Rare Pathology Research Articles

Case Report: Sclerosing lymphocytic lobulitis of the breast: case report and literature review

Sclerosing lymphocytic lobulitis, also known as diabetic mastopathy, is a rare and benign pathology predominantly observed in young women with type I diabetes, typically with degenerative complications. Clinically, the lesion can mimic carcinoma. Biopsy remains the gold standard for a definitive diagnosis. Radical surgery is generally unnecessary when histological verification can be achievedusing core needle biopsy. This case report details the clinical and radiological findings, as well as the management of a 33-year-old patient with a 22-year history of type I diabetes who presented with sclerosing lymphocytic lobulitis.

Mesenteric cysts.

<b>Introduction:</b> Mesenteric cysts (MCs) are a rare pathology of the abdominal cavity (with an incidence of 1/27.000 to 1/250.000 admissions) and less than 1000 cases have been described in the specialized literature.<b>Aim:</b> To identify the characteristics of clinical manifestations, diagnostics, and treatment of MCs.<b>Materials and methods:</b> Within 2001-2017, 17 consecutive patients with MCs were observed. Ultrasound scan (n = 17), computed tomography (n = 15), and magnetic resonance imaging (n = 1) were used to diagnose MC.<b>Results:</b> The mean age of patients with MCs was 43.4 3.5 years (range from 19 to 67), with a significant predominance of women (n = 14, 82.4%). The main clinical symptoms of MCs included abdominal pain (n = 9, 52.9%), pain + abdominal discomfort (n = 4, 23.5%), abdominal pain + nausea (n = 2, 11.8%), and abdominal discomfort (n = 2, 11.8%). In 13 (76%) cases, a palpable mass was noted in the abdominal cavity. According to imaging studies, the largest size of the MCs was 12.1 1.2 cm (from 5 to 20 cm) and the smallest - 8.6 0.8 cm (from 4 to 15 cm). The density of MC contents by computed tomography data varied from 2 to 26 HU. All patients underwent surgery via laparotomy. MCs were significantly more often located in the mesentery of the large bowel (n = 14, 82.4%) as compared to the small intestine (n = 3, 17.6%). The MCs of the small intestine in all cases were located in the mesentery of the jejunum, whereas in the colon lesions, they were distributed as follows: cecum (n = 1, 7.1%), ascending colon (n = 3, 21.4%), transverse colon (n = 7, 50%), and sigmoid colon (n = 3, 21.4%). Most MCs (n = 16, 94.1%) were enucleated, and only in one case segmental resection of small intestine with MCs was performed. The histopathological examination revealed: simple mesothelial MC - in 8 (47.1%), simple lymphatic MC - in 7 (41.2%), and lymphangioma - in 2 (11.7%) cases.<b>Conclusions:</b> MC is a rare pathology of abdominal organs. The clinical manifestations of MCs are nonspecific, and the final diagnosis becomes apparent only during surgery. In most cases, it is possible to perform MC enucleation without long-term recurrences.

Rectal duplication in association of other developmental malformations

Purpose of the study. It is to show the difficulty of making a diagnosis and choosing surgical treatment as a rare disease - duplication of the rectum in combination with other anomalies of the development of organs and systems.Material and research methods. Under our supervision there were 2 children diagnosed with congenital anomaly of the gastrointestinal tract. Isolated duplication of the rectum. Perineal hypospadias. Narrowing of the vesicoureteral segment on the right. Ureterohydronephrosis Ⅰ degree on the right.Results. A control study did not reveal any pathology in the internal organs of the children. Ultrasound of the urinary system: the pyelocaliceal system is not dilated, no symptoms of megaureter were detected. Children do not lag behind their peers in mental and physical development. Today, the children’s condition is satisfactory, there are no complaints, they are under the supervision of a pediatric surgeon.Conclusion. Duplication of the rectum is an isolated rare pathology in children in combination with malformations of the genitourinary system, with very diverse clinical manifestations, which poses a problem in making a diagnosis before surgery. Open surgery is safer and good results can be achieved.

