Bullous Lesions Research Articles

The role of serum tryptase in the diagnosis and monitoring of pediatric mastocytosis: a single-center experience.

IntroductionIn children, cutaneous mastocytosis (CM) is considered to be a benign disease associated with mast cell mediator-related symptoms. However, systemic mastocytosis (SM) and anaphylaxis may also occur. Since the basal serum tryptase (bsT) level reflects mast cell burden, its determination is recommended in the diagnosis and follow-up.AimTo compare clinical presentation and the course of mastocytosis in children with normal and clearly elevated bsT levels as well as to assess its usefulness in the diagnosis and monitoring of the course of the disease.Material and methodsA retrospective analysis of 102 medical records of children with mastocytosis diagnosed and followed-up in the Gdansk Mastocytosis Center in 2014 was performed.ResultsMaculopapular CM (MPCM) was diagnosed in 91 (89.22%) children, diffuse cutaneous mastocytosis (DCM) in 7 (6.86%) and mastocytoma in 4 (3.92%). The presence of flushing and bullous lesions was more frequent in children with bsT levels > 20 ng/ml in comparison with those who had bsT ≤ 20 ng/ml (p = 0.002 and p = 0.03, respectively). Anaphylaxis occurred in 2 MPCM children with bsT levels in normal ranges. In all of the 3 children with persistently and clearly elevated bsT levels, bone marrow biopsy revealed no mast cells infiltrates corresponding to SM.ConclusionsAlthough mastocytosis children with clearly elevated bsT levels frequently develop mediator-related symptoms, the occurrence of anaphylaxis in this age group may be difficult to predict. In pediatric cases with suspected SM, the bsT level is one of the crucial parameters considered before the decision on bone marrow biopsy.

Bullous Henoch–Schonlein purpura with involvement of face

Henoch–Schonlein purpura (HSP) with facial involvement with bullous rashes are extremely rare. A 12-year-old boy presented with abdominal pain and features of arthritis. He also had multiple purpuric rashes over his lower limbs. Gradually, he developed bullous rashes which were seen on his legs and hands and progressed to involve the face. He was confirmed to be suffering from HSP from clinical presentation and skin biopsy. The child responded well to oral steroids. Bullous lesions may be seen in HSP. However, there is neither prognostic significance of this nor does it alter the management. Other causes of bullous lesions should be ruled out. As facial involvement is associated with renal and gastrointestinal involvement, these children should be monitored for sequelae.

Risk factors associated with local complications of erysipelas: a retrospective study of 152 cases.

Erysipelas is a common skin infection. Hemorrhagic, bullous, abcessing and necrotic lesions are the major local complications. However, their occurrence factors are not clearly known. The aim of this study is to identify the risk factors associated with the occurrence of local complications of Erysipelas. Medical records from all patients hospitalized with local complications of erysipelas admitted to the Military Hospital of Rabat between 2005 and 2015, were retrospectively studied. Using an univariate and multivariate statistical study, the main characteristics were compared with those from patients with erysipelas without local complications. In total, 152 patients were analysed, of whom 72 had local disease complications. Using univariate analysis, the factors significantly associated with disease complications were found to be: age ≤ 50 years, female gender, heart disease, smoking, taking antibiotics or non-steroid anti-inflammatory drug before hospitalization, and accelerated sedimentation rate. However, in multivariate analysis, taking antibiotics before hospitalization (OR 5.15, 95% CI 1.28 to 20.72, P = 0.01) and accelerated sedimentation rate (OR 5, 15, 95% CI 1.00 to 1.06, P = 0.001) were the only independent factors associated with complicated erysipelas. Our study showed that prior antibiotics taking and higher sedimentation rate are independent risk factors for local complications of erysipelas. Patients with these characteristics should be carefully evaluated and monitored.

Amyloidosis and Anesthesia.

