Mucosal Involvement Research Articles

Accuracy of serological tests in diagnosing mucosal leishmaniasis.

In this serum panel-based study, we evaluated the accuracy of serological tests originally developed for visceral leishmaniasis (VL), for diagnosis of mucosal leishmaniasis (ML). A total of five tests were evaluated, four of which are registered at the National Agency of Sanitary Surveillance (Agência Nacional de Vigilância Sanitária-ANVISA) (RIDASCREEN® Leishmania Ab from R-Biopharm AG., Leishmania ELISA IgG + IgM from Vircell S.L., IFI Leishmaniose Humana-BioManguinhos, and IT-LEISH® from Bio-Rad Laboratories, Inc.), and the other a direct agglutination test (DAT-LPC) prototype kit developed at Fiocruz. The panel was composed of 40 serum samples from patients with confirmed ML and 20 from patients with mucosal involvement and negative parasitological/molecular tests for leishmaniasis and confirmation of another etiology. All cases were treated from 2009 to 2016 in a referral center for leishmaniasis in Belo Horizonte, Minas Gerais, Brazil (Instituto René Rachou, Fiocruz). Diagnostic accuracy, based on the cut-off point for VL diagnosis, was 86.2% with RIDASCREEN® Leishmania Ab, 73.3% with Leishmania ELISA IgG + IgM, and 66.7% with IFI Leishmaniose Humana, while IT-LEISH® and DAT-LPC had the lowest accuracy (38.3%), despite high specificity (100% and 95%, respectively). New cut-off points defined with sera from ML patients improved accuracy from 86.2 to 89% (p = 0.64) and 73.3 to 88% (p = 0.04) for RIDASCREEN® Leishmania Ab and Leishmania ELISA IgG + IgM, respectively. Moreover, these tests presented greater sensitivity and immunoreactivity in patients with moderate/severe clinical ML forms. The data of this study suggest that ELISA assays can contribute to laboratory diagnosis, especially for patients with moderate or severe mucosal involvement.

Epidemiological, clinical and therapeutic aspects of dermatitis herpetiformis at Yalgado Ouédraogo University Hospital Centre, Burkina Faso

Background: Dermatitis herpetiformis is a rare autoimmune bullous dermatosis that predominantly affects Caucasians, adults or children, with a sex ratio of 1.8. Materials and Methods: The aim of this study was to document the clinical and epidemiological profiles of dermatitis herpetiformis and to detail its treatment in a hospital setting in order to increase our knowledge about this disease in our context and so to improve its management. We conducted a retrospective, cross-sectional study of the records of all patients seen in consultation or hospitalized at the dermatology department of Yalgado Ouédraogo University Hospital Centre, Ouagadougou, a public hospital in Burkina Faso, from January 2016 to December 2020. Results: We collected 14 cases (0.12%) of dermatitis herpetiformis among 11,456 patients seen. The mean age was 8 years (ranging from 4 to 27 years). The sex ratio was 1.33. The majority of the patients were schoolchildren living in rural areas (8 cases). The duration of the disease ranged from five days to one year (mean duration: 59.35 days). Eight patients had a history of digestive problems such as abdominal pain and diarrhea. Pruritis was the principal functional sign. In all patients, the lesions were polymorphous: disseminated vesicular bullae, papules, erosive, and excoriated lesions, sometimes forming clusters. Mucosal involvement was rare (3 cases). A gluten-free diet and dapsone 2 mg/kg/day were proposed to all patients and resulted in the improvement of the lesions. Conclusion: Our study confirmed that dermatitis herpetiformis is rare in our context. It is more frequent in young children and predominantly affects boys. It is intensely pruritic, and generalized polymorphous lesions were present in all our patients. Treatment is essentially based on a gluten-free diet and dapsone, which is a therapeutic test in the absence of supplementary investigations to establish a definitive diagnosis. Key words: Dermatitis Herpetiformis; Clinical Medicine; Dapsone; Gluten Intolerance

Vesicular contact dermatitis following sulfacetamide eye drop instillation raising the specter of vesiculobullous mucocutaneous syndrome – Case presentation

Allergic reactions to ocular drops are a well-known side effect and usually present as eczematous dermatitis localized to periocular skin. However, rarely, the reactions can be severe and present with systemic involvement leading to vision or even life-threatening consequences. We report a case of vesicular allergic contact dermatitis following instillation of an over-the-counter sulfacetamide eye drop in right eye with involvement of oral mucosa, raising a threat of mucocutaneous vesiculobullous disorders. Prompt discontinuation of ocular drops followed by treatment with systemic and topical steroids, topical lubrication, and fornix glass rod sweeping prevented vision- and life-threatening sequalae.

