-
Journal of Pediatric Urology Jun 2024It is estimated that approximately one out of 200 boys has the diagnosis of lichen sclerosus (LS), previously referred to as BXO (balanitis xerotica obliterans). Severe...
INTRODUCTION
It is estimated that approximately one out of 200 boys has the diagnosis of lichen sclerosus (LS), previously referred to as BXO (balanitis xerotica obliterans). Severe progressive disease is rare however, mismanagement of urethral tissues may contribute to progression of LS.
STUDY DESIGN
The current literature regarding the management of severe lichen sclerosus was reviewed alongside our management of seven patients with ages ranging from six to ten years of age with severe lichen sclerosus who required surgical intervention. These patients were identified out of a busy pediatric practice that saw 5507 patients during the four-year span. Based on the pathophysiology of lichen sclerosus, urethral anatomy, and our management an algorithm was developed for medical and surgical management.
RESULTS
All patients received initial medical treatment with topical steroids. Three patients underwent urethral mobilization and serial biopsy. One of these patients with severe disease required a second distal urethral mobilization. Three patients were treated with circumcision, and one is responding well to topical steroids after complex reconstruction. None developed postoperative urethral disease.
DISCUSSION
Lichen sclerosus affects squamous epithelium but can extend to unaffected tissue if traumatized via the Koebner phenomenon. There are no randomized control trials for the management of the disease. Thus, appropriate early management with avoidance of urethral dilation or incision may prevent extension down the urethra that can lead to severe stricture disease. Several authors identified this as one of the worst forms of stricture diseases to manage. Based on the pathophysiology of the disease and our 4-year experience treating patients, we propose an algorithm for management of severe lichen sclerosus in boys. The diagnosis of lichen sclerosus in boys requires a high level of suspicion, and early biopsies should be obtained if suspected. If identified before circumcision or meatotomy, initial treatment should be medical. If the patient fails topical steroid therapy, circumcision and biopsy are the initial recommended surgical approach. Optimally, a biopsy with the first meatotomy establishes the diagnosis. If the disease persists, urethral mobilization may represent a curative treatment as it advances healthy urethra and allows complete removal of distal squamous epithelium instead of traumatic repeated dilations or incisions.
CONCLUSION
This paper summarizes the available literature on the management of severe LS and provides a flow diagram based on the pathophysiology of the disease and our experience sever cases.
PubMed: 38918118
DOI: 10.1016/j.jpurol.2024.06.007 -
Journal of Pediatric Surgery May 2024We describe meatal outcomes for boys undergoing circumcision to treat Lichen Sclerosus (LS/BXO) with a focus on those who underwent meatotomy/meatoplasty at circumcision...
PURPOSE
We describe meatal outcomes for boys undergoing circumcision to treat Lichen Sclerosus (LS/BXO) with a focus on those who underwent meatotomy/meatoplasty at circumcision and factors associated with post-circumcision meatal intervention.
METHODS
Retrospective review of patients undergoing circumcision for histologically confirmed LS between 2011 and 2020. Statistical testing was by Chi2 and multivariate analysis.
RESULTS
382 patients underwent circumcision at a mean of 9.1 years (SD 2.9). At circumcision, LS on the glans was documented in 213/365 (58%). Meatal involvement was documented in 74/382 (19%); 25/382 (6.5%) had a meatotomy, 94/382 (25%) had meatal calibration/dilatation and 234/367 (64%) were prescribed post-operative topical steroids. Patients with LS glans or meatal involvement were more likely to have a meatotomy (p = 0.0013) and to receive post-operative steroids (OR 5, p = 0.0001). Post circumcision, 40/382 (10%) required a median of 1 subsequent procedure (range 1-5), 10 (2.6%) underwent dilatation, 30 (7.4%) had a meatotomy. Patients undergoing meatotomy at circumcision had an odds ratio (OR) of 1.2 for subsequent meatotomy (p = 0.027). Analysis based on requirement for any subsequent procedure identified an OR of 3.1 for having had a meatotomy at circumcision (p = 0.022) and an OR of 6.0 of receiving post-operative steroids (p=<0.001).
