Síndrome de úlceras en piernas de carácter familiar y comienzo precoz / Familial Leg Ulcer Syndrome of Early Onset

Introducción: Las úlceras en las piernas son llagas sin sanar o lesiones abiertas de etiología multifactorial. Constituyen una patología importante en la práctica diaria de los profesionales de la salud en todos los niveles de asistencia. Objetivo: Presentar un caso infrecuente con una afección genética hereditaria familiar que provocó lesiones ulcerosas en las extremidades inferiores. Presentación del caso: Paciente masculino de 30 años con lesiones ulcerosas en ambos miembros inferiores, de 18 años de años de evolución, muy dolorosas con signos de infección local severa. Presentó diagnóstico de úlceras inespecíficas en ambos miembros inferiores. Fue ingresado con toma de su estado general, gran limitación de la deambulación, dolor intenso en ambas piernas, lesiones abundantes ulcerosas sucias diseminadas en ambas piernas, de fondo amarillento, con secreción amarilla clara, muy fétida. Se realizó estudio clínico, humoral, imagenológico, microbiológico y anátomo-histopatológico. Conclusiones: Se diagnostica síndrome de úlceras en piernas de carácter familiar y comienzo precoz, de herencia recesiva ligada al cromosoma X. Se requieren estudios a mayor escala para evaluar las contribuciones de los factores genéticos en la génesis de esta enfermedad, los cuales podrían ser la clave para comprender mejor su desarrollo(AU)

Introduction: Leg ulcers are unhealed sores or open lesions of multifactorial etiology. They constitute an important pathology in the daily practice of health professionals at all levels of care. Objective: To report an infrequent case with a familial hereditary genetic condition that caused ulcerative lesions in the lower limbs. Case report: We report the case of a 30-year-old male patient with ulcerative lesions on both lower limbs, 18 years of evolution, very painful with signs of severe local infection. He had diagnosis of nonspecific ulcers in both lower limbs. He was admitted with poor general condition, great limitation of ambulation, intense pain in both legs, abundant dirty yellowish ulcerative lesions scattered on both legs, and light yellow, very foul-smelling discharge. A clinical, humoral, imaging, microbiological and anatomical-histopathological study was performed. Conclusions: The diagnosis was familial leg ulcer syndrome of early onset, recessive inheritance linked to the X chromosome, is diagnosed. Larger scale studies are required to assess the contributions of genetic factors in the genesis of this disease, which could be the key to better understand its development(AU)

[Subcutaneous infection by Curvularia pallescens in a lung transplant recipient: First report in Spain]. / Micosis cutánea por Curvularia pallescens en un paciente trasplantado pulmonar: primer caso descrito en España.

BACKGROUND: Curvularia is a filamentous dematiaceous fungus increasingly recognized as a pathogen in immunocompromised patients. The most common clinical entities associated with this fungus are allergic sinusitis, cutaneous infection and keratitis. In this article, a report on the first clinical case of Curvularia pallescens cutaneous infection in Spain and its treatment is described. CASE REPORT: A 68 year-old man with a history of lung transplantation presented to Dermatology Unit due to a skin lesion in the knee that had been evolving for 6 months. A skin biopsy was performed for its study. In the histopathological study, an intense and non-specific inflammatory reaction in the dermis was observed, and with Grocott stain and periodic acid Schiff abundant septate hyphae and spores were found in the dermis. The culture of the sample revealed a filamentous fungus whose microscopic examination allowed to identify the genus as Curvularia. Using MALDI-TOF mass spectrometry and molecular identification, the fungus was finally identified as Curvularia pallescens. The patient underwent surgical resection of the lesion and was treated with posaconazole, evolving favorably. CONCLUSIONS: The species of Curvularia should be considered causal agents of fungal skin infections in immunosuppressed patients. This clinical case, which showed good clinical response after surgical resection and treatment with posaconazole, is the first described in Spain due to this species.

Sickle Cell Anemia and Comorbid Leg Ulcer Treated With Curative Peripheral Blood Stem Cell Transplantation.

