Pachydermoperiostosis Mimicking Acromegaly: A Case Report.

Pachydermoperiostosis is a rare, hereditary disease commonly presenting with digital clubbing, pachyderma, and periosteal hypertrophy. Coarsening of facial features and spade-like enlargement of hands and feet may give rise to a diagnostic dilemma between pachydermoperiostosis and acromegaly. This report highlights a case of a 36-year-old man who presented with broadening of hands and feet, facial skin thickening, and edematous and drooping eyelids for the last 10 years. There was no history of similar presentation in his family. Such clinical presentation in corroboration with normal growth hormone level and prominent radiological abnormalities prompted us to make a diagnosis of pachydermoperiostosis.

Cutaneous Cysts with Nail Dystrophy in a Young Female: A Classical Association.

Pachyonychia Congenita (PC) refers to a group of autosomal dominant disorders with variable clinical presentations. While nail dystrophy and plantar keratoderma are the most consistent features in all the variants, a myriad of other manifestations has been observed. This report highlights a case of young female presenting with multiple asymptomatic cutaneous cysts associated with plantar kearatoderma and nail dystrophy. Similar nail changes were evident in her son also. Such clinical presentation, in corroboration with histopathological evaluation of the cutaneous cyst prompted us to make a diagnosis of Pachyonychia Congenita type II.

The Bumpy Face of Che Guevara: An Interesting Case.

Placement of decorative tattoo on the skin may lead to various immunological, infective, and coincidental complications. Inoculation of human papillomavirus leading to development of verruca is an uncommon complication of tattoos. The present report highlights the development of verruca vulgaris, developing after 2 years of tattooing in a young male.

Dual Infection with Mycobacterium tuberculosis and Mycobacterium leprae at Same Site in an Immunocompetent Patient: An Unusual Presentation.

Occurrence of two mycobacterial infections Tuberculosis & Leprosy in a single immunocompetent person and at single site is very unusual even in endemic countries like India. We report the case of 23yr old male, who previously presented with trophic ulcer under the ball of right great toe one yr back and diagnosed as a case of Multibacillary leprosy, now presented with chronic discharging sinus, ulceration, granulation and crusting over same site even under treatment with MDT MB. Such clinical presentation, in corroboration with proper diagnostic test likes AFB staining and BACTEC culture evaluation with no evidence immunosuppression prompted us to make a diagnosis of dual infection with M. leprae and M. tuberculosis at same site.

A systematic review and meta-analysis on the efficacy of intravesical therapy for bladder pain syndrome/interstitial cystitis.

Bladder pain syndrome/interstitial cystitis (BPS/IC) is a chronic disease characterised by persistent irritating micturition symptoms and pain. The objective was to compare the clinical efficacy of currently available products for intravesical therapy of BPS/IC and to assess their pharmacoeconomic impact. A Pubmed/Medline database search was performed for articles on intravesical therapy for BPS/IC. A total of 345 publications were identified, from which 326 were excluded. Statistical evaluation was performed with effect size (ES) assessment of symptom reduction and response rates. The final set of 19 articles on intravesical BPS/IC therapy included 5 prospective controlled trials (CTs), the remaining were classified as uncontrolled clinical studies. The total number of patients included was 801, 228 of whom had been evaluated in a CT. For CTs, the largest ES for symptom reduction as well as response rate was observed for high molecular weight hyaluronic acid (HMW-HA), with similar findings in two uncontrolled studies with HMW-HA. The number needed to treat to achieve a response to intravesical therapy was 2.67 for intravesical pentosan polysulphate and 1.31 for HMW-HA which were superior to all other instillates. HMW-HA was significantly superior in cost effectiveness and cost efficacy to all other instillation regimes. The present meta-analysis combined medical and pharmacoeconomic aspects and demonstrated an advantage of HMW-HA over other instillation agents; however, direct comparisons between the different products have not been performed to date in properly designed controlled studies.

Uncommon Presentation of a Common Histiocytic Tumor: A Rare Entity.

Juvenile xanthogranuloma (JXG) is the most common form of non-Langerhans cell histiocytic lesion, characterized by benign, usually asymptomatic, self-healing yellowish brown papulonodular lesions of skin and other organs in the absence of metabolic disorder. The cells of origin of JXG are dermal dendrocytic cells. Histopathologically there is dermal infiltration of foamy or non-foamy histiocytes with or without giant cell. Immunohistochemistry shows CD68 positivity with CD1a and S-100 negativity of lesional cells although S-100-positive JXG have been reported. JXG may be associated with neurofibromatosis type one (NF 1) with increased risk of juvenile chronic myelogenous leukemia and other hematological malignancies. Our case was S-100 immunoreactive multiple, cutaneous JXGs with NF 1 without any visceral involvement or malignant complication. We are presenting this case due to its rarity.

