-2180-188 peptide vaccine for 4 sessions.One week after the last vaccination,C57BL/6 mice were sacrificed,and spleen lymphocytes were collected and then cultured with the TRP-21180-188 peptide and interleukin-2 (IL-2) for 5 days,with lymphocytes untreated with the TRP-2180-188 peptide or IL-2 serving as the control group.Mitomycin-treated B16F10 murine melanoma cells and TRP-2180-188 peptide-stimulated lymphocytes were co-cultured with the presence of supernatants of 293T cells that had been cultured for 48 hours (blank control group),TIM-3-containing supernatants (TIM-3 group) and Ig-tail-containing supernatants (negative control group) separately.After 24 and 48 hours of co-culture,cell counting kit-8 (CCK-8) assay was performed to estimate the proliferative activity of lymphocytes,enzyme-linked immunosorbent assay (ELISA) to determine the supernatant levels of interferon (INF)-γ and tumor necrosis factor (TNF)-α,flow cytometry to determine the percentage of CD8 + T cells in the co-culture system.Results Enzyme digestion and sequence analysis showed that the TIM-3 gene was successfully inserted into the eukaryotic expression plasmid.After 48-hour culture,TIM-3 and Ig-tail expressions were detected in the supernatants of 293T cells transfected with the recombinant plasmid and empty plasmid respectively.As CCK-8 assay showed,the proliferative activity of lymphocytes was significantly lower in the TIM-3 group than in the blank control group and negative control group after 24-and 48-hour culture (78.06％ ± 6.37％ vs.100.00％ ± 10.42％ and 108.70％ ± 9.90％ at 24 hours,42.93％ ± 5.93％ vs.100.00％ ± 6.24％ and 168.00％ ± 2.98％at 48 hours,all P ＜ 0.05),so was the ratio of cellular proliferative activity at 48 hours to that at 24 hours (all P ＜ 0.05).Compared with the blank control group and negative control group,the TIM-3 group showed significantly decreased supernatant levels of IFN-γ and TNF-α after 24-hour (IFN-γ:192.96 γ 5.05 ng/L vs.216.44 ± 7.85 ng/L and 223.67 ±7.79 ng/L,both P＜ 0.05;TNF-α:58.43 ± 0.26 ng/L vs.26.43 ± 0.01 ng/L and 86.85 ± 1.12 ng/L,both P＜ 0.05) and 48-hour culture (IFN-γ:54.95 ± 0.57 ng/L vs.230.06 ± 4.23 ng/L and 167.24 ± 3.33 ng/L,both P ＜ 0.05;TNF-α:30.23 ±0.26 ng/L vs.26.84 ± 0.20 ng/L and 45.34 ± 0.22 ng/L,both P ＜ 0.05).In addition,the median percentage of CD8+ T cells was significantly increased in the TIM-3 group compared with the blank control group and negative control group after 24-and 48-hour culture (3.30％ vs.0.421％ and 2.22％ at 24 hours,4.06％ vs.0.577％ and 0.691％ at 48 hours,all P＜ 0.05).Conclusion TIM-3 in vitro can suppress the proliferative activity of and secretion of IFN-γand TNF-α by lymphocytes,but increase the percentage of CD8 + T cells in the co-culture system of TRP-2180-188 peptide-stimulated lymphocytes and B16F10 cells.
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Objective To measure the expression of CC chemokine ligand 18(CCL18)in cutaneous malignant melanoma (CMM) tissues, and to explore its clinical significance, as well as relationship with vascular endothelial growth factor (VEGF) and Ki67 antigen expressions. Methods Immunohistochemistry was performed to measure CCL18, VEGF and Ki67 expressions in 58 paraffin?embedded CMM tissue specimens, as well as CCL18 expression in 20 paraffin?embedded pigmented nevus specimens, and immunofluorescence assay to confirm the expression of CCL18 in fresh CMM tissue specimens. Correlations of CCL18 expression with CMM clinicopathologic features, VEGF and Ki67 expressions were analyzed. Results CCL18 was detected in 49 (84.48%) of 58 paraffin?embedded CMM specimens, but in none of the 20 paraffin?embedded pigmented nevus specimens, with a significant difference in the positive rate of CCL18 between the CMM group and pigmented nevus group(χ2=45.46, P0.05). In addition, the expression of CCL18 in CMM tissues was positively correlated with that of VEGF(rs = 0.727, P 0.05). Immunofluorescence assay showed CCL18 expression in the cytoplasm of tumor cells in CMM tissues. Conclusion CCL18 is highly expressed in CMM tissues, and may be involved in tumor invasion and metastasis.
