Search

CSR is a challenging disease to understand and treat, since its pathogenesis remains elusive and multifactorial. CSR is an idiopathic disorder characterised by a localised serous detachment of sensory retina at the macula secondary to leakage from the choriocapillaries through one or more hyperpermeable RPE sites. In Ayurvedic literature, all the conditions which cause impairment of vision are included under Timiraroga. The present case CSCR, can be included under Sannipathikatimira. The treatment procedure Vamana is contraindicated in Timira but CSR is a disease in which excessive accumulation of serous fluid. So, it can be considered as Ekamgajasopha. The first line of treatment of Sopha is Apatarpana. Vamana is one of the Apatarpana procedures. So, Vamana can be incorporated in CSR. Management of CSR usually involves the use of laser photocoagulation, photodynamic therapy with verteporfin and anti VEGF agents in case of choroidal neovascularization related to CSR. As per Ayurvedic classics this condition can be included under Timiraroga. This article describes a case report of 47 years old male patient presented with CSR. In present study, Sadyovamana is found very effective to improve his visual acuity. OCT studies showed almost total absorption of subretinal fluid after treatment.

Introduction Vitiligo is an acquired, idiopathic disorder clinically characterized by amelanotic lesions on the skin which cause significant impairment of patients' quality of life. A variety of treatments have been proposed, with inconsistent results. In the last decades, the use of platelet-rich plasma (PRP) is receiving increasing interest as a potential effective technique in the treatment of several dermatological diseases, including vitiligo. Objective We conducted a review with the aim to identify studies that documented the use of PRP for vitiligo. Materials and methods Electronic databases of MEDLINE, EMBASE and Cochrane Central Register of Controlled Trials (CENTRAL) from inception to November 2019 have been searched using different combinations of the following terms: "platelet-rich plasma", "platelet gel", "platelet-rich fibrin", "PRP" and "vitiligo". Results We identified 6 clinical studies consistent with our research, with a total of 253 patients, listing and discussing the obtained results. In all reports, all treated patients showed a stable vitiligo, and a significantly higher improvement in the PRP groups was always observed compared to control groups. Regarding the side effects, PRP in vitiligo patients is useful and without important side effects. Conclusion PRP is a promising treatment for stable vitiligo lesions in different body sites. The possible use of PRP in combination with traditional therapeutic options and the standardization of processing protocols represents a very fertile field for future research. Larger clinical trials with longer time of observation would provide solid evidence regarding the effectiveness of PRP for the treatment of vitiligo.

Osteochondritis dissecans of the elbow is an idiopathic disorder of the cartilage and subchondral bone. It occurs most frequently in the capitellum of adolescent overhead athletes. For patients with an unstable OCD limited to less than 50% of the capitellum without involvement of the lateral column, arthroscopic debridement with loose body removal and microfracturing is the first choice of treatment. The goal is to remove loose bodies and create a stable cartilage rim. To place the portals without damaging the neurovascular structures, it is important to have a thorough knowledge of the anatomy of the elbow. Pain relief and increased range of motion can be expected after arthroscopy, but the return to pre-injury level is variable.

Scleromyxedema is a chronic, idiopathic disorder associated with monoclonal gammopathy, and characterized by dermal mucin deposition. However, systemic manifestations are frequent, including neuromuscular symptoms. We herein present a 71-year-old man who developed a vacuolar myopathy in a context of a known scleromyxedema, and we compare our observation with the nineteen other cases found in the medical literature. Such an association (especially with suggestive skin abnormalities) has to be known for two reasons. First, this diagnosis might be quite challenging because the myopathy may precede the typical skin changes. Secondly, conversely to other forms of vacuolar myopathy, some of the symptoms may respond (even partially) to immunomodulatory and/or immunosuppressant therapeutics.

Lichen planus is a chronic, idiopathic disorder which usually affects skin and mucosal surfaces. While oral mucosa is frequently involved, esophageal localization is uncommon, and it usually manifests with dysphagia. It has also been associated with squamous cell carcinoma. Underdiagnosing of esophageal lichen planus often leads to a delay in treatment. There are also no clear recommendations for treatment of this disorder. Systemic corticosteroids are usually the first-line therapy, but different other therapeutic approaches are also used, with a various rate of response. We present the case of a patient with esophageal lichen planus complicated with esophageal stenosis, who was rather resistant to treatment.

Pre-eclampsia (PE) is an idiopathic disorder of pregnancy characterized by proteinuric hypertension and still one of the most important causes of maternal and fetal mortality...