Oral Myiasis in Anterior Palate - A Rare Case Report & Review of Literature

Oral myiasis was coined by Reverend F. William Hope in 1840. It is a rare pathology and risk to the patients in humans suffering from myiasis. It has a higher incidence in rural areas and in developing countries having hot climatic conditions such as tropical and sub-tropical zones of Africa and America. Most of the infestations are associated with various predisposing factors such as poor oral hygiene, suppurative lesions, senility, alcoholism, and debilitating conditions and reported in post-extraction socket, trauma. The diagnosis is made basically by the presence of maggots. This article deals with oral myiasis in cerebral palsy female patient. The treatment consisted of manual removal of the maggots by topical application of turpentine oil and surgical debridement of the wound Keywords: Diptera fly, human myiasis, ivermectin, musca domestica, myiasis, parasitic infection

Rare Broad Ligament Cavernous Hemangioma Mimicking Advanced Endometriosis: A Case Report.

Cavernous hemangioma within the female genital tract is an extremely rare pathology, characterized by irregular vascular spaces containing blood or thrombus. We present a unique case of a 42-year-old primiparous woman who presented with typical endometriosis symptoms such as dysmenorrhea, dyspareunia, and heavy menstrual bleeding. The patient also experienced complex postpartum symptoms, which were misdiagnosed as cholecystitis and retained placental products. Imaging studies suggested deep infiltrative endometriosis with extraovarian endometriotic lesions. Surgical exploration revealed a hemangioma within the right anterior broad ligament alongside peritoneal endometriosis lesions. The hemangioma itself expresses estrogen and progesterone receptors in stromal cells. The presence of steroid hormone receptors strongly suggests symptom alleviation during the menstrual cycle and the postpartum period. The coexistence of cavernous hemangioma and endometriosis in the broad ligament, previously unreported, and symptomatic overlap between the two conditions complicates diagnosis and management, emphasizing the need for comprehensive evaluation integrating clinical symptoms and imaging findings.

Late diagnosis of chronic inflammatory demyelinating polyneuropathy on the background of misdiagnosed alcoholic polyneuropathy: a clinical case

Chronic inflammatory demyelinating polyneuropathy is a rare immune-mediated polyneuropathy pathology of the peripheral nervous system. The presence of etiological factors in the patient, such as type 1 and 2 diabetes mellitus, alcohol intoxication in the anamnesis or other causes of polyneuropathy, which are often found in practice, can disguise chronic inflammatory demyelinating polyneuropathy and lead to delayed diagnosis of potentially curable polyneuropathy, which further increases the likelihood of disability of the patient and a significant decrease in the quality of his life. The clinical case under consideration is a demonstration of such diagnostic difficulties, which led to a delayed diagnosis of chronic inflammatory demyelinating polyneuropathy against the background of alcohol abuse in the debut of neurological disorders.

Intracranial complications secondary to acute bacterial sinusitis requiring neurosurgical intervention before and after the onset of the COVID-19 pandemic.

Intracranial complications of acute bacterial sinusitis are rare pathologies that occur in children, and are associated with significant neurological morbidity and mortality. There is a subjective concern among neurosurgeons that the incidence of this rare disease has increased since the onset of the novel COVID-19 pandemic. The primary objective of this study was to review the presentation and management of patients admitted at the authors' institution with intracranial extension of sinusitis, to better understand the local disease burden relative to the COVID-19 pandemic. This is a single-center retrospective observational cohort study. The patients underwent neurosurgical intervention for intracranial extension of sinusitis between January 1, 2007, and March 1, 2023. The historical cohort was defined as those patients who presented prior to March 2020. Clinical covariates such as surgical and microbiological data were collected and analyzed. A total of 78 patients (55 historical, 23 new) were included; they had a median age of 11.7 years and a male predominance of 69.2%. There was a significant increase in the annual rate of neurosurgical intervention for suppurative intracranial extension of acute bacterial sinusitis after the onset of the COVID-19 pandemic, with an average of 4.2 cases per year prior to March 2020 compared to 7.7 cases per year after that date (p = 0.013). This increase was largely driven by the unprecedented case volume of 13 cases in 2022. Patients in the new cohort were older (p = 0.009) and more likely to have Pott's puffy tumor/frontal bone osteomyelitis (p = 0.003) at the time of presentation than patients in the historical cohort. Patients in the new cohort had lower rates of readmission within 30 days of discharge than those in the historical cohort (p = 0.047). In both cohorts, patients with seizure on presentation were more likely to have neurological sequelae at last follow-up (p = 0.004), which occurred at a median of 2.9 months after discharge. Clinicians encountering pediatric patients presenting with persistent symptoms of acute bacterial sinusitis must have a high index of suspicion for suppurative intracranial extension. Prompt neuroimaging and subsequent neurosurgical intervention are critical to ensure timely diagnosis and treatment. The results in this study show a significant increase in the number of neurosurgical interventions for suppurative intracranial extension of sinusitis per year after the onset of the COVID-19 pandemic. Further research is needed to understand the underlying pathophysiology of this clinical phenomenon.