CONCLUSIONS
Meatal stenosis following circumcision for LS requiring meatal intervention affected 10% of boys. Meatotomy at circumcision increased the likelihood of subsequent meatal intervention and is therefore not recommended.
LEVEL OF EVIDENCE
Level III.
PubMed: 38862295
DOI: 10.1016/j.jpedsurg.2024.05.010 -
Cureus May 2024A 57-year-old African-American male presented with urinary retention secondary to a history of balanitis xerotica obliterans (BXO) concurrent with penile carcinoma....
A 57-year-old African-American male presented with urinary retention secondary to a history of balanitis xerotica obliterans (BXO) concurrent with penile carcinoma. BXO, characterized by chronic, sclerosing inflammation of the male external genitalia, presents significant clinical challenges due to its progressive nature and potential for complications. The patient experienced recurrent episodes of urinary retention, leading to multiple hospital visits and disease progression, prompting a comprehensive evaluation and intervention. The patient's medical history revealed a complex array of comorbidities, including penile carcinoma secondary to BXO, urethral strictures, and meatal stenosis. Clinical assessment, including bedside bladder ultrasound and laboratory investigations, confirmed urinary retention secondary to urethral stricture, necessitating urological consultation. Management strategies involved Foley catheter placement, urethral dilation, and pharmacological interventions for pain management. Subsequent follow-up and imaging evaluations identified an increased risk of carcinoma development, highlighting the importance of surveillance and early intervention in patients with BXO. This case report highlights the intricate clinical manifestations and therapeutic considerations encountered in managing BXO and its associated pathologies.
PubMed: 38832208
DOI: 10.7759/cureus.59555 -
Journal of Clinical Medicine May 2024: Data on the incidence and comorbidity of Lichen sclerosus (LS), based on validated nationwide population-based registries, remains scarce. : To explore the incidence...
: Data on the incidence and comorbidity of Lichen sclerosus (LS), based on validated nationwide population-based registries, remains scarce. : To explore the incidence and association of comorbidities with LS in Sweden, emphasizing its potential links to malignancies and autoimmune disorders. : A population-based retrospective open cohort study was conducted using the National Patient Register to identify all individuals diagnosed with LS (ICD-10 code L90.0) from 1 January 2001 to 1 January 2021. The study included 154,424 LS patients and a sex and age matched control group of 463,273 individuals to assess the incidence and odds ratios for various cancers and premalignant conditions. : The incidence of LS in Sweden was 80.9 per 100,000 person per year, with higher incidence in females (114.4) than in males (47.2). LS patients showed an increased odds ratio for vulvar cancer (OR = 8.3; 95% CI = 7.5-9.0), penile cancer (OR = 8.9; 95% CI = 7.3-11.0), prostate cancer (OR = 1.2; 95% CI = 1.1-1.2), testicular cancer (OR = 1.4; 95% CI = 1.1-1.7), bladder cancer (OR = 1.1; 95% CI = 1.1-1.2), breast cancer (OR = 1.4; 95% CI = 1.3-1.4), leukoplakia of the vulva (OR = 253.5; 95% CI = 221.9-289.6), and leukoplakia of the penis (OR = 5.1; 95% CI = 4.9-5.4). : This study underscores the significantly increased association of various cancers and premalignant conditions in LS patients, highlighting the critical need for efficacious treatment and diligent follow-up. The association between LS and autoimmune diseases further necessitates comprehensive investigation to understand the underlying mechanisms and clinical management implications. Future research is essential to confirm these findings and elucidate the role of LS in cancer development.