Endomyocardial fibrosis (EMF) is an idiopathic disorder of the tropical and subtropical regions of the world, considered endemic in tropical and subtropical Africa and is characterised by development of restrictive cardiomyopathy. Although accurate epidemiological data are lacking, EMF is estimated to be the most common form of restrictive cardiomyopathy globally. We report on a young patient presenting with a stroke diagnosed with EMF and review the role of cardiovascular magnetic resonance in evaluation of EMF.

Background: Pre-eclampsia is an idiopathic disorder of pregnancy characterized by proteinuric hypertention and still one of the important causes of maternal and fetal mortality.The identification of its predisposing factors before and during early stage of pregnancy will help in reducing the mortality.Objective:The objective of the present study is to determine the risk factors for pre-eclampsia among pregnant women in a tertiary level hospital.Methods: This study was conducted in the Department of Obstetrics and Gynaecology of a tertiary care hospital in Chittagong, Bangladesh, from January to June 2015.A total number of 50 pregnant women with pre-eclampsia who admitted in this hospital were selected as study group.This was hospital based descriptive study.Results: Most of the partcipants were within 21-30 years of age group and mean age 24.06 ± 3.71.The factors that were found to be significant predictors of risk for development of PE were primigravida, low socioeconomic condition,family history of PE & hypertension, past history of PE and hypertention, past history of diabetes mellitus was also associated with development of PE.Chatt Maa Shi Hosp Med Coll J; Vol.16 (1); Jan 2017; Page 29-32

Background: Spontaneous coronary artery dissection (SCAD) is an uncommon idiopathic disorder predominantly affecting young, otherwise healthy women. Rare familial cases reveal a genetic predisposition to disease. The aim of this study was to identify a novel susceptibility gene for SCAD. Methods: Whole-exome sequencing was performed in a family comprised of 3 affected individuals and filtered to identify rare, predicted deleterious, segregating variants. Immunohistochemical staining was used to evaluate protein expression of the identified candidate gene. The prevalence and spectrum of rare ( Results: We identified a rare heterozygous missense variant within a highly conserved β-integrin–binding domain of TLN1 segregating with familial SCAD. TLN1 encodes talin 1—a large cytoplasmic protein of the integrin adhesion complex that links the actin cytoskeleton and extracellular matrix. Consistent with high mRNA expression in arterial tissues, robust immunohistochemical staining of talin 1 was demonstrated in coronary arteries. Nine additional rare heterozygous missense variants in TLN1 were identified in 10 sporadic cases. Incomplete penetrance, suggesting genetic or environmental modifiers of this episodic disorder, was evident in the familial case and 5 individuals with sporadic SCAD from whom parental DNA was available. Conclusions: Our findings reveal TLN1 as a disease-associated gene in familial and sporadic SCAD and, together with abnormal vascular phenotypes reported in animal models of talin 1 disruption, implicate impaired structural integrity of the coronary artery cytoskeleton in SCAD susceptibility.

Immune thrombocytopenia (ITP) is an idiopathic disorder which could betriggered by various factors including; drugs, rheumatic diseases and malignancies. We aimedto present a 66 year old woman diagnosed with ITP whose history was relevant for influenzavaccine and non-steroidal anti-inflammatory drug (diclofenac) use. She responded well tostandard ITP therapy. In conclusion, medications should be elaborately questioned in patientswith thrombocytopenia, especially, when a diagnosis of ITP was considered.

Nevoid hyperkeratosis of nipple and areola was first described by Tauber in 1923. It is a rare idiopathic disorder characterized by papular warty excrescences on the nipple and areola or both. Histopathology shows hyperkeratosis, acanthosis, papillomatosis, elongation of rete ridges, filiform interconnecting pattern, increased pigmentation in the basal layer, and sparse perivascular lymphocytic infiltrate. We report two cases of nevoid hyperkeratosis of nipple because of rarity of this condition.

The Melkersson-Rosenthal syndrome is characterized by the presence of the classic triad: orofacial granulomatosis, facial paralysis, and fissured tongue. However, all 3 symptoms appear only in 8% to 25% of the cases. Cases with only 2 symptoms are considered oligosymptomatic forms. The orofacial granulomatosis is an idiopathic disorder and represents the most common symptom of the syndrome. We report the case of an 81-year-old man with a complaint of painless swelling on the lower lip of 3 months’ duration. During the clinical exam, a fissured tongue was noticed and there was no history of facial paralysis. An incisional biopsy was performed and a noncaseating granulomatous inflammation was observed histopathologically. No foreign material was present and special stains for fungal and bacteria were negative, confirming the diagnosis of granulomatous cheilitis. The patient was treated with systemic corticosteroid and there is a complete remission of the labial edema after 10 months of follow-up.