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A 59?year?old female patient, who received bilateral lower limb amputation 39 years ago, presented with eczematoid changes in both lower limbs for over 20 years, and with chronic granuloma?like lesions complicated by verrucous hyperplasia for more than 10 years. There were large areas of infiltrative and proliferative lesions with exudation and peripheral erythema at the amputation sites in both knee joints. The lesions were hard with tenderness on palpation. Microscopic examination of lesional scales with 10%KOH showed negative results for fungi. However, three times of culture on the Sabouraud dextrose agar(SDA)medium all grew the same kind of fungus, and the front side and reverse side of its filamentous colony were white and orange yellow respectively. Microculture showed that linear hyaline conidiophores came out from lageniform mother cells with conidia ascending alongside. The conidia looked like dark brown eye lens, with an equatorial germ slit. Based on these findings, this fungus was identified as Arthrinium phaeospermum. Periodic acid?Schiff (PAS) staining showed scattered hyphae in the stratum corneum. The internal transcribe spacer(ITS)sequence of the isolated fungus showed 99%consistency with that of Arthrinium phaeospermum. The patient was diagnosed with cutaneous Arthrinium phaeospermum infection, and treated with oral itraconazole capsules 200 mg/d for 16 days. One month later, follow?up showed satisfactory outcomes.
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Objective To investigate clinical and pathological features of primary cutaneous CD30 + anaplastic large cell lymphoma(PC-ALCL). Methods Clinical and pathological data were collected from 7 patients with PC-ALCL and analyzed retrospectively. Results Of the 7 patients, 6 were male and 1 was female, with an average age of 52 years. PC-ALCL was characterized by solitary (n = 3)or multiple (n = 4)erythematous nodules, lumps and/or plaques with (n = 6)or without (n = 1)ulceration. Systemic involvement was observed in none of the 7 patients. Histopathological examination showed diffuse distribution of tumor cells in the dermis, which were large with rich cytoplasm and atypical nuclei. Mitotic figures were seen. An immunohistochemical study of tumor cells showed positive staining for CD30 and cytotoxic protein, but negative staining for CD20, CD56,anaplastic lymphoma kinase(ALK). Epstein-Barr virus-encoded RNA in situ hybridization was negative. Conclusions PC-ALCL is a rare primary cutaneous low-grade malignant T-cell lymphoma, which can be confirmed by clinical manifestations as well as histopathological and immunohistochemical examinations. It usually has good prognosis with rare systemic involvement and metastasis.
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Objective To investigate the clinicopathologic features and differential diagnosis ofacroangiodermatitis (AM).Methods Clinical and pathological data on 12 patients with AM were retrospectively reviewed.Results Clinical manifestations of AM consisted of circumscribed brown to violaeeous macules,plaques,nodules and ulceration.Lesions were located in bilateral legs in 6 patients,and in unilateral legs in the other 6 patients.Histopathological examination revealed an increased number of lobular or clump-shaped capillaries and small veins whose lumens were round and regular,swelling of vascular endothelial cells,and different degrees of erythrocyte extravasation,hemosiderin deposition,dermal fibrosis and sparse infiltrates of inflammatory cells.The lesions were histologically located in the superficial dermis in 3 cases,in the upper and middle dermis in 8 cases,and in the entire dermis in 1 case.Immunohistochemical studies showed that vascular endothelial cells stained positive for CD31 and CD34,while perivascular cells stained negative for CD34.Conclusions AM has specific clinical and pathological manifestations,and pathological examination is essential for the diagnosis of AM.
