Mucocutaneous Disorder Research Articles

Mucocutaneous Manifestations in Patients with Chronic Kidney Disease: A Cross-sectional Study.

Background:Chronic kidney disease (CKD)-associated mucocutaneous manifestations significantly impair the quality of life but often remain understudied. They may also vary across regions, socioeconomic and nutritional status, and racial differences.Objectives:To study the patterns of mucocutaneous disorders and their prevalence in CKD patients irrespective of clinical stage or dialysis status.Materials and Methods:122 (M:F = 77:45) patients aged 21‒85 (Mean ± SD = 57.5 ± 14.0) years having CKD for 3 month to 5 years were studied for mucocutaneous manifestations. Fifty (41%) patients were on hemodialysis for 1‒42 months. Detailed medical history, clinical and mucocutaneous examination, and lab investigations were performed. KOH mounts, skin biopsy, Gram's and Giemsa staining, bacterial or fungal cultures were performed as required.Results:Xerosis in 93 (76.2%), skin pallor in 61 (50%), pruritus in 57 (46.7%), pigmentation in 47 (38.5%), and purpura in 18 (14.8%) patients were the major dermatoses. Bullous lesions and perforating folliculitis occurred in 3 (2.5%) patients each. Major nail abnormalities were pallor (in 35.2%), absent lunula (in 23.8%), nail discoloration (in 18%), and “half-and-half nails” in 16.4% patients, respectively. Hair abnormalities included sparse scalp and body hairs (in 35.2% and 13.1%, respectively) and lusterless hair in 12.3% patients. Coated tongue (in 14.8%), xerostomia (in 12.3%), and macroglossia with teeth indention (in 7.4%) patients were the mucosal manifestations.Conclusions:Xerosis, pruritus, skin pallor/pigmentary changes, nail pallor, absent lunula, nail discoloration, sparse hairs, coated tongue, xerostomia, macroglossia, and infections were the most common mucocutaneous manifestations in the studied patients irrespective of hemodialysis status. Cold and dry climates might be additional aggravators for xerosis/pruritus. Lifelong follow-up may be needed to reduce the morbidity associated with CKD/hemodialysis specific dermatoses appearing over a period.

Multivariant lichen planus of lips and gingiva: Report of a case and a review

Oral lichen planus (OLP) is a relatively common inflammatory mucocutaneous disorder that frequently involves the oral mucosa. There are many etiological factors, and some among them are stress, diabetes mellitus, and hypothyroidism. An important complication of OLP is the development of oral squamous cell carcinoma, which led the World Health Organization to classify OLP as a potentially malignant disorder. This article reports a multivariant (reticular, erosive, and papular) case of lichen planus that has affected a 40-year-old medically compromised female who was managed successfully and still under follow-up.

Current trends in the management of Stevens–Johnson syndrome: A call for a paradigm shift in ophthalmic care in Nigeria

Stevens–Johnson syndrome (SJS) is a severe blistering mucocutaneous disorder, which affects the skin and at least two mucous membranes that very often includes the eyes. The ophthalmic complications, though considered the most devastating of all the complications of the disease in survivors, are often a time the last to be attended to, with consequent life-changing sequelae. Medical therapy has been the mainstay of ophthalmic care in our locality, and these have not been shown to improve the long-term outcome of the disease. Glass rod synechiolysis, previously practiced in some centers, has been largely abandoned. Appropriate proactive interventions such as lubrication, topical antibiotics, and steroids are advocated in the acute phase. Surgical management to remove the membranes and the use of improvised symblepharon rings prevent adhesions. Amniotic membrane grafting or mucous membrane grafting for lid margin keratinization and forniceal scarring if implemented will also take advantage of a window of opportunity to ameliorate the severity of the long-term sequelae requiring more specialized and expensive interventions for vision restoration. The critical role of the ophthalmologist in the management of patients with SJS for the prevention of corneal blindness, therefore, cannot be overemphasized.