STEVENS–JOHNSON SYNDROME AND TOXIC EPIDERMAL NECROLYSIS: PANORAMIC REVIEW

Introduction: Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are infrequent diseases represented by disseminated epidermal necrosis and skin sloughing. They have a representative mortality and morbidity, their early diagnosis and treatment is necessary to provide good results in affected individuals. Objective: to detail the current information related to Stevens-Johnson syndrome and toxic epidermal necrolysis, description, clinical presentation, pathophysiology, diagnosis, differential and treatment. Methodology: a total of 52 articles were analyzed in this review, including review and original articles, as well as clinical cases, of which 44 bibliographies were used because the other articles were not relevant for this study. The sources of information were PubMed, Google Scholar,CrossRef, and Cochrane; the terms used to search for information in Spanish, Portuguese, and English were: Stevens-Johnson, toxic epidermal necrolysis, and immunologic burn. Results: Almost all affected individuals present mucosal involvement, with two or more mucosal surfaces involved in up to approximately 80% of the cases. Oral involvement is more frequent, accompanied by mucositis and ulceration in almost all cases. Ocular involvement is also frequent in 60-100% of patients with SJS/TEN, and can present as conjunctival hyperemia and sometimes even complete epidermal detachment of the ocular surface. Gynecologic involvement also varies according to severity, however it is seen in approximately 77% of affected individuals. The precise incidence of genitourinary involvement in individuals affected in the acute phase of SJS/NET is not known. Drugs are the factors most frequently associated with SJS and NET, however, infection, especially Mycoplasma pneumonia, has been implicated. In approximately 15-30% of cases, no causative agent can be identified.

CPC02 Delayed widespread lichenoid drug reaction with radioisotopic response in a patient treated with nivolumab

Abstract The introduction of immune checkpoint inhibitors for the treatment of a wide variety of malignancies has led to a rise in cutaneous immune-related adverse events (irAEs), including autoimmune and autoinflammatory skin conditions, which typically present within the first 16 weeks of treatment—on average, 3.6 weeks after drug initiation. Cutaneous irAEs occur in approximately 8.7% of patients receiving antiprogrammed cell death type 1 therapy (PD-1). Nivolumab is an antibody against PD-1 widely used for various cancers. As the application of these therapies expands, understanding of their adverse cutaneous effects is essential. We present a 76-year-old woman with metastatic renal cell carcinoma, who developed a widespread pruritic eruption 18 months after commencing nivolumab. Examination revealed a papular and vesicular rash affecting the arms, legs, anterior chest, face, hands and feet. There was a confluent well-demarcated rectangular area of grouped papules on the right side of her back; this site had been treated with radiotherapy for localized rib metastases 1 month prior to onset of rash. There was no mucosal involvement and no systemic symptoms. Three skin biopsies were taken, which showed vacuolar alteration of the basal layer and a band-like inflammatory cell infiltrate at the dermoepidermal junction, with focal areas of subepidermal separation. Direct immunofluorescence was negative, and skin swabs were negative for bacterial and viral infection. A diagnosis of delayed bullous lichenoid drug reaction secondary to nivolumab was made. The area involving the previous radiotherapy field was considered to be a radioisotopic response rather than a radiation recall response as she was already on nivolumab when it occurred. She discontinued nivolumab and commenced oral prednisolone and her skin settled over 5 weeks. Lichenoid dermatitis is a known adverse reaction associated with PD-1 therapy, with improved survival outcomes noted in patients with lichenoid irAEs on PD-1 therapy. Typically, it occurs within 16 weeks, with a documented range of 1–266 days. This patient developed a delayed lichenoid eruption at approximately 550 days, with concurrent radioisotopic response at a previous radiotherapy site. Radiotherapy-induced lichen planus is rare, with only one case report documenting involvement outside the irradiated field. We found no previous reports of nivolumab causing lichenoid drug eruption after such a protracted period. This case adds to the growing literature around PD-1 inhibitor-associated cutaneous eruptions.

