Spectrum Of Mucocutaneous Manifestations Research Articles

Cerebriform patterns in dermatology

Dermatology has vast spectrum of mucocutaneous manifestations in respect of morphology of the lesions, their arrangement, distribution and other feature. Cerebriform pattern is one of many interesting presentation which has been described in some skin conditions in English literature. Here in this article, those conditions have been compiled and described in brief.

Clinical characteristics of oral mucosal lesions in patients with systemic lupus erythematosus and their association with clinical and laboratory parameters

Introduction. Systemic lupus erythematosus (SLE) is an autoimmune disease that includes a broad spectrum of mucocutaneous manifestations. Objectives. To characterize the clinical spectrum of oral mucosal lesions in patients with SLE and to analize their association with clinical and laboratory parameters. Methods. We performed a cross-sectional study with systematic oral evaluations in SLE adult patients. Systemic and cutaneous lupus activities were recorded. We collected epidemiologic, clinical, and laboratory data. Statistical analysis included the kappa coefficient, X2 test, Fisher’s exact test and Mann-Whitney U-test, adjusting for multiple comparisons according to Bonferroni’s method. Results. A total of 181 patients (92.8% females) were included, with a median age of 37 (range 16-76) years. Cutaneous, systemic, and oral manifestations of lupus erythematosus (LE) activity were found in 31.5%, 23.8% and 18.8% of patients, respectively. Higher titres of anti-double-stranded (ds) DNA antibodies were detected in patients with LE-related oral lesions (LEOL) when compared to those without LEOL [356 (82-1083) UI vs 45 (0-417) UI; p=0.02). LEOL did not correlate to cutaneous (k=0.380) nor systemic (k=0.228) LE-activity (p<0.01). Conclusions. Oral manifestations related to SLE were significantly associated to anti double-ds DNA antibodies. LEOL were independent of cutaneous and systemic activity.

Effects of CD4 cell count and antiretroviral therapy on mucocutaneous manifestations among HIV/AIDS patients in Yunnan, China

The affecting factors of mucocutaneous manifestations inhuman immunodeficiency virus (HIV)/acquired immunodeficiency syndrome (AIDS) patients remain unclear in China. A retrospective analysis was conducted among HIV/AIDS patients in Yunnan, China. The demographic data, mucocutaneous manifestations, CD4 cell counts, and antiretroviral therapy (ART) regimens were collected. The effects of CD4 cell count and ART on the spectrum of mucocutaneous manifestations were evaluated. Among 508 HIV/AIDS patients, 86.0% of cases showed mucocutaneous manifestations. The average CD4 cell count (176 cells/μl) of the patients with manifestations was significantly lower than those without manifestations (328 cells/μl) (P<0.001). Diseases such as herpes zoster, oral candidiasis, condyloma acuminatum, genital herpes, oral leukoplakia, talaromycosis, cryptococcosis, and HIV-PPE (pruritic papular eruption) were represented quite frequently in patients with CD4 cell count <200 cells/μl (P<0.05), but eczema was suffered by those with CD4 cell count ≥200 cells/μl (P<0.05). ART could decline the incidence of herpes zoster, oral candidiasis, condyloma acuminatum, genital herpes, oral leukoplakia, talaromycosis, and cryptococcosis (P<0.05). Mucocutaneous manifestations are closely related to the CD4 cell count and can be used as early predictors of HIV/AIDS and immune status in clinic. ART could reduce the incidence of certain mucocutaneous manifestations.