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Objective To explore the clinicopathologic features of pityriasis lichenoides et varioliformis acuta (PLEVA).Methods A retrospective analysis was performed.Clinical and histological data were collected from 60 patients with PLEVA.The clinicopathologic features of PLEVA were analyzed.Results Among the 60 patients with PLEVA,32 (53.3％) were aged 2-18 years,and 28 (46.7％) aged 19-49 years.Skin lesions were distributed in a diffuse pattern in 50 (83.3％) patients,in a central pattern in 2 (3.3％) patients,and in a peripheral pattern in 8 (13.4％) patients.Nineteen (31.6％) patients had a history of upper respiratory infection.Histopathological examination revealed liquefactive degeneration of basal cells and perivasculitis in the dermis in all the 60 cases,neutrophil abscess formation in the stratum corneum in 26 (43.3％) cases,keratinocyte necrosis in the epidermis in 41 (68.3％) cases,generalized liquefactive degeneration in 30 (50.0％) cases,migration of lymphocytes into the epidermis in 43 (71.6％) cases,Pautrier's microabscess formation in 2 cases,varying degrees of extravasation of erythrocytes into the epidermis in 46 (76.7％) cases,fibrinoid necrosis of blood vessel walls in the dermis in 3 cases.PLEVA progressed into granuloma fungoides in 1 patient.Twenty patients underwent immunohistochemical examination,and 3 of them showed monoclonal hyperplasia of T cells.Conclusions PLEVA has characteristic clinical manifestations,and the combination of pathological and clinical examination is the gold standard for its diagnosis.
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Objective To investigate the diagnosis and differential diagnosis of dermatofibrosarcoma protuberans (DFSP). Methods Totally, 50 patients with DFSP visiting the Institute of Dermatology, Chinese Academy of Medical Sciences from 1998 to 2014 were enrolled. The clinical manifestations, histopathological and immunohistochemical features, treatment and prognosis of DFSP were retrospectively reviewed. Results The average age at onset of DFSP was (29.5 ± 15.9)years in the 50 patients, with a mean disease duration of 9.57 years. Skin lesions most frequently occurred on the trunk(n = 33, 66.0%), followed by the extremities, head and neck. DFSP was characterized by atrophic patches or plaques in 13 cases (26.0%), multiple nodules varying in size and arising on atrophic plaques or patches in 30 cases (60.0%), single or multiple nodules arising on normal skin in 7 cases (14.0%). Histologically, the tumor consisted of uniform infiltrative spindle cells arranged in a storiform or cartwheel pattern. In addition, the tumor cells expressed CD34 and vimentin. Twenty patients experienced recurrence at the primary site after resection of skin lesions with a recurrence rate of 43.5%. No distant metastasis or death occurred in these patients. Conclusions DFSP usually has various skin manifestations, is easily misdiagnosed, and can be confirmed based on histopathological and immunohistochemical findings. Local recurrence of DFSP is common, and may occur for many times after surgical excision, but lymphatic and distant metastases are rare.
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A 44-year-old male presented with a neoplasm on the buccal side of the right nasolabial fold for more than two months.Dermatological examination showed a hemispherical bulge sized 1.5 cm × 1.5 cm with central crater-like ulceration on the buccal side of the right nasolabial fold,as well as a crescent-shaped elevation measuring 1.5 cm × 2.5 cm above the hemispherical lesion.Histopathology of the hemispherical lesion revealed irregularly downward proliferation of epidermis,crater-like holes filled with eosinophilic keratinous plug in the center which were surrounded by collar-shaped epithelial cell projections.Small neutrophil abscesses were found in the clumps of epithelial cells,and massive lymphocyte infiltration with a clear bottom boundary was observed around the proliferating epithelial cells.Histopathologic examination of the crescent lesion showed multiple irregularly-shaped lobular-like structures of various sizes with sebaceous glands at different degrees of maturity in the mid dermis,which were surrounded by proliferating connective tissue.Immunohistochemical studies showed that the squamous cells stained positive for cytokeratin (CK),CK5,CK14,CK17,carcinoembryonic antigen (CEA) and epithelial membrane antigen (EMA) in the keratoacanthoma,and the sebaceous cells for CK,CK5,CK14 and EMA in the sebaceous adenoma.The pathological diagnosis was keratoacanthoma and sebaceous adenoma.The patient was diagnosed with moderately and poorly differentiated rectal adenocarcinoma in 2008.A diagnosis of Muir-Torre syndrome presenting as keratoacanthoma and sebaceous adenoma was finally made.
