Clinical interpretation of the Uremic Pruritus in Dialysis Patients (UP-Dial) scale: a novel instrument for the assessment of uremic pruritus.

BACKGROUND: The Uremic Pruritus in Dialysis Patients (UP-Dial) scale is valid and reliable for uremic pruritus (UP) assessment. However, it remains unknown how the scores should be interpreted in clinical practices. OBJECTIVES: To establish a clinical interpretation of the UP-Dial by identifying severity cut-off scores according to the disease severity and burden of pruritus. METHODS: This cross-sectional study developed a classification system for the UP-Dial scores using the patient-based anchors method. From May 2012 through January 2017, 697 dialysis patients were screened. Of these, a total of 258 met the criteria for UP and completed the UP-Dial scale and three sets of patient-assessed anchor questions: (i) global UP intensity by visual analogue scale (VAS)-UP, (ii) Dermatology Life Quality Index and (iii) global kidney disease-related quality of life. The cut-off scores were generated based on the kappa (κ) coefficient of agreement and the area under receiver operating characteristic curve (AuROC) statistics. Subgroup analyses were performed to explore associations between patient characteristics and the UP-Dial severity bands. RESULTS: The proposed scores were ≤12 (mild), 13-21 (moderate) and ≥22 (severe) with κ coefficient ranging between 0.39 and 0.46. Assigned scores were associated with all patient-based anchors. The highest association was with the VAS-UP, and AuROC was 0.80 (mild; 95% CI, 0.75-0.86), 0.66 (moderate; 0.60-0.71) and 0.83 (severe; 0.77-0.89). In subgroup analysis according to patient characteristics, we did not find any significant difference. CONCLUSIONS: The estimated UP-Dial severity band can facilitate the interpretation of UP in practice-based research settings and can be used to support treatment decisions.

Development of a multidimensional assessment tool for uraemic pruritus: Uraemic Pruritus in Dialysis Patients (UP-Dial).

BACKGROUND: Dialysis patients with uraemic pruritus (UP) have significantly impaired quality of life. To assess the therapeutic effect of UP treatments, a well-validated comprehensive and multidimensional instrument needed to be established. OBJECTIVES: To develop and validate a multidimensional scale assessing UP in patients on dialysis: the Uraemic Pruritus in Dialysis Patients (UP-Dial). METHODS: The development and validation of the UP-Dial instrument were conducted in four phases: (i) item generation, (ii) development of a pilot questionnaire, (iii) refinement of the questionnaire with patient recruitment and (iv) psychometric validation. Participants completed the UP-Dial, the visual analogue scale (VAS) of UP, the Dermatology Life Quality Index (DLQI), the Kidney Disease Quality of Life-36 (KDQOL-36), the Pittsburgh Sleep Quality Index (PSQI) and the Beck Depression Inventory (BDI) between 15 May 2012 and 30 November 2015. RESULTS: The 27-item pilot UP-Dial was generated, with 168 participants completing the pilot scale. After factor analysis was performed, the final 14-item UP-Dial encompassed three domains: signs and symptoms, psychosocial, and sleep. Face and content validity were satisfied through the item generation process and expert review. Psychometric analysis demonstrated that the UP-Dial had good convergent and discriminant validity. The UP-Dial was significantly correlated [Spearman rank coefficient, 95% confidence interval (CI)] with the VAS-UP (0·76, 0·69-0·83), DLQI (0·78, 0·71-0·85), KDQOL-36 (-0·86, -0·91 to -0·81), PSQI (0·85, 0·80-0·89) and BDI (0·70, 0·61-0·79). The UP-Dial revealed excellent internal consistency (Cronbach's α 0·90, 95% CI 0·87-0·92) and reproducibility (intraclass correlation 0·95, 95% CI 0·90-0·98). CONCLUSIONS: The UP-Dial is valid and reliable for assessing UP among patients on dialysis. Future research should focus on the cross-cultural adaptation and translation of the scale to other languages.

Chronic generalized fibrotic skin lesions from disseminated leishmaniasis caused by Leishmania martiniquensis in two patients from northern Thailand infected with HIV.