Severity and Features of Epistaxis in Children with a Mucocutaneous Bleeding Disorder

To use standardized bleeding questionnaires to compare the severity and patterns of epistaxis in children with a mucocutaneous bleeding disorder and control children. The epistaxis sections of the Pediatric Bleeding Questionnaire (PBQ) administered to pediatric patients with von Willebrand disease or a platelet function disorder and healthy control children were reviewed. Scores and features of epistaxis (frequency, duration, onset, site, seasonal correlation, and need for medical/surgical intervention) were recorded. A PBQ epistaxis score ≥2 was defined as clinically significant. The Katsanis epistaxis scoring system was administered to eligible patients, ie, with ≥5 episodes of epistaxis per year. PBQ epistaxis scores were obtained for 66 patients, median age 12 years (range 0.6-18.3 years), and 56 control children. The median PBQ epistaxis score in patients was 2 vs 0 in control children (P <.0001). All of the features of epistaxis, except spontaneous onset, occurred in a significantly greater proportion of patients than control children with epistaxis. A total of 50% of the patients were graded as having severe epistaxis by the Katsanis epistaxis scoring system, and 30 of these (91%) had a clinically significant PBQ epistaxis score. Standardized bleeding questionnaires are useful in the assessment of epistaxis severity and pattern and may help to distinguish children with and without a mucocutaneous bleeding disorder.

Evidence for a contributory role of a xenogeneic immune response in experimental epidermolysis bullosa acquisita.

Autoimmune diseases affect a large fraction of the population in Western countries. To elucidate the underlying causes, autoantibody transfer-induced mouse models have been established that greatly contributed to the understanding of the pathophysiology of these diseases. However, the role of a potentially co-occurring murine xenogeneic immune response to commonly utilized rabbit anti-mouse IgG remains poorly understood. Using the established rabbit anti-mouse type VII collagen (COL7) IgG-induced mouse model of the mucocutaneous blistering disorder epidermolysis bullosa acquisita (EBA), we found in this study a profound T- and B-cell response along with an altered cytokine expression profile in draining lymph nodes of mice injected with the xenogeneic IgG. This was associated with the formation of circulating and skin-bound mouse anti-rabbit IgG in wild-type but not CD154-deficient or B-cell-deficient JHT mice challenged with pathogenic rabbit IgG. Development of EBA skin lesions was attenuated in the two mouse strains lacking a B-cell response at later observation time points, but was not affected in mice treated with the T-cell trafficking blocker FTY720. Collectively, our results implicate a host's xenoreactive immune response to rabbit anti-mouse COL7 IgG, a confounding effect that may contribute to immune complex-driven inflammation and tissue damage in this antibody transfer-induced EBA mouse model, especially at later time points. In this regard, it may be recommended to finish the evaluation of results obtained by experiments employing antibody-transferred mouse models within the first 2weeks after the pathogenic antibody injection.

Assessment of griseofulvin in the management of lichen planus

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; &lt;span lang="EN-IN"&gt;Oral lichen planus (OLP) is a common mucocutaneous disorder that affects 1-2% of the adult general population and slight predominance in females has been observed. The success rate is not satisfactory with these modalities of treatment, so there is a clear need for alternative therapy. This study was done to evaluate the efficacy of griseofulvin in the treatment of lichen planus.&lt;/span&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; &lt;span lang="EN-IN"&gt;The present study was conducted in the department of dermatology in the medical institution for the period of one year. The study included the initial assessment of 60 patients who were diagnosed with lichen planus (LP). Patients with both sexes and age between 15-60 years who agreed to come on follow up examination were included. All patients were treated with griseofulvin 500 mg/day for 6 months. Response of treatment was assessed by clinical examination at each subsequent visit (every two weeks)&lt;/span&gt;.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; &lt;span lang="EN-IN"&gt;Among patients with OLP, there was complete response in 27%, moderate improvement in 51%, and no response in 22% of cases. Complete clinical response of cutaneous LP was seen in 18% cases, no response was found in same number of patient, 64% no. of cases showed moderate response. &lt;/span&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; &lt;span lang="EN-IN"&gt;Griseofulvin gives complete improvement in 27% cases and moderate improvement in 51% cases in OLP and it gives complete improvement in 16% cases in cutaneous LP after treatment of 6 months. This study was done on a small scale and without any control group, so conclusive comments could not be passed.&lt;/span&gt;&lt;/p&gt;

In situ delivery of corticosteroids for treatment of oral diseases.

In many mucocutaneous disorders, corticosteroids therapy is currently central. Systemic therapy is restricted to severe disorders whereas topical applications are considered as the first-line treatment. The oral cavity environment, the medication form and other factors related to the delivery method are key factors for the therapy efficiency and effectiveness. Current marketed medications are not able to avoid wrong drug exposure and scarce patients' compliance. Innovative in situ delivery systems are able to prolong the drug retention time on the mucosa and to avoid the drawbacks of conventional formulations. This review is intended to give a general overview of oral mucocutaneous pathologies and highlight the potential of new technologies in designing innovative delivery systems able to release corticosteroids in situ for the treatment of various oral cavity disorders.