DP17 A very rare presentation of linear IgA disease during pregnancy

Abstract We present a very rare immunobullous dermatosis, linear IgA disease (LAD), that developed during pregnancy, to highlight its clinical features, management and important safety considerations for the neonate. During her first pregnancy, a 32-year-old Japanese woman of 27 weeks’ gestation developed an intensely itchy blistering rash on her limbs, trunk and face without periumbilical predilection. The blisters were tense, clustering in an annular configuration, with subsequent development of blistering and erosions involving the oral and genital mucosae. Medications included a pregnancy multivitamin only. A lesional skin biopsy revealed a subepidermal blister containing neutrophils and eosinophils. Direct immunofluorescence of perilesional skin demonstrated linear deposition of IgA along the basement membrane zone. Linear IgA disease was diagnosed. Initial treatment consisted of erythromycin and daily clobetasol ointment. Owing to inadequate symptom control and blister progression, dapsone 50 mg daily was initiated with prompt improvement in her condition and cessation of new blistering. A postpartum flare necessitated a dose increase to 75 mg daily. Her baby’s glucose-6-phosphate dehydrogenase (G6PD) level was found to be normal at birth. She proceeded to breastfeed her son, and monitoring of his haemoglobin level was normal. Although her clinical presentation initially raised the possibility of pemphigoid gestationis (PG), there was no periumbilical involvement, and the morphology of blistering in conjunction with mucosal involvement was more suggestive of LAD. The immunofluorescence results also helped to distinguish LAD from PG. The new onset of LAD during pregnancy is very unusual and has been rarely reported (Ikkaku N, Tateishi N, Oda Y et al. Linear immunoglobulin A bullous dermatosis developing during late pregnancy. J Dermatol 2017; 44:e44–5). Pre-existing LAD typically improves during pregnancy, although postpartum flares are recognized. Pemphigoid gestationis has been associated with an increased risk of prematurity and intrauterine growth restriction, but this has not been reported with LAD during pregnancy, possibly due to its rarity. Both the diagnosis of LAD and its mainstay treatment—dapsone—have important potential implications for the neonate. As for other immunobullous diseases, transplacental transfer of pathogenic antibodies may result in transient, usually mild, neonatal blistering. Although dapsone can be safely used during pregnancy and breastfeeding, the risk of neonatal haemolysis and methemoglobinaemia necessitates the assessment of the G6PD level and haemoglobin monitoring in the neonate. Multidisciplinary input from the dermatology, obstetric and paediatric teams is crucial to ensure appropriate counselling and care of the affected mother and baby.

P87 Therapeutic outcomes in xanthoma disseminatum with cladribine: a long-term experience of six patients from a tertiary care institute

Abstract Xanthoma disseminatum is a rare, non-Langerhans-cell histiocytosis with systemic involvement that has limited treatment options and response. Cladribine has been previously reported in few case reports and a single case series to induce long-term remission of cutaneous lesions. The aim was to study the treatment outcomes in a long-term follow-up of patients with xanthoma disseminatum, treated with cladribine. This was an ambispective case series of six patients diagnosed with xanthoma disseminatum on the basis of clinical and histopathological features. All patients were treated with monthly cycles of cladribine (2-chlorodeoxyadenosine) 0.14 mg kg−1 daily for 5 days a month, ranging from two to eight cycles. The follow-up period ranged from 2 months to 4.75 years. There were four males and two females in our series, with mean (SD) age of 25.3 (13.1) years (range 9–48). Disease duration at presentation ranged from 1.5 to 8 years. All patients presented with skin-coloured to yellowish-brown papules in a periorbital, perioral and flexural distribution. Four of six (67%) patients had mucosal involvement. All but one patient had associated diabetes insipidus. Half the patients had bone involvement, and two showed extensive infiltrates in the brain causing neurological symptoms. Patients receiving at least three cycles (n = 5) reported a mean improvement of 72% in cutaneous lesions at the end of the follow-up period, allowing for the avoidance of tracheostomy in a patient with laryngeal involvement. Imaging (magnetic resonance imaging of the brain, positron emission computed tomography) repeated in four patients after 3–6 cycles showed evidence of significant improvement in extracutaneous disease. Adverse effects were noted in four (67%) patients, including transient leukopenia in three, post-infusion fever in one and proximal renal tubular acidosis in one. All side-effects were resolved after the cessation of therapy and/or with supportive treatment; no serious infections were reported. Cladribine is a purine analogue that has an antiproliferative effect on histiocytes and lymphocytes, as well as an immunomodulatory action. It has emerged as an effective therapeutic modality for patients with xanthoma disseminatum that necessitates further evaluation for dosage and safety.

