The diagnostic difficulties of juvenile vulvar pemphigoid.

Mucous membrane pemphigoid is a rare autoimmune disease affecting mucosal surfaces. Pediatric cases are exceptionally rare, one subtype being vulvar pemphigoid. Juvenile vulvar pemphigoid can be challenging to diagnose due to its rarity and subtle initial symptoms. We present a case of an 8-year-old girl successfully diagnosed early in the disease course via histopathology, and immunofluorescence. Detecting MMP can be complex due to variations in epitope binding typically not included in commercial ELISA assays, necessitating comprehensive workup. Missed diagnosis may lead to progression to systemic involvement with severe consequences; thus, timely diagnosis and treatment are crucial.

[Morphological predictors of water-electrolyte disorders in patients with preventive ileostomy after rectal resection for cancer]. / Issledovanie morfologicheskikh prediktorov vodno-elektrolitnykh narushenii u patsientov so sformirovannoi preventivnoi ileostomoi pri rezektsii pryamoi kishki po povodu raka.

OBJECTIVE: To analyze morphological changes in wall of functioning and non-functioning small intestine in patients with preventive ileostomy and to determine histological predictors of water-electrolyte disorders. MATERIAL AND METHODS: We prospectively analyzed 57 patients >18 years old who underwent rectal resection with preventive ileostomy between January 2022 and November 2023. Anthropometric data included gender, age, body mass index, ECOG and ASA classes. Complications associated with large losses through ileostomy were water-electrolyte disorders, dehydration and acute renal failure with repeated hospitalization. Morphological analysis implied intraoperative full-layer biopsy of small intestine on anterior abdominal wall (ileostomy). Intraoperative biopsy of efferent and afferent loops was also carried out. Tissue samples were examined by light microscopy. We analyzed mean height of mucous membrane villi and depth of crypts, as well as their ratio. Fibrosis and swelling of submucosa were evaluated too. The results were analyzed in the SPSS Statistics 20 software. RESULTS: Mean height of intestinal villi <465 microns (p=0.028), ratio of their height to crypt depth <4.38 (p=0.034) and submucosal fibrosis (p=0.031) significantly affected malabsorption and readmission of patients. The risk of readmission was 11.5 and 5.5 times higher in univariate analysis. Multivariate analysis revealed in-hospital dehydration with resumption of infusion therapy as a predictor of readmission (p=0.046). CONCLUSION: Ileostomy is a certain stress for the patient's body. Not every patient is able for adaptation. One of the adaptation mechanisms is hypertrophy of mucous membrane villi involved in digestion. This mechanism is less pronounced in patients with repeated hospitalizations. Preoperative morphological examination of ileum mucosa may be an additional objective predictor of possible complications of preventive ileostomy.

Ocular Mucous Membrane Pemphigoid: The Effect of Risk Factors at Presentation on Treatment Outcomes.