Aim:The aim of this article is to provide a view of amyloidosis and discuss implications for the anesthetic management of patients with this condition.Material and Method:Urine samples from patients with plasma cell dyscrasias were obtained from a urine bank that gathers urine samples from patients who gave research use consent for specimens that would otherwise be considered waste.Results:Patients with amyloidosis may present to the anesthesiologist for procedures relating to diagnosis, surgery relating to the underlying condition (e.g., bronchial laser and organ transplant), or for incidental surgery. The condition carries a significant risk of perioperative morbidity and mortality.Conclusion:The term amyloid was coined by Virchow in the mid 19th century, meaning “starch like.” Amyloidosis is a disease complex, in which there is an abnormal deposition of extracellular hyaline material with particular staining characteristics and which contains protein fibrils embedded in a relatively amorphous ground substance. There are numerous clinical manifestations, the onset is insidious, and the diagnosis may not be made in a patient undergoing anesthesia and surgery for an apparently straightforward problem. Unexpected complications such as heart or kidney failure may arise, either before operation or in the postoperative period. Bullous lesions of the skin or oral mucosa and extensive areas of purpura are but two of the ways, in which amyloidosis may first present. The disease spectrum may be inherited or acquired, localized or systemic, and life threatening or an incidental finding.

Bullous Erythrodermic Mycosis Fungoides: A Case Report

Bullous mycosis fungoides is a rare entity of cutaneous T-cell lymphomas; however, it can be particularly aggressive with an unfavorable prognosis. Mechanism of formation of bullous lesions in bullous lymphoma remains poorly understood, it can be explained by excessive epidermotropism or tumor infiltration’s toxicity. We report the case of a bullous erythrodermic mycosis fungoides.

An infant with bullous mastocytosis: A rare form of bullous disorder

Bullous mastocytosis (BM) is a rare variant of diffuse cutaneous mastocytosis where there is disorderly infiltration of the skin by mast cells. We report a 4-month-old male infant with this rare disease who presented with wrinkled tough skin and generalized multiple bullous lesions. Diagnosis was done by serum tryptase level and histopathological examination. The patient responded well to oral corticosteroid and antihistaminic therapy. Although a congenital disorder, timely diagnosis and treatment can be potentially rewarding in patients with BM.

Diagnosis of Vesicullo Bullous Lesions- Simplified

Introduction Vesiculo bullous lesions are a distinct group of oral disorders characterized by the formation of vesicle or bullae. And it is uncommon to see vesicle and bullae intra orally because due to constant masticatory pressure vesicles and bullae get ruptured and it becomes ulcers and erosi

Occult primary pulmonary synovial sarcoma presenting as recurrent spontaneous pneumothorax and explosive progression

Primary pulmonary synovial sarcoma (PPSS) is a relatively rare neoplasm with highly progressive potential. We present an extremely rare case of PPSS presenting as recurrent pneumothorax with bullous lesions. Bullectomy was performed at the local hospital. Unfortunately, the patient was initially misdiagnosed as atypical carcinoid. Although a negative resection margin was obtained during the first surgery and a remedial operation and chemotherapy followed, the patient developed severe disease progression and died soon after. This report demonstrates that PPSS can easily be misdiagnosed and should be seriously considered in the differential diagnosis of pneumothorax.