CPC03 Triad of generalized rash, peripheral eosinophilia and coexisting obstructive airway disease

Abstract An 87-year-old woman was referred to the acute dermatology service with a 1-week history of a generalized painful rash. She was under the care of the medical team for treatment of pneumonia with symptoms of a cough, fevers and shortness of breath. Her past medical history included adult-onset asthma, hyponatraemia and hypertension. She had never smoked. On examination, she had erythematous smooth papules and plaques on her limbs and torso with palmoplantar involvement, alongside purple macules and patches where older lesions were present. There was no lymphadenopathy and no mucosal involvement. She had an oxygen requirement of 2 L per minute via nasal cannula. Initial blood tests showed eosinophils of 2.8 × 109 cells L−1 and a C-reactive protein (CRP) of 77 mg L−1. A chest radiograph showed bilateral ill-defined shadowing. Two skin punch biopsies were performed for histology and immunofluorescence. Vasculitis screen identified positive antinuclear antibodies (anti-Ro) and reduced complement C4 with negative antineutrophil cytoplasmic antibody (ANCA). The patient remained unwell with increasing CRP and peripheral eosinophilia (up to 9.2 × 109 cells L−1) with rash progressing to exhibit a reticular pattern. Alongside bilateral patchy ground glass opacification on chest computed tomography, a clinical diagnosis of eosinophilic granulomatosis with polyangiitis (EGPA) was made. Differential diagnoses included viral exanthem, erythema multiforme and urticarial vasculitis. Screening for cardiac, renal and sinus involvement was undertaken. The patient improved clinically following the initiation of systemic steroids and the peripheral eosinophilia returned to baseline. Skin histology reinforced the clinical diagnosis of EGPA, demonstrating a dermal and perivascular eosinophilic infiltrate with focal extension into vessel walls. EGPA is a rare small- to medium-sized vessel vasculitis with an annual incidence of 0.9–2.4 per million and a median age at diagnosis of 49–59 years. The clinicopathological hallmarks, as in this case, are asthma with peripheral eosinophilia and pulmonary eosinophilic infiltrates. Median time from onset of asthma to presentation with EGPA is 5–9 years. EGPA remains a clinical diagnosis, reinforced by serological and histological findings, although ANCA is reported to be negative in 60–70% of cases. Systemic steroids are the first-line therapy, with a role for second-line immunosuppressants such as methotrexate and rituximab. The key learning points from this case are to keep an open mind in considering a systemic cause for cutaneous manifestations, especially when the patient does not fulfil the usual demographic picture.

Is transition between subtypes of pemphigus possible? A series of pemphigus vulgaris patients showing the transition to pemphigus foliaceus