PURPOSE: Analyze the influence of risk factors at presentation in the long-term immunosuppressive therapy (IMT) outcomes of ocular mucous membrane pemphigoid (OMMP). DESIGN: Retrospective multicenter study. PARTICIPANTS: Patients with OMMP seen at the Duke Eye Center, Tecnologico de Monterrey, and Hospital Clinic of Barcelona from 1990 to 2022. METHODS: Data at presentation on demographics, direct immunofluorescence, ocular findings, sites of extraocular manifestations (EOMs), and previous treatments in patients with a clinical or laboratory diagnosis of OMMP, were analyzed with multivariable analysis and Kaplan-Meier plots to identify factors associated with adverse outcomes. MAIN OUTCOME MEASURES: (1) Inflammatory control (no conjunctival inflammation in both eyes at 3 months on IMT); (2) relapse (new-onset inflammation after absolute control in either eye); (3) progression (≥ 1 cicatrizing stage progression in either eye); and (4) vision loss (≥ 2 Snellen lines). RESULTS: A total of 117 patients (234 eyes), 61% (71/117) of whom were women, with a mean age of 66.6 (SD: 12.4) years (range: 37-97 years) and median follow-up of 34 months (interquartile range: 16-66 months; range: 3-265 months), were enrolled. Inflammatory control was achieved in 57% of patients (67/117), with high-risk EOM (HR-EOM), including esophageal, nasopharyngeal, and/or genital involvement (adjusted odds ratio [aOR]: 12.51; 95% confidence interval [CI]: 2.61-59.99; P = 0.002) and corneal scarring (aOR: 3.06; 95% CI, 1.15-8.14; P = 0.025), as significant risk factors for persistent inflammation. Disease relapse, progression, and vision loss occurred in 20% of patients (23/117), 12% of patients (14/117), and 27% of patients (32/117), respectively. Baseline corneal scarring was a risk factor for relapse (adjusted hazard ratio: 4.14; 95% CI: 1.61-10.62; P = 0.003), progression (aOR: 11.46; 95% CI: 1.78-73.75; P = 0.010), and vision loss (aOR: 3.51; 95% CI: 1.35-9.10; P = 0.010). HR-EOM was associated with stage progression (aOR, 34.57; 95% CI, 6.57-181.89; P<0.001) and vision loss (aOR, 8.42; 95% CI, 2.50-28.42; P = 0.001). No significant differences were found between IMT regimes and relapse (P = 0.169). CONCLUSIONS: Ocular mucous membrane pemphigoid presenting with HR-EOMs and corneal scarring has an increased risk of stage progression and vision loss. Corneal scarring and severe inflammation at baseline were associated with an increased risk of relapse. A disease progression staging system incorporating both the HR-EOMs and corneal involvement is required to predict the visual outcome of OMMP better. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Arabic Web-Based Information on Oral Lichen Planus: Content Analysis.

BACKGROUND: The use of web-based health information (WBHI) is on the rise, serving as a valuable tool for educating the public about health concerns and enhancing treatment adherence. Consequently, evaluating the availability and quality of context-specific WBHI is crucial to tackle disparities in health literacy and advance population health outcomes. OBJECTIVE: This study aims to explore and assess the quality of the WBHI available and accessible to the public on oral lichen planus (OLP) in Arabic. METHODS: The Arabic translation of the term OLP and its derivatives were searched in three general search platforms, and each platform's first few hundred results were reviewed for inclusion. We excluded content related to cutaneous LP, content not readily accessible to the public (eg, requiring subscription fees or directed to health care providers), and content not created by health care providers or organizations (ie, community forums, blogs, and social media). We assessed the quality of the Arabic WBHI with three standardized and validated tools: DISCERN, Journal of the American Medical Association (JAMA) benchmarks, and Health On the Net (HON). RESULTS: Of the 911 resources of WBHI reviewed for eligibility, 49 were included in this study. Most WBHI resources were provided by commercial affiliations (n=28, 57.1%), with the remainder from academic or not-for-profit affiliations. WBHI were often presented with visual aids (ie, images; n=33, 67.4%). DISCERN scores were highest for WBHI resources that explicitly stated their aim, while the lowest scores were for providing the effect of OLP (or OLP treatment) on the quality of life. One-quarter of the resources (n=11, 22.4%) met all 4 JAMA benchmarks, indicating the high quality of the WBHI, while the remainder of the WBHI failed to meet one or more of the JAMA benchmarks. HON scores showed that one-third of WBHI sources had scores above 75%, indicating higher reliability and credibility of the WBHI source, while one-fifth of the sources scored below 50%. Only 1 in 7 WBHI resources scored simultaneously high on all three quality instruments. Generally, WBHI from academic affiliations had higher quality scores than content provided by commercial affiliations. CONCLUSIONS: There are considerable variations in the quality of WBHI on OLP in Arabic. Most WBHI resources were deemed to be of moderate quality at best. Providers of WBHI could benefit from increasing collaboration between commercial and academic institutions in creating WBHI and integrating guidance from international quality assessment tools to improve the quality and, hopefully, the utility of these valuable WBHI resources.

Real-Time Experience of Abrocitinib for the Treatment of Mucous Membrane Pemphigoid: A Case Report.