PubMed: 38792303
DOI: 10.3390/jcm13102761 -
Cureus Feb 2024Contrasting ethical and legal arguments have been made concerning neonatal male circumcision (NMC) that merit the first systematic review on this topic. We performed... (Review)
Review
Contrasting ethical and legal arguments have been made concerning neonatal male circumcision (NMC) that merit the first systematic review on this topic. We performed PRISMA-compliant keyword searches of PubMed, EMBASE, SCOPUS, LexisNexis, and other databases and identified 61 articles that met the inclusion criteria. In the bibliographies of these articles, we identified 58 more relevant articles and 28 internet items. We found high-quality evidence that NMC is a low-risk procedure that provides immediate and lifetime medical and health benefits and only rarely leads to later adverse effects on sexual function or pleasure. Given this evidence, we conclude that discouraging or denying NMC is unethical from the perspective of the United Nations Convention on the Rights of the Child, which emphasizes the right to health. Further, case law supports the legality of NMC. We found, conversely, that the ethical arguments against NMC rely on distortions of the medical evidence. Thus, NMC, by experienced operators using available safety precautions, appears to be both legal and ethical. Consistent with this conclusion, all of the evidence-based pediatric policies that we reviewed describe NMC as low-risk and beneficial to public health. We calculated that a reduction in NMC in the United States from 80% to 10% would substantially increase the cases of adverse medical conditions. The present findings thus support the evidence-based NMC policy statements and are inconsistent with the non-evidence-based policies that discourage NMC. On balance, the arguments and evidence reviewed here indicate that NMC is a medically beneficial and ethical public health intervention early in life because it reduces suffering, deaths, cases, and costs of treating adverse medical conditions throughout the lifetimes of circumcised individuals.
PubMed: 38405642
DOI: 10.7759/cureus.54772 -
Frontiers in Immunology 2023Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected... (Review)
Review
Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.
Topics: Scleroderma, Systemic; Humans; Diagnosis, Differential
PubMed: 37600771
DOI: 10.3389/fimmu.2023.1180221 -
Indian Journal of Plastic Surgery :... Jun 2023Clinical classification of the urethrocutaneous fistulas (UCFs) was designed to help the surgeons in (1) categorizing the fistulas, (2) selecting appropriate...
Clinical classification of the urethrocutaneous fistulas (UCFs) was designed to help the surgeons in (1) categorizing the fistulas, (2) selecting appropriate treatments, (3) keeping record at presentation and discharge, and (4) transferring information while referring a patient with recurrent fistula to a higher center. This retrospective study comprised of 68 patients with UCFs who reported in the "Hypospadias and VVFs Clinic" between 2004 and 2016. The study was performed to determine the incidence or etiology of the UCFs. It was rather performed to classify fistulas into different categories depending on the number of fistulas: A (5 fistulas), B (16 fistulas), C-a (28 fistulas), C-b (4 fistulas), D (4 fistulas), and E (11 fistulas). Category A fistulas healed conservatively. Category B fistulas underwent transection of the fistula tracts (tractotomy), purse-string closure, or multilayered closure (fistulorrhaphy). Category C-a fistulas were reenforced by preputial or penile skin flaps or waterproofing flaps. Category C-b fistulas underwent re-tubularization of their neourethral plates and eccentric closure of peno-preputial skin. The urethral plates of category D fistulas were re-tubularized after 3 to 6 months and cover was provided by the Cecil-Culp procedure. Category E fistulas had associated hairy urethra, stricture distal urethra, stricture with diverticulum, perifistular scar-induced chordee, long narrow urethral plate, balanitis xerotica obliterans (BXO), and short reconstructed neourethra. Accordingly, appropriate corrective measures were taken. Miscellaneous category F was excluded from the study. Except for one in category D, none of the patients had any recurrence of fistula. One patient of category E had residual diverticulum. The designed clinical classification of UCFs is simple. Treatment was in accordance with reconstructive ladder wherein complexity of treatment paralleled with increasing complexity of fistulas.
PubMed: 37435345
DOI: 10.1055/s-0043-1761598 -
Cureus May 2023Background Lichen sclerosus et atrophicus (LSEA) is a chronic inflammatory dermatosis of genital and extragenital sites with a prevalence ranging from 9% in prepubertal...
