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Skin and Connective Tissue Diseases - Top 30 Publications

Ankylosing spondylitis associated with Still's disease: should it be considered a pathophysiologic link or a simple association?

Ankylosing spondylitis is a chronic inflammatory rheumatism; it is part of the group of spondyloarthrites. General signs such as fever and weight loss are of little importance. Adult Still's disease is a rare systemic condition, a diagnosis of exclusion commonly characterized by high hectic fever, rash, arthritis and various systemic manifestations. Few cases of ankylosing spondylitis associated with adult Still's disease have been described in the literature. We here report the case of a 31-year old patient followed up for ankylosing spondylitis presenting with fever which had lasted for a long time and clinico-biological signs compatible with adult Still's disease. A possible pathophysiologic link between the two diseases may be suggested, even if their simultaneous occurrence has been rarely reported in the literature.

Bullous pemphigoid and Parkinson's disease: about a case.

Bullous pemphigoid (BP) is an organ-specific autoimmune disease which can be associated with many pathologies including degenerative neurological diseases such as Parkinson's and Alzheimer's disease. Studies have suggested the possibility of cross-immune responses through "epitope spreading". Bullous dermatosis has been reported to be secondary to neurological disease, at intervals ranging from a few months to a maximum of a few years. Then is it an unusual association or a causation? It has been suggested that drug consumption, decubitus lesions, traumatic events as well as reduced immunity can be triggers for BP in patients with neurological disease. We report the case of a 93-year old patient with a 10-year history of advanced Parkinson's disease, hospitalized for the treatment of a common bullous pemphigoid confirmed by histology and immunohistochemistry. The patient was treated with oral corticosteroids. After a week of treatment, the patient died due to septic shock. Neurological disorders represent a real risk factor for BP. BP could be considered as a marker for neurological disorder. These associations are of broad interest, because they can play a role in the etiopathogenesis of BP and contribute to a complete understanding of the causes of these neurodegenerative diseases.

Xanthelasmoid mastocytosis: a rare form of cutaneous mastocytosis.

Mastocytosis is a rare disease characterized by the abnormal accumulation of mast cells in the skin and possibly in other organs. It can occur in a variety of forms; xanthelasmoid mastocytosis(XM) is a very rare form classified as papulo-nodular. Clinically, it appears as buff-yellow soft papules or nodules of variable size. Triggerings are those of classic mastocytosis. Darier's sign is often absent. Histology shows dense infiltrate of mast cells in the deep dermis. This clinical form can be singled out because of the persistence of lesions beyond puberty without the additional risk of systemic involvement. We here report the case of a 18-month old female infant, with no previous medical history. Maculopapular, intensely pruritic, ovalaires, brownish lesions with a buff-yellow centre, with elastic consistency and different sizes first occurred at the age of 8 months. The initial interview of parents revealed that the infant had had episode of flush especially caused by warmth. Darrier's sign was negative. Skin biopsy showed dermal infiltrate of mast cells, allowing to retain the diagnosis of xanthelasmoid mastocytosis. Complementary examinations as well as trypsin dosage were normal. Treatment was based on the exclusion of drugs and of factors participating in mast cells degranulation and antihistamines.

Bean's syndrome in children: about two cases.

Diffuse angiomatosis or Bean's syndrome is a rare disease characterized by venous malformations mainly involving the skin and the digestive tract which can result in hemorrhage of variable severity. This study reports the case of two children aged 5 and 9 and a half years respectively with diffuse angiomatosis who had been treated in the Department of Emergency Paediatric Surgery over the years. The diagnosis was based on rectal bleeding and/or melenas causing severe anemia requiring regular transfusions in both patients as well as skin angiomas occurrence at the level of the limbs. Radiological evaluation showed the presence of multiple lesions at the level of the jejunum and ileum consistent with small intestinal angiomatosis in the child aged 9 and a half years. It didn't show abdominal lesions in the child aged 5 years. The two patients were admitted to the operating block. Angiomas were surgically individualized. Some of them were actively bleeding. Enterotomy was performed. Postoperative sequelae was marked by the stop of the bleedings. This study aims to update the current understanding of this rare pathology as well as the benefit of surgical treatment in controlling the complications caused by this pathology and in reducing the frequency of transfusions.

Primary Sjögren's Syndrome.

Case 7-2018: A 25-Year-Old Man with New-Onset Seizures.

Prevention of pharyngocutaneous fistula in salvage total laryngectomy: role of the pectoralis major flap and peri-operative management.

This study aimed to evaluate the impact of an onlay pectoralis major flap in reducing the incidence of pharyngocutaneous fistula after salvage total laryngectomy and determine the complications of pectoralis major flap reconstruction.

Aberrations and clinical significance of BRAF in malignant melanoma: A series of 60 cases in Chinese Uyghur.