Background: Mucous membrane pemphigoid (MMP), a rare autoimmune vesiculous and erosive disorder, may affect multiple mucous membranes, with the oral cavity being the most commonly affected site. Its treatment depends on the site(s) of mucosal involvement and disease severity. Patients and Methods: A 62-year-old female patient with MMP that predominantly involved the oral cavity strongly rejected systemic corticosteroid or immunosuppressive agents and was successfully treated with abrocitinib, a highly selective JAK-1 inhibitor with a good safety profile. Results: The case demonstrated good efficacy and safety profile of abrocitinib for the treatment of MMP with predominant oral involvement. Conclusion: Abrocitinib is a promising agent for the treatment of MMP with oral involvement.

Linear IgA bullous dermatosis of childhood: Retrospective single-center cohort.

Linear IgA bullous dermatosis (LABD) is a rare autoimmune blistering disorder impacting children and adults. In this single-center retrospective chart review of pediatric patients with LABD at a large tertiary referral center, we report the unifying and unique clinical features of 10 pediatric patients. Patients typically presented with the "cluster of jewels" sign (n = 6; 60%), mucous membrane involvement (n = 5; 50%) and had a mean disease duration of 38 months; six patients (60%) required inpatient admission for management of their skin disease, including all five patients who had mucous membrane involvement. Our findings suggest that pediatric LABD may be a disease with high morbidity and may be associated with severe complications when mucous membranes are involved.

Gingival Manifestations in Oral Chronic Autoimmune Bullous Diseases: A Retrospective Study.

Background and Objectives: Desquamative gingivitis (DG) is a clinical term indicating "peeling gums" and is associated with different oral manifestations. In this study, we aimed to assess the association between DG and autoimmune blistering mucocutaneous diseases (ABMD) with oral manifestations. Materials and Methods: A retrospective study including 88 patients diagnosed between 1998 and 2019 with ABMD (intraepithelial and subepithelial autoimmune blistering diseases) was performed at the Oral Medicine Department, Faculty of Dentistry, "Carol Davila" University of Medicine and Pharmacy in Bucharest. For each patient, the sociodemographic and anamnestic data, as well as clinical features of oral lesions (location), histological evaluation, and direct immunofluorescence data were collected. Results: Most of the patients involved in the study were female (78.4%). In total, 34 patients (38.63%) were diagnosed with subepithelial autoimmune diseases (SAD) and 54 (61.36%) had intraepithelial autoimmune diseases (IAD). Differences in the anatomic distribution of oral involvement were found between SAD and IAD. The presence of DG was significantly more common in patients with SAD compared to those with a diagnosis of IAD. Conclusions: Specific anatomical locations of the oral lesions are significantly associated with different subtypes of ABMD, with gingiva and hard palate mucosa being more involved in SAD and the soft palate and buccal mucosa in IAD. Desquamative gingivitis is a clinical sign that raises diagnostic challenges for several conditions in oral medicine.

Survey: Preferred practice patterns in the diagnosis of mucous membrane pemphigoid amongst cornea specialists.

PURPOSE: To evaluate preferred diagnostic tools and treatment decision-making factors in cases suspicious of mucous membrane pemphigoid (MMP) amongst ophthalmologists and cornea specialists. METHODS: Web-based survey, consisting of 14 multiple choice questions, posted to the Cornea Society Listserv Keranet, the Canadian Ophthalmological Society Cornea Listserv, and the Bowman Club Listserv. RESULTS: One hundred and thirty-eight ophthalmologists participated in the survey. Eighty-six percent (86%) of respondents were cornea trained and practiced in either North America or Europe (83%). Most respondents (72%) routinely perform conjunctival biopsies for all suspicious cases of MMP. For those who do not, fear that biopsy will exacerbate inflammation was the most common reason to defer investigation (47%). Seventy-one percent (71%) performed biopsies from perilesional sites. Ninety-seven percent (97%) ask for direct (DIF) studies and 60% for histopathology in formalin. Most do not recommend biopsy at other non-ocular sites (75%), nor do they perform indirect immunofluorescence for serum autoantibodies (68%). Immune-modulatory therapy is started following positive biopsy results for most (66%), albeit most (62%) would not let a negative DIF influence the choice of starting treatment should there be clinical suspicion of MMP. Differences in practice patterns as they relate to level of experience and geographical location are contrasted to the most up-to-date available guidelines. CONCLUSION: Responses to the survey suggest that there is heterogeneity in certain practice patterns for MMP. Biopsy remains an area of controversy in dictating treatment plans. Identified areas of need should be targeted in future research.