Lichen planus (LP) is an idiopathic disorder that presents with cutaneous and genital manifestations. Esophageal LP (ELP) was first described by Al-Shihabi and Jackson [J Laryngol Otol 1982;96:567-571] in 1982. Only approximately 80 cases have been documented in the literature since. It is a rare and underrecognized disorder, leading to a delay in diagnosis and a lack of standardized management. We describe the presentation, diagnosis, and management of 6 cases of ELP, at a tertiary institution, because we believe that an increasing awareness of this condition can help identify more cases and increase our understanding of this interesting condition.

Crohn's disease is an idiopathic disorder of the gut thought to be caused by a combination of environmental and genetic factors in susceptible individuals. It is characterized by chronic transmural inflammation of the terminal ileum and colon, with typical transmural lesions. Complications, including fibrosis, mean that between 40 and 70% of patients require surgery in the first 10 years after diagnosis. Presently, there is no evidence that the current therapies which dampen inflammation modulate or reverse intestinal fibrosis. In this review, we focus on cytokines that may lead to fibrosis and stenosis and the contribution of experimental models for understanding and treatment of gut fibrosis., Instituto de Estudios Inmunológicos y Fisiopatológicos

Scleromyxedema is a chronic, idiopathic disorder associated with monoclonal gammopathy and characterized by dermal mucin deposition.1 A rare but severe complication of scleromyxedema is dermatoneuro syndrome (DNS), which presents with a flu-like prodrome, skin papules, fever, seizures, and coma. It can have catastrophic systemic effects with a substantial mortality rate of 21%2,3 requiring a multidisciplinary diagnostic approach for early detection and management.

Lichen planus (LP) is a chronic idiopathic disorder involving the skin and mucosal surfaces. Although oral mucosal involvement is common, esophageal LP (ELP) is uncommon and also under-reported. Here, we present a case of ELP who was symptomatic with dysphagia for a year, but was untreated. Increasing awareness of this condition can help identify more cases and increase our understanding of this uncommon but interesting condition.

Purpose To describe the long-term outcomes of peripheral hypertrophic subepithelial corneal degeneration. Design Retrospective case series. Participants Twenty-two patients under the care of the External Disease Service, Moorfields Eye Hospital. Methods All patients matching clinical diagnostic criteria were included. Symptomatic patients were managed either conservatively or were offered superficial keratectomy in progressive cases where symptom control was inadequate. All excised tissue was examined histologically. Main Outcome Measures Clinical phenotype, symptoms, recurrence rate after surgery, and histopathologic results. Results Twenty-two white patients (20 women and 2 men; age range, 27–88 years; median age, 42 years) had peripheral, usually bilateral (20/22 [91%]), elevated circumferential peripheral subepithelial corneal opacities and adjacent abnormal limbal vasculature, with or without pseudopterygia in 9 patients (41%), among whom 7 (32%) patients had bilateral disease. Apart from the abnormal vasculature, there were no signs of chronic ocular surface inflammation. Symptoms were ocular surface discomfort in 10 patients (45%), reduced vision in 4 patients (18%), and both of these in 5 patients (23%). Three patients (14%) were asymptomatic. There was no treatment in 6 patients (27%), topical lubricants in 8 patients (36%), spectacles in 1 patient (5%), and superficial keratectomy in 7 patients (32%; 5 bilateral), which was repeated for incomplete primary excision in 1 eye of 2 patients (9%) and for a bilateral recurrence in 1 patient (5%). Median follow-up after excision was 5 years (range, 1–11 years). Histopathologic changes were similar to those observed in pterygia (vascular component) and Salzmann's nodular degeneration (corneal component). Conclusions Peripheral hypertrophic subepithelial corneal degeneration is an uncommon, usually bilateral, idiopathic disorder, occurring mostly in white women with a distinct phenotype. The condition was first described in 2003. Further cases among some patients have been described in more recent case series of Salzmann's nodular degeneration and diffuse keratoconjunctival proliferation, as well as in the previously described familial pterygoid corneal degeneration. Surgical excision was required in 30% of patients in this series, with infrequent short-term recurrences. The cause is uncertain. Financial Disclosure(s) The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Purpose Central serous chorioretinopathy (CSC) is an idiopathic disorder characterized by serous detachments of the neurosensory retina and/or the retinal pigment epithelium affecting the macular area in the majority of cases. The objective of this study was to describe choroidal findings in patients with acute and chronic CSC based on enhanced depth imaging optical coherence tomography analysis. Methods This is a cross-sectional, noninterventional study performed at Luigi Sacco University Hospital of Milan. Inclusion criteria were the presence of diagnosed (acute or chronic) CSC and being 18 years or older. Patients were evaluated with Spectralis spectral-domain optical coherence tomography enhanced depth imaging by 2 operators. The main features analyzed were intrachoroidal hyperreflective spots and hyperreflective choroidal vessel walls, as actual measurements of wall thickness could not be performed. Results Patients with chronic CSC had hyperreflective spots in 83.3% of the cases and hyperreflective choroidal vessel walls in 75%, whereas patients with acute course had the same alterations in 33% and 6.7% of cases, respectively. Conclusions These findings, if proven, may be indicative of chronic forms, thus guiding more accurate treatments and guiding clinicians through more accurate prognosis.

