Search

BackgroundPolyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy, and skin changes (POEMS) syndrome is a rare paraneoplastic disease with a high prevalence of skin symptoms. Few studies have focused on skin responses to systemic treatment of this disease.ObjectiveTo evaluate skin responses after treatment with low-dose lenalidomide plus dexamethasone and determine their relationship with vascular endothelial growth factor (VEGF) and hematological responses.MethodsA total of 41 consecutive Chinese patients who were at least 18 years of age with newly diagnosed POEMS syndrome were enrolled. 36 of them completed 12 cycles of treatment. Skin, serum VEGF, hematological and neurological responses were documented at 1, 2, 3, 6, 9, and 12 months during treatment.ResultsSkin manifestations were not associated with serum VEGF levels at baseline. Of the patients with hyperpigmentation, hemangiomas, hypertrichosis, white nails, or acrocyanosis separately, 26/34 (76.5%), 11/30 (36.7%), 14/15 (93.3%), 21/21 (100%), and 4/4 (100%) achieved complete responses at 12 months. Reduction in hyperpigmentation after 12 months reflected a good VEGF response (P = 0.017).ConclusionLow-dose lenalidomide plus dexamethasone therapy is effective in reversing skin changes in patients with POEMS syndrome.Clinical Trial RegistrationClinicaltrials.gov, identifier NCT01816620

Objective To analyze the disease constitution, accuracy of clinical and pathological diagnoses of skin biopsy samples in Peking Union Medical College Hospital. Methods A total of 29 987 patients subjected to skin biopsy were collected from Department of Dermatology, Peking Union Medical College Hospital from June 2010 to November 2018, and clinical and histopathological diagnoses of these skin biopsy samples were analyzed retrospectively. Results According to the results of histopathological diagnosis, confirmed diagnoses of these patients could be classified into 33 categories and 242 kinds. Common disease categories included epidermal tumors (2 931 cases, 9.77%) , connective tissue diseases (2 809 cases, 9.37%) , melanocytic tumors (2 078 cases, 6.93%) , erythematous scaly pustular dermatoses (1 376 cases, 4.59%) , lichenoid dermatoses (1 291cases, 4.31%) , allergic or eczematous skin diseases (1 282 cases, 4.28%) and infectious skin diseases (1 156 cases, 3.86%) . Common skin diseases included scleroderma (1 887 cases, 6.29%) , pigmented nevus (1 755 cases, 5.85%) , seborrheic keratosis (1 136 cases, 3.79%) , eczema (1 089 cases, 3.63%) , psoriasis (881 cases, 2.94%) , lichen planus (867 cases, 2.89%) , lupus erythematosus (638 cases, 2.13%) , pemphigus (549 cases, 1.83%) , and basal cell carcinoma (501 cases, 1.67%) . Poor consistency was observed between clinical diagnosis and histopathological diagnosis of lichen planus, bullous pemphigoid, granuloma annulare and hypereosinophilic dermatitis. Conclusions Common disease categories of the skin biopsy samples in Peking Union Medical College Hospital were epidermal tumors, connective tissue diseases, melanocytic tumors, erythematous scaly pustular dermatoses, lichenoid dermatoses, and allergic or eczematous skin diseases. Poor consistency was observed between clinical and pathological diagnosis in some skin diseases, and understanding of these diseases should be improved. Key words: Biopsy; Connective tissue diseases; Nevi and melanomas; Skin diseases, papulosquamous; Lichenoid eruptions; Skin diseases, eczematous

Epidermolysis bullosa acquisita (EBA) is a rare chronic subepidermal bullous autoimmune disease. The occurrence of acquired hemophilia A (AHA) is low and so the coexistence of EBA and AHA is extremely rare. We herein described a case of EBA coexisting with AHA and a case of EBA coexisting with AHA and hepatitis B. These EBA may be related to the pathogenesis of AHA. In this study, we analyzed the clinical features in the two Chinese cases of EBA coexisting with AHA, and found esophageal hemorrhage and hematemesis were the main symptoms of both patients. Cyclosporin, prednisone and lamivudine effectively control EBA with AHA and hepatitis B. The dose of cyclosporin should be more than 4 mg/kg per day and the period of treatment should be longer than 5 months to reduce the risk of EBA co-occurring with AHA.

The pathological grade of esophageal carcinoma is highly determinant of patient prognosis, but it still cannot be adequately evaluated preoperatively. Compared with conventional diffusion-weighted imaging (DWI), intravoxel incoherent motion (IVIM) diffusion-weighted MRI can separate true molecular diffusion and perfusion in tissues and has been shown to be useful in characterizing malignant tumors. There is no report that compared IVIM and conventional DWI in grading esophageal carcinoma.To prospectively determine the diagnostic performance of conventional DWI and IVIM models in differentiating the pathological differentiated grade of esophageal carcinoma.Prospective.A cohort comprising 81 patients with newly diagnosed esophageal squamous cell carcinoma (ESCC) between December 2015 and August 2017 were evaluated.3.0T, axial echo-planer imaging, fast spin echo (FSE) sequence, IVIM sequence (b = 0, 20, 50, 80, 100, 150, 200, 400, 600, 800, 1000, 1200).Apparent diffusion coefficient (ADC), true ADC (ADCGames-Howell test; diagnostic accuracy; Spearman correlation; intraclass correlation coefficient; and Bland-Altman analysis. Receiver operating characteristics (ROC) curves.ADCADC1 Technical Efficacy: Stage 2 J. Magn. Reson. Imaging 2019;49:253-261.