BackgroundPemphigus vulgaris (PV) and pemphigus foliaceus (PF) are subtypes of pemphigus with distinct clinical and laboratory features. The transition between these two subtypes has rarely been reported previously. MethodsThe data of PV patients who exhibited clinical and immunoserological transition to PF during the follow-up period were retrospectively evaluated regarding their demographical, clinical, and laboratory characteristics. ResultsAmong 453 patients diagnosed with PV, 13 (2.9%) patients exhibited clinical and immunoserological transition from PV to PF. The mean age of PV patients at the time of diagnosis was 39.8 ± 14.7 (19‒62) years and 7 (53.8%) of them were female. These patients showed clinical and immunoserological transition from PV to PF after a period ranging from 4 months to 13 years (mean 36.2 ± 41 months). In addition to typical clinical features of PF, all patients had positive anti-desmoglein-1 and negative anti-desmoglein-3 antibody levels after the clinical transition had occurred without any mucosal involvement. During a mean 7.8 ± 5.8 (2‒21) years of follow-up period after the transition from PV to PF, only one female patient had experienced a re-transition to PV characterized by a relapse of disease involving mucosal surfaces with positive anti-desmoglein-3 antibody levels following a 5-year period of remission period without treatment. Study limitationsSingle-center study with a retrospective study design. ConclusionOur series is the largest group of patients reported to show the transition from PV to PF to date with a long follow-up period. The reason behind the disappearance of anti-desmoglein-3 antibodies and the pathogenesis of this phenomenon is not yet elucidated.

Detection of herpes simplex viruses in the oral lesions of patients with pemphigus vulgaris: Is it diagnostic or predictive of disease severity?

Some studies emphasise the relationship between the herpes simplex virus (HSV) and pemphigus. Although the possible role of HSV in the pathogenesis of pemphigus and the severity of the disease is obscure, we aimed to evaluate the presence of herpes simplex viruses (HSV 1/2) in the oral lesions of patients with pemphigus vulgaris and also assess its association with disease severity and types of lesions. A retrospective study was conducted on collected data in the form of collecting paraffin blocks, slides, and relevant pathology reports and referring to patients' medical records. A questionnaire containing details on the degree of skin, scalp, and mucosal involvement (Pemphigus Disease Area Index (PDAI)) was fulfiled. The immunoassay result was also collected to check the anti-desmoglein 3 and 1 antibodies (using ELISA technique). In this study, 52 patients of pemphigus vulgaris with oral lesions (case) and 52 patients with oral lesions not related to the disease (control) were evaluated. HSV1 was detected in 13.5% of oral pemphigus lesions and 1.9% of the control group (p=0.0598). There were no positive cases of HSV2 in either group. There was no significant association between the positivity of HSV1 and the site of lesions (p=1.00) or disease severity (p=0.28). However, we found a strong correlation between the PDAI disease severity score with the titre of the AntiDsg3 antibody (r=0.487, p=0.001) and AntiDsg1 antibody (r=0.309, p=0.026). This study demonstrated a significant prevalence of HSV1 in oral pemphigus lesions, and acyclovir therapy may play a significant role in managing these patients. However, HSV's role in the pathogenesis of pemphigus vulgaris cannot be clearly determined.

Deadly drug rashes: Early recognition and multidisciplinary care.

Potentially deadly drug rashes include Stevens-Johnson syndrome/toxic epidermal necrolysis, drug reaction with eosinophilia and systemic symptoms, acute generalized exanthematous pustulosis, and drug-induced vasculitis. Differentiating them can be a challenge. Factors to consider include timing of rash to drug exposure, rash distribution and clinical appearance, and the presence of systemic features such as mucosal involvement, organ failure, or eosinophilia. Various scoring systems aid in the diagnosis, but skin biopsy is the gold standard. Prompt identification and withdrawal of the suspected offending agent are the crucial first steps in management.

Drug-induced Stevens Johnson syndrome and toxic epidermal necrolysis: Interpreting the systematic reviews on immunomodulatory therapies.