Bullous Pemphigoid Induced by Vildagliptin

Bullous pemphigoid (BP) is an uncommon chronic, autoimmune, and subepidermal disease (1). Tense blisters occur over normal or erythematous skin. (1) BP may be localized or generalized. It may involve both skin of extremities and trunk and mucosa. Pruritus may precede bullae formation by weeks or months. It can be associated with drugs, UV irradiation, and X-ray therapy (1). There is a number of reports on bullous pemphigoid (BP) induced by DPP-IV inhibitors (vildagliptin, sitagliptin) used for treatment of type 2 DM (2-6). Although skin lesions were not observed at an increased incidence in clinical trials, there have been post-marketing reports of bullous and exfoliative skin lesions. The enzyme dipeptidyl peptidase (DPP-IV) degrades glucagon like peptide 1 (GLP-1), which is a potent stimulator of insulin production and secretion (7). DPP-IV is expressed by diverse tissues including skin (7). In the skin, many cell types (including keratinocytes) express DPP-4, giving rise to cytokine production, tissue differentiation and collagen metabolism (7). Some factors Bullous pemphigoid (BP) is an uncommon chronic, autoimmune, and subepidermal disease. Tense blisters occur over normal or erythematous skin. It can be associated with drugs. There is a number of reports on bullous pemphigoid (BP) induced by DPP-IV inhibitors (vildagliptin, sitagliptin, saxagliptin). DPP-IV (CD26), present as a cell surface molecule on immune cells, also plays an important costimulatory role in immune activation. We present a case of BP induced by vildagliptin. A 59 yr old male patient who was diagnosed type 2 DM had initial haemoglobin A1c level of 12.90%. Initial therapy with premix biphasic aspart insulin bid was switched to metformin and vildagliptin 50/1000 mg combo pill bid after A1c level dropped to 5.7% at 9 months of insulin therapy, Five months after vildagliptin was started, tense vesicles 8-10 in number with an erythematous base developed over forearms and cruris. Histologic examination of the lesions confirmed bullous pemphigoid. Oral antidiabetics were discontinued. He was followed up with diet alone. The lesions regressed spontaneously after cessation of antidiabetics and clobetasol propionate cream bid treatment. A1c was 5.7% 5 months after discontinuation of vildagliptin and metformin. In the literature onset of BP lesions took 10 days to 2 years. Mostly the patients were on combo therapy with metformin. The lesions improved dramatically after cessation DPP-IV inhibitors avoiding necessity for systemic treatment for BP. Elder males predominate. This is the first case of BP induced by DPP-IV inhibitors Turkey.

Bullous keratopathy in a yellow-headed caracara (Milvago chimachima) treated with a modified third eyelid flap

ABSTRACT: A Yellow-headed Caracara (Milvago chimachima) was submitted to the Ophthalmology Service of the Federal University of Bahia with a corneal abnormality. During ophthalmic evaluation the right cornea was stained positively with fluorescein; a blurred bullous lesion, with irregular surface, compatible with the diagnosis of bullous keratopathy was found. This is a rare condition in a bird which was treated successfully with a modified third eyelid flap associated with antibiotic and hyaluronic acid eye drops. The adopted therapeutic proved to be simple to implement and viable for repair of the bullous keratopathy in the Yellow-headed caracara.

Lenalidomide for refractory cutaneous manifestations of pediatric systemic lupus erythematosus.

Objective Cutaneous manifestations of pediatric systemic lupus erythematosus cause significant morbidity. Lenalidomide, a thalidomide analogue, has shown promise treating cutaneous lupus erythematosus in adults. Our objective was to evaluate lenalidomide's efficacy and safety in treating refractory cutaneous manifestations of pediatric systemic lupus erythematosus. Methods We performed a retrospective chart review of 10 adolescents who received lenalidomide for recalcitrant cutaneous lupus erythematosus. Information was gathered at drug initiation and 6-month follow-up. The Wilcoxon matched-pairs signed-rank test was used to assess change in quantitative parameters of disease activity. Results Nine subjects were girls and six were African-American. Indications for lenalidomide treatment included alopecia, nasal and oral ulcers, extensive malar rash, discoid lesions, bullous lesions, panniculitis, cutaneous vasculitis, and Raynaud's phenomenon with digital ulcerations. Within 6 months, all patients demonstrated complete or near resolution based on physician report. Prednisone dose decreased from a mean 23.5 mg (SD± 13.3) to 12.25 mg (SD± 9.2) ( P= 0.008). Sedimentation rate decreased from a mean 29 mm/hour (SD± 31.5) to 17 mm/hour (SD± 18.1) ( P= 0.004). Lenalidomide was well tolerated. Conclusion Lenalidomide is an effective and safe treatment for a spectrum of dermatological conditions in pediatric systemic lupus erythematosus. Its use may allow a reduction in prednisone dose and decreased disfigurement. Prospective study is needed to clarify lenalidomide's role in treating cutaneous manifestations of systemic lupus erythematosus.

