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Reactive pustular eruptions (RPEs) can manifest in a variety of conditions, including pustular psoriasis (PP) and adult-onset immunodeficiency syndrome due to anti-interferon-γ autoantibody (AOID). These RPEs can be attributed to different causes, one of which is genetic factors. However, the genetic basis for pustular skin diseases remains poorly understood. In our study, we conducted whole-exome sequencing on a cohort of 17 AOID patients with pustular reactions (AOID-PR) and 24 PP patients. We found that 76% and 58% of the AOID-PR and PP patients, respectively, carried rare genetic variations within the filaggrin (FLG) gene family. A total of 12 out of 21 SNPs on FLG had previously received clinical classifications, with only p.Ser2706Ter classified as pathogenic. In contrast, none of the FLG3 SNPs identified in this study had prior clinical classifications. Overall, these variations had not been previously documented in cases of pustular disorders, and two of them were entirely novel discoveries. Immunohistochemical analysis of skin biopsies revealed that FLG variants like p.Ser860Trp, p.Gly3903Ter, p.Gly2440Glu, and p.Glu2133Asp caused reductions in FLG levels similar to the pathogenic FLG p.Ser2706Ter. These results highlight rare FLG variants as potential novel genetic risk factors contributing to pustule formation in both AOID and PP.
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Povo Asiático , Proteínas Filagrinas , Proteínas de Filamentos Intermediários , Polimorfismo de Nucleotídeo Único , Humanos , Proteínas de Filamentos Intermediários/genética , Feminino , Masculino , Povo Asiático/genética , Adulto , Pessoa de Meia-Idade , Sequenciamento do Exoma , Predisposição Genética para Doença , Psoríase/genética , Psoríase/patologia , Idoso , Interferon gama/genética , Interferon gama/metabolismo , Autoanticorpos/imunologia , Pele/patologia , Pele/metabolismoRESUMO
Pustular skin diseases, with pustular psoriasis (PP) being the prototype, are immune-mediated diseases characterized by the presence of multiple pustules, resulting from neutrophil accumulation in the layer of epidermis. Sterile skin pustular eruption, like PP, is also observed in 20-30% of patients with adult-onset immunodeficiency syndrome (AOID) and anti-interferon γ autoantibodies (IFN-γ), leading to challenges in classification and diagnosis. While the mechanism underlying this similar phenotype remains unknown, genetic factors in relation to the immune system are suspected of playing an important role. Here, the association between human leukocyte antigen (HLA) genes, which play essential roles in antigen presentation, contributing to immune response, and the presence of skin pustules in AOID and PP was revealed. HLA genotyping of 41 patients from multiple centers in Thailand who presented with multiple sterile skin pustules (17 AOID patients and 24 PP patients) was conducted using a next-generation-sequencing-based approach. In comparison to healthy controls, HLA-B*13:01 (OR = 3.825, 95%CI: 2.08-7.035), C*03:04 (OR = 3.665, 95%CI: 2.102-6.39), and DQB1*05:02 (OR = 2.134, 95%CI: 1.326-3.434) were significantly associated with the group of aforementioned conditions having sterile cutaneous pustules, suggesting a common genetic-related mechanism. We found that DPB1*05:01 (OR = 3.851, p = 0.008) and DRB1*15:02 (OR = 3.195, p = 0.033) have a significant association with pustular reaction in AOID patients, with PP patients used as a control. A variant in the DRB1 gene, rs17885482 (OR = 9.073, p = 0.005), was observed to be a risk factor for PP when using AOID patients who had pustular reactions as a control group. DPB1*05:01 and DRB1*15:02 alleles, as well as the rs17885482 variant in the DRB1 gene, were proposed as novel biomarkers to differentiate PP and AOID patients who first present with multiple sterile skin pustules without known documented underlying conditions.
