Mucosal Involvement Research Articles

An Unusual Case of Solitary Oral Leishmaniasis in an Elderly Woman

Leishmaniasis is a chronic inflammatory disease caused by a flagellate protozoan from the genus Leishmania, transmitted by an insect vector belonging to either the genus Phlebotomus spp. or Lutzomyia spp. This disease is considered one of the most prevalent infectious diseases worldwide due to its high detection rate and the morbidity it causes. A 70-year-old woman from a low-income background, with no significant medical or drug history and no other general constitutional symptoms, presented with an asymptomatic, exophytic, granulomatous growth that has a reddish hue. The growth is located on the lingual aspect of the mandibular anterior teeth region and has been progressing over the past six months. Radiographic features suggested associated chronic periodontitis along with hyperdense calcified deposits. On excisional biopsy and histopathology, the excised specimen revealed characteristic Leishmania amastigotes and pseudoepitheliomatous hyperplasia. An abdominal ultrasound was advised to rule out any visceral involvement, but no significant findings were observed. The present case is particularly intriguing due to oral mucosal involvement by Leishmania sp. without associated primary visceral or cutaneous lesions, especially given the male predilection (M:F=7:5) and the most common site involved being the posterior palatal region for Leishmaniasis. The present case represents a rare instance. Considering the difficult socio-economic circumstances, the involvement of the World Health Organization (WHO) demonstrates an understanding of the need for collaboration in supporting underprivileged communities.

Toxic epidermal necrolysis and Steven Johnson syndrome in Sri Lanka; clinical course, HLA-B*1502 allele and its association to carbamazepine

Steven Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) occur frequently with carbamazepine among the carriers of HLA-B*1502 allele. The study evaluated the clinical course and strength of this association in Sri Lanka.Twenty four consecutive patients with SJS/TEN from multiple dermatology centres were assessed for demographics, history and clinical course. Carbamazepine induced cases with a group of healthy control (10) were tested for HLA-B*1502 using multiplex PCR with sequence specific primers.The sample had a mean age of 41.6 years; female: male ratio of 3:1. Antiepileptics were the aetiological cause in 12 (50%), amongst 9 (37.5%) taken carbamazepine. The HLA-B*1502 allele was positive only in one patient but none in the control group (10). The mucosal involvement was common (95.8%).Carbamazepine is the commonest single therapeutic agent implicated in SJS/TEN, although the association with the HLA-B*1502 allele is weak in our sample. Early mucosal involvement is a reliable clinical sign useful for the diagnosis.

Hereditary C1-inhibitor deficiency angioedema (C1-INH-HAE) in children – practical considerations

Hereditary angioedema is a rare, autosomal dominant, potentially life-threatening disorder usually resulting from C1-esterase deficiency or dysfunction. Clinical manifestations include unpredictable, acute, recurrent episodes of well-demarcated oedema occurring without pruritus, most commonly affecting the face, trunk, extremities, but also, due to mucosal involvement, the respiratory and gastrointestinal systems. Although oedema resolves spontaneously within 2–5 days, the skin lesions are disfiguring, painful and disrupt daily functioning, while abdominal attacks are very painful and often lead to hospital admissions and unnecessary surgery. In addition, laryngeal oedema, which affects at least half of patients, is life-threatening and, in many cases, leads to fatal asphyxia. Therefore, early diagnosis and implementation of proper therapeutic management as soon as possible are crucial. In recent years, significant advances have been made in the treatment of hereditary angioedema. Initially, only intravenous drugs administered during attacks were available in medical facilities. Patient education has allowed for home therapy. The next step was the introduction of subcutaneous injections, which were more convenient for patients. Finally, long-term prophylactic treatment, which completely relieves symptoms and allows for a normal life, has gained importance. Unfortunately, the high cost of these therapies remains a problem. This paper reviews in detail the clinical picture and differential diagnosis of the disorder, as well as modern treatment approaches, with special focus on the distinctive features in children.