Transitional cell carcinoma of the ureteric stump: a systematic review of the literature.

OBJECTIVE: To present a review of the literature using evidence-based criteria for diagnosis and treatment of malignant growths in the ureteric remnant following nephrectomy for non-malignant disease. METHODS: A database search using the key search words was performed, producing a total of 16 articles published between 1952 and 2009. The Oxford Centre for Evidence-Based Medicine classification was used. Statistical significance was tested by Pearson correlation. Demographic data, reason for nephrectomy, symptoms, time to diagnosis since initial nephrectomy, imaging modality and treatment option chosen, as well as histology and overall survival were reviewed. RESULTS: Analysis was possible for 33 out of 63 cases reported in the literature. There was a male predominance (82%). Visible, painless haematuria was the presenting symptom in 72% of cases. Open ureterectomy was performed in the majority of patients (85%), while none had laparoscopic surgery. Transitional cell carcinoma was found in 66% of cases. Mean follow-up was 2.7 years. Metastases were detected in 36% and correlated significantly with cancer-specific mortality (95% CI: p < 0.001). Tumour stage, grade and cell type did not correlate significantly with mortality. CONCLUSION: Gross, painless haematuria is a feature highly suggestive of neoplastic change. Diagnosis often involves multimodality imaging and endoscopy. Complete ureterectomy with removal of bladder cuff, previously resected endoscopically, is the treatment of choice. Metastases at diagnosis and follow-up carry a worse prognosis.

Effective management of lower urinary tract dysfunction in idiopathic Parkinson's disease.

Parkinson's disease, also known as paralysis agitans, is a progressive degenerative disorder of the central nervous system, with onset usually between the ages of 50 and 65 years, and is associated with loss of dopaminergic neurons in the subsantia nigra and the presence of Lewy bodies. It is characterized by the triad of resting tremor, muscular rigidity and bradykinesia. Often-accompanying abnormalities include disorders of equilibrium, posture and autonomic function, including micturition. Symptoms from the lower urinary tract add a significant comorbidity factor in these patients. The incidence and prevalence of lower urinary tract dysfunction rise with increasing progression of the underlying neurological disease. They present a troublesome and difficult to treat health issue with a profound impact on the patient's quality of life. Storage symptoms seem to predominate. In the long term, renal function might be compromised, mainly as a result of elevated intravesical pressure. Various conservative, minimally-invasive and surgical treatment options are available to prevent harmful sequelae, and to improve the quality of life of these patients. We present an overview of current and prospective treatment strategies.

Mucocutaneous and demographic features of systemic sclerosis: a profile of 46 patients from eastern India.

BACKGROUND: Systemic sclerosis (SSc) is a multisystem connective tissue disorder of uncertain etiology. The clinical picture is frequently dominated by prominent cutaneous manifestations that have diagnostic and prognostic significance. The objective of the present study was to find out the demographic profile and the relative frequencies and characteristics of different mucocutaneous features of SSc in a group of patients from eastern India. In addition, we sought to compare the frequency and pattern of the findings in the limited versus the diffuse variety of the disease. MATERIALS AND METHODS: This was a cross-sectional, clinical observational study. Consecutive patients of SSc attending the dermatology O.P.D. of a tertiary care hospital of eastern India over 3 years were enrolled to the present study. RESULTS: A total of 46 patients (41 females and 5 males; mean age 29.6±12.3 years) of SSc were evaluated. Among mucocutaneous manifestations Raynaud's phenomenon was present in 39 (84.8%) patients. Other cutaneous features included dyspigmentation (40, 86.9%), sclerodactyly (38, 82.6%), inability to open the mouth (38,82.6%), mat-like telangiectasia (11,23.1%), fingertip ulceration and scarring (29,63%), cutaneous calcinosis (1,2.2%), digital gangrene in (2,4.3%), generalized pruritus (4,8.7%), cutaneous small vessel vasculitis (2,4.3%), chronic urticaria (2,4.3%), flexion contractures of the fingers (13,28.3%), and amputation of the digits (3,6.5%). Mucosal changes were observed in 10 (21.7%) patients and nail changes were seen in 13 (28.2%) patients. Diffuse cutaneous SSc was noted in 27 (58.7%) patients and limited cutaneous SSc was seen in the remainder. Thirty-six (78.2%) patients tested positive for ANA. CONCLUSION: The present study provides a snapshot of the spectrum of the demographic and mucocutaneous manifestations of SSc in the eastern Indian population. We have not observed any statistically significant differences between dcSSc and lcSSc in terms of mucocutaneous manifestations in the studied population.