Allogeneic bone marrow transplantation or peripheral blood stem cell transplantation (PBSCT) are the only curative therapies for patients with sickle cell disease (SCD). Once the patients have successfully undergone transplantation and engrafted, the hallmark of hemolytic anemia resolves, and normal hemoglobin levels are achieved. Some transplant protocols exclude patients with open wounds, including leg ulcers, because of infection risks associated with transplantation and long-term immunosuppression required to prevent graft-versus-host disease. Recalcitrant and recurrent leg ulcers are a serious complication of SCD and a determinant of morbidity. Here, we report the case of a 37-year-old man with sickle cell anemia and a chronic leg ulcer, who underwent PBSCT, engrafted successfully, and then had complete healing of his ulcer 16 months posttransplant.

Photodynamic Therapy With Methylene Blue for Skin Ulcers Infected With Pseudomonas aeruginosa and Fusarium spp. / Terapia fotodinámica con azul de metileno en úlceras cutáneas infectadas con Pseudomonas aeruginosa y Fusarium spp.

Photodynamic therapy (PDT) is a therapeutic modality with significant antimicrobial activity. We present 2 cases of chronic lower limb ulcers in which fungal and bacterial superinfection complicated management. PDT with methylene blue as the photosensitizer led to clinical and microbiological cure with no significant adverse effects. PDT with methylene blue is a valid option for the management of superinfected chronic ulcers, reducing the use of antibiotics and the induction of resistance.

Invasive Candida krusei infection and Candida vasculitis of a leg ulcer in an immunocompetent patient: A case report.

A 71year old female Caucasian farmer without any known immunosuppression presented with a painful ulcer of her right lower leg after a trauma caused by a wood billet. There was no response to empirical antibacterial treatment. An ulcer biopsy showed an invasive Candida infection of the soft tissue and leucocytoclastic vasculitis. Voriconazole treatment was followed by wound healing. Invasive Candida infection and localized Candida vasculitis represent a rare cause of persisting leg ulcers. The similar clinical picture of chronic venous leg ulcers might blur the true cause and refractory cases should therefore promptly be processed by histopathological diagnostics.

Long-Term Outcome of Patients with Peripheral Arterial Disease and Tissue Loss Stratified to a Nonrevascularization Approach.

BACKGROUND: To evaluate the long-term outcome of patients presenting with peripheral artery disease (PAD) and tissue loss that were stratified in our limb preservation program to receive aggressive wound care without revascularization. METHODS: Veterans presenting with PAD and nonhealing wounds were prospectively enrolled into our Prevention of Amputation in Veterans Everywhere (PAVE) program. Patients were stratified according to management strategies, which include: revascularization, primary amputation, palliative limb care, and aggressive local wound care without revascularization (conservative group). This study focuses on the conservative cohort. Wound presentation, type of wound care provided, wound care-associated procedures, healing rates, revascularization, major amputation, wound recurrences, management of recurrent wounds, and patient survival were analyzed. RESULTS: Between January 2006 and November 2014, 601 patients were prospectively enrolled in our PAVE program. A total of 203 limbs in 183 patients with 231 wounds were allocated to the conservative group based on a validated pathway of care. Mean follow-up for this cohort was 33.6 months (range, 1.5-104). Complete wound healing was achieved in 148 limbs (73%). The mean time to healing was 4.1 months. Twenty-four limbs (11.8%) received "late revascularization" (beyond 6 months from enrollment). Overall limb preservation was 90% at 4 years, with 57% freedom from wound recurrence. In patients with recurrence over 80% were successfully managed without revascularization. Limb loss was attributed to infection in most cases. CONCLUSIONS: In this selected group, an initial approach with aggressive wound care without revascularization appears justified with good limb salvage. Long-term analysis demonstrated a notable incidence of wound recurrence (43%) albeit most recurrences can be successfully managed without the need for late revascularization and no increased incidence of limb loss.