Treatment of infantile hemangioma with topical β-blockers in pediatric practice: a review of the literature

The purpose of this review is to acquaint the audience with the benefits of infantile hemangioma (IH) treatment with local β-blockers, which are safe for the child, allow for the complete cure of hemangiomas without surgery, complications, scars, and cosmetic defects, and have practically no systemic side effects. We aimed to analyze the effectiveness and limitations of the use of local β-blockers, to show the possibility and expediency of IH management in pediatric practice, and to demonstrate that the goal of therapy is not the treatment of complications, but the prevention of their occurrence and complete resolution of the hemangioma without surgical intervention. The use of topical β-blockers, such as timolol gel or solution, provides a non-surgical, non-invasive treatment option for IH, minimizing the systemic exposure and possible side effects associated with oral β-blockers. Local treatment is best started before the age of 2 months - at an early stage, when IHs are small in size, potentially preventing the need for more invasive treatment methods. Topical use is associated with a lower risk of systemic side effects, such as hypotension or bradycardia, that can occur with oral β-blockers. Topical treatment can be applied at home, which can be more convenient for parents and caregivers, and also increases the opportunity for treatment and follow-up by pediatricians. In case of natural disasters or military operations, treatment can be done remotely using teledermatology. The effectiveness of local β-blockers mostly depends on the age at which treatment was started: the earlier it was started, the higher the effectiveness. Treatment of complicated IH with deep soft tissue, mucosal, or airway involvement usually requires a combination of systemic propranolol and topical β-blockers or other interventions such as laser therapy. The choice of treatment should be selected individually according to the degree of risk of IH, its size, localization, child's age, weight, and other parameters in each clinical case. β-adrenoblockers are the most modern, effective, non-surgical, and safe method of treating IH, they can be used both for systemic and local application. IH can be successfully treated by pediatricians and dermatologists. No conflict of interests was declared by the authors.

Endometriosis with colonic mucosal colonisation: a diagnostic confounder.

Secondary mucosal colonisation by a carcinoma originating from a distant site is a pattern of metastasis to the intestines and hepatobiliary tract and a mimic of primary neoplasia. Although endometriosis is considered benign, its ability to spread widely underscores its quasi-neoplastic nature. After noting that endometriotic glands can colonise the colonic mucosa along the basement membrane, mimicking metastatic disease, we conducted an intradepartmental review of intestinal specimens showing endometriosis obtained from 2016 to 2023 to characterise and quantify the incidence of this phenomenon. Material from 38 lower gastrointestinal specimens with a primary or ancillary diagnosis of endometriosis was identified from our surgical pathology database. Slides were reviewed, documenting the extent and micro-anatomic location affected by endometriosis, with a focus on identifying examples showing mucosal colonisation. The most common site of involvement was the distal colon (23 cases; 11 of rectum, 9 of sigmoid colon and 3 of rectosigmoid) followed by the appendix (N=10), cecum (N=2), small intestine (N=2) and 'colon not otherwise specified' (N=1). Mucosal involvement was identified in eight cases (21%), half of which demonstrated seamless colonisation of the epithelium by endometriotic glands. In two of these, the procedure was prompted by the presence of a rectal mass or stricture with concern for malignancy. Endometriosis occasionally (4/38; 10.5%) colonises colonic epithelium, potentially mimicking a metastasis or intraepithelial neoplasia/dysplasia. Although unusual, this phenomenon was observed in half of specimens from patients with mucosal involvement in whom a mass or stricture suggested malignancy, a potentially misleading pattern of endometriosis.

Sexual quality of life in patients with pemphigus: A case-control study.

Pemphigus, an autoimmune blistering disease that affects the skin and mucous membranes, significantly impairs the quality of life (QoL) of affected individuals. While there are a variety of QoL measurement tools available for assessing this disease, there is a lack of studies that specifically evaluate the sexual QoL of patients with pemphigus. This case-control study aims to investigate the impact of the disease on sexual activity as well as its overall effect on QoL. Fifty pemphigus patients, who were referred to the Dermatology Department at the University Hospital of Brescia in the period March 2019-September 2021, completed several QoL surveys, including the 36-item Short Form Health survey (SF-36), the 12-Item General Health Questionnaire (GHQ-12), the Autoimmune Bullous Disease Quality of Life (ABQOL) and either the International Index of Erectile Function (IIEF) or the Female Sexual Function Index (FSFI). The severity of the disease was assessed using the Pemphigus Disease Area Index (PDAI). Differences in QoL surveys between the case and control groups were analysed using either the t-test or the Wilcoxon-Mann-Whitney test. The correlation between QoL surveys in pemphigus patients and disease severity were analysed using Spearman's coefficient (r). The results revealed a marked impairment in overall QoL among patients with pemphigus compared to the healthy control subjects. Significant differences were observed in various domains of QoL, including physical health, mental well-being, social functioning and, notably, sexual health. Furthermore, disease severity as evaluated by the PDAI showed correlations with specific aspects of health status, and disease-specific QoL demonstrated associations with nearly all domains of health status. No significant correlations were found between sexual activity, mucosal involvement or steroid therapy and PDAI scores or disease-specific QoL measures. These findings emphasize the significant impact of pemphigus on patients' well-being, with particular attention to the impaired sexual activity.