Chronic eosinophilic pneumonia (CEP) is an idiopathic disorder characterized by an abnormal and marked accumulation of eosinophils in the interstitial and alveolar spaces of the lung. Idiopathic chronic eosinophilic pneumonia is reported to comprise anywhere from 0-2.5% of cases within the registries of interstitial lung disease. Diagnosis is based on the clinical constellation of symptoms, characteristic radiographic findings and peripheral blood or BAL eosinophilia, in the absence of infection or drug-induced eosinophilia. There is no consensus on the dose and duration of treatment, but most authors recommend initial doses of prednisone at 0.5–1 mg/kg/day with gradual tapering of the dose for total treatment duration of 6–12 months.

Papular mucinosis is an uncommon, idiopathic disorder characterized by dermal mucin deposition and increased collagen in the skin and internal organs. Its clinical presentation is characterized by dome-shaped, flesh colored papules that are closely spaced or linearly arranged. Papular mucinosis has been individually associated with several entities that include discoid lupus erythematosus, systemic lupus erythematosus and monoclonal gammopathy of undetermined significance. We encountered a 60-year-old woman with papular mucinosis in the setting of three concurrent disorders: discoid lupus erythematous, systemic lupus erythematosus and IgG paraproteinemia. Furthermore, we have reported the first case in North America of papular mucinosis being successfully treated with intralesional hyaluronidase.

Primary cricopharyngeal dysfunction (PCD) is a rare idiopathic disorder of the upper esophageal sphincter (UES), characterized by oropharyngeal dysphagia, frequent aspiration, and narrowing at the level of the UES. Cricopharyngeal myotomy (CPM) has been used to treat oropharyngeal dysphagia of different causes including anatomic, neuromuscular, iatrogenic, inflammatory, neoplastic, and idiopathic; however, the indications for CPM and predictors of its outcome are not clearly defined. We report a case of PCD with hypertonic UES caused by a structural abnormality localized in the cricopharyngeus muscle, visualized as a cricopharyngeal bar, which we treated successfully by CPM, achieving long-term relief.

Trachyonychia or rough nails, may present as an idiopathic disorder of the nails or it can be associated with other dermatological conditions. The dystrophic nail findings seen in trachyonychia are characterized by brittle, thin nails, with excessive longitudinal ridging. The most common histopathologic features associated with trachyonychia are spongiosis and exocytosis of inflammatory cells into the nail epithelia; typical features of lichen planus or psoriasis can also be detected. Determining the cause of trachyonychia is challenging. Treatment is often unsatisfactory, although in general it should be aimed at the underlying cause, if found. In most cases, the nail abnormalities improve spontaneously.

Sweet's syndrome is an acute febrile neutrophilic dermatosis which usually presents as an idiopathic disorder but can also be drug induced, associated with hematopoetic malignancies and myelodysplastic disorders, and more, infrequently, observed in autoimmune disorders. Sweet's syndrome has been reported in three cases of neonatal lupus, three cases of hydralazine-induced lupus in adults, and in nine pediatric and adult systemic lupus erythematosus (SLE) patients. We describe three additional adult cases of Sweet's associated with SLE and provide a focused review on nondrug-induced, nonneonatal SLE and Sweet's. In two of three new cases, as in the majority of prior cases, the skin rash of Sweet's paralleled underlying SLE disease activity. The pathogenesis of Sweet's remains elusive, but evidence suggests that cytokine dysregulation may be central to the clinical and pathological changes in this condition, as well as in SLE. Further research is needed to define the exact relationship between the two conditions.