Surgical management of chondrosarcomas of the skull-base and temporal bone.

To analyze the overall long-term outcome of surgically treated skull base and temporal bone chondrosarcomas. The medical records of patients with surgically treated skull base and temporal bone chondrosarcomas between 1983 and 2024 were thoroughly evaluated. Out of a total of over 5000 skull base surgeries performed at our center, only 29 patients had histopathologically confirmed chondrosarcomas of the skull base and temporal bone. The mean of patients age was 45.6, and the male-to-female ratio was 1.9:1. The most common symptoms included hearing loss (58.6%), tinnitus (41.4%), diplopia (31%), dysphonia (24.1%), dysphagia (20.7%), vertigo (10.3%), and dizziness (10.3%). The most frequent locations of lesions among the 29 patients are as follows: petroclival region (34.5%), jugular foramen (27.6%), petrous apex (17.2%), middle ear (13.8%), others (3.4%). TO, IFTA, IFTB, IFTC, POTS, and combined surgical approaches were commonly used. The rate of gross total removal and recurrence was 82.6% and 13.8% respectively. The follow-up duration of 6 patients was more than five years and less than ten years whereas ten patients had more than ten years of follow-up. Chondrosarcoma of the skull base and temporal bone is a very rare pathology. Due to its multiple potential sites of origin and histological specificity, it presents us with significant challenges. Gross total removal is the primary treatment for chondrosarcoma of the skull base and temporal bone. Personalized decision-making should be considered based on the following aspects: tumor, patient, and surgeon's factors. Postoperative radiation therapy is complementary to surgical treatment in grades II and III lesions to achieve long-term survival.

Adult Thornwalt Cyst: A Rare Case Report.

Nasopharyngeal cysts are rare benign entity, smaller in size and usually asymptomatic. They are mostly diagnosed incidentally on MRI. Larger cysts commonly presents with spasmodic and obstructive symptoms. Thornwaldt cysts are congenital cysts originating from the mid- line of the nasopharynx just above the upper border of the superior constrictor muscle. They represent primitive communication between notochord remnants and the pharyngeal endoderm. We report this case because of its unique presentation in a 35year old male. Patient presented with nasal blockage, nasal discharge, snoring, ear pain and aural fullness, intermittent neck pain and neck stiffness. Preoperative evaluation included diagnostic nasal endoscopy (DNE) and Magnetic resonance imaging (MRI-scan). A large cystic mass of size 2.8cm × 3cm was diagnosed on DNE and MRI. Transnasal Marsupialisation and excision of the cyst was planned using powered instruments. Post operatively, patient noticed marked improvement in the obstructive symptoms. Regular follow up was done 6 monthly for 2years. No recurrence was noted on DNE. Although a rare pathology in adulthood, it should be kept as a differential diagnosis for cervical pain and neck stiffness.

Robotic-assisted ectopic mediastinal parathyroidectomy: a single institution experience and operative review for the thoracic surgeon.

Parathyroidectomy remains the only definitive cure for primary hyperparathyroidism (PHPT). In rare cases, ectopic hyperfunctioning glands are located in the mediastinum, necessitating a thoracic surgical approach. The objective of this project was to review a single high-volume institutional experience of this presentation, with specific attention to the use of a robotic-assisted thoracic surgery (RATS) approach. This was a single-center, 5-year retrospective cohort study. All patients who underwent RATS mediastinal mass resection (MMR) for PHPT at the University of Colorado Anschutz Medical Campus were targeted for inclusion. Patient cases were reviewed for demographics, history, operative data, laboratory values, and postoperative course. Eight patients underwent RATS-MMR for PHPT between 2018-2023. Median [interquartile range] operative time was 178 [138-213] minutes, and length of stay was 2.0 [1.5-2.0] days. One patient experienced post-operative chylothorax requiring dietary modification. There were no other 30-day complications or readmissions. Final pathology confirmed intrathymic parathyroid tissue in all patients. All patients achieved cure of PHPT. The robotic-assisted approach has low morbidity and associated hospital length of stay and can be safely used to cure PHPT. As this is a rare pathology with an infrequently utilized surgical approach, it is important to critically discuss the diagnostic evaluation and operative course, aimed at educating the thoracic surgeon who may encounter and assist in the management of these patients.