Drug-induced Stevens-Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN) are non-immunoglobulin E-mediated severe cutaneous adverse reactions with a high risk of morbidity, mortality, and physical and mental health impact. These are associated with certain high-risk drugs, human leukocyte antigen (HLA)-specific genotypes and ethnicities. HLA class I-restricted oligoclonal CD8 cytotoxic T-cell responses occur at the tissue level in SJS/TEN. Cytotoxic T cells are the T effector cells that result in keratinocyte apoptosis (cell death) mediated by T effector molecules granzyme B, perforin, granulysin, gamma interferon, tumor necrosis factor-alpha, and lipocalin-2. The clinical hallmarks of SJS/TEN include fever, ≥2 mucosal involvements (ocular, oral, and genital), and positive Nikolsky sign with epidermal detachment. Systematic reviews on immunomodulatory treatments remain limited by the paucity of randomized controlled trials, heterogeneity of studies, and non-standardization of outcome measures. Preventive HLA genotype screening before the prescription of carbamazepine and allopurinol may further reduce the incidence of SJS/TEN. The role of immunomodulatory treatments in SJS/TEN is at present not supported by robust evidence from systematic reviews given the lack of randomized controlled trials. The evidence for improved survival with off-label use of corticosteroids plus intravenous immunoglobulins, ciclosporin plus intravenous immunoglobulins, and ciclosporin alone has not been demonstrated by network meta-analyses and meta-regression. In the real-world clinical setting, systemic corticosteroids (in SJS and overlap SJS/TEN), ciclosporin, and etanercept (in TEN) appear to be the off-label treatments currently most widely used.

Pediatric Psoriasis: Clinical Aspects and Comorbidities: A Study of 50 Patients in Morocco

Aims: The objective of our study was to describe the epidemiological and clinical characteristics of pediatric psoriasis, as well as metabolic comorbidities and cardiovascular diseases.
 Study Design: Retrospective descriptive study.
 Place and Duration of Study: Dermatology department of the CHU of Rabat Morocco over a two- and half-year period.
 Methodology: We conducted a retrospective descriptive study collecting the cases of psoriasis in children followed in the pediatric dermatology consultation of Ibn Sina University Hospital of Rabat Morocco over a two- and half-year period.
 Results: We collected 50 patients. A female predominance was notedwith a sex ratio of 0.58. Concerning the antecedents; Parental consanguinity was identified in 8 % of cases, family history of psoriasis in only 6% and the atopy in 16%. The triggering factors were an infection in 12% of cases and psychological trauma in 6 % of cases. Concerning the metabolic comorbidity, one case of diabetes (2%), one case of obesity (2%) and three cases of overweight (6%) were noted. however, no cases of dyslipidaemia were reported.Psoriasis vulgaris was the most frequent clinical presentation (48 %), followed by guttate psoriasis (34%), inverted psoriasis (10%), napkin psoriasis (4 %) and blaskoline psoriasis (2 %). Palmoplantar involvement was observed in 10 % of cases, nail involvement in 22% and scalp involvement in 40 %. Oral mucosal involvement was noted in only one patient.

717 Management of Gynecologic Involvement in Stevens-Johnson Syndrome (SJS)/ Toxic Epidermal Necrolysis Syndrome (TENS)

Abstract Introduction Stevens-Johnson Syndrome (SJS)/ Toxic Epidermal Necrolysis Syndrome (TENS) are rare and potentially life-threatening dermatologic conditions that require interdisciplinary management (Shanbhag et al., 2020). One of the most painful and traumatic is vaginal mucosal involvement. Gynecologic complications have been historically undermanaged, and lack of acute intervention can lead to dyspareunia, adhesion formation, and increased risk for vaginal cancers (Kaser et al., 2011). Unfortunately, treatment is variable but typically involves use of vaginal dilators, which can be painful, and often traumatic for both patient and provider. The purpose of this study was to analyze the utilization of these treatments and to observe any sequelae from gynecological involvement. Methods Retrospective chart review was completed for all female patients with a diagnosis of SJS/TENS who were admitted to a single center burn unit from 2015-2022. Trends in age, gynecologic involvement, wound care strategies, and outcomes were assessed. Results Twenty-nine patients met inclusion criteria. These patients ranged from 9-82 years of age with a mean of 43 (STD 21). Ten percent died in the hospital, 14% were transferred to another hospital, and the remaining patients were discharged to home or a rehabilitation facility. Of these 29 SJS/TENS patients, 59% had gynecologic involvement of which 52% received gynecologic interventions. Gynecologic interventions included use of vaginal dilators (67%), topical steroids (60%), antifungal ointment (33%), and menstrual suppression (13%). For pediatric populations, patient discomfort and family concerns limited pelvic exam and vaginal interventions. Accommodations for pediatric patients included completion of examination and treatment under anesthesia and education of parents about the treatment process. While 2 patients were confirmed to have no vaginal adhesions in follow up appointments, documentation of future vaginal complaints was not available for most patients. Conclusions Gynecologic management is an important part of SJS/TENS treatment with wide variability in practice patterns not only nationally but at a single setting institution. Outcomes from treatment versus non treatment with steroids +/- dilation is equivocal. Providers must be better at screening for symptoms in outpatient follow up. When forming new algorithms, special consideration among pediatric populations and invasive management needs to be considered. Applicability of Research to Practice There is wide practice variability on the gynecological management of SJS/TENS patients with variable outcomes. Longitudinal studies on outpatients would better help create algorithms for patient management.