Collapsing glomerulopathy is a proliferative disease defined by segmental or global wrinkling of the glomerular basement membranes associated with podocyte proliferation. These lesions are particularly poor responders to standard therapies. First described as an idiopathic disorder or following HIV infection, it is now associated with a broad group of diseases and different pathogenetic mechanisms, which participate in podocyte injury and mitogenic stimulation. Because of this etiologic heterogeneity, there is clear need for new therapeutic approaches to target each variant of this entity. Historical background, terminology, morphologic and phenotypic features, and suggested mechanisms are reviewed in this manuscript.

Scleromyxedema is a rare, chronic and persistent idiopathic disorder characterized by a generalized papular eruption due to dermal mucin deposition with an increase in dermal collagen. Patients usually have associated paraproteinemia. We describe the case of a 59-year-old gentleman with features of scleromyxedema, who had severe pruritus, scalp involvement, unrestricted mobility and associated peripheral eosinophilia, but no monoclonal gammopathy.

Weber–Christian disease is an idiopathic disorder characterized by chronic non-specific inflammation. In extremely rare cases, the Weber–Christian disease can involve the pericardium and the myocardium by a granulomatous process with a myocyte degeneration and interstitial fibrosis. We report about a 65-year-old man with known Weber–Christian disease confirmed by a biopsy from the nodules revealed lobular panniculitis with mixed cell infiltrate. He …

William Aldren Turner (1864-1945), in his day Physician to the National Hospital, Queen Square, and to King's College Hospital, London, was one of the major figures in the world of epileptology in the period between Hughlings Jackson in the latter part of the 19th century and the advent of electroencephalography in the 1930s. Although he also made contributions to knowledge in other areas of neurology, and with Grainger Stewart wrote a competent textbook on that subject, Turner's main professional interest throughout his career seems to have been epilepsy. On the basis of a series of earlier, rather heavily statistical, personal publications dealing with various aspects of the disorder, he authored what became a well-accepted monograph entitled Epilepsy - a study of the idiopathic disorder, which appeared in 1907, and he also gave the 1910 Morison lectures in Edinburgh on the topic. His writings on epilepsy over a period of three decades consolidated knowledge rather than led to significant advances, but helped maintain interest in the disorder during a rather long fallow phase in the development of the understanding of its nature.

Granular parakeratosis is an acquired, idiopathic disorder of keratinization typified by retention hyperkeratosis. It usually occurs in women at intertriginous sites. There have been only 2 reports of infants with granular parakeratosis to our knowledge. We describe 3 additional infants with granular parakeratosis. We demonstrate that infantile granular parakeratosis exhibits 2 clinical patterns: bilateral linear plaques in the inguinal folds; and erythematous geometric plaques underlying pressure points from the diaper. A thick, flakelike scale is present in both forms and is characteristic. Diaper wearing appears to play an important role in the genesis of infantile granular parakeratosis but the mechanisms are unclear. Therapeutic responsiveness to topical agents is ambiguous, however, spontaneous clearance after months to 1 year appears to be the rule.

Eosinophilic cellulitis (EC) is a rare idiopathic disorder, first described as a "recurrent granulomatous dermatitis with eosinophilia", that mimics cellulitis of infectious origin. We describe here a previously healthy 11-year-old girl who experienced fever and tender erythematous patch lesions after trauma to her knees. Because of the relapsing cellulitis-like skin lesions, skin biopsies were taken, resulting in a diagnosis of EC. The patient responded well to oral prednisolone but experienced side effects and relapse during dose tapering. She was switched from prednisolone to cyclosporine. Her EC remained under control, and she showed no evidence of relapse after discontinuation of cyclosporine.

Wegener granulomatosis (WG) is an uncommon, idiopathic disorder that is characterized by granulomatous inflammation of the upper and lower respiratory tract, disseminated vasculitis involving small and medium-sized vessels, and focal segmental glomerulonephritis. Approximately 25% to 50% of patients have nervous system involvement. The brain is less frequently involved. We report a case of a 74 year-old previously well woman who presented with rapidly progressing WG that culminated in subarachnoid hemorrhage. Only six cases of subarachnoid hemorrhage in the setting of WG have previously been reported. We review the common presenting signs and symptoms, diagnosis, treatment, and complications of WG.