Cutaneous manifestations in renal diseases

In addition to general skin changes like pallor or dryness and the frequent, often excruciating nephrogenic pruritus, specific diseases in patients with renal failure may occur. Acquired perforating dermatoses are usually also highly pruritic. Calciphylaxis is asevere disease with poor prognosis. Nonhealing wounds with superinfection and progression to sepsis are characteristic. Bullous lesions can be caused by disturbances in porphyrin metabolism. Nephrogenic systemic fibrosis is adisease which was first described in 2000. Its incidence is already on the decline. Furthermore, this article provides an overview of systemic diseases which have both skin symptoms and kidney changes. These include connective tissue diseases, vasculitis or sarcoidosis and amyloidosis. After akidney transplantation, particular attention must be paid to the development of skin tumors and infections. The last part of this article is dedicated to genodermatoses with skin and renal involvement, where numerous causative mutations have already been characterized. Knowing the correlations of characteristic skin symptoms and specific, potentially life-threatening kidney disease is important in order to initiate further investigations and steps such as referral to nephrologists at an early stage.

A abordagem precoce de ferida complexa em uma unidade de emergência: uma experiência de êxito.

Introdução: Ferida complexa é o termo utilizado para identificar as feridas de difícil cicatrização. A abordagem precoce impacta no atendimento levando a menor tempo de internação, reduzindo agravos à saúde e os custos com internação. Objetivo: Relatar uma abordagem precoce de uma lesão causada por erisipela bolhosa pós-traumática. Método: trata-se de um relato de caso, realizado na Unidade de Emergência Referenciada. Resultado: Paciente do sexo feminino, 63 anos, deu entrada na UER após ter sido vítima de atropelamento com trauma em membro inferior esquerdo e fratura de tíbia e fíbula, evoluiu com edema e um dia após a sua admissão iniciou quadro de hiperemia, dor e lesões bolhosas. Foi avaliada por enfermeiros e submetida precocemente a desbridamento instrumental conservador do tecido necrótico proveniente da lesão bolhosa e terapia tópica com Solução de PHMB 0,2%, curativo não aderente com troca a cada 3 dias e óxido de zinco em bordas. Dez dias após sua admissão as lesões mediam (lesão 1) 9x18cm e (lesão 2) 3x2,5cm. No dia seguinte, a paciente encaminhada para o Centro Cirúrgico e posteriormente para a enfermaria. Teve alta médica dois dias após. A paciente compareceu à UER posteriormente para reavaliação com ambas as lesões já epitelizadas. Discussão: –A abordagem precoce adequada realizada possibilitou o crescimento do tecido de granulação e a epitelização completa de ambas as feridas em um curto intervalo de tempo. Conclusão: O cuidado com feridas complexas deve ser amplamente discutido nos serviços de emergência para promover um cuidado de enfermagem excelência.

Staphylococcal Scalded Skin Syndrome in Child. A Case Report and a Review from Literature.