Ovarian neoplasms in pediatric gynecology (literature review)

Ovarian neoplasms in children and adolescents are rare pathologies (2.6–3.0 per 100,000 patients a year), however, they are sufficiently relevant in pediatric gynecology nowadays. The age of the patients highly correlates with different histological types of ovarian neoplasms. And this is a reason for discussing this disease in different age groups apart. Unfortunately, there are a number of unresolved issues connected with the etiology, pathogenesis and diagnosis. This paper discusses recent information related with the subject “ovarian neoplasms in pediatric gynecology” and particularly questions which require further study.The aim of the work is to conduct a review of recent literature which corresponds to this subject and to discover questions which require further study.The analysis revealed that the etiology and pathogenesis of a number of rare neoplasms has not been discovered fully yet and that can be the cause of the delayed disease diagnostics. Apart from this there are no tools for the preoperative stratification of the risk of malignancy and that can lead to more frequent radical operations for benign tumors.

Kidney autotransplantation with renal arteries injury in a patient with Takayasu arteritis

Takayasu arteritis (AT) is a systemic vasculitis of large vessels. As a rule, AT develops in patients under 50 years of age and is characterized by vasculitis, often granulomatous, with a predominant lesion of the aorta and/or its main branches. The article describes a clinical case of a patient with bilateral lesions of the renal arteries caused by AT, who underwent kidney autotransplantation. The first manifestations of psoriasis in the patient occurred after psychological stress 15 years after surgery. The described clinical observation of multi-stage surgical treatment of Takayasu arteritis, which ended favorably, is presented by us in order to demonstrate the capabilities of modern medical technologies in both diagnosis and surgical treatment of multi-vessel lesions in Takayasu arteritis. The combination of AT and psoriasis is a rare pathology, the problems of diagnosis and treatment of which require further study.

Intracranial Cysts: A Single-Institution Experience With 27 Surgically Managed Cases.

Introduction Intracranial cysts (ICs) are rare pathologies that are often found incidentally during radiological examinations. They may appear in various brain regions and are categorized as normal, congenital, traumatic, or tumor-associated variants. ICs can be asymptomatic or cause symptoms, such as headaches, visual impairments, or seizures, depending on their size and location. Severe complications include obstructive hydrocephalus, loss of consciousness, and intracranial bleeding. Surgical excision is the most accepted type of management in most ICs. Objectives This study aimed to evaluate 27 surgically managed ICs in a tertiary hospital focusing on their clinical, radiological, histopathological, surgical outcomes, and prognosis to enhance understanding and management of these rare, benign cysts. Methodology This retrospective cohort study included 27 surgically managed ICs with pathological confirmation in King Abdulaziz Medical City, National Guard Health Affairs, Jeddah, Saudi Arabia, from May 2016 to May 2023. All extracranial and nonsurgically managed cysts have been excluded from this study.Data on demographics, clinical presentations, radiological features, surgical outcomes, and follow-up were retrospectively extracted and analyzed. MRI and CT scans were reviewed to determine cyst characteristics. Surgical outcomes and postoperative complications were recorded. Data were collected via Google Forms and analyzed using the JMP Pro software. Ethical approval was obtained from King Abdullah International Medical Research Center, Jeddah, Saudi Arabia. Results The study included 27 ICs: 11 (40.74%) colloid cysts, six (22.22%) epidermoid cysts, five (18.51%) adamantinomatous craniopharyngiomas, two (7.40%) neuroepithelial cysts, and one each of Rathke's cleft cyst (3.70%), xanthogranuloma (3.70%), and dermoid cyst (3.70%). All 27 cases were surgically managed (100.00%), with gross total resection achieved in 14 (51.85%) cases.Only 12 cases (44.44%) did not develop any surgical complications. Twenty-two cases (81.48%) experienced an improvement in the preoperative presenting symptoms. During the follow-up, only three cases (11.11%) had evidence of recurrence. Conclusion This study analyzed 27 ICs of various histopathological types. Each type showed distinct clinical and radiological features. Surgical management generally improved preoperative symptoms with low mortality and recurrence rates, although complications were common. Identifying specific radiological features is crucial for an accurate preoperative diagnosis and optimal surgical outcomes.