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Objective To estimate the diagnostic value of interstitial infiltration pattern for early morphea.Methods Twenty-five cases of early morphea pathologically characterized by interstitial infiltration of inflammatory cells were collected from 2010 to 2012.The clinicopathological features of these cases were retrospectively analyzed.Results The average clinical course was 7.5 months.The primary manifestation was edematous dark erythematous plaques,and interstitial or mixed infiltrate of inflammatory cells was the characteristic histopathological presentation.After anti-inflammatory treatment,lesions markedly improved or disappeared in 70％ of these patients.Conclusions Interstitial infiltration of inflammatory cells is a rare histologic pattern in early morphea.To learn and recognize this pattern may be beneficial to the diagnosis and treatment of early morphea.
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Objective To investigate the expressions of Na+/H+ exchanger regulatory factor 1 (NHERF1) and β-catenin in extramammary Paget's disease tissue as well as their significance.Methods Immunohistochemistry was performed to detect the protein expressions of NHERF1 and β-catenin in paraffin-embeded tissue samples from 18 patients with in situ and 22 patients with invasive extramammary Paget's disease.Results There was a high expression of NHERF1 protein in 18 (81.82％) invasive and 7 (38.89％) in situ extramammary Paget's disease samples (x2 =7.78,P ＜ 0.01).Statistical differences were observed in the membrane expression rate and cytoplasmic or nuclear expression rate of β-catenin between invasive and in situ extramammary Paget's disease tissue samples (0 (0/22) vs.33.33％ (6/18),x2 =8.63,P ＜ 0.01; 81.82％ (18/22) vs.44.44％ (8/18),x2 =6.08,P ＜ 0.05).In extramammary Paget's disease in situ tissue samples,the expression of NHERF1 was negatively correlated with the cytomembrane expression of β-catenin (ρ =-0.488,P ＜ 0.01),but positively correlated with the cytoplasmic or nuclear expression of β-catenin (ρ =0.623,P ＜ 0.01),and there was a negative correlation between the cytomembrane and cytoplasmic or nuclear expression of β-catenin (ρ =-0.572,P ＜ 0.01).Conclusions There is an abnormal expression of NHERF1 and β-catenin in extramammary Paget's disease tissue,which may be associated with the initiation,progression,and invasion of primary extramammary Paget's disease.
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Objective To assess the relationship of methylation status of CpG islands in the promoter region of insulin-like growth factor binding protein 7 (IGFBP7) gene with the expression of IGFBP7 gene in human melanoma cell lines and primary melanocytes.Methods Primary melanocytes from human forcskin tissue as well as 4 human melanoma cell lines,including A375,M14,SK-MEL-1 and MV3,were used in this study.Bisulfite sequencing PCR (BSP) was applied to detect the methylation status of 54 CpG sites in the 5'-flanking promoter region of IGFBP7 gene in all of the melanoma cell lines and primary melanocytes.Results As hierarchical cluster analysis showed,IGFBP7-positive cells (including A375,M14 and SK-MEL-1 ) differed significantly from IGFBP7-negative cells (including MV3 cells and primary melanocytes) in the methylation pattern of IGFBP7 gene promoter region.Conclusion The methylation status of CpG island in the promoter region of IGFBP7 gene may be associated with its expression in melanoma cell lines.
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Objective To analyze the clinical and histopathologic features of eruptive microvenular hemangioma (EMVH). MethodsThe clinical and histological findings in six patients with EMVH were retrospectively reviewed.Results The patients were young or middle-aged,with a median age of onset of 34 years.The ratio of males to females was 1 ∶ 1.Clinically,all the patients experienced a sudden development of widespread,asymptomatic,brunneus,flat-topped papules and plaques measuring from 0.1 to 2.0 cm in diameter.Histopathologic examination revealed a poorly circumscribed proliferation of small,irregularly branched,thin-walled blood vessels haphazardly arranged between collagen fiber bundles in the dermis.The lumina of the blood vessels were often collapsed.There was no cellular atypia or mitosis.A variable degree of sclerosis was noted in the surrounding stroma.Immunobistochemical study revealed that the endothelial cells stained positive for CD34,CD31,and factor Ⅷ-related antigen.Conclusions The diagnosis of EMVH is proposed,and awareness of this disease may benefit its early diagnosis and avoidance of unnecessary treatment.