Malignant melanoma (MM) is a highly malignant melanocytic tumor, it occurs mostly in the skin, the mucous membrane close to the skin, but also in the tunicae rhagoides and the pia mater. The Uyghur is the largest ethnic group living in the Xinjiang Uyghur Autonomous Region of China, accounting for 46% of the total population of 20 million. Large-scale studies on MMs in Asian countries are limited. This study aimed to investigate BRAF mRNA expression and mutations in Chinese Uyghur patients with MMs and to identify the clinical features associated with these parameters.Formalin-fixed, paraffin wax-embedded tumor sections from 60 MMs were analyzed for BRAF expression using reverse transcription polymerase chain reaction (RT-PCR). Exons 11 and 15 of BRAF were analyzed for the presence of mutations using PCR and DNA sequencing. Sixty MMs were followed by mobile phone for survival analysis.BRAF mRNA expression was higher in MMs than in pigmented moles and normal skin tissues. Fourteen of 60 MMs had BRAF mutations. The frequency of BRAF mutations was significantly higher in patients younger than 60 years (10/28, 4/32, P = .02). A significant difference was observed in the frequency of BRAF mutations among specimens of mucosal, acral, chronic sun-induced damage (CSD), and non-CSD MMs (2/10, 3/19, 8/25, 1/6, P = .002). No significant association was found among BRAF mutations, sex, ulceration, or lymph node metastasis. MMs lymph node metastasis (hazard ratio 2.54 [95% confidence interval 1.062 - 6.066], P = .01) affected survival.This study indicated that BRAF mutations and expression might serve as independent adverse prognostic factors in melanoma.

Ultrasonographic manifestations of a rare granular cell tumor of the accessory breast: A case report.

The ultrasound manifestations of granular cell tumor (GCT) is a consequence of the histopathological characteristic of the tumor and can be distinguished from breast cancer.

Nursing care of a boy seriously infected with Steven-Johnson syndrome after treatment with azithromycin: A case report and literature review.

Stevens-Johnson syndrome (SJS) is an acute blistering disease of the skin and mucous membranes. SJS in children is not common but potentially serious disease. But the epidemiology of SJS in China is not well defined.

Induced abortion and breast cancer: An updated meta-analysis.

Different epidemiological studies have indicated conflicting information about the association of induced abortion (IA) with breast cancer risk. A recent meta-analysis with prospective evidences did not support the positive association between IA and breast cancer risk. Thus, we in our meta-analysis study have tried to analyze this specific association.We searched all relevant articles from an English-language literature using Pubmed, Embase, and Cochrane databases, until December 10, 2016. All the statistical analyses were performed on case-control studies, using Review Manager Software 5.3 (Cochrane Collaboration, Oxford, UK).Our meta-analysis results based on 25 studies, including 5 studies with Chinese patients, indicated that there was no association of IA with breast cancer (OR = 1.08, 95% CI 0.98-1.19, P = .1). However, significant heterogeneity was observed, and thus further subgroup analyses were conducted. The combined OR of subjects with only 1-time IA was 1.03, 95% CI 0.90 to 1.18, P = .63, while for subjects with 2 or more IAs, it was 1.06, 95% CI 0.86 to 1.30, P = .58. In addition, the ORs of subjects, with 1st IA age either less than 30 or older than 30, were 1.05, 95% CI 0.88 to 1.26, P = .59, and 1.18, 95% CI 0.93 to 1.49, P = .17, respectively. These observations indicated that number of IAs and the age of 1st IA were not associated with breast cancer risk. Due to lack of dose-response relationships, it is difficult to say if number of IAs contributed into statistical heterogeneity. But after subgroup analysis, the age at the 1st IA appeared to impact the statistical heterogeneity. The different reproductive history appears to account for the high heterogeneity among individual studies. Also analysis of nulliparous women showed no significant difference in the association of IA and breast cancer (OR = 1.02, 95% CI 0.86-1.21, P = .85). However, parous women had higher IA rate in case group than control group (OR = 1.11, 95% CI 1.02-1.20, P = .01). Ethnicities might also result in high heterogeneity; thus, we conducted subgroup analyses on Chinese subjects, importantly, with 5 studies having Chinese patients, and did not observe any difference in the incidence of IA and its association with breast cancer between case and control groups (OR = 1.05, 95% CI 0.97-1.13, P = .21).After subgroup analysis, our study showed that IA might increase the risk of breast cancer in parous women, but in the nulliparous, IA was not significantly associated with an increased risk of breast cancer.

Lupus erythematosus panniculitis in a 10-year-old female child with severe systemic lupus erythematosus: A case report.

Lupus erythematosus panniculitis (LEP) is a rare subset of lupus erythematosus. The incidence of LEP in systemic lupus erythematosus (SLE) ranges from 2% to 5%. In the previous literature, most LEP patients were women aged from 20 to 60 years, while pediatric cases were rare, all of whom appeared on their own without SLE.A rare LEP in a 10-year-old female child with severe SLE is presented.

Treating Onchocerciasis in Regions in Which Loa loa Is Endemic.

Breast-Cancer Recurrence after Stopping Endocrine Therapy.

Optimal Compression Therapy and Wound Care for Venous Ulcers.

Venous leg ulcers remain a major public health issue with significant economic impact. Two main components of the management of patients with venous leg ulcers are compression therapy and wound care. This article addresses principles and specific aspects of compression therapy and focal wound care for patients with venous leg ulcers.

Pathophysiology of Chronic Venous Disease and Venous Ulcers.

Chronic venous disease and venous leg ulceration are a common disease affecting millions of individuals. The fundamental problem is venous hypertension with resultant clinical manifestations of venous disease including varicose veins, skin changes, and venous leg ulceration. The pathophysiology leading to venous hypertension is complex and multifactorial, involving genetic predisposition, environmental factors, hormones, endothelial dysfunction, inflammatory cells and molecules and activation on the endothelium and vein wall, and disturbances in the balance of cytokines and matrix metalloproteinases. Understanding the pathophysiology of chronic venous disease and venous leg ulcers identifies cellular pathways, biomarkers, metabolic signatures, and cellular cross-talk for targeted therapy.

Impact of the 21-gene recurrence score assay in clinical treatment and prognosis analysis for patients with hormone receptor positive early-stage breast cancer.