Spectrum of Mucocutaneous Manifestations among Patients with Various Liver Disorders

Spectrum of Mucocutaneous Manifestations among Patients with Various Liver Disorders

Stevens-Johnson syndrome/toxic epidermal necrolysis spectrum for the rheumatologist

Stevens-Johnson syndrome/toxic epidermal necrolysis (SJS/TEN) represent a spectrum of mucocutaneous manifestations characterized by widespread epidermal and/or mucosal detachment from dermis. It occurs due to Type IV hypersensitivity leading to keratinocyte apoptosis. Many extrinsic and intrinsic defects in the apoptotic pathway have been postulated to result in its dysregulation. The characteristic skin lesions of SJS/TEN include ill-defined, coalescing, red macules with necrotic centers followed by extensive epidermal detachment. Mucosa is involved in up to 90% of cases causing erosions and crusts that may precede or follow skin lesions. Supportive care is the mainstay of treatment and if not started promptly may result in high mortality and morbidity. Although many immunosuppressive drugs have been tried in treatment, no treatment has proven beneficial except cyclosporine which has been shown to retard the progression of SJS/TEN. A patient with SJS/TEN as a presenting feature poses two-fold challenge to a rheumatologist as it could be a manifestation of lupus or side effects of a myriad of drugs used by the rheumatologist.

Spectrum of mucocutaneous manifestations in an Asian cohort of patients with leukemia.

Literature on cutaneous manifestations of leukemia is limited. To determine the pattern of mucocutaneous manifestations in adult Asian patients with leukemia and to establish their relation with the leukemia type. After previous consent, 196 consecutively registered patients with leukemia aged ≥18 years were recruited. All patients were prospectively followed for 3 months to evaluate the patterns of mucocutaneous involvement. The mucocutaneous manifestations were categorized into specific lesions with leukemic infiltration and non-specific lesions. Seventy-nine (40.3%) of 196 (males 128 and females 68) recruited patients showed one or more mucocutaneous manifestations. The total number of complaints observed was 87 with mean number of dermatoses per patient being 0.44. Specific manifestations (leukemia cutis) were present in six (3.06%) and nonspecific mucocutaneous manifestations in 73 (37.2%, reactive dermatoses n = 21 and infections n = 52). Cutaneous viral infections were significantly associated with acute lymphoblastic leukemia (P < 0.005). Antiviral prophylaxis with acyclovir significantly reduced the incidence of varicella-zoster infection (P = 0.016). Cutaneous manifestations are common in Asian patients with leukemia, and a thorough cutaneous examination will aid in their management.

Spectrum of mucocutaneous manifestations in 277 patients with joint hypermobility syndrome/Ehlers-Danlos syndrome, hypermobility type.

Cutaneous manifestations are a diagnostic criterion of Ehlers-Danlos syndrome, hypermobility type (EDS-HT) and joint hypermobility syndrome (JHS). These two conditions, originally considered different disorders, are now accepted as clinically indistinguishable and often segregate as a single-familial trait. EDS-HT and JHS are still exclusion diagnoses not supported by any specific laboratory test. Accuracy of clinical diagnosis is, therefore, crucial for appropriate patients' classification and management, but it is actually hampered by the low consistency of many applied criteria including the cutaneous one. We report on mucocutaneous findings in 277 patients with JHS/EDS-HT with both sexes and various ages. Sixteen objective and five anamnestic items were selected and ascertained in two specialized outpatient clinics. Feature rates were compared by sex and age by a series of statistical tools. Data were also used for a multivariate correspondence analysis with the attempt to identify non-causal associations of features depicting recognizable phenotypic clusters. Our findings identified a few differences between sexes and thus indicated an attenuated sexual dimorphism for mucocutaneous features in JHS/EDS-HT. Ten features showed significantly distinct rates at different ages and this evidence corroborated the concept of an evolving phenotype in JHS/EDS-HT also affecting the skin. Multivariate correspondence analysis identified three relatively discrete phenotypic profiles, which may represent the cutaneous counterparts of the three disease phases previously proposed for JHS/EDS-HT. These findings could be used for revising the cutaneous criterion in a future consensus for the clinical diagnosis of JHS/EDS-HT.

Spectrum of mucocutaneous manifestations in human immunodeficiency virus-infected patients and its correlation with CD4 lymphocyte count.