Addressing Challenges in Diagnosis, Differential Diagnosis, and Treatment of Pemphigus: A Case Series.

Pemphigus is a rare autoimmune disease characterized by skin blisters and erosions, with or without mucosal involvement. The clinical presentation of pemphigus can resemble other bullous diseases, leading to challenges in diagnosis. This report aims to address the challenges in diagnosing and treating oral pemphigus. Three patients, ranging in age from 26 to 55 years, complained of a sore throat and mouth canker sores. Extra-oral examination revealed dry lips in case 1, while serosanguinolenta crust on the lip that bled easily was found in case 2. Intra-oral examinations in all cases showed multiple painful, sloughing-covered, erosive lesions on the entire oral mucosa. The histopathological examination of case 1 revealed pemphigus foliaceous, whereas cases 2 and 3 showed pemphigus vulgaris. Secondary syphilis-like pemphigus was given as a differential diagnosis in case 2 due to the histopathological changes not being specific. The patients were instructed to maintain oral hygiene and treated with corticosteroid, analgesic, antifungal, and anti-inflammation mouthwash, as well as vitamins and minerals. All cases showed improvement in oral lesions within 14 days to a month. In conclusion, pemphigus may mimic other bullous diseases, making diagnosis challenging. A comprehensive clinical and laboratory assessment is necessary to provide accurate diagnosis and treatment.

Pyogenic Granuloma in Kidney Transplant Recipient

Objectives: This manuscript describes a clinical case of pyogenic granuloma with atypical presentation and evolution, and outlines a brief bibliographic review on the nosological entity. Introduction: Pyogenic granuloma is a common benign skin lesion, usually small, with some more common locations and predominance in females in adulthood. It has a correlation with trauma and medications. Its pathophysiology is in the initial stages of understanding. The typical histopathological alteration is a lobular arrangement of capillaries at the base. Treatment is surgical, with a low recurrence rate; there are non-surgical alternatives. Case report: We report a case of a 25-years-old renal transplant patient who develops a giant Pyogenic granuloma in the right forearm after a mild trauma, 2 years after transplantation from a deceased donor. He performs successive surgical excisions, with several recurrences, which disappeared only after discontinuation of tacrolimus and radiotherapy. The peculiarities of this case are: post-kidney transplant status, large size, recurrences after surgery and radiotherapy.. Conclusions: A patient with pyogenic granuloma, with atypical evolution, had a favorable outcome after identification and removal of a causative factor and alternative therapy. However, this report has limited potential for generalization. There are few publications of case series involving unusual situations, in addition do the involvement of the oral mucosa, paraungual/subungual regions and cases in pregnant women. Radiotherapy is a poorly performed intervention, which can be of value in refractory cases. Further studies are needed to achieve a greater understanding of this pathology and to describe treatment alternatives.

Management and long-term clinical outcomes of patients with stage IVA cervical cancer with bladder involvement

ObjectiveTo describe the long-term outcomes of patients with stage IVA cervical cancer, a rare and deadly disease for which long-term toxicity data are scarce, to guide clinician counseling and survivorship support. MethodsIn a retrospective review of a prospectively maintained database, we identified 76 patients with stage IVA cervical cancer with biopsy- or MRI-proven bladder mucosal involvement who received definitive radiotherapy (external beam radiotherapy [EBRT] alone or EBRT plus brachytherapy) with or without chemotherapy at our institution between 2000 and 2020. We used Kaplan-Meier modeling to estimate recurrence-free survival (RFS) and overall survival (OS) and used proportional hazard modeling to identify clinical variables associated with recurrence or survival. We performed actuarial competing risk modeling for severe late toxicity (grades 3 to 5, occurring >6 months of follow-up) and vesicovaginal fistulae (VVF), censoring for pelvic recurrence and death, and made comparisons between potential predictors using Gray's test and binary logistic regression. ResultsThe median follow-up time was 76 months (interquartile range 58–91). The median OS duration was 35 months (range, 18-not reached), and the 2- and 5-year OS rates were 53.6% and 40.9%, respectively. OS and RFS did not differ significantly between patients who received EBRT alone (N = 18) or EBRT plus brachytherapy (N = 49). Current smoking was a strong predictor of severe late toxicity, whose incidence was 14% at 2 years and 17% at 10 years. The VVF incidence was 24% at 2 years and 32% at 10 years. ConclusionPatients with stage IVA cervical cancer, even those who receive EBRT alone, can have long-term survival. These patients should be followed closely for late radiation-related toxicity.