Background Lichen sclerosus et atrophicus (LSEA) is a chronic inflammatory dermatosis of genital and extragenital sites with a prevalence ranging from 9% in prepubertal patients to 50% in postmenopausal patients. Chat generative pre-trained transformer (ChatGPT) is an artificial intelligence tool designed to assist humans based on supervised and reinforcement techniques. In this study, we aimed to evaluate the characteristics of patients with LSEA using ChatGPT. Methods In this retrospective study, we included all patients who presented to the outpatient dermatology department during 2017-2022 at a tertiary care teaching hospital in South India. Information regarding demographic data, characteristics of LSEA, comorbidities, and associated autoimmune disorders was gathered using a medical chart review. Following data analysis and drafting of the manuscript, the utility of ChatGPT-3 and ChatGPT-4 in finalizing the draft was assessed. Results Of 20 patients diagnosed with LSEA, 16 (80%) and four (20%) patients were females and males, respectively. Of them, 50% of female patients had attained menopause. While 65% of patients had genital LSEA, 30% of patients had extragenital LSEA only, and 5% of patients had both genital and extragenital LSEA. Furthermore, four (20%) patients were prepubertal children. Of four male patients, two (50%) were younger than 18 years of age, and one patient was diagnosed with balanitis xerotica obliterans. The commonest associated features in LSEA included joint involvement (30%), hypertension (25%), and anemia (15%). Rare concomitant disorders included psoriasis, asthma, and basal cell carcinoma over the nose. Conclusions LSEA may be confused with other various dermatoses, such as morphea, vitiligo, and lichen planus. A high index of suspicion is required, especially in children, to diagnose it early and intervene to prevent further complications. Its relationship with autoimmune disorders and comorbidities warrants further large-scale studies. ChatGPT was unreliable in the literature search due to the provision of non-existent citations. ChatGPT-4 was better than ChatGPT-3 since it provided few true publications. ChatGPT was used in this study to summarize the articles identified by the authors during the literature search and to correct grammatical errors in the final draft of the manuscript.
PubMed: 37323348
DOI: 10.7759/cureus.38987 -
Frontiers in Medicine 2023Lichen sclerosus (LS) is an underdiagnosed inflammatory mucocutaneous condition affecting the anogenital areas. Postmenopausal women are predominantly affected and, to a... (Review)
Review
Lichen sclerosus (LS) is an underdiagnosed inflammatory mucocutaneous condition affecting the anogenital areas. Postmenopausal women are predominantly affected and, to a lesser extent, men, prepubertal children, and adolescents. The etiology of LS is still unknown. Hormonal status, frequent trauma and autoimmune diseases are well-known associations for LS, yet infections do not seem to be clear risk factors. LS pathogenesis involves factors such as a genetic predisposition and an immune-mediated Th1-specific IFNγ-induced phenotype. Furthermore, there is a distinct expression of tissue remodeling associated genes as well as microRNAs. Oxidative stress with lipid and DNA peroxidation provides an enabling microenvironment to autoimmunity and carcinogenesis. Circulating IgG autoantibodies against the extracellular matrix protein 1 and hemidesmosome may contribute to the progression of LS or simply represent an epiphenomenon. The typical clinical picture includes chronic whitish atrophic patches along with itching and soreness in the vulvar, perianal and penile regions. In addition to genital scarring, and sexual and urinary dysfunction, LS may also lead to squamous cell carcinoma. Disseminated extragenital LS and oral LS are also reported. The diagnosis is usually clinical; however, a skin biopsy should be performed in case of an unclear clinical picture, treatment failure or suspicion of a neoplasm. The gold-standard therapy is the long-term application of ultrapotent or potent topical corticosteroids and, alternatively, topical calcineurin inhibitors such as pimecrolimus or tacrolimus. Collectively, LS is a common dermatological disease with a so far incompletely understood pathogenesis and only limited treatment options. To foster translational research in LS, we provide here an update on its clinical features, pathogenesis, diagnosis and (emerging) treatment options.
PubMed: 36873861
DOI: 10.3389/fmed.2023.1106318