Zero prevalence of hepatitis B and hepatitis C infections in clinicopathologically proven lichen planus cases: a cross sectional study at a tertiary care centre in south India

&lt;p class="abstract"&gt;&lt;strong&gt;Background:&lt;/strong&gt; Lichen planus (LP) is a common chronic mucocutaneous inflammatory disorder of unknown etiology. Several editorials, chapters, studies have suggested an association between hepatitis C virus and hepatitis B virus (to a lesser extent) in LP. This association was not seen in various parts of the world including India.&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Methods:&lt;/strong&gt; Patients attending the dermatology outpatient department with lichen planus confirmed by histopathologic examination were included in the study and along with clinic-epidemiological data, all the patients were tested for hepatitis B and hepatitis C infection.&lt;strong&gt;&lt;/strong&gt;&lt;/p&gt;&lt;p class="abstract"&gt;&lt;strong&gt;Results:&lt;/strong&gt; A total of 84 patients of clinico-pathologically proven lichen planus were included in the study out of which 31 were males and 53 were females. The most common age group of lichen planus in our study group was 40-50 years. Major histopathological findings were basal cell degeneration (92.7%), hyperkeratosis (89%), pigment incontinence (73%) and lymphohistiocytic infiltration (70%). All patients in this study group tested negative for HBsAg and anti-HCV antibodies.&lt;/p&gt;&lt;p&gt;&lt;strong&gt;Conclusions:&lt;/strong&gt; Although numerous studies around the world have suggested an association between HCV and HBV infections with lichen planus- multiple subsequent studies including the present study shows contrasting results especially in this part of the world. The authors believe routine screening of lichen planus patients for presence of hepatitis B and hepatitis C infection is not warranted, particularly in Indian population. &lt;/p&gt;

Mucocutaneous disorders complicating inflammatory bowel disease and related mechanisms

Mucocutaneous disorders complicating inflammatory bowel disease and related mechanisms

Chinese Systemic Lupus Erythematosus Treatment and Research Group Registry IX: Clinical Features and Survival of Childhood-Onset Systemic Lupus Erythematosus in China.

Background:Approximately 15–20% cases of systemic lupus erythematosus (SLE) are diagnosed in children. There have been a few studies reporting the epidemiological data of pediatric-onset SLE (cSLE) in China, neither comparing the differences between cSLE and adult-onset SLE (aSLE). The aim of this study was to describe the impact of age of onset on clinical features and survival in cSLE patients in China based on the Chinese SLE Treatment and Research group (CSTAR) database.Methods:We made a prospective study of 225 cSLE patients (aged < 16 years) and 1759 patients aged 16–50 years based on CSTAR registry. We analyzed initial symptoms, clinical presentations, SLE disease activity, damages, and outcomes of cSLE, as well as compared with aSLE patients.Results:The mean age of cSLE patients was 12.16 ± 2.92 years, with 187 (83.1%) females. Fever (P < 0.001) as well as mucocutaneous (P < 0.001) and renal (P = 0.006) disorders were found to be significantly more frequent in cSLE patients as initial symptoms, while muscle and joint lesions were significantly less common compared to aSLE subjects (P < 0.001). The cSLE patients were found to present more frequently with malar rash (P = 0.001; odds ratio [OR], 0.624; 95% confidence interval [CI], 0.470–0.829) but less frequently with arthritis (P < 0.001; OR, 2.013; 95% CI, 1.512–2.679) and serositis (P = 0.030; OR, 1.629; 95% CI, 1.053–2.520). There was no significant difference in SLE disease activity index scores between cSLE and aSLE groups (P = 0.478). Cox regression indicated that childhood onset was the risk factor for organ damage in lupus patients (hazard ratio 0.335 [0.170–0.658], P = 0.001). The survival curves between the cSLE and aSLE groups had no significant difference as determined by the log-rank test (0.557, P = 0.455).Conclusions:cSLE in China has different clinical features and more inflammation than aSLE patients. Damage may be less in children and there is no difference in 5- year survival between cSLE and aSLE groups.

Is chronic ulcerative stomatitis a variant of lichen planus, or a distinct disease?