Clinical and radiological variants of the nail–patella syndrome: Why is it important to make a correct diagnosis?

BACKGROUND: Nail–patella syndrome is an autosomal dominant disease caused by LMX1B mutations, characterized by a combination of dermatological and musculoskeletal abnormalities. The classic tetrad of the nail–patella syndrome includes hypoplasia of the nail plates, absence or hypoplasia of the patella, elbow joint contractures, often accompanied by posterior subluxation of the radial head, presence of “iliac horns” on radiographs of the pelvic bones, and renal changes. However, its diagnosis and treatment are not described in sufficient detail in domestic and foreign literature. AIM: This study aimed to analyze the variability of clinical and radiological manifestations in children with nail–patella syndrome. MATERIALS AND METHODS: Nine patients with nail–patella syndrome were examined and treated (aged 4 months to 14 years). All children presented to the H. Turner National Medical Research Center for Children’s Orthopedics and Trauma Surgery with a diagnosis of congenital multiple arthrogryposis. The examination results excluded this diagnosis. The diagnosis of nail–patella syndrome has been verified. RESULTS: Various variants of the nail–patella syndrome have been identified, with lesions to only the elbow joints (n = 3), damage to the upper and lower extremities (n = 5), and damage to only the lower extremities (n = 1). All patients had hypoplasia of fingernail plates. However, the pathognomonic sign – “iliac horns” on radiographs of the pelvic bones – was noted only in 33% of cases. Regarding functional disorders of the kidneys, proteinuria was observed in one patient, which appeared at the age of 15 years. In two cases, the parents suffered from this disease. A genetic examination was performed in three patients: a pathogenic variant in LMX1B was detected in a heterozygous state. All patients underwent conservative treatment for knee and elbow joint contractures and foot deformities. When casting, the presence of dislocation of the radial head or patella was considered. Surgical treatment was performed on five patients. The results of surgery on the lower extremities (knee contractures, foot deformities, and hip dislocation) were good in 87% of the cases. Recurrence of flexion contractures in the elbow joints was observed both after conservative and after surgical treatment in all cases; however, their severity varied. CONCLUSIONS: Clinical observations were considered to attract the attention of doctors of different specialties to a rare pathology. Moreover, a molecular genetic study is recommended for the timely diagnosis of the nail–patella syndrome, determining the treatment techniques and disease prognosis.

Peritoneal bile granuloma formation at the site of caesarean surgical scar

Bile granuloma is a rare complication associated with cholecystectomy about which few case reports have been written. Herein we present the case of a patient who underwent laparoscopic bilateral salpingectomy for the purpose of sterilization and was incidentally found to have a linear collection of bile granulomas along the site of adhesions from her cesarean section. We also present a review of case reports on bile granuloma that have presented in OB/GYN and suggest that this rare pathology should be cited as a differential more often when considering intra-abdominal pathology, particularly for pelvic surgeons. We argue that this condition is likely more common than previously thought and suggest that without further research into this topic, routine biopsy or removal of encountered lesions may not always be necessary in patients at low likelihood for having a malignant pathology.

A clinical case of isolated non-compact left ventricular myocardium in a 29-year-old patient

BACKGROUND: Non-compact left ventricular myocardium is a rare heterogeneous pathology, which in the two-layer structure of the myocardium. There is no generally accepted definition of this form of pathology for both echocardiography and cardiac magnetic resonance imaging. Non-compact left ventricular myocardium can occur at any age and is often asymptomatic. Treatment of non-compact left ventricular myocardium is nonspecific and symptomatic. DESCRIPTION: This study describes a clinical case of a young patient who was diagnosed with non-compact left ventricular myocardium. The disease debuted in the form of cardiac arrhythmia. Holter monitoring showed frequent ventricular extrasystole and atrioventricular block of the first and second degrees. Moreover, echocardiography revealed a decrease in global contractile function of the left ventricle. The diagnosis was made after magnetic resonance imaging of the heart, which revealed the presence of non-compact myocardium of the anterior, lateral, and inferior walls of the left ventricle in the apical and middle segments. Sustained rhythm disturbances were not induced in electrophysiological research by rapid and programmed stimulation. The patient was prescribed antiarrhythmic therapy, followed by echocardiography Holter monitoring. In the future, the patient needs regular monitoring by a cardiologist. CONCLUSION: Non-compact left ventricular myocardium should be diagnosed at an early stage, so that life expectancy can be increased owing to timely treatment of heart failure and the use of oral anticoagulants, antiarrhythmic drugs, cardiac resynchronization therapy, cardioverter-defibrillator implantation, and heart transplantation when other treatment options are ineffective.

