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Objective To report a case of hemophagocytic lymphohistiocytosis syndrome (HLH) secondary to systemic hydroa vacciniforme lymphoproliferative disorder (systemic HV-LPD) and review relevant literature, in order to improve understanding of the disease. Methods Clinical data of the case, laboratory examination, histopathology, immunohistochemistry, TCR gene rearrangement, and whole exon gene sequencing were collected for analyses. Results A 19 years old man was admitted to the hospital due to generalized papules, blisters, necrotic scabs and fever for 7 months. The clinical manifestations included hydroa vacciniforme-like rashes, lymphad-enopathy, splenomegaly, pancytopenia, hypertriglyceridemia, decreased NK cell activity, high levels of sIL-2r, positive for VCA-IgA and EA-IgA, and elevated EBV DNA load. The whole exon sequencing did not show pathogenic gene mutations. Histopathological changes in the skin lesions included liquefaction degeneration of epidermal and follicular basal cells, pigmentary incontinence, lymphocytic infiltrates of the epidermis and follicular epithelium, perivascular and perifollicular infiltrates of lymphocytes and eosinophils in the dermis, extravasation of erythrocytes, as well as focal infiltrates of atypical lymphoid cells around appendages and small blood vessels. Pathologic mitosis was visible. Immunohistochemistry showed CD3 (+), CD5 (+), CD20 (individual +), CD30 (small patch +), CD79a (individual +), CD4 (+), CD7 (+), CD8 (+), TIA-1 (+), CD56 (scattered lymphocyte +), ≈ 50% of Ki-67 (+), and in situ hybridization EBER (+). TCRβ gene monoclonal rearrangements were detected. According to the results of clinical and auxiliary examination, patient was diagnosed with HLH secondary to systemic HVLPD. After 8 months of follow-up, the patient passed away while waiting for transplantation of allogeneic hematopoietic stem cells. Conclusions Systemic HVLPD is rare and requires vigilance for the development of secondary hemophagocytic syndrome. The awareness of hemophagocytic syndrome should be improved to avoid possible misdiagnosis and delayed treatment. [ABSTRACT FROM AUTHOR]

Byline: Jiao. Zhang, Ruzeng. Xue, Eryi. Lin, Huiqin. Pan, Yongfeng. Chen Sir, Lichen sclerosus is a chronic mucocutaneous inflammatory disorder that preferentially affects female genitalia. It is characterized clinically by [...]

Byline: Yanan. Jiang, Ruzeng. Xue, Weijia. Wang, Yongfeng. Chen Sir, According to the World Health Organization-European Organization for Research and Treatment of Cancer (WHO-EORTC), folliculotropic mycosis fungoides (FMF) is categorized [...]

Background: Quick and accurate diagnosis of primary cutaneous amyloidosis (PCA) may be difficult because its symptoms are often subtle and nonspecific. Objective: We sought to review the literature on the roles of various staining methods in the diagnosis of amyloidosis and demonstrate added benefits of using rapid 4,6-diamidino-2-phenylindole (DAPI) staining in the diagnosis of PCA. Methods: Three groups of cases, namely, PCA, neurodermatitis, and prurigo nodularis, were retrieved from a computerized pathology database for study, and their paraffin-embedded tissue blocks were cut following standard procedures. The tissue sections were stained with three stains: hematoxylin-eosin (HE), Congo red, and DAPI stains, and examined under the microscope to compare the staining patterns of these three methods. We also performed amyloid keratin and apolipoprotein E (APOE) staining on the sections of PCA in order to further support our conclusion. The PCA sections were read by junior and senior dermatopathologists for comparison. Results: The sensitivity of DAPI staining for PCA was significantly higher than that of Congo red staining and HE staining (p < 0.001). This statement holds true whether the experiment was grouped in one sample or was divided into groups of junior and senior dermatopathologists (p < 0.001). The DAPI-positive staining areas, except for the nuclei, were consistent with the amyloid deposition areas. In this study, DAPI staining had a sensitivity of 98.6% and a specificity of 100%. Conclusion: DAPI staining could serve as a useful technique to establish the diagnosis of PCA, and its high efficacy in diagnosing PCA makes it less dependent on the experience levels of the evaluators. Additionally, the binding of DAPI to the A-T-rich sequence of double-stranded DNA suggests that amyloid may contain DNA or a similarly structured nucleic acid. [ABSTRACT FROM AUTHOR]

Byline: Ruzeng. Xue, Wei. Su, Xiaoping. Pei, Lining. Huang, Amira. Elbendary, Zhiqiang. Chen Sir, Verruciform xanthoma is a rare benign mucocutaneous lesion which was first described by Shafer in 1971. [...]

Byline: Ruzeng. Xue, Liyan. Yuan, Huaiqiu. Huang Sir, Papular elastorrhexis is a rare disorder of elastic tissue that occurs predominantly during adolescence. It was first described by Bordas et al [...]

Buschke‐Ollendorff syndrome (BOS; OMIM 166700) is a rare autosomal dominant disorder characterized by the existence of connective tissue nevus and/or osteopoikilosis. The skin lesions usually present as firm, yellow, or flesh‐colored papules and nodules, which may coalesce into plaques and increase in size and number over time. We present a case of a 26‐year‐old male with multiple subcutaneous nodules on the waist and thigh for more than 20 years. Being deeply seated, his skin lesions were not visible and could only be appreciated by palpation. Accordingly, pathology showed an increase in thick, crossed, or paralleled, elastic fibers arranged between the collagen bundles in the lower part of the reticular dermis and the subcutaneous fat with mucin deposition. Heterozygous point mutation in exon 8 of the LEMD3 gene was detected, which confirmed the diagnosis of BOS. The deeply situated nature of skin lesions noted in our case has not been reported in the literature of BOS. Our case thus expands the clinical and pathological features of the disease. [ABSTRACT FROM AUTHOR]

Early syphilis can rarely cause erythema multiforme-type eruptions as well as triggering erythema multiforme (EM). EM-like lesions in secondary syphilis are characterized by clinical features of EM and laboratory tests consistent with secondary syphilis and the skin histology shows predominantly a plasma cell infiltrate with the presence of treponemes. When EM is triggered by early syphilis, the skin histology shows mixed inflammatory cells usually in the absence of treponemes in the skin lesion. There may also be mixed histology with the presence of treponemes in the absence of a plasma cell infiltrate and vice versa. We describe a case of secondary syphilis presenting as EM with bullae and histology showing EM features without a plasma cell infiltrate but positive for Treponema pallidum by immunohistochemical staining. The patient was also coinfected with cytomegalovirus, human immunodeficiency virus, and anal warts. The EM eruptions resolved with treatment for secondary syphilis with benzathine penicillin G. [ABSTRACT FROM AUTHOR]