Peyronie's disease is an idiopathic disorder of the penis that produces erectile dysfunction. It affects mainly the tunica albuginea. We describe our preliminary results with extracorporal shockwave therapy (ESWT) as a new noninvasive modality for the treatment of Peyronie's disease.In this study, 24 patients aged 36 to 67 years were treated with ESWT on the Lithostar overhead-module (Siemens). All our patients had unsuccessful medical treatment before ESWT. The average plaque was 7x15 mm. The number of shockwaves ranged from 15,000 to 25,000 (18-21 kV) delivered in four to ten sessions. Most patients needed local anesthesia before therapy.Four patients (17%) showed marked improvement and complete remission of the penile deviation. Six patients (25%) showed partial remission with painless erections after treatment. Four patients had painless erections after treatment but still had some penile deviation. In 10 patients (41%), ESWT failed, necessitating subsequent penile surgery.Our preliminary results with a response rate of 59% with ESWT for Peyronie's disease, including a 17% complete remission rate, is encouraging. However, further multicenter studies will have to prove if ESWT is a real therapeutic option for this disease.

The human body is largely composed of similar organs the contractile chambers which are, therefore, prone to similar disorders. Their functions and dysfunctions mainly depend on their constitutent muscles any one of which may overact or underact. Muscles are paired, so every muscular action is a double effect. Failure of normal cooperation and even conflict between coupled muscles is a common idiopathic disorder. Internecine conflict between paired muscles leads to rupture, dislocations and extravasations. Depending on its nature, dislocated matter can initiate various problems, micro-organisms make infections, cells make neoplasms, etc.

OBJETIVO: Apresentação do perfil epidemiológico das patologias fonoaudiológicas de fala e linguagem, de causa idiopática, especificamente relacionado à prevalência dessas desordens na população infantil de 1 a 11 anos de idade. MÉTODO: As crianças foram avaliadas nos aspectos de fala, linguagem e sistema miofuncional oral. Estabelecido o diagnóstico fonoaudiológico, foram os mesmos classificados segundo a manifestação predominante. Após agrupadas as categorias das desordens, foram diferenciadas as idades e aplicados os cálculos de prevalência. RESULTADOS: De um total de 2.980 crianças, 125 delas eram portadoras de desordens fonoaudiológicas (prevalência de 4,19). A prevalência geral mais elevada foi referente à faixa etária de 3 a 8 anos, sendo a fase crítica dos 4 aos 5 anos. As patologias de manifestação primária mais prevalentes foram, em ordem de freqüência: distúrbios articulatórios, defasagens na aquisição e desenvolvimento da linguagem oral e desordens miofuncionais orais e de funções neuro vegetativas. CONCLUSÃO: As desordens fonoaudiológicas constituem importante segmento nos agravos à saúde infantil, sendo necessário que sejam urgentemente estruturados programas fonoaudiológicos preventivos e curativos. Em sua precariedade, o sistema de saúde brasileiro não oferece uma rede de apoio para o atendimento aos portadores de patologias da comunicação, existindo apenas esforços isolados em algumas unidades de saúde. OBJECTIVE: The study of the epidemiological aspect of speech-language idiopathic disorders. The profile relates to the prevalence rate among children from 1 to 11 years old. METHOD: The subjects were assessed with regard to speech, language and myofunctional oral aspects. After diagnosis, the subjects were classified according to their principal disorder. Then the disorders were placed in separate age groups. The prevalence rates were applied. RESULTS: The sample for this study included 2,980 children, 125 with primary communicative disorders (prevalence rate = 4.19). The highest general prevalence related to the 3 to 8 year old age-group. The critical phase was that from 4 to 5 years of age. The most prevalent idiopathic speech-language disorders were: articulatory disorders; then oral language delays and miofunctional and neurovegetative disturbances. DISCUSSION: It is important to note that the communication disorder prevalence rate is very significant during childhood. It is a serious problem in Brazil, because although we have more critical cases there are no national preventive programs for these pathologies. It is, therefore, expected that this problem will increase rapidly in the near future if there isn't governmental action.

Parkinson’s disease is an idiopathic disorder of the central nervous system, characterized by resting tremors, muscular rigidity, slow and decreased movements. Oral rehabilitation of these patients requires special care, especially in those cases where the patient’s socioeconomic status is not good and patient cannot come several times for fabrication of a complete denture. This clinical report presents a case of a Parkinson’s patient who was completely rehabilitated in 3 appointments using special techniques. Border molding, final impression and jaw relation procedures were done in one appointment by using a custom tray with detachable handles and occlusal rims.