Chronic ulcerative stomatitis is an immune-mediated mucocutaneous disorder characterized clinically by erosions or ulcers. Most cases are limited to the mouth. The histopathological features are non-specific or mimic those of oral lichen planus, and studies by immunofluorescent microscopy are essential for definitive diagnosis. The defining immunopathogenic mechanism is the binding of IgG to the nuclear protein deltaNp63alpha of keratinocytes in the basal and parabasal cell layers of the oral stratified epithelium. DeltaNp63alpha functions as a regulator of epithelial stem cell activity and as an antiapoptotic agent and regulates the expression of cell-to-cell and cell-to-basement membrane adhesion molecules. The autoimmune IgG-deltaNp63alpha interaction is thought to result in damage to the structural attachment of keratinocytes to one another and to the epithelial basement membrane zone and in dysregulation of the cell cycle and apoptosis of basal keratinocytes with the development of erosions or ulcers. The aims of treatment are to suppress the pathogenic immunoinflammatory responses, to prevent local infection and to promote healing. The purpose of this article is to provide a succinct review of the diagnostic, clinical and etiopathogenic features of, and treatment guidelines for chronic ulcerative stomatitis, and to argue that this disease should be regarded as a variant of oral lichen planus, rather than as a distinct entity.

Immunopathogenic Oral Diseases: An Overview Focusing on Pemphigus Vulgaris and Mucous Membrane Pemphigoid.

Pemphigus vulgaris, mucosal pemphigoid (mucous membrane pemphigoid), lichen planus, discoid lupus erythematosus and erythema multiforme are a group of immune-mediated mucocutaneous disorders characterised clinically by the formation of blisters, erosions or ulcers. The oral mucosa is often affected, and sometimes the disease is limited to the mouth. The target antigens, autoreactive immune responses, microscopic features, treatment and prognosis vary from one disease to the other. Treatment aims to eliminate exogenous risk factors, suppress the pathogenic immuno-inflammatory reactions, promote healing and prevent infection. The aim of this article is to provide the general dental practitioner with a succinct overview of the diagnostic, clinical, aetiopathogenic features and characteristics of, as well as treatment guidelines for oral pemphigus vulgaris and oral mucosal pemphigoid. Early diagnosis and treatment could prevent severe consequences of the disease in their full-blown forms.

Erythema multiforme: A case report

Erythema multiforme (EM) is a life threatening mucocutaneous disorder where early diagnosis and management is of utmost important. Among various etiological agents, viral etiology is the most common and is characterized by ulcerations, erosions, and bleeding within the mucosa associated with encrustations and tissue tags. Dermal counterparts present with target iris lesion. Here, we report a case of viral-induced EM major where there are classical oral and dermal lesions in a 28-year-old male patient.

Genital Ulcers in Women with HIV Proposed Revision on Management Algoritm

More than 100,000 people has been infected by HIV (Human Immunodeficiency Virus) in Indonesia. Mucocutaneous disorders occur in most patients with HIV during the course of the disease, it is caused by acquired immunodeficiency or the effects of the HIV therapy. One of the most important clinical manifestations is genital ulcers. A specific attention is needed to identify the clinical manifestations of genital ulcers in HIV patients to find the etiology so that the treatment of the case can be more appropriate. There are 4 cases of genital ulcers in women with AIDS (Acquired Immune Deficiency Syndrome). Both VDRL test (Venereal Disease Reseach Laboratory) and TPHA (Trepenoma pallidum Haemagglutination) test gave negative results. Two cases has successful therapy with acyclovir 400 mg three times for 2 weeks. Test results for herpes simplex antibodies is 2 positive. The incidence of genital ulcers, especially in the chronic form, increases along with the incidence of the growing AIDS epidemic. A prospective study in Paris shows that the most common cause of genital ulcers in patients with AIDS is syphilis. All of these four cases have negative results of VDRL and TPHA, so it is necessary to consider another diagnosis as the cause of genital ulcers in immunocompromised patients that often give atypical clinical symptoms. The accuracy of the diagnosis is necessary to provide adequate treatment, especially in immunocompromised patient.