Metabolic syndrome in patients with lichen planus: Acase-control study.

Lichen Planus (LP) is a chronic dermatosis affecting the skin and mucous membranes. Chronic inflammation and oxidative stress in patients with LP is a trigger predisposing to Metabolic Syndrome. To study the association of Metabolic Syndrome in patients with LP. A hospital-based prospective case-control study was conducted from April 2021 to January 2023 including 75 histopathologically confirmed patients with LP and 82 age and sex-matched controls according to the inclusion and exclusion criteria. Metabolic Syndrome was diagnosed using Modified National Cholesterol Education Programme Adult Treatment Panel III criteria. Statistical analysis of the data was performed using Statistical Package for the Social Sciences software, version 26. The chi-square test was used for data analysis. The majority (30.6%) of the patients belonged to the age group 31-40years. The mean age of patients with LP was 46.13±14.9years. Female predominance (69.3%) was observed in our study. Patients with classic LP (54.6%) were predominantly observed. Metabolic Syndrome was significantly prevalent in LP patients than in controls (32% vs. 13.4%, p=0.005, OR 3.037) and was significantly associated with morphology (only oral mucosal involvement, 61.5%, p 0.027, OR 3.9), severity (severe LP, 58.6%, p<0.001, OR 7.79), and duration of the disease (≥6months, 55.5%, p 0.001, OR 5.42). 71% of Metabolic Syndrome was observed in females (p 0.847). Among patients with metabolic syndrome, the majority belonged to the age group between 31 and 40years (37.5%, p 0.378). Systolic and Diastolic Blood Pressure values (≥130/85mm of Hg), Serum Triglycerides (≥150mg/dl), and Low-Density Lipoprotein (>130mg/dl) were significantly elevated, and High-Density Lipoprotein (<40mg/dl) was significantly low in LP than in controls (p<0.05). The study showed a significant association of Metabolic Syndrome in patients with LP. Thus, patients with LP need to be screened to avoid complications associated with Metabolic Syndrome that is, Diabetes Mellitus, Cardiovascular Disease, colorectal cancer, and stroke.

Pyoderma gangrenosum: A single-center case series study of 32 cases

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis with multiple different clinical presentations and associated comorbidities. It often presents as ulcerated lesions with a violet/erythematous border and an irregular undercut margin. In this largest single-centre case series study in Antalya-Turkey, we reviewed 32 PG patients diagnosed consecutively within the last 5-year period. Consistent with the literature, PG morphologically often presented with the ulcerative clinic (90.6%), and inflammatory bowel disease (15.6%) was the most common etiologic factor. In our study, female gender predominance (78.2%), lower extremity localization (93.7%), and the rate of multiple ulcers at diagnosis (90.6%) were more prominent than in the literature. Unlike the literature, oral mucosa involvement and syndromic form (both, 6.2%) of the disease were detected more frequently. Our results indicate that PG patients may show differences according to geographical and ethnic differences and/or characteristics of the healthcare institution.

Clinical and Radiological Characteristics of Central Compartment Atopic Disease in Korea

Background and Objectives Central compartment atopic disease (CCAD) has been studied with increasingly more importance as a distinct subtype of chronic rhinosinusitis with nasal polyp (CRSwNP). Studies have shown that CCAD in Western populations show a strong association with allergy and typical features of medial nasal structure and sinus involvement. The purpose of this study was to evaluate the clinical characteristics of CCAD and compare them to those of other subtypes of CRS in Korea.Subjects and Method A chart review was performed retrospectively at two tertiary rhinologic centers for patients who were treated with bilateral endoscopic sinus surgery for CRS with or without nasal polyp from January 2019 to December 2021. Patients were divided into three groups, namely CCAD, CRSwNP, and CRS without nasal polyps (CRSsNP), and demographics, clinical characteristics, and imaging data were compared among the three groups.Results A total of 150 patients were included in this study (50 patients in each group). CCAD showed similar nasal symptoms with CRSwNP, but higher rates of olfactory impairment than CRSsNP did. Allergy prevalence, serum total immunoglobulin E, and serum eosinophil were significantly higher in CCAD than in CRSwNP or CRSsNP. In CT scans of paranasal sinuses, CCAD showed significantly less haziness of maxillary sinus and higher ethmoid/maxillary score ratio than the other two groups. In addition, CT scans showed mucosal involvements of central compartment in CCAD.Conclusion CCAD has characteristic endoscopic and imaging findings, showing a high prevalence of allergy and a strong association with the markers of type 2 inflammation. Therefore, CCAD is a distinct disease of CRSwNP and its allergic etiology should be considered in the management of patients with CCAD phenotype.