[Shaping approaches to standardization of oropharynx mucous membrane assessment of immunological parameters]. / Formirovanie podkhodov k standartizatsii issledovaniya immunologicheskikh parametrov slizistoi obolochki rotoglotki.

Basis of acute pharyngitis pathogenesis is an inflammatory process at the entrance gate of the infection. Therefore, local immunity study proves to be the most informative. Difficulty in making that type of assessment is lack of generally accepted reference values and biological sampling techniques. OBJECTIVE: Validation of biological sampling techniques to study the parameters of local mucosal immunity in oropharynx acute inflammatory diseases. MATERIAL AND METHODS: 30 people with acute catarrhal pharyngitis with intoxication syndrome were examined. The sampling was carried out in 7 different ways. 1. Collect saliva samples using the passive drool collection method. 2. Collect saliva, using cotton swabs placed into the mouth for 3 minutes. 3. Cotton swabs wrapped around a metal probe was placed on palatine tonsils and lateral walls of the oropharynx. 4. Instead of a cotton swab, a disc of laboratory filter paper with a diameter of 0.7 cm was used. 5. Scrape by the mucous membrane of the palatine tonsils and lateral walls of the oropharynx were made with a cytobrush. 6. Using a cytobrush, scrapings were made from the mucous membrane of only the posterior pharyngeal wall, excluding the region of the palatine tonsils. 7. Using a cytobrush to make scrapings from the only palatine tonsils mucous membrane. RT-PCR was used to determine IL-1ß mRNA. RESULTS: Minimal IL-1ß mRNA values were detected in saliva collected by passive flow (0.095 [0; 3.45] units) and on a cotton swab (0.21 [0.1; 3.82] units). IL-1ß mRNA in the material collected by methods No. 3 and No. 4 on a cotton swab and a paper disk did not differ significantly from each other. Its level was higher than in saliva and lower than in scrapings. The maximum result was revealed with method No. 5 when simultaneously taking scrapings from the palatine tonsils and posterior pharyngeal wall mucous membrane (4.76 [0.92; 8.13] units). The expression of IL-1ß mRNA in the material obtained by methods No. 6 and No. 7 did not differ significantly from each other. CONCLUSION: Separated scrapings collecting from the palatine tonsils or posterior pharyngeal wall mucous membrane will allow assessing the inflammatory response autonomously in the lymphoid tissue and separately on the mucosa of the posterior pharyngeal wall. The mucosal scraping technique was the most effective for assessing cytokines in the oropharyngeal mucosal membrane.

[Pathogenetic approach in the treatment of inflammatory diseases of the nose and paranasal sinuses]. / Patogeneticheskii podkhod v terapii vospalitel'nykh zabolevanii nosa i okolonosovykh pazukh.

In recent years, inflammatory diseases of the nose and paranasal sinuses have been on the rise. In addition to infectious diseases, in the modern world a large percentage of the population suffers from allergic diseases. The approach to therapy and the choice of a drug should take into account the pathogenesis of the inflammatory reaction in the nasal cavity and paranasal sinuses. By exerting its effect, the drug should reduce hyperemia and swelling of the nasal mucosa, reduce the level of mucus secretion, improve the drainage of the paranasal sinuses, i.e. possess vasoconstrictive and anti-allergic properties. As such a drug, you can use the combined intranasal spray Frinozol, which basically contains cetirizine and phenylephrine. The use of Frinozol in the complex treatment of inflammation of the mucous membrane of the nasal cavity and paranasal sinuses contributes to the rapid and pronounced weakening of the symptoms of the disease, and is also the key to successful therapy.

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

Introduction: 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. Methods: 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. Results: 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. Discussion: 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.

Paraneoplastic autoimmune Laminin-332 syndrome (PALS): Anti-Laminin-332 mucous membrane pemphigoid as a prototype.