BACKGROUND: Leishmaniasis is a newly emerging infection in Thailand. Most of the previous human cases have presented with the clinical features of visceral leishmaniasis and were mainly found in southern Thailand. Here we report the first two patients from northern Thailand presenting with disseminated cutaneous leishmaniasis. OBJECTIVES: To determine the nature of the infection of leishmaniasis and to identify the species of parasite responsible. METHODS: Clinical investigations included the taking of biopsy samples and histology. Parasitological diagnosis was performed by establishment of Leishmania promastigote cultures, and identification was performed by DNA sequencing of four independent gene loci (ribosomal RNA internal transcribed spacer 1; large subunit of RNA polymerase II; heat shock protein 70; RPL23a intergenic sequence). RESULTS: Both patients were infected with HIV, and had multiple cutaneous lesions and accompanying visceral leishmaniasis. They had similar cutaneous manifestations characterized by chronic generalized fibrotic lesions, which were more prominent on traumatic areas. In both patients the parasite was identified as Leishmania martiniquensis. This is a recently described species that is distinct and only distantly related to the classical agents of cutaneous leishmaniasis in Asia (Leishmania major and Leishmania tropica) or of visceral leishmaniasis (Leishmania donovani and Leishmania infantum). Each patient responded well to therapy with intravenous amphotericin B followed by oral itraconazole. CONCLUSIONS: Leishmania martiniquensis is a cause of cutaneous leishmaniasis in Thailand.

Cutaneous tuberculosis in three HIV-infected patients.

Although tuberculosis is a common disease in patients infected with HIV, cutaneous presentation is not commonly found. The authors report three HIV-infected patients with cutaneous tuberculosis and lung involvement. Patient 1 presented with a nodular skin lesion on the right forearm and the diagnosis was confirmed by histopathology and PCR study. Patients 2 and 3 presented with generalized erythematous papules and vesicopustules on the trunk and extremities. Culture grew M. tuberculosis in patient 2 and M. tuberculosis DNA was detected in the skin lesion of patient 3 by the PCR method.

Uncommon manifestations of opportunistic infections in an HIV infected patient.

A case of an HIV infected patient complicated with Penicillium marneffei and Rhodococcus equi infection is reported. He presented with chronic ulcer as pyoderma gangrenosum-like on his right calf and submandibular lymphadenitis as scrofuloderma-like. Penicillium marneffei and Rhodococcus equi were isolated from the ulcer and lymph node respectively.

Congenital erythropoietic porphyria: a case report.

Congenital erythropoietic porphyria is a rare autosomal recessive disorder of heme synthesis resulting from deficiency of uroporphyrinogen III synthase (UROIIIS). It is the most severe porphyria. The clinical manifestations are markedly variable due to the different mutation in the UROIIIS gene. We recently diagnosed a case of congenital erythropoietic porphyria. A 9-year-old boy presented with recurrent ulcers on the skin especially dorsum of the hands and feet since aged 3. The physical examination revealed ulcers on the dorsum of the feet, mutilation of the fingers, fluorescent erythrodontia, and darkening and hypertrichosis of the sun exposed area. Laboratory findings showed mild hemolysis, red urine, increased serum alkaline phosphatase level, and fluorescence of the red blood cell and urine. The histopathology was consistent with porphyria. The urine and plasma porphyrin levels confirmed the diagnosis of congenital erythropoietic porphyria. The administration of oral ultracarbon and topical zinc oxide has been tried.

Bacillary angiomatosis and mycobacterium infection coexisting in a cutaneous lesion in a patient with AIDS.

Bacillary angiomatosis is a recently recognized bacterial infectious disease. It mainly affects patients with acquired immunodeficiency syndrome. The presence of coexistent infections of more than one pathologic process in skin lesions in patients with AIDS has been demonstrated. We report a patient with AIDS in whom both bacillary angiomatosis and mycobacterium infection were documented within the same cutaneous lesion.

Disseminated cryptococcosis presenting as molluscum-like lesions in three male patients with acquired immunodeficiency syndrome.

Cryptococcosis is an opportunistic infection caused by a ubiquitous encapsulated yeast, Cryptococcus neoformans. The pulmonary infection is primary site and most frequently self-limited and may be asymptomatic. The most common recognized site of disseminated cryptococcosis is the central nervous system. The cutaneous cryptococcosis is rare and nonspecific. More recently, in patients with AIDS, some cases of cutaneous cryptococcosis resembling molluscum contagiosum have been described and occasionally appeared before systemic signs and symptoms. To our knowledge, this has not previously been reported in Thailand. This is a report of 3 males with acquired immunodeficiency syndrome who developed disseminated cryptococcosis with cutaneous lesions resembling molluscum contagiosum. Skin findings in disseminated cryptococcosis indicate a poor prognosis, however, earlier recognition and treatment would improve survival.

Disseminated cutaneous cytomegalic inclusion disease resembling prurigo nodularis in a HIV-infected patient: a case report and literature review.

Cytomegalovirus infection is common disease but most of the healthy patients are asymptomatic. Cytomegalovirus infection is a serious problem in immunocompromised patients particularly in acquired immunodeficiency syndrome. About 90 per cent of patients with AIDS develop active cytomegalovirus infection and up to 25 per cent may experience life threatening infection due to the virus. However, cytomegalovirus cutaneous lesion have rarely been reported. A 40-year-old Thai man presented with multiple prurigo nodularis-like lesions on both legs and feet. The investigation revealed consistent with acquired immunodeficiency syndrome associated with cytomegalovirus cutaneous infection.