Juvenile Bullous Pemphigoid with Similarity Clinical Features to Chronic Bullous Disease of Childhood

Introduction: Autoimmune bullous disease (ABD) is a specific autoimmune skin disease characterized by subepidermal vesicles and bullae. In pediatrics, there is an autoimmune bullous disease controversy with clinical clusters of jewel on Chronic bullous disease of childhood (CBDC) but dominant IgG autoantibodies. A similar clinical presentation challenges diagnosis; thus, supporting studies on direct immunofluorescence (DIF) as a specific autoantibody factor are necessary. Case: A 10-year-old boy with clinically tense-walled blisters with or without an erythematous base is itchy all over his body, with a predominance of palmoplantar, neck, inguinal, and axillary, presented by erosions, crusts, and a partial cluster of jewels lesion with mucosal involvement. A complete blood count shows leukocytosis and eosinophilia. Hematoxylin-eosin (HE) staining revealed a subepidermal cleft with eosinophil predominance, while direct immunofluorescence (DIF) revealed IgG deposits in the basal membrana and intercellular parts of the basal stratum. Discussion: Histopathology reveals subepidermal blisters of eosinophil dominance, and direct immunofluorescence (DIF) examination reveals IgG autoantibody deposit dominance, verifying the diagnosis of juvenile bullous pemphigoid. Given that BP180 (BPAG2) is one of the autoantigens involved, there may be similarities between the histology and clinical presentation of Bullous pemphigoid (BP) and CBDC. Conclusion: Autoimmune bullous diseases are difficult to differentiate clinically, histological studies and direct immunofluorescence are necessary to make a diagnosis.

Adenocarcinomas of the Gynecologic Tract Involving the Urinary Bladder: A Series of 16 Cases Potentially Mimicking Urothelial Malignancy.

There is limited literature describing gynecologic adenocarcinomas involving the urinary bladder and potential diagnostic pitfalls. To describe key features distinguishing metastatic (or extension of) gynecologic adenocarcinomas from urothelial carcinomas with glandular differentiation. Retrospective review of surgical pathology cases of gynecologic adenocarcinomas involving the bladder from 2 different institutions, retrieved from surgical pathology archives, was performed. Morphologic features were recorded, along with immunohistochemistry results when available. Electronic medical records were reviewed for clinical and radiographic information. Sixteen cases of gynecologic adenocarcinomas (9 endometrial endometrioid adenocarcinomas, 4 endometrial serous carcinomas, 2 high-grade tubo-ovarian serous carcinomas, and 1 cervical adenosquamous carcinoma) involving the bladder were identified. All included cases had mucosal involvement potentially mimicking primary bladder neoplasms, including 4 cases originally diagnosed as urinary carcinomas. Tumors expressed keratin 7 (12 of 13; 92%), PAX8 (11 of 12; 92%), estrogen receptor (11 of 15; 73%), p16 (8 of 11; 73%), progesterone receptor (8 of 14; 57%), GATA3 (5 of 12; 42%), and p63 (3 of 11; 27%); all tumors were negative for keratin 20 (0 of 12). Features supportive of Müllerian origin included prior history of gynecologic malignancy, lack of morphologic heterogeneity in nonendometrioid tumors, and immunophenotypic coexpression of PAX8 and estrogen receptor with absent GATA3. Potential pitfalls seen in a subset of cases included misleading radiologic and cystoscopic findings, replacement of the overlying urothelial mucosa by tumor mimicking precursor lesions, focal GATA3 and/or p63 positivity, and areas of squamous differentiation in tumors of endometrioid histology. A combination of clinical history, certain morphologic features, and proper selection of immunohistochemical stains is key for the correct diagnosis of secondary gynecologic adenocarcinomas involving the urinary bladder.