Course of Niemann – Pick disease type A/B in the context of hematopoietic stem cell transplantation

Niemann – Pick disease type A/B (NPAB) is a rare severe inherited disease from the group of accumulation diseases with a defect in the acid sphingomyelinase gene (Niemann-Pick disease types B, A/B) (ASMD). Symptoms of damage to the nervous system and internal organs manifest in infancy, leading to disability, fatalities in childhood. NPAB is so far incurable. Optimal management of the disease requires a multidisciplinary team of physicians, specialists. The basis of therapy is the elimination of existing/forming complications, symptomatic treatment. Enzyme replacement therapy as a means of modifying the course of this disease is expected to slow down the progression of pathologic manifestations of the disease not related to the central nervous system lesions. Single cases of hematopoietic stem cell transplantation (HSCT) have been described in the treatment of ASMD, which is one of the new methods aimed at normalizing the level of acid sphingomyelinase, blood parameters, as well as reducing the severity of pathological visceral manifestations. However, the development of complications during HSCT, absence of positive therapeutic effect in severe CNS lesions does not allow to widely implement this method. Taking into account the contradictory data on the efficacy of HSCT in ASMD, further clinical studies are required. Analysis of 2 clinical cases of NPAB in children from the same family allowed us to reveal differences in the course and outcomes of the disease at verification of the diagnosis at birth followed by HSCT. Difficulties in diagnosing this extremely rare pathology, which requires a multidisciplinary approach, justify the need to improve methods of early diagnosis, including the organization of genetic risk determination, introduction of prenatal genetic testing before pregnancy.

Neonatal cytosteatonecrosis: Hypercalcemia with nephrocalcinosis

Cytosteonecrosis (CSN) is a rare pathology that is poorly described in the medical literature. Its pathogenesis is still poorly elucidated, and it generally develops in the neonatal period. Diagnosed clinically, it presents as indurated, purplish skin patches on fair skin or hyperchromic patches on black skin

A "C" triad: cirrhosis and cholangitis due to Caroli disease.

Abstract Caroli disease (CD) is a rare congenital pathology of probable autosomal recessive inheritance, it is part of the fibrocystic liver diseases and is included in group V of the Todani classification. It is characterized by saccular or cystic dilations of the intrahepatic bile ducts, sometimes associated with congenital liver fibrosis, and is then called Caroli syndrome (CS). The usual clinical manifestation is recurrent abdominal pain secondary to intrahepatic or vesicular stones, and acute cholangitis crises with the consequent formation of intrahepatic abscesses.

Undiagnosed Behçet’s disease presenting as Fournier’s gangrene in undiagnosed Behçet’s disease: a case report of a young adult male

Introduction and importance: Behçet’s disease is a rare systemic condition, with the mean age of patients being ~30 years. It affects men more than women. Behçet’s disease should be considered in any patient with a systemic inflammatory disease characterized by recurrent orogenital ulcers and uveitis. Fournier’s gangrene is a rare life-threatening bacterial genital infection that acquires at any age with male predominance. Case presentation: A healthy 32-year-old male presented with fatigue, fever, oral ulcers, and discrete scrotal ulcers. He had Fournier’s gangrene and a new diagnosis of Behçet’s disease. He was treated with intravenous antibiotics with surgical debridement. Before discharge, the patient was commenced on 1 mg/day of colchicine. During the follow-up visits, we noted small ulcerations without necrosis on the scrotum, which we started with oral prednisolone and oral methotrexate. He remains under their care 6 months later. Clinical discussion: The patient had recurrent oral ulcerations followed by genital aphthous ulceration and acneiform eruptions that met Behçet’s diagnosing criteria. Fournier’s gangrene on the scrotum was diagnosed due to the physical examination, radiological findings, and histological findings. A PubMed search reveals one similar case report. This patient’s only risk factor for Bechet’s was his ethnicity and male gender for Fournier’s gangrene. Conclusion: The young adult patient presented unusually, with a diagnosis of Fournier’s gangrene superimposed on undiagnosed Behçet’s disease. An understanding of the epidemiology and risk factors can help in the diagnosis of these rare pathologies.