Background Recurrent peripheral facial palsy is uncommon in children. It mostly occurs as an idiopathic disorder and to a lesser extent in the setting of some infectious, genetic, or systemic disorders. However, its association with familial Mediterranean fever has not been reported before. Patient We present a 14-year-old girl who experienced three episodes of right-sided peripheral facial palsy during a 9-month interval. She had a diagnosis of familial Mediterranean fever (homozygous with M694V mutation) and she had been receiving colchicine for 8 years. Recurrent peripheral facial palsy could be a neurological manifestation of vasculitis in familial Mediterranean fever. Conclusion Recurrent peripheral facial palsy may be a manifestation of familial Mediterranean fever in children.

Background: Lichen myxedematosus (LM) is a rare idiopathic disorder characterized by papules, plaques, and/or nodules in the skin secondary to mucin deposition and variable dermal fibrosis in the absence of thyroid disease. Case reports are the predominant literature on LM due to its rarity, and the data are limited regarding the disease. No standard treatment regimen exists, and the response to treatment varies. Objective: This report adds to the limited literature on atypical LM associated with thyroid dysfunction and contributes to the growing body of evidence supporting intravenous immunoglobulin (IVIg) as effective therapy for extensive cases. Methods and Results: We report an unusual case of atypical LM associated with hypothyroidism, central nervous system (CNS) disturbances, and atrial fibrillation in a 64-year-old male. The patient experienced remarkable improvement within 3 months of beginning IVIg treatment; however, he required repeat therapy due to the recurrence of CNS symptoms 3 months after treatment. Conclusion: We agree with previous authors that thyroid dysfunction alone should not preclude a diagnosis of LM. Additionally, we contribute to the increasing evidence of IVIg being an effective treatment, particularly in the setting of systemic complications or acute worsening of LM.

Erythromelalgia is a rare neurovascular peripheral disorder of unknown cause that affects hands or feet causing painful skin redness in affected area. The attacks are periodic and are commonly triggered by heat, pressure, mild activity, exertion, insomnia or stress. Erythromelalgia can occur either as a primary or idiopathic disorder, or as secondary forms associated with hematologic diseases. Drugs have been implicated in several skin disorders but only punctual single cases of erythromelalgia have been related to drug therapies. Here we describe two cases of erythromelalgia associated with penicillin treatment, a new non-described association. Key words: Erythromelalgia, penicillin, drug

Apocrine chromhidrosis is a rare, chronic, idiopathic disorder, characterized by the excretion of pigmented sweat. A 26-year-old woman presented with a 3-year history of dark blue secretions on bilateral malar cheeks. On examination, upon pressure on the cheeks, a dark blue fluid was expressed, which appeared to arise primarily from the follicle. The patient had not appreciated it, but on examination of her axillae, a very subtle blue coloring was observed. Histopathologic examination revealed apocrine glands in the deep reticular dermis. Bluish cytoplasmic granules were observed in the apocrine epithelium lining, which correspond to lipofuscin granules. The diagnosis of apocrine cromhidrosis was made. We began treatment with 20 percent aluminum chloride hexahydrate solution and capsaicin cream with poor tolerance. Finally, we treated with botulinum toxin type A with a successful response. We report a case of facial and axillary apocrine cromhidrosis with good response to botulinum toxin type A.

Nevoid hyperkeratosis of the nipple and/or areola is a rare and idiopathic disorder with fewer than 50 cases reported in the literature. We report two cases, discuss the clinical and histological findings, and review the literature pertaining to its pathogenesis and therapy. It is hoped that, through this report, nevoid hyperkeratosis of the nipple and/or areola becomes a more recognizable and reported entity, thereby stimulating future studies regarding its pathogenesis and therapy.

Langerhans cell histiocytosis is a rare idiopathic disorder, characterized by the infiltration of 1 or more organs by large mononuclear cells. It can develop at any age. Pulmonary Langerhans cell histiocytosis (PLCH) is found more frequently and is better described in adults than in children. The PLCH findings on computed tomography (CT) scans include nodular opacities, thin-walled cysts (often bizarre in shape), and marked parenchymal fibrosis. The purpose of this study was to compare the chest CT findings between adults and children with PLCH.We retrospectively analyzed the chest CT findings in a series of 7 children and 12 adults. Two independent observers reviewed the CT studies of all cases, and then correlated their observations. Statistical analysis was done with the Fisher exact test.The mean ages were 8 years (3 mo to 16 y) for the pediatric group and 39 years (21 to 59 y) for the adults. Extrapulmonary disease was present in 3 (43%) children and 1 (8.3%) adult. All the adults reported smoking, and only 1 pediatric patient (16 y old) had a history of smoking. The CT findings were characterized by the presence of cysts, fibrosis, and nodules, with the findings being similar in size and appearance for both populations. However, the distribution was different; the subpleural parenchyma in the costophrenic recess was spared in the entire adult group, but not spared in any of the pediatric patients.The CT findings of PLCH were similar in the adult and the pediatric populations, with the exception that costophrenic recess subpleural parenchyma was spared in the adult group but demonstrated pathology in the pediatric group.