Diagnostic and prognostic values of conjunctival and oral biopsies analyzed by direct immunofluorescence in patients with mucous membrane pemphigoid.

Mucous membrane pemphigoid (MMP) is diagnosed on the basis of a characteristic clinical picture (a predilection for mucosal involvement and scarring in the affected tissues) and a positive direct immunofluorescence (DIF) result. In this study, we compare the diagnostic and prognostic values of conjunctival and oral biopsies analyzed by DIF in patients with MMP. Sixteen patients with MMP and mucosal involvement as a predominant symptom were classified into three groups based on the clinical picture. Oral and conjunctival DIF were performed on all patients. Our study showed that patients with simultaneous oral and conjunctival involvement had a positive oral DIF in 83% and a positive ocular DIF in 100% of the examined cases, respectively. Patients with isolated ocular MMP had a positive oral DIF in 50% and a positive ocular DIF in 66% of the examined cases, respectively. Patients with only oral involvement with MMP had a positive oral DIF in 100% and a positive ocular DIF in 50% of the examined cases, respectively. Oral biopsy should be performed first and is usually sufficient for the diagnosis, even in patients with exclusively ocular MMP, whereas in patients without clinical ocular involvement, ocular DIF is positive in half of the cases and may be a predictive factor for ocular lesions in the future.

Outcomes of Squamous Cell Carcinoma of the Lip Treated With Mohs Micrographic Surgery: A Retrospective Cohort Study.

Cutaneous squamous cell carcinomas (cSCCs) of the lip have been reported to be at higher risk for poorer post-treatment outcomes. To examine outcomes of patients with SCC of the lip treated with Mohs micrographic surgery (MMS) and identify factors for recurrence. This retrospective review of a single tertiary referral center's Mohs case logs from 2010 to 2019 identified cases of lip SCC. Clinicopathologic characteristics and outcomes (local recurrence [LR], metastasis, and disease-specific death) were reviewed. One hundred ninety cases of SCC of the lip were identified and demonstrated that MMS offered a disease-free survival of 96.8% over an average follow-up period of 42 months. Younger age (61 vs 74 years p = .006), increased MMS stages ( p = .009), and higher American Joint Committee on Cancer and Brigham and Women's Hospital T stages were risk factors for LR. Immunosuppression, large tumor size, mucosal lip involvement, aggressive histology, and perineural invasion were not associated with LR. The results of this study show that SCC of the lip behaved similarly to cSCC outside the lip area, and that both primary and recurrent lesions can be treated effectively with MMS.

In Situ Patch Test and Repeated Open Application Test for Fixed Drug Eruption: A Multicenter Study

Few studies have evaluated allergy workup in fixed drug eruption (FDE) in a large population. To evaluate the sensitivity of a standardized allergy workup for diagnosing the cause of FDE, with a focus on in situ repeated open application tests (ROATs). In a retrospective multicenter study, we analyzed the practice of conducting a complete allergy workup for the etiological diagnosis of FDE. It consisted of 3 steps: in situ patch tests (PTs) for all cases except pure mucosal involvement, followed by in situ ROAT if in situ PT results were negative, and finally a drug challenge (DC). The in situ ROAT involved daily application of the suspected drug on a previously affected FDE site for 7 days. Of 98 suspected FDE cases, 61 patients (median age 61 y; male-to-female ratio 1.8) with a complete allergy workup were included. In 4 cases, even the DC yielded negative results. Among the remaining 57 patients with a positive workup, implicated drugs included paracetamol (12 cases), β-lactams (11 cases), imidazoles (9 cases, including 5 with metronidazole), nonsteroidal anti-inflammatory drugs (8 cases), iodinated contrast media (4 cases), cotrimoxazole (3 cases), and various other drugs in 10 patients. The diagnosis was confirmed by in situ PT in 17 of 54 cases (31.5%), in situ ROAT in 14 of 40 cases (35%) (with 4 cases showing remote reactivation of FDE sites), and DC in 26 cases. The sequential allergy workup involving successively in situ PT, in situ ROAT, and DC is a reliable and safe method for diagnosing the cause of FDE. In situ tests exhibited a sensitivity of over 50%.