Paediatric balanitis xerotica obliterans: an 8-year experience.

OBJECTIVE: Balanitis Xerotica Obliterans (BXO) is rarely described in the paediatric population. We report our 8-year experience, at Harold Wood Hospital, with BXO in circumcised boys. MATERIALS AND METHODS: Pathological tissue diagnoses of BXO from 1997 to 2005 were extracted from our histopathology database. Patient records were reviewed and demographic features, clinical presentation, referral history, operative procedure(s) and postoperative course were recorded. RESULTS: A total of 40 patients had a confirmed tissue diagnosis of BXO. Mean patient age was 9.6 years. The most common referral diagnoses were phimosis (70%), balanitis (25%) and only 2 patients (5%) had a referral diagnosis of BXO. 35 (87%) underwent curative circumcision and had no recurrence at a median follow-up of 13.5 months. A total of 5 patients (15%) had BXO involvement of the meatus and underwent circumcision combined with meatotomy or meatoplasty. No patients required extensive plastic operations of the penis. CONCLUSIONS: The incidence of BXO in paediatric age group may be higher than previously reported. Early recognition of this condition is important because of the associated urethral stricture formation. Hence, we recommend that all circumcision specimens are sent for histopathological analysis (especially in areas where circumcision is widely practiced for ritual and religious reason) and patients should be followed up appropriately.

Urinary tract dysfunction in Parkinson's disease: a review.

INTRODUCTION: Parkinson's disease is an extrapyramidal neurological disorder. Although motor symptoms are a predominant feature of the condition, non-motor symptoms have also been recognized. Urinary symptoms are frequently present in patients affected with Parkinson's disease (PD). Symptoms such as urgency, frequency, nocturia and urge incontinence significantly impact the patient's quality of life. We discuss the urinary dysfunction seen in patients with Parkinson's disease and consider the pathophysiology, important differentials, the investigations and management options for such patients. MATERIALS AND METHODS: An extensive search was performed using the PubMed(®)/EMBASE(®) databases to identify the available literature on urinary disturbances and Parkinson's disease. Reference was also made to current national guidelines on Parkinson's disease. RESULTS: Urinary disturbances are frequently observed in sufferers of Parkinson's disease resulting in significant impact to the individual's quality of life. Studies report that storage symptoms are present in 57-83% of patients, whereas voiding symptoms are seen in 17-27% patients. Out of all the urinary symptoms, nocturia is the most common complaint in >60% patients with PD. Urgency occurs in 33-54% of patients, whilst frequency is experienced by 16-36% of patients. Detrusor overactivity (DO) is the commonest cystometric abnormality in patients with PD. The rate of neurogenic DO in patients with PD is 45-93%. The main differential to consider is Multiple System Atrophy (MSA) in which all patients are ultimately afflicted with urinary disturbance. It is well recognized that patients initially diagnosed with PD may in fact have MSA, and it is important to distinguish the two as their urological management is different. Patients presenting with refractory LUTS with concurrent PD should undergo full urodynamic investigation including cystometry, flowmetry and ultrasonography before treatment is initiated. DISCUSSION: Referral to a urologist is advised in those with persistent or refractory urinary complaints. Urodynamic evaluation allows determination of the underlying bladder disorder; however, post-void residuals suffice in the uncomplicated patient. The pathophysiology of urinary dysfunction and current investigation and treatment modalities are discussed.

The practice of the first check cystoscopy following radiotherapy for the treatment of muscle-invasive (T3N0M0) bladder cancer: a UK national survey.

OBJECTIVES: To determine the current trends in the follow-up of patients with muscle-invasive bladder cancer following radiotherapy, among consultants in the United Kingdom and the republic of Ireland in terms of cystoscopic survey and imaging of the upper tracts. METHODS: A national postal survey was carried out in 2006 including 602 urologists registered with the British Association of Urological Surgeons (BAUS). RESULTS: About 40% of the consultants participated in the survey. Seventy-seven per cent performed cystoscopy 3 months post-radiotherapy and 78% used rigid cystoscopy. The majority (91%) did not take routine random biopsies at the time of the first cystoscopy if it looked normal. Seventy-eight per cent of the participants requested upper-tract imaging in the presence of cystoscopic abnormalities, and long-term follow-up was recommended by 75%. CONCLUSIONS: There is a wide-range of practice regarding the first check cystoscopy following radiotherapy for muscle-invasive bladder cancer and it could prove the foundation for more randomised trials to determine the evidence-based practice.