Background: Myeloid leukemia cutis is the terminology used for cutaneous manifestations of myeloid leukemia. Objective: The purpose of this study was to study the clinical, histopathological and immunohistochemical features of myeloid leukemia cutis. Methods: This was a retrospective study of clinical and pathological features of 10 patients with myeloid leukemia cutis. Results: One patient developed skin lesions before the onset of leukemia, seven patients developed skin infiltration within 4-72 months after the onset of leukemia, and two patients developed skin lesions and systemic leukemia simultaneously. Of these patients, five presented with generalized papules or nodules, and five with localized masses. The biopsy of skin lesions showed a large number of tumor cells within the dermis and subcutaneous fat layer. Immunohistochemical analysis showed strong reactivity to myeloperoxidase (MPO), CD15, CD43 and CD45 (LCA) in most cases. NPM1 (nucleophosmin I) and FLT3-ITD (Fms-like tyrosine kinase 3-internal tandem duplication) mutations were identified in one case. Five patients with acute myelogenous leukemia and one patient with chronic myelomonocytic leukemia died within two months to one year after the onset of skin lesions. Study limitations: This was a retrospective and small sample study. Conclusions: In patients with myelogenous leukemia, skin infiltration usually occurs after, but occasionally before, the appearance of hemogram and myelogram abnormalities, and the presence of skin infiltration is often associated with a poor prognosis and short survival time. myeloid leukemia cutis often presents as generalized or localized nodules or masses with characteristic pathological and histochemical findings.

Non-bullous congenital ichthyosiform erythroderma (NBCIE) is a hereditary disorder of keratinization caused by pathogenic variants in genes encoding enzymes important to lipid processing and terminal keratinocyte differentiation. Impaired function of these enzymes can cause pathologic epidermal scaling, significantly reduced skin barrier function. In this study, we have performed a focused, genetic analysis of a probrand affected by NBCIE and extended this to his consanguineous parents. Targeted capture and next-generation sequencing was performed on NBCIE associated genes in the proband and his unaffected consanguineous parents. We identified a homozygous nonsense variant c.814C&gt, T (p.Arg272*) in ALOXE3 (NM_001165960.1) in the proband and discovered that his parents are both heterozygous carriers of the variant. The clinical manifestations of the proband’s skin were consistent with NBCIE, and detailed histopathological assessment revealed epidermal bulla formation and Majocchi’s granuloma. Infection with Trichophyton rubrum was confirmed by culture. The patient responded to oral terbinafine antifungal treatment. Decreased skin barrier function, such as that caused by hereditary disorders of keratinization, can increase the risk of severe cutaneous fungal infections and the formation of Majocchi’s granuloma and associated alopecia. Patients with NBCIE should be alerted to the possible predisposition for developing dermatophytoses and warrant close clinical follow-up.

A 32-year-old Chinese woman presented for further investigation with a 6-year history of polyuria and polydipsia, a 4-year history of vulva ulceration and a 2-year history of gingival swelling. A biopsy of the vulva lesion showed diffuse infiltration of medium-sized cells with lobulated, grooved, vesicular nuclei. Occasional mitoses were present. The infiltrate of the cells showed reactivity with antibody to S-100 protein and CD1a, confirming the diagnosis of Langerhans' cell histiocytosis (LCH). Cephalic magnetic resonance imaging scanning revealed that an 8 x 7 x 8 mm3 large mass was located at the pituitary stalk. An X-ray film of the jaw showed sharply demarcated rarefaction of the right posterior portion of the mandible. In women with diabetes insipidus and genital ulcer, LCH should be considered in the differential diagnosis.

The current report presents an extremely rare case of a 41-year-old female with advanced gastric cancer who developed scalp metastasis during the period of systemic chemotherapy. The patient did not exhibit any rash or plaque at the initial physical examination. Following the 11th cycle of chemotherapy, the patient complained of pain on the scalp and a pink lesion was identified in the parietal region on physical examination, which increased in size and became darker and ulcerated. Pathological biopsy of the lesion and cranial magnetic resonance imaging confirmed the diagnosis of scalp metastasis. The patient succumbed to the disease one month later. The English literature was searched in the PubMed database and four cases of gastric cancer metastatic to the scalp were found. The present report discusses the common clinical presentations of these four cases in combination with the current case.

We describe a 33-year-old woman with a 2-year history of rheumatoid arthritis-like joint changes and a 1-year history of papulonodules on the fingers, ears, oral mucosa, forearm, elbows and thighs, and erythematous plaques on the buttocks. Six months after the onset of the cutaneous lesions she had dyspnoea, 3 months later she developed a husky sound. Biopsies from the nodules on the ear and left forearm revealed multinucleated giant cells with eosinophilic 'ground-glass' cytoplasm. Bronchoscopy revealed that there were nodules in the bronchus. A biopsy taken from a nodule from the bronchus was consistent with multicentric reticulohistiocytosis. Fibrostic laryngoscopy showed a mass 1.5 x 2.0 cm(2) in size on the left aryepiglottic fold and posterior commissure. Magnetic resonance imaging revealed a large pleural effusion and pericardiac effusion. Ultrasound revealed splenomegaly and peritoneal fluid. Combination therapy with prednisone, cyclophosphamide and methotrexate significantly improved cutaneous and joint symptoms. The huge cutaneous erythematous plaques and the generalized systemic involvement make this case interesting.