Tata Laksana Terintegrasi Sindrom Stevens-Johnson (SJS) dan Nekrolisis Epidermal Toksik (NET)

Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are acute life-threatening mucocutaneous reactions characterized by extensive epidermal necrosis and epidermal detachment. The incidence of SJS in Japan is 2.3 cases/100,000 people/per year. The mortality rate of SJS-TEN in Saudi Arabia is 1 per 10 cases (10%). SJS-TEN can be caused by drugs, idiopathic, infection, post-bone marrow transplant, immunization, radiotherapy, and genetics. The pathogenesis of SJS-TEN is suspected as a result of a cytotoxic reaction to keratinocyte cells which causes massive apoptosis. Clinical manifestation begins with prodromal symptoms followed by erythematous macular lesions and atypical target lesions that may confluence, then develop into bullous, oozing erosions with mucosal involvement. Vital sign observation is important to predict prognosis. A diagnostic procedure is necessary if another diagnosis is being considered. Management of SJS-TEN includes cessation of suspected drugs, early treatment such as fluid resuscitation, early nutrition, treatment and evaluation of eyes and skin lesions. Multiorgan involvement can occur in SJS-TEN so multidisciplinary management is needed for optimal therapy. Corticosteroids and cyclosporin A provide beneficial effects on SJS-TEN. SCORTEN is used to evaluate prognosis.

Oral lichen planus post COVID-19 vaccination: report of two cases

Manifestations of сoronavirus disease 2019, caused by SARS-CoV-2 virus, are diverse and can vary from asymptomatic infection to respiratory failure needing intensive care. Vaccines against SARS‐CoV‐2 focusing on the spike glycoprotein have shown promise in diminishing the spread of Covid‐19. Adverse effects, however, are associated with every vaccination. Currently, there are literature reports of some cases of lichen planus eruption following COVID-19 vaccine injection. Oral lichen planus is a relatively common immune-mediated dermatologic disorder that can occur isolated or associated with cutaneous lichen planus. Postvaccination lichen planus has previously been reported in the literature as a rare adverse event following various vaccinations, especially hepatitis A and B, influenza, and varicella. The majority of patients presented with classic cutaneous lichen planus, although some reports highlighted primarily mucosal involvement. Herein we describe two rare cases of erosive-ulcerous oral lichen planus among SARS-CoV-2 vaccine recipients. The first is a case of a 67-year-old male who developed mucocutaneous lichen planus 5 days after administration of the single-dose of Ad26.COV2.S COVID-19 vaccine. In the second case, a 56-year-old female presented with reactivation of oral lichen planus after receiving a first dose of Pfizer COVID-19 vaccination 2 days earlier. The mechanism of the association between vaccines and lichen planus is not known, it is attributed to cell-mediated autoimmunity. Although there is currently no evidence of a causal relationship between SARS-CoV-2 vaccination and lichen planus, these clinical observations suggest that the COVID-19 vaccine is a possible key initiating event leading to the development or recurrence of this disease.

Oropharyngolaryngeal manifestations in severe toxic epidermal necrolysis: a single-center's retrospective case series.

Toxic epidermal necrolysis is a rare and life-threatening mucocutaneous disease. Although mucosal ear, nose, and throat (ENT) involvement is common, little is known about the characteristics, treatment modalities, and outcomes of these lesions. The aim of this study was to evaluate ENT mucosal lesions in severe toxic epidermal necrolysis patients and analyze their characteristics, treatment modalities, and outcomes, as well as proposing a management algorithm to prevent long-term debilitating sequalae of these lesions. This is a retrospective review of toxic epidermal necrolysis cases treated at the tertiary burns unit of the Lausanne University Hospital CHUV, Switzerland, between 2006 and 2019. Out of 19 patients with severe toxic epidermal necrolysis, 17 (89%) underwent a complete ENT examination at admission and 14 (82%) had ENT mucosal involvement. Five (26.3%) patients died during the stay in the intensive care unit. Of the 16 patients who received maximal care, 13 (81%) required orotracheal intubation for a median time of 16 (IQR: 14) days. Out of the 14 patients who survived, four (29%) had long-term ENT complications consisting of synechiaes necessitating subsequent endoscopic procedures. Those four patients all required mechanical ventilation with an orotracheal tube and suffered from hypopharyngeal synechiaes as well as oral and endonasal synechiaes in individual cases. This study suggests a high prevalence of ENT synechiaes in patients with severe toxic epidermal necrolysis and requiring orotracheal intubation. Periodic ENT examination could prevent mature synechiae formation in these patients. We propose an algorithm to prevent long-term sequalae in ENT mucosal involvement.