IMPORTANCE: Laminin-332 is an important component of the basement membrane. Recently, autoantibodies to Laminin-332 have been described in several autoimmune diseases. Many of these autoimmune diseases have a high incidence of malignancy. The importance of Laminin-332 autoantibodies and its relationship to malignancy is highlighted by using Laminin-332 Pemphigoid (LM-332Pg) as a prototype. OBJECTIVE: To identify several autoimmune diseases that have autoantibodies to Laminin-332 present, and to determine the prevalence of malignancy in them. Using Laminin-332 Pemphigoid (LM-332Pg) as a prototype, to compare clinical profiles of LM-332Pg patients with and without cancer. By identifying the temporal detection of cancer, can the influence of autoantibodies to Laminin-332 on prognosis be determined. EVIDENCE REVIEW: A literature search was conducted to identify autoimmune and inflammatory diseases in which autoantibodies to Laminin-332 were present. Subsequently, the rate of malignancy in these autoimmune diseases was determined. A search for publications on LM-332Pg patients to determine cancer rates and clinical outcomes to examine if a relationship can be proposed, was performed. FINDINGS: Autoantibodies to Laminin-332 were detected in recent studies of systemic lupus erythematosus (SLE), psoriasis, bronchiolitis obliterans (BO), graft-vs-host disease (GVH), bullous pemphigoid (BP), lichen planus (LP), epidermolysis bullosa acquisita (EBA), and membranous glomerulonephropathy (MGN). A high incidence of cancer rate was reported in these autoimmune diseases including primary Sjögren's syndrome (pSS), systemic sclerosis (SS), dermatomyositis (DM), multiple sclerosis (MS), immune thrombocytopenia purpura (ITP), and rheumatoid arthritis (RA). Data analysis demonstrated that LM-332Pg patients had a higher risk of developing ovarian, uterine, lung, gastric cancers and leukemia. The incidence for breast cancer was lower, when compared with global cancer rates. Patients diagnosed with cancer after the presence of LM-332Pg had higher rates of mortality and lower rates of remission, compared to those diagnosed with cancer prior to the discovery/diagnosis of LM-332Pg. When studied, levels of Laminin-332 autoantibodies correlated with the presence or absence of malignancy. CONCLUSIONS AND RELEVANCE: Preliminary analysis suggests that autoantibodies to Laminin-332 are present in multiple autoimmune diseases, which also have a high incidence of malignancy. Detailed analysis of available data highlights that patients who developed LM-332Pg after cancer was diagnosed, had a more favorable prognosis, compared to patients who developed cancer when LM-332Pg was previously present. Preliminary data would suggest that autoantibodies to Laminin-332 could serve as an important biomarker in certain patients, for correlation with possible incidence of malignancy.

Clinical use of cryopreserved ultra-thick human amniotic membrane for anophthalmic socket contracture.

PURPOSE: To study the use of ultra-thick human amniotic membrane for management anophthalmic socket contracture. METHODS: A prospective study done at King Khaled Eye Specialist Hospital, Riyadh, Saudi Arabia. Thirty-six patients (45 eyelids) were involved. Contracted socket caused by trauma, previous surgery or radiotherapy, delay in use of prosthesis, Congenital Anophthalmia/Microphthalmia, and Anophthalmia secondary to Enucleation/Evisceration were included in the study. RESULTS: Thirty-three patients (42 eyelids) underwent fornix reconstruction with cryopreserved ultra-thick human amniotic membrane. Mean ± SD age at surgery was (40.90 ± 17.32) years. Mean follow up was 10.5 months. Grade II fornix contracture was the most common type in 23 (54.8%) eyelids. The most common involved primary diagnosis was Anophthalmia secondary to Enucleation/Evisceration (n = 13). The incidence of pyogenic granuloma (PG) after surgery was seen in 8 eyelids (19.0%). CONCLUSION: Anophthalmic contracted socket secondary to significant history of multiple PG excision (> 5 times) and secondary to enucleation/evisceration were associated with good surgical outcome. Cryopreserved ultra-thick human amniotic membrane is an ideal material for the management of anophthalmic socket contracture.

[Prospects for the use of autofluorescence endoscopy in rhinosurgery]. / Perspektivy primeneniya autofluorestsentnoi endoskopii v rinokhirurgii.