An efficacy study of itraconazole in the treatment of Penicillium marneffei infection.

OBJECTIVES: to evaluate the efficacy and safety of itraconazole in treating P. marneffei infection. METHODS: Ten patients with previously untreated P. marneffei infection were given oral itraconazole at a dose of 200 mg twice a day for 2 months, followed by a dose of 100 mg once a day for 1 month. Efficacy was determined by the clinical and microbiological cure. RESULTS: All but one patient were seropositive for human immunodeficiency virus (HIV). Two patients died during therapy. Clinical improvement was evident in 8 patients. In 7 of these, the mean duration for becoming culture negative was 57 days. Five patients presented with relapse of P. marneffei infection within four months after completion of treatment. CONCLUSIONS: Itraconazole was shown to be effective in the initial treatment of P. marneffei infection. Relapse after treatment is common and long-term suppressive therapy is recommended.

Penicillium marneffei infection in patients infected with human immunodeficiency virus.

From June 1990 to August 1991, 21 patients infected with the human immunodeficiency virus (HIV) presented with systemic mycosis caused by Penicillium marneffei. Between August 1987 and August 1991, only five patients were observed who had P. marneffei infection but not HIV infection. The clinical presentation included fever, cough, and generalized papular skin lesions. For 11 of these 21 patients, the presumptive diagnosis of P. marneffei infection could be made by microscopic examination of Wright's-stained bone marrow aspirate and/or touch smears of skin specimens obtained by biopsy several days before the results of culture were available. Initial clinical response to treatment with either parenteral amphotericin B or oral itraconazole was favorable in most patients. Epidemiological and clinical evidence suggest that this systemic mycosis is caused by an important opportunistic pathogen and that it should be included in the differential diagnosis of AIDS, at least for countries in areas of endemicity, i.e., Southeast Asia and China.

Studies of human leprosy lesions in situ using suction-induced blisters: cell changes with IgM antibody to PGL-1 and interleukin-2 receptor in clinical subgroups of erythema nodosum leprosum.

To examine the immunopathogenesis of type 2 erythema nodosum leprosum (ENL) reactions in leprosy, we studied cellular and soluble immunologic components of skin lesions in 57 patients with reactions (19 acute ENL and 38 chronic ENL), 61 active patients without reactions, and 33 control patients whose leprosy had been treated and cured. Cells, IgM antibody to PGL-1 and Tac peptide levels were obtained from fluid aspirated from blisters induced by suction directly over representative skin lesions. During ENL reactions: a) the lesions in chronic ENL showed a decreased number of CD8+ (T-suppressor) cells and increased helper/suppressor ratio as compared to those in acute ENL and non-reactional leprosy; b) Tac peptide and IgM antibody to PGL-1 levels were elevated in the chronic ENL lesions; c) and systemic administration of corticosteroids appeared to cause a reduction in the intralesional CD4+ cell population and IgM antibody to PGL-1 but did not change CD8+ cell population and the levels of Tac peptide in the lesions. The elevated levels of Tac peptide were localized in the skin lesions while increased levels of IgM anti-PGL-1 seemed to be filtered from the peripheral blood. We conclude that spontaneous lymphocyte activation in situ, primarily of decreased CD8+ and relatively increased CD4+ cells, are important features of chronic, recurrent ENL reactions and may be an intermittent or cyclic phenomenon during the reaction. Understanding the mechanisms of these spontaneous changes in immunity in leprosy will enlarge our knowledge of reactions and of the underlying determinants of delayed type hypersensitivity and cell-mediated immunity in leprosy, which in turn will allow us to realize the potential for artificially manipulating these responses as proposed with vaccines or immunotherapy.

Cutaneous manifestations of disseminated Penicillium marneffei mycosis in five HIV-infected patients.

Five cases of disseminated Penicillium marneffei mycosis of Thai HIV patients, presented between November 1990 and April 1991, have been investigated. All patients were male and had bone marrow involvement. They showed mucocutaneous lesions with papules predominantly on the faces and upper extremities (5 cases), palatal papules (3 cases), and chronic genital ulcer (2 cases). P. marneffei was cultured, and the specific tissue form of the fungus was demonstrated histopathologically from skin and bone marrow specimens. In future, with increasing awareness of mucocutaneous lesions, disseminated penicilliosis in HIV-infected patients might be diagnosed sooner, thus leading to earlier therapy and better prognosis of the disease.