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Psoríase , Dermatopatias Vesiculobolhosas , Adulto , Humanos , Antígenos de Histocompatibilidade Classe II , Antígenos HLA/genética , Psoríase/diagnóstico , Psoríase/genética , AutoanticorposRESUMO
Background: A dysregulated immune response has been implicated in Sweet syndrome (SS) pathogenesis; however, cytokine profiles across different conditions associated with SS - including adult-onset immunodeficiency (AOID) due to anti-interferon (IFN)-γ autoantibodies - remain unknown. Objective: To investigate alterations in inflammatory cytokines in skin lesions of distinct subtypes of SS. Methods: Skin biopsies were collected from 42 AOID- and 52 non-AOID-associated SS patients and 18 healthy controls. The comparative immunohistochemical study was conducted using monoclonal antibodies against interleukin (IL)-1ß, IL-6, IL-17, IFN-γ, and tumor necrosis factor-α on paraffin-embedded sections. The quantitative percentage positivity and intensity were calculated using computer-based image analysis. Results: The results showed stronger and more diffuse dermal immunoreactivity for IFN-γ and IL-17 in the AOID-associated (p < 0.001 and p < 0.001, respectively) and non-AOID-associated SS (p < 0.001 and p < 0.001, respectively) groups. However, no significant differences in the levels of these two cytokines were observed between the AOID- and non-AOID-associated SS groups. Increased expression of IFN-γ together with IL-17 was also noted in almost all subtypes among non-AOID-associated SS. Conclusions: These results demonstrate that IFN-γ and IL-17 are implicated in immunopathology of all SS subtypes, including AOID-associated SS, despite the presence of anti-IFN-γ autoantibodies.
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Citocinas , Síndrome de Sweet , Adulto , Humanos , Citocinas/metabolismo , Interleucina-17 , Autoanticorpos , Fator de Necrose Tumoral alfaRESUMO
Psoriasis is a chronic inflammatory skin disease, and its pathogenesis remains unclear. Although many studies have demonstrated the role of serum interleukin-31 (IL-31) in psoriasis, only one study has examined histopathological expression in lesional skin. This study aimed to investigate the expression of IL-31 in skin biopsy specimens of psoriasis patients compared to healthy subjects and identify its possible correlation to disease severity and itch intensity. Psoriasis patients and healthy volunteers were recruited. Four-millimeter punch biopsy was performed at the lesional skin of psoriasis patients and normal skin of healthy subjects. Expression of IL-31 was measured by immunohistochemistry. Baseline characteristics, disease activity, itch intensity, and related laboratory results were collected. Twenty-six biopsy specimens of psoriasis patients and 10 tissue samples of healthy subjects were evaluated. Epidermal and dermal psoriasis lesions had significantly higher IL-31 expression compared to the healthy skin (P < 0.001). However, there was no significant difference in lesional expression of IL-31 by disease severity or itch intensity. Increased IL-31 expression in the lesions of psoriasis patients suggests the involvement of IL-31 in the pathogenesis of psoriasis.
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Psoríase , Humanos , Epiderme/metabolismo , Interleucinas/metabolismo , Prurido , Psoríase/metabolismo , Pele/metabolismoRESUMO
BACKGROUND: Generalized pustular psoriasis (GPP; MIM 614204) is a rare and severe pustular autoinflammatory skin disease in which acute generalized erythema and scaling develop with numerous sterile pustules. GPP shares skin manifestations, especially pustular skin reaction, with adult-onset immunodeficiency (AOID) with anti-interferon-γ autoantibodies, an autoimmune disease. METHODS: Clinical examinations and whole-exome sequencing (WES) were performed on 32 patients with pustular psoriasis phenotypes and 21 patients with AOID with pustular skin reaction. Immunohistochemical and histopathological studies were performed. RESULTS: WES identified three Thai patients presenting with similar pustular phenotypes-two with a diagnosis of AOID and the other with GPP. A heterozygous missense variant chr18:g.61325778C>A NM_006919.2: c.438G>T; NP_008850.1: p.Lys146Asn; rs193238900 in SERPINB3 was identified in two patients: one with GPP and the other with AOID. The other patient who had AOID carried a heterozygous missense variant chr18:g.61323147T>C NM_006919.2: c.917A>G; NP_008850.1: p.Asp306Gly in SERPINB3. Immunohistochemical studies showed overexpression of SERPINA1 and SERPINB3, a hallmark of psoriatic skin lesions. CONCLUSIONS: Genetic variants in SERPINB3 are associated with GPP and AOID with pustular skin reaction. The skin of patients with GPP and AOID carrying SERPINB3 mutations showed overexpression of SERPINB3 and SERPINA1. Clinically and genetically, GPP and AOID appear to share pathogenetic mechanisms.