In this study, 100 HIV-positive cases (63 men, 37 women) with skin findings were included. The mean CD4 T cell count was 253 cells/mm(3). A total of 235 dermatological manifestations were seen. The common infectious dermatoses were candidiasis (21%), Staphylococcal skin infections (20%), dermatophytoses (14%) and herpes zoster (6%). Among the non-infectious dermatoses were papular pruritic eruptions (20%), xerosis/ichthyosis (20%) and seborrhoeic dermatitis (16%). Statistically significant association (p < 0.05) with CD4 T cell count was seen in pyodermas, dermatophytoses and papular pruritic eruptions. Adverse drug reactions, diffuse hair loss, straightening of hairs and pigmentary changes were also noted. Although there was an absence of Kaposi's sarcoma in our study, a case of verrucous carcinoma of penis was noted.

Mucocutaneous manifestations of HIV‐infected patients in the era of HAART in Guangxi Zhuang Autonomous Region, China

Human immunodeficiency virus (HIV) infection and acquired immunodeficiency syndrome (AIDS) are frequently associated with diverse mucocutaneous manifestations. However, few studies of HIV/AIDS-related mucocutaneous manifestations have been reported in China. To investigate the occurrence of mucocutaneous disorders and their relationship with the degree of immunosuppression in 348 HIV-infected Chinese patients. The influence of highly active antiretroviral therapy (HAART) on the spectrum of mucocutaneous manifestations was also evaluated. A cross-sectional study was performed on 348 HIV-infected Chinese patients seen at the Guangxi Longtan Hospital from August 2010 to November 2010. Collected information included demographic data, HIV-associated mucocutaneous disorders, CD4 cell count, AIDS-defining illness and antiretroviral therapy. In this study, 62.9% of all patients had mucocutaneous disorders. The prevalence of mucocutaneous disorders in the patients who had received HAART was lower than in those without HAART (54.05% vs. 88.76%, P < 0.001). The prevalence of mucocutaneous disorders was higher in the patients with CD4 < 200 cells/mm(3) in comparison to those with CD4 ≥ 200 cells/mm(3) (P < 0.05). The most common mucocutaneous disorders were oral candidiasis (28.47%), Penicillium marneffei infection (11.49%), drug eruptions (10.06%) and pruritic papular eruption (PPE 5.75%). Oral candidiasis, P. marneffei infection and PPE were significantly more prevalent in patients with a CD4 cell count below 200 cells/mm(3) , but frequency of drug eruptions was not related to the level of CD4 cell counts. Patients treated with HAART had decreased rates of herpes simplex, oral candidiasis and P. marneffei infection, but increased rates of drug eruptions. A wide range of mucocutaneous disorders were observed in HIV-infected Chinese patients. Oral candidiasis, P. marneffei infection and PPE may be the signs of advanced HIV infection. HAART had an impact on the spectrum of HIV-associated mucocutaneous disorders.

Mucocutaneous manifestations of chikungunya fever: A study from an epidemic in coastal Karnataka

Background:Chikungunya fever (CF) epidemic has recently re-emerged in India affecting large population. Mucocutaneous manifestations are an important clinical feature of the disease. We have reviewed mucocutaneous manifestations of the disease during a recent epidemic in coastal Karnataka.Patients and MethodsSeventy-five “suspect cases” of CF with dermatological manifestations were examined.Results:We categorized the mucocutaneous manifestation into six groups: 1. skin rash, 2. apthae like ulcers, 3. pigmentary changes, 4. desquamation, 5. exacerbation of the existing dermatoses, 6. miscellaneous. Skin rashes were the most commonly observed skin changes followed by apthae like ulcers and pigmentary changes. Desquamation of the skin over the face is a new manifestation observed by us.Conclusion:wide spectrum of mucocutaneous manifestations is observed in CF. We have provided a classification for these manifestations, which may help in better recognition and uniform recording in future.