Evaluation of Early Surgical Treatment Results Considering Venous Leg Ulcers.

UNLABELLED: Lower leg ulcers are characterized by a chronic and recurrent course, being considered as a difficult therapeutic problem. Based on epidemiological data chronic ulcers concern 0.2 to 2% of the Western European population. The above-mentioned problem affects women more often than men, and increases with age. Treatment and recurrence prevention methods remain under discussion and research. The aim of the study was to evaluate the early surgical treatment results of venous leg ulcers at the Department of Traumatology and Emergency Medicine, Medical University of Lublin. MATERIAL AND METHODS: We evaluated patients hospitalized at the Department of Traumatology and Emergency Medicine, Medical University of Lublin during the period between 2009-2014. Inclusion criteria were as follows: venous leg ulceration, full documentation. The study group comprised 62 patients subjected to treatment for leg ulcerations, including 35 women and 27 men, aged between 30 and 94 years. The treatment method included the excision of the ulcers with the deep fascia, and the implantation of the mesh graft (thickness of skin) directly into the exposed muscle. RESULTS: Amongst patients evaluated at discharge, the healing of the transplant was as follows: in case of 44 patients 90-100%, in 8 patients - 75-89%, in 5 patients 50-74% and in 5 patients less than 50%. The predominating bacteria collected from ulceration samples was Pseudomonas aeruginosa (32%) and Staphylococcus aureus (35%). CONCLUSIONS: The patient outcome shows that surgery is an effective method considering treatment of venous leg ulcers. Earlier qualification for this type of surgery will improve patient outcome.

[Latent-disseminated tuberculosis revealed by atypical skin ulcerations]. / Tuberculose disséminée paucisymptomatique révélée par des ulcérations cutanées atypiques.

INTRODUCTION: Cutaneous tuberculosis (CT) is rare in industrialized countries. Given the clinicopathological polymorphism and the difficulty of isolating the pathogen, diagnosis can be difficult. The condition may be associated with other known locations of the disease or in rare cases, it may be a tell-tale sign, as in our case, in which leg ulcers revealed paucisymptomatic disseminated tuberculosis. OBSERVATION: A 67-year-old man was referred for rapidly extensive ulcers of the right leg contiguous to debilitating arthritis of the knee of unknown aetiology for 18 months. Earlier investigations revealed thymoma and a pulmonary nodule considered to be sarcoidosis. A skin biopsy showed a granulomatous eosinophilic-rich infiltrate and vasculitis of the small vessels. Screening of the skin sample and gastric aspirate for Koch Bacillus (BK) was negative. A diagnosis of sarcoidosis was made. A positive QuantiFERON test eventually led to the correct diagnosis. On further testing of bronchoalveolar fluid and a synovial biopsy, culture for Mycobacterium tuberculosis (MT) was positive. The PET scan showed high metabolism in the prostate, bone, spleen, liver, nodes and heart. The quad- and then dual-antibiotic antitubercular therapies produced a rapid improvement but treatment was continued over 12 months, given the persistence of high metabolism on PET-CT scan and the low blood rifampicin concentration. DISCUSSION: A CT should be considered in the presence of giant-cell granulomas, even in the absence of caseous necrosis, and where both direct examination and culture for the skin are negative. Our case also underlines the importance of an extensive workup to rule out disseminated disease even if the patient is not symptomatic.

Skin ulcers caused by Serratia marcescens: three cases and a review of the literature.

UNLABELLED: Serratia marcescens is a Gram-negative, encapsulated, motile, anaerobic, non-sporulating bacillus that belongs to the Enterobacteriaceae family. It is found in water, soil, plants, food, and garbage. S. marcescens is an opportunistic pathogen. It usually causes nosocomial infections, such as lung and genitourinary infections, sinusitis, otitis, endocarditis, and sepsis. Skin infections caused by S. marcescens are rare. To describe three new cases of skin ulcers of the leg caused by S. marcescens and review the relevant literature. We investigated three patients admitted for ulcers on the leg. RESULTS: In two patients, post-traumatic aetiology was concluded. The modality of infection was not identified for the other patient. One patient was diabetic. All patients recovered with specific antibiotic therapy (ciprofloxacin, ceftriaxone and levofloxacin, respectively). Skin ulcers due to S. marcescens are very rare. The three cases presented here add to the limited literature of skin infections caused by S. marcescens.