Manifestations of Various Diseases and Changes in The Body in Children Suffering from Drug Allergy

It is characterized by polymorphic rashes in the form of erythema, target-shaped papules, which can progress to vesicular and bullous lesions, on the site of which erosions form. Rashes are mainly localized on the hands, feet, upper and lower extremities. Mucosal involvement may occur. Erythema multiforme is a polyetiological disease mainly based on hypersensitivity reactions to drugs or infection, but in some cases associated with other pathological conditions, in particular with Kawasaki disease [68]. Treatment of patients is based on the withdrawal of causative drugs or the treatment of existing infectious diseases. In some cases, the course is recurrent, which is due to unresolved antigenic stimulation.

Five- and 10-year survival in extramammary Paget's disease: A focus on wide local excision.

This study aimed to analyze extramammary Paget's disease (EMPD)-specific survival, overall survival, and recurrence rate (RR) in patients with EMPD in South Korea, with a focus on wide local excision. We retrospectively reviewed the medical records of patients with EMPD from 1993 to 2020 at Kyungpook National University Hospital. We determined the survival and RRs after wide local excision. A total of 95 patients (66 males and 29 females; mean age 67.4 years) were included. The 5-year disease-specific survival and overall survival were 91.8% and 79.3%, respectively, whereas the 10-year rates were 81.6% and 64.7%, respectively. No significant sex differences were observed. Seventy-five patients (78.9%) underwent wide local excision. Mucosal involvement and lymphadenopathy were identified as the significant prognostic factors of disease-specific survival in multivariate analysis. The RR was 14.7% in patients who underwent wide local excision: seven local, two regional, and two distant metastases, with a mean recurrence-free interval of 42.3 months. Based on the survival and RRs obtained, surgical treatment of EMPD with wide local excision provides fair curative resection. Wide local excision can be a feasible treatment option for extramammary Paget's disease.

Manifestations of Various Diseases and Changes in the Body in Children Suffering from Drug Allergy

It is characterized by polymorphic rashes in the form of erythema, target-shaped papules, which can progress to vesicular and bullous lesions, on the site of which erosions form. Rashes are mainly localized on the hands, feet, upper and lower extremities. Mucosal involvement may occur. Erythema multiforme is a polyetiological disease mainly based on hypersensitivity reactions to drugs or infection, but in some cases associated with other pathological conditions, in particular with Kawasaki disease [68]. Treatment of patients is based on the withdrawal of causative drugs or the treatment of existing infectious diseases. In some cases, the course is recurrent, which is due to unresolved antigenic stimulation

Clinical Approach to Ocular Cicatricial Pemphigoid.