Mucocutaneous Disorders of Pediatric HIV in South West Nigeria

Nigeria has the world's highest burden of pediatric HIV. In the face of paucity of monitoring tests in Nigeria, we studied the spectrum of pediatric mucocutaneous manifestations and evaluated their clinical utility as surrogate markers for immunodeficiency and plasma viral load levels. Cross-sectional study comparing mucocutaneous manifestations in 155 HIV-positive children aged 12 weeks to 14 years with 155 HIV-negative children. Relationships between mucocutaneous manifestations in HIV-infected patients and their immunologic and virologic indices were analyzed. Mucocutaneous lesions were seen in 53.5% of HIV-infected children compared with 18.1% of the controls. Prevalence of lesions increased with worsening levels of immunodeficiency and increasing viral loads (P < .01). Oral candidiasis, angular stomatitis, and fluffy hair were associated with more severe degrees of immunodeficiency. Mucocutaneous disorders are common in HIV-infected children. Oral candidiasis and nutritional dermatoses can be used as surrogates for advanced or severe immunodeficiency.

The Role of the Glucocorticoid System in Anchorageindependence during Progression of Squamous Cell Carcinoma

Background. Topical and systemic corticosteroids are widely used for the treatment of a variety of mucocutaneous disorders including those with malignant potential. We have shown previously that normal keratinocytes regulate the local concentration of active steroids, as well as synthesize hydrocortisone de novo following stimulation with ACTH. The role of locally produced hydrocortisone in tumour progression is unclear. Methods. The human keratinocyte cell line HaCaT derived from irradiated facial skin and c-Ha-ras- transfected HaCaT cell clones (I-7, II-3, RT-3) demonstrate increasing tumorigenic and metastatic potential in vivo and are ideally suited to studies of keratinocyte tumour progression. The formation of multicellular aggregates (MCAs) in vitro was used as a paradigm of metastatic potential in vivo. Cortisol levels were assessed by ELISA. Results. Non-tumorigenic (HaCaT), benign (I-7), malignant with low metastatic potential (II-3) and malignant with high metastatic capacity (RT-3) keratinocytes secreted basal levels of cortisol, activated cortisone and differentially synthesized cortisol de novo in the presence of ACTH; malignant cells (II-3 and RT-3) produced the highest levels of the steroid. When the cells were cultured in the absence of a substrate,

A rare case of isolated extragenital bullous lichen sclerosus

Lichen sclerosus et atrophicus is a rare, chronic, mucocutaneous disorder of unknown etiology predominantly involving the anogenital skin and mucosa. It is rarely seen exclusively at an extragenital site. A 40-year-old man presented with multiple asymptomatic clear fluid-filled vesicles with shiny atrophic papules over bilateral thighs and the calf region for a period of 4 months. Histopathology confirmed the diagnosis of bullous lichen sclerosus. The patient showed significant improvement with topical clobetasol propionate 0.05% and tacrolimus 0.1% ointment.

Oral mucosal lichen planus in childhood

Lichen planus is a relatively common mucocutaneous disorder in adults, but it is rarely reported in children. Much less data is available regarding lichen planus in children. This is a report with intraoral lesions of lichen planus. Lichen planus, although reportedly rare in childhood, should be considered in the diagnosis of hyperkeratotic or erosive lesions of oral mucosa in children.

Treatment modalities of oral lichen planus: an update

Oral lichen planus is an immunologically based, chronic, inflammatory, mucocutaneous disorder of undetermined etiology. It is a relatively common disorder affecting stratified squamous epithelia. It is of special importance due to its malignant potential and can be a source of morbidity. The management of oral lichen planus should therefore address both the transformation rate as well as the patient symptoms. Care and management of such patients challenges even the most experienced clinician. There is currently no cure for OLP. Treatment is aimed primarily at reducing the length and severity of symptomatic outbreaks. The review critically analyses the various options in the literature and discusses the practical management from the Indian perspective.

The Correlation between Desquamative Gingivitis Associated-Diseases and Plaque-Induced Periodontal Disease

Desquamative gingivitis (DG) is a gingival manifestation of systemic mucocutaneous disorders such as mucous membrane pemphigoid, oral lichen planus, and pemphigus vulgaris. The lesion is very painful, so affects the patient’s ability to do proper oral hygiene practices. This may be a potential risk factor for long-term periodontal health. However, there is some controversy about the relationship between the existence of DG and periodontal status. Although the correlation between DG-associated diseases and periodontal status is not to be certain, early diagnosis and appropriate treatment including adequate plaque control and removal of local factors is very important for preventing the progression of diseases and destruction of periodontal tissues.