Cutaneous ulcers in a returning traveller: a rare case of imported diphtheria in the UK.

We describe a case of cutaneous diphtheria in the UK, presenting as lower leg ulcers in a returning traveller, and discuss the epidemiology, significance and public health implications of this disease and the therapeutic options available. A 65-year-old woman presented with a 6-week history of multiple ulcers appearing on her legs following a holiday in Kenya. Culture of biopsy tissue grew Corynebacterium diphtheriae. A cascade of therapeutic and public health interventions followed, many of which were terminated once the isolate was confirmed as nontoxigenic. Cutaneous diphtheria is a rare, notifiable disease in the UK, but is common in tropical countries, and is most often seen in the West as a traveller's disease. Corynebacteria are common skin commensals, and without appropriate clinical details, laboratories may not recognize C. diphtheriae/Corynebacterium ulcerans. This is likely to have led to under-reporting and under-recognition of the condition.

[Autologous mesenchymal stem cells and cutaneus autograft as a treatment for chronic ulcer secondary to diabetes mellitus 2]. / Células troncales mesenquimales autólogas e injerto cutáneo autólogo para tratamiento de una úlcera crónica secundaria a diabetes mellitus tipo2.

BACKGROUND: Diabetes mellitus 2 has become a global problem. It is estimated that 15% to 25% of patients could develop a chronic ulcer in their life, and nearly 33% of direct care costs of the diabetes mellitus 2 is spent on treating these ulcers. Mesenchymal stem cells have emerged as a promising cell source for the treatment of these ulcers. CLINICAL CASE: The case is presented of a 67 year-old male with a history of diabetes mellitus, acute myocardial infarction, and food ulcer chronic involving right foot and part of his leg. He was treated with mesenchymal stem cell management, resulting in skin graft integration and full coverage of the lesion. CONCLUSION: The implementation of mesenchymal stem cell techniques for treatment of chronic ulcer is feasible. The impact on the population would lead to a significant improvement in their quality of life and reduce healthcare spending.

Leg ulcer and osteomyelitis due to methicillin-susceptible Staphylococcus aureus infection after fracture repair treatment: a case highlighting the potential role of prostaglandin E1 vasodilator.

Prostaglandins appear to reduce biofilm formation and chronicization of infections, and stimulate a rapid and effective clearance of infecting micro-organisms. We report a case of recovery from methicillin-susceptible Staphylococcus aureus (MSSA) osteomyelitis after multidisciplinary management with antibiotics, anti-thrombotics and prostaglandin E1 (PGE1) vasodilator, in a patient with tibial plateau fracture repaired with internal fixation devices. A 47-year-old HIV-negative male with chronic ulcer on the proximal third of the left leg was admitted to the Orthopaedic Unit of the Orestano Clinic in Palermo, Italy, for suspected osteomyelitis. A biopsy of the skin ulcer and blood cultures were performed and resulted positive for MSSA. Labelled leukocyte scintigraphy confirmed osteomyelitis. No clinical improvement was observed after combined antibiotic treatment with rifampicin plus trimethoprim-sulfamethoxazole. The patient underwent a 4-day therapeutic cycle: PGE1 (alprostadil 60 mg/day IV) combined with nadroparin calcium plus gentamicin, followed by treatment with aminaftone plus sulodexide plus levofloxacin. At discharge, the patient's painful symptoms had completely resolved and the ulcer had cicatrized; recovery from osteomyelitis was confirmed by scintigraphy. This treatment protocol including PGE1 may result in a significant improvement in quality of life and functional status of patients with a reduction in direct and indirect costs and economic benefit for the National Health Service.