An 11-year-old girl presented with a waddling gait andrestricted abduction of both hips. A pelvic radiographshows bilateral deepening of the acetabular cavity (Fig. 1).All other radiographs were normal. Syndromes like Marfan,Stickler, Beals, Kniest and Ehlers-Danlos were ruled out.Complete blood counts, calcium, phosphorus, alkalinephosphatase, rheumatoid factor and antinuclear antibodieswere within normal limits.A center-edge angle greater than 40° and medializationof the medial wall of the acetabulum past the ilioischial lineare the diagnostic features for protrusio acetabulum in ananteroposterior pelvic radiograph [1]. The protrusio acetab-ulum is caused by a primary idiopathic disorder andsecondary infectious, metabolic, inflammatory, traumatic,neoplastic and genetic disorders [2]. Primary protrusioacetabulum is common in females, bilaterally, and inadolescents. The triradiate cartilage closure is effective inreversing or stopping the protrusio before 10 years of age[1]. Early diagnosis and treatment reduce long-termdisability and secondary osteoarthritis in patients.References

Constipation has been traditionally viewed and classified as a functional or idiopathic disorder. However, evidence has been accumulating that suggests how constipation might be considered as due to abnormalities of the enteric nervous system, since alterations of this system, not evident in conventional histological examination, may be present in these patients. These abnormalities often consist in decrease or loss of the enteric glial cells, a pathological finding present in most types of constipation so far investigated. In this article we will discuss these evidences, and will try to consider constipation no more as a simple functional or idiopathic disorder but as a form of enteric neuro-gliopathy.

• Langerhans cell histiocytosis replaces the other descriptive terms for this idiopathic disorder that included histiocytosis X and eosinophilic granuloma.

Pulmonary alveolar microlithiasis (PAM) is a rare idiopathic disorder, usually with familial association, leading to alveolar deposition of calcium phosphate microliths. A 48-year-old female patient was referred to our clinic for bone scintigraphy because of severe osteoporosis (L1–L4 T score was −3.5 ; serum calcium values and 24-h calciuria in normal range). Bone scintigraphy showed bilateral, intense pulmonary 99mTc- methylenediphosphonate uptake, which led us to clinical suspicion of PAM. The chest radiograph revealed extensive, bilateral micronodular sand-like appearance, predominantly in the lower lung fields. The diagnosis of PAM was confirmed by transbronchial lung biopsy. Although the bone scintigraphy finding is sometimes negative in the early stage of PAM , this imaging method could be very useful in the detection of PAM.

Pseudotumor cerebri is usually thought of as an idiopathic disorder of obese young women. A variety of systemic illnesses have been described as having pseudotumor cerebri as an associated condition. This syndrome has been infrequently reported as a primary feature of central nervous system involvement by vasculitides or collagen vascular disease such as systemic lupus erythematosus. The authors report a nine-year-old child who presented with pseudotumor cerebri as a major sign of systemic vasculitis. Although a vasculitis was suspected and high dose gluco-corticosteroids administered, it was only after a sudden fatal pulmonary hemorrhage that the postmortem examination revealed the overwhelming nature of the vasculitis, consistent with the polyangiitis overlap syndrome. This is the first reported case of pseudotumor cerebri in association with the polyangiitis overlap syndrome. Familiarity with ocular signs of vasculitides may facilitate early diagnosis and specific treatment.

Chronic inflammatory demyelinating polyneuropathy (CIDP) may develop as an isolated, idiopathic disorder or may occur with a temporal relationship to other systemic disorders. We report two cases of chronic inflammatory demyelinating polyneuropathy and membranous glomerulonephropathy (MGN) occurring in the same patients. In each case, the two disorders manifested in close proximity to each other. These two cases supplement previously reported cases of MGN as a coincident and potentially causally related comorbidity to CIDP. We review previous reports of MGN associated with the full spectrum of inflammatory demyelinating polyneuropathies (IDP).