Human immunodeficiency virus and pemphigus vulgaris: An interesting association

Pemphigus vulgaris is an autoimmune blistering disorder requiring immunosuppressive therapy with corticosteroids. If pemphigus vulgaris occurs in a seropositive patient having immunosuppression and underlying opportunistic infections (OI) steroid therapy proves to be harmful unless the patient is given treatment for OI or antiretroviral therapy. The frequency and spectrum of mucocutaneous manifestations associated with human immunodeficiency virus (HIV) infection increase with clinical and immunological deterioration. In contrast autoimmune-mediated disorders tend to improve with HIV disease progression. We discuss a case of pemphigus vulgaris infected by HIV. Co-presentation of pemphigus vulgaris and HIV raises many clinical and therapeutic issues. The mutual impact of such co-presentation needs to be explored thoroughly. (excerpt)

The spectrum of mucocutaneous manifestations during the evolutionary phases of HIV disease: an emerging Indian scenario.

Third world countries, including India, lack sophisticated investigations to assess the progression of HIV disease. Hence, this study was undertaken to determine the clinical mucocutaneous markers of HIV disease and to establish its relationship with the stage of the disease. This was an observational institutional study of 75 patients with mucocutaneous disorders and HIV infection recruited over a period extending from September of 1996 to June of 1998. The patients with mucocutaneous lesions were staged according to the Centers for Disease Control classification system for HIV infection (1986). The most frequent mode of acquisition of HIV infection was heterosexual contact (96%). The patients were broadly categorized into two groups. The AIDS group was comprised of patients who were in group IV and the early HIV infection group included patients in group II and III; none were detected in group I. Forty-eight cases belonged to the AIDS group, and 27 belonged to the early HIV infection group. A total of 207 dermatoses were diagnosed and grouped as fungal, viral, bacterial, or miscellaneous. The common mucocutaneous disorders in order of frequency observed in this study were: candidiasis, dermatophytosis, herpes simplex, oral aphthae, xerosis/ichthyosis, scabies, HPV infection, molluscum contagiosum, and psoriasis. Xerosis/acquired ichthyosis and giant molluscum contagiosum were characteristically seen in group IV of HIV disease, whereas oral candidiasis, oral aphthae, papular dermatitis of HIV, and psoriasis were early warning signs. The mean number of dermatoses per patient in group IV was 3.15; in group III, it was 2.41; and in group II, it was 1.5. There was a statistically significant difference between the early HIV infection group and AIDS group with regard to number of dermatoses. Apart from syphilis and human papilloma virus infection, the treatment outcomes were satisfactory.

Mucocutaneous manifestations in systemic amyloidosis

It has long been recognized that certain characteristic mucocutaneous symptoms and signs may act as important clues in diagnosing the early stage of systemic amyloidosis. Since the first description of clinical cutaneous lesions of primary amyloidosis by Koenigstein 1 in 1925, the spectrum of mucocutaneous manifestations in this disorder has been well documented. 2–7 There is no truly satisfactory classification of systemic amyloidosis; however, systemic amyloidosis could be simply classified as follows: 1. 1. Primary systemic amyloidosis: usually no evidence of preceding or coexisting disease but paraproteinemia or plasma-cell dyscrasia can be found. 2. 2. Amyloidosis associated with multiple myeloma. 3. 3. Secondary systemic amyloidosis: evidence for coexistence of previous chronic inflammatory or infectious conditions. 4. 4. Heredofamilial amyloidosis. Based on the result of amyloid protein analysis, Glenner 8 has proposed a more detailed clinical-chemical-pathological classification for human amyloidoses. It appears, however, that this classification is overly complex for clinicians, thus its clinical usage seems rather limited. In primary systemic and myeloma-associated amyloidosis, deposits consist mainly of light-chain (AL) amyloid protein produced as a result of plasma-cell dyscrasia. In a large series of 236 cases of systemic amyloidosis, 4 56% were of primary type and 26% were multiple-myeloma patients. During the period 1978–1987, we have found 14 cases of systemic amyloidosis in our hospital; primary systemic amyloidosis with a type of paraprotein and amyloidosis secondary to multiple myeloma occurred in nine cases (64%) and five cases (36%), respectively. Multiple myeloma associated with amyloidosis has been widely studied in medicine. It has been pointed out that amyloidosis develops in 6–15% of patients with myeloma. 9 In our hospital, five cases (4%) out of 159 myeloma patients showed an association with systemic amyloidosis.