The condition of blood supply and microcirculation in the mucous membrane is considered to be the main factor determining the consistency of local flaps of the nasal mucous membrane. OBJECTIVE: To assess the possibility of using autofluorescence endoscopy to control the condition of flaps of the nasal mucous membrane after surgical interventions. MATERIAL AND METHODS: The assessment of the processes of biological destruction of 15 fragments of the mucous membrane of the inferior nasal concha, obtained during intranasal interventions with endoscopic technique in autofluorescence mode, followed by histological examination at different periods of observation, has been conducted. The results of the proposed method were tested in 84 patients with chronic and intraoperative defects of the nasal septum after the surgical intervention. RESULTS: It has been shown that autofluorescence endoscopy can be used to assess the viability of flaps of the nasal cavity's mucous membrane. A change of the autofluorescence signal to a whitish and orange shades indicates the presence of ischemia and necrosis. Method sensitivity was 92.3%, specificity was 95.8% and total accuracy was 95.4%. CONCLUSION: This method is promising for predicting the long-term outcomes of surgical treatment of the nasal septum's perforation in relation to the risk of reperforation forming, as well as for assessment of the functional viability of local flaps in plastic reconstruction of defects of the anterior skull base.

[Pemphigoid diseases in older adults]. / Pemphigoiderkrankungen bei älteren Menschen.

Pemphigoid diseases are a group of bullous autoimmune diseases characterized by autoantibodies against structural proteins of the dermal-epidermal junction. With a steadily growing aging population, pemphigoid diseases are emerging as a significant medical challenge, because they occur primarily in older individuals. The by far most common disease is bullous pemphigoid, which is clinically characterized by tense blisters, erosions, erythema or urticarial plaques, while severe pruritus is the leading subjective symptom. Mucous membrane pemphigoid predominantly affects surface-close mucous membranes with painful erosions and blisters as well as frequently scarring usually in the mouth, nose, and eyes. Anti-p200 pemphigoid clinically resembles bullous pemphigoid but is much less common. Diagnosis of these diseases involves the combination of clinical evaluation, lesional histopathology, direct immunofluorescence microscopy of a perilesional biopsy and serology. Topical and systemic corticosteroids are the mainstay of pemphigoid diseases treatment. Depending on the severity of the disease, various potentially corticosteroid-sparing therapies, such as dapsone, doxycycline, methotrexate, azathioprine and mycophenolate may be used. In severe courses, treatment with rituximab, cyclophosphamide, intravenous immunoglobulins or immunoadsorption are second- or third-line treatment options. Patients are best managed in centers experience with the management of pemphigoid diseases. Updated national and international guidelines for the diagnosis and treatment of bullous pemphigoid and mucous membrane pemphigoid have recently been published.

COVID-19 associated severe mucocutaneous blistering eruptions: A case series.

Mucocutaneous eruptions are associated with numerous infectious processes and can present as erythema multiforme (EM), reactive infectious mucocutaneous eruption (RIME), Stevens Johnson syndrome (SJS), or toxic epidermal necrolysis (TEN). Limited reports have detailed the association of these eruptions with SARS-CoV-2 infection. We present a series of eight cases of severe mucocutaneous blistering eruptions associated with SARS-CoV-2 infection. A retrospective case series was performed at six tertiary medical centers from March 1, 2020 to August 1, 2022. Inclusion criteria were met with a clinical diagnosis of EM, RIME, SJS, or TEN and a positive SARS-CoV-2 test (rapid antigen or polymerase chain reaction) less than 4 weeks prior to onset of dermatologic manifestation. Data was collected at time of each patient encounter. Eight patients met criteria with six pediatric patients (<18 years of age) having a median age of 15 years and two adult patients (>18 years of age) having a median age of 36 years. Patients were found to have a clinical diagnosis of RIME in 85.7% of cases. Oral mucosal involvement was the most common clinical finding (100%), followed by ocular (50.0%), urogenital (50.0%), and skin (37.5%) involvement. Evaluation did not reveal any additional infectious triggers in four patients. Evidence of possible concurrent or previous infectious triggers were identified in four patients. This case series highlights the development of severe mucocutaneous eruptions in association with COVID-19 infection, as well as the potential contributing role of concurrent or prior infections.