To evaluate the demographic data, ocular and systemic findings, clinical management, and outcomes of patients with ocular cicatricial pemphigoid (OCP). The medical records of 11 patients diagnosed as having OCP in the ophthalmology department of Ege University between 2008 and 2021 were evaluated retrospectively. The patients' mean follow-up time was 14±5.76 months. All eyes (100%) had conjunctival involvement and 18 (81.81%) had corneal involvement. According to the Tauber staging system, 7 (31.81%), 8 (36.36%), and 7 (31.81%) of the eyes were stage 2, 3, and 4, respectively. The diagnosis was confirmed in 6 (66.66%) of 9 patients who underwent biopsy. Amniotic membrane transplantation was performed in 7 eyes, entropion surgery in 2 eyes, and electrocauterization for trichiasis in 5 eyes. Systemic involvement was observed in 45.45% (5/11) of patients, most commonly oral mucosal involvement (18.18%). Review of medical records showed that alkylating agents, steroids, and dapsone were used in patients treated before 2020. Mycophenolate mofetil was preferred to be used in combination with corticosteroids. Although treatment responses before mycophenolate mofetil usage could not be evaluated well because of loss to follow-up, 4 (66.66%) of 6 patients who received steroid treatment combined with mycophenolate mofetil showed partial or complete clinical remission. No serious side effects and drug withdrawal were observed. OCP is a sight-threatening autoimmune disease that affects older adults. Although positive biopsy results are valuable for diagnosis, negative results do not exclude the diagnosis. The main treatment is systemic immunosuppressives. Disease activity can be suppressed, especially with early initiation of drug therapy. These patients require a multidisciplinary approach. Especially in the presence of isolated ocular findings, ophthalmologists should be able to make the decision to start immunosuppressive treatment, and systemic treatment should not be delayed.

Post-acute sequelae of COVID-19 is characterized by diminished peripheral CD8+β7 integrin+ T cells and anti-SARS-CoV-2 IgA response

Several millions of individuals are estimated to develop post-acute sequelae SARS-CoV-2 condition (PASC) that persists for months after infection. Here we evaluate the immune response in convalescent individuals with PASC compared to convalescent asymptomatic and uninfected participants, six months following their COVID-19 diagnosis. Both convalescent asymptomatic and PASC cases are characterised by higher CD8+ T cell percentages, however, the proportion of blood CD8+ T cells expressing the mucosal homing receptor β7 is low in PASC patients. CD8 T cells show increased expression of PD-1, perforin and granzyme B in PASC, and the plasma levels of type I and type III (mucosal) interferons are elevated. The humoral response is characterized by higher levels of IgA against the N and S viral proteins, particularly in those individuals who had severe acute disease. Our results also show that consistently elevated levels of IL-6, IL-8/CXCL8 and IP-10/CXCL10 during acute disease increase the risk to develop PASC. In summary, our study indicates that PASC is defined by persisting immunological dysfunction as late as six months following SARS-CoV-2 infection, including alterations in mucosal immune parameters, redistribution of mucosal CD8+β7Integrin+ T cells and IgA, indicative of potential viral persistence and mucosal involvement in the etiopathology of PASC.

Sporotrichosis in the nasal mucosa: A single-center retrospective study of 37 cases from 1998 to 2020.

Sporotrichosis is a subcutaneous or implantation mycosis caused by some species of the genus Sporothrix. Rio de Janeiro state, Brazil, experiences hyperendemic levels of zoonotic sporotrichosis, with increasing cases of disseminated disease, especially in people living with HIV (PLHIV). Involvement of the nasal mucosa is rare and occurs isolated or in disseminated cases, with a delayed resolution. This study aimed to describe the epidemiological, clinical, and therapeutic profiles of 37 cases of sporotrichosis with involvement of the nasal mucosa treated at the ear, nose, and throat (ENT) outpatient clinic of the Instituto Nacional de Infectologia Evandro Chagas, Fundação Oswaldo Cruz, from 1998 to 2020. Data were reviewed from the medical records and stored in a database. The Mann-Whitney test was used to compare the means of quantitative variables, and Pearson chi-square and Fisher's exact tests were used to verify the association between qualitative variables (p<0.05). Most patients were males, students or retirees, with a median age of 38 years, residents in the municipality of Rio de Janeiro, and infected through zoonotic transmission. Disseminated sporotrichosis forms in patients with comorbidities (mostly PLHIV) were more common than the isolated involvement of the mucosa. The main characteristics of lesions in the nasal mucosa were the presence/elimination of crusts, involvement of various structures, mixed appearance, and severe intensity. Due to therapeutic difficulty, itraconazole was combined with amphotericin B and/or terbinafine in most cases. Of the 37 patients, 24 (64.9%) healed, with a median of 61 weeks of treatment, 9 lost follow-up, 2 were still treating and 2 died. Immunosuppression was determinant to the outcome, with worse prognosis and lower probability of cure. Notably in this group, the systematization of the ENT examination for early identification of lesions is recommended to optimize the treatment and outcome of the disease.