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Síndromes de Imunodeficiência , Doenças da Imunodeficiência Primária , Psoríase , Dermatopatias Vesiculobolhosas , Humanos , Interleucinas/genética , Psoríase/genética , Pele/patologia , Mutação , Dermatopatias Vesiculobolhosas/patologia , Doenças da Imunodeficiência Primária/patologiaRESUMO
Secukinumab demonstrated high efficacy and favorable safety profile in patients with moderate-to-severe plaque psoriasis (PsO) in clinical trials. However, understanding of patient characteristics and clinical outcomes in real world in Thailand is still limited. To describe patient characteristics, effectiveness and safety of secukinumab in Thai PsO patients. This retrospective study analyzed data from medical records of adult PsO patients who initiated secukinumab at 7 dermatology centers from September 2017 to April 2021. Study outcomes included patient characteristics and changes in Psoriasis Area and Severity Index (PASI) score from baseline at weeks 4 and 16 after secukinumab initiation. Adverse events were recorded. Subgroup analyses by adherence rate and completeness of loading dose were performed. Of 163 patients, the mean (SD) age was 44.0 (14.0) years. Most patients (84.7%) were previously treated with topical therapy while 62.0% and 21.5% of patients had received systemic and biologic therapy, respectively. The mean baseline PASI score was 15.4 (9.3). Overall, the mean PASI score improved by 58.0% at week 4 and 78.4% at week 16. Statistically significant differences in PASI approvement were revealed among subgroups of patients with different loading dose and adherence rate. Adverse effects were reported in 8.0% of patients. The characteristics of patients in this study were slightly different from clinical trials in terms of demographic and clinical characteristics, as well as PsO treatment. Secukinumab was effective and safe in Thai patients with PsO, especially among those with complete loading dose and a higher adherence rate.
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Anticorpos Monoclonais , Psoríase , Adulto , Humanos , Estudos Retrospectivos , Tailândia , Anticorpos Monoclonais/efeitos adversos , Índice de Gravidade de Doença , Resultado do Tratamento , Psoríase/diagnóstico , Psoríase/tratamento farmacológico , Psoríase/induzido quimicamenteRESUMO
BACKGROUND: Chronic spontaneous urticaria (CSU) is a common skin disease and has a significant impact on patients' quality of life. The aim of treatment is complete symptom control. AIM: To identify potential factors associated with antihistamine-refractory isolated CSU and to determine the factors that predict response to second-generation H1 antihistamines at dosages from one- to fourfold. METHODS: We conducted a retrospective cohort study, which included adult patients diagnosed with isolated CSU and had complete symptom control. Clinical and laboratory findings were compared between the patients who were responsive to second-generation H1 antihistamines (< fourfold) and those who were refractory to a fourfold dose. Clinical and laboratory data were compared by dosage in the antihistamine-responsive group. RESULTS: There were 182 isolated CSU patients who met the study criteria, of whom 150 (82.4%) were responsive to treatment with up to a fourfold dose of second-generation H1 antihistamines, while 32 (17.6%) were refractory. In univariate analysis, age at onset, body mass index, baseline Urticaria Activity Score-7 (UAS7), white blood cell count, total neutrophil count, neutrophil-lymphocyte ratio, platelet count, and new generation antihistamines were significantly higher in the antihistamine-refractory group. According to multivariate analysis, baseline UAS7 was the only independent factor associated with antihistamine-refractory isolated CSU (odds ratio 1.14, 95% CI 1.01-1.28, P = .03). In the antihistamine-responsive group, white blood cell count tended to predict response to antihistamine treatment (P < .001, 0.