Staphylococcal scalded skin syndrome (SSSS) is the medical term used to define a skin condition induced by the exfoliative toxins produced by Staphylococcus aureus. The disorder is also known as Ritter disease, bullous impetigo, neonatal pemphigus, or staphylococcal scarlet fever. The disease especially affects infants and small children, but has also been described in adults. Prompt therapy with proper antibiotics and supportive treatment has led to a decrease in the mortality rate. The current case report describes the clinical progress of a patient with generalized erythema and fever, followed by the appearance of bullous lesions with tendency to rupture under the smallest pressure, and with extended areas of denudation. The patient aged four years and six months was admitted to our clinic to establish the aetiology and treatment of a generalized bullous exanthema, followed by a skin denudation associated with fever and impaired general status. Based on clinical and paraclinical examinations a diagnosis of Staphylococcal scalded skin syndrome was established which responded favourably to antibiotic treatment, hydro-electrolytic re-equilibration, and adequate local hygiene. Staphylococcal infection can represent a problem of significant pathological importance sometimes requiring a multidisciplinary approach involving paediatricians, dermatologists, infectious diseases specialists, and plastic surgeons.

Érythème polymorphe postinfectieux de l’enfant

Postinfectious erythema multiforme is an uncommon skin disease in childhood that can have a strong impact, especially in infants if there is involvement of the mucous membranes. The lesion is targeted (central bullous lesions with three concentric circles). Its diagnosis is typically made clinically. Atypical forms are the highly inflammatory, mainly bullous type, with exclusive involvement of the mucous membrane, or recurrent erythema multiforme. The diagnosis of erythema multiforme is frequently suspected in children with urticaria multiforme. Kawasaki disease and toxic epidermal necrolysis may have a target-like skin aspect with mucosal involvement, and should be considered when planning treatment. The two major infectious etiologies in children are Mycoplasmapneumoniae and Herpes simplex. More recently, postvaccination erythema multiforme has been reported with the majority of vaccines used in pediatric practice. The prognosis is usually good but requires observation of the mucosal involvement because of the risk of serious complications. The treatment of erythema multiforme is essentially symptomatic, with etiology-related treatment.

Hailey Hailey disease-a rare case report

Hailey-Hailey disease is an autosomal dominant acantholytic disorder relatively uncommon in India. It is characterized by painful, pruritic, foul smelling vesicles and bullous lesions with erosions in intertriginous areas such as the neck, axillae, inframammary areas and groin. The defect in ATPC2 gene leads to calcium channel dysfunction which results in defect in epidermis. Fungal infection, intertrigo, psoriasis, extramammary Paget's disease, acanthosis nigricans, pemphigus vegetans and Darier's disease are some of the dermatosis from which it needs to be differentiated. Histopathology has a key role in diagnosis of Hailey-Hailey disease with characteristic ‘dilapidated brick wall' appearance. Hailey-Hailey disease can be easily misdiagnosed by general physicians due to lack of knowledge of this uncommon disease as it resembles dermatosis involving intertriginous areas. Due to its relapsing and remitting course there is a need to have effective treatment options. We report a case of 45 year old middle aged female who presented with on and off itchy papules & vesicles over right inframammmary region around the umbilicus & medial aspect of right thigh. Skin biopsy revealed a diagnosis of Hailey-Hailey disease. This case is being reported due to its rare occurrence.

Integrative Medicine Atlas of Skin Diseases

Evidence shows that Ayurveda and biomedical systems of medicine recognize the same disease albeit with minor difference in clinical description. We illustrate through clinical photos of Lymphoedema, Vitiligo, Psoriasis, Lichen Planus, bullous lesions, etc. the interpretation of diseases when both doctors of Ayurveda and Allopathy examine the patients together. Comparable clinical features in allopathy for Ayurveda's Vatha, Pitta and Kapha doshas are shown for different presentations in patients. This atlas is a guide for Integrative Medicine clinical methods to develop Sthaneeya vikruthi table, the first step in integrating Ayurveda and Allopathy for any disease and leads to the integration of Ayurveda and biomedicine at treatment level in dermatology irrespective of background understanding of the disease.

Terbinafine : Drug-induced lupus erythematodes and triggering of psoriatic skin lesions

Based on the technical information that oral terbinafine must be used with caution in patients with pre-existing psoriasis or lupus erythematosus, the literature was summarized. Terbinafine belongs to the drugs able to induce subcutaneous lupus erythematosus (SCLE)-with arelatively high risk. The clinical picture of terbinafine-induced SCLE may be highly variable and can also include erythema exsudativum multiforme-like or bullous lesions. Thus, differentiation of terbinafine-induced Stevens-Johnson syndrome or toxic epidermal necrolysis may be difficult. Therefore, terbinafine should be prescribed with caution in patients who show light sensitivity, arthralgias, positive antinuclear antibodies or have ahistory of SLE or SCLE. Case reports include wide-spread, but mostly nonlife-threatening courses, which did not require systemic therapy with steroids or antimalarials in every case. Terbinafine is also able to induce or to aggravate psoriasis. The latency period seems to be rather short (<4weeks). Terbinafine therefore is not first choice if asystemic therapy with antimycotics is indicated in apatient with psoriasis or psoriatic diathesis. Azole derivatives according to the guidelines may be used as an alternative.

Clinical application of near-infrared thoracoscope with indocyanine green in video-assisted thoracoscopic bullectomy.

Failure to identify all the possible bullous lesions was considered an important reason for the higher recurrence rate after the VATS bullectomy. We applied the latest near-infrared (NIR) thoracoscope with indocyanine green (ICG) to detect bullous lesions for patients with spontaneous pneumothorax. Two male patients with spontaneous pneumothorax and poorly identified bullae intraoperatively were included in this pilot study. An NIR thoracoscope with two different doses of ICG injection (0.2 and 0.6 mg/kg) was used to detect bullous lesions during VATS bullectomy. Partial lung resections of the bullous lesions were performed under syncretic mode. Data was managed with ImageJ software. No procedure-related complications were observed. The fluorescent signal was detected in normal lung tissue 10.5 seconds (mean, 10-11 seconds) after the ICG bolus, and lasted up to 525 seconds (mean, 480-570 seconds). The bullous lesions showed an obviously decreased fluorescent densities comparing to adjacent normal tissue. At the dosage of 0.6 mg/kg, ICG emits sufficient fluorescence to demonstrate the precise border of bullae, with the max SBR of 6.32. All resected specimens were confirmed as bullous lesions microscopically. NIR thoracoscope with intravenous ICG is a safe, accurate and real-time method to detect bullous lesions of lung tissue difficult to be found under normal light in human subjects. NCT02611245 (https://register.clinicaltrials.gov/).

Immunohistochemical study of toll-like receptors 2, 4, and 9 expressions in pemphigus and bullous pemphigoid lesions.

Pemphigus and bullous pemphigoid (BP) are severe autoimmune skin diseases. Whether innate immunity could be a trigger or a part of the pathogeneses is unknown. Toll-like receptors (TLRs) are important components of the innate immune system, with no previous evaluation of TLRs in autoimmune bullous diseases. This work aims to investigate TLRs 2, 4, and 9 expressions in pemphigus and bullous pemphigoid. Thirty-six patients with pemphigus vulgaris (PV), pemphigus foliaceus (PF), bullous pemphigoid (BP), and six healthy controls were studied. Skin biopsies from the patients and the controls were examined immunohistochemically for TLR2, 4, and 9 expressions. The TLR4 expressed mainly at the basal layer of epidermis in controls, but in the cases with autoimmune bullous diseases, TLR4 staining located at basal layer and suprabasal layer, even superficial layer of epidermis. The immunostaining-intensity-distribution (IID) index of TLR4 in patients with PF (13.83, P=0.001), PV (13.08, P=0.003), and BP (11.42, P=0.042) were significantly higher than that of the controls (6.17). TLR2 and TLR9 showed no significantly changes at epidermal expression (P>0.05) compared with controls. There was no correlation found between the expressions of these TLRs. This work, thus, shows a re-localization of TLR4 expression sites with increased expression in pemphigus and bullous pemphigoid lesions. Targeting TLR4 signaling is expected to be a novel treatment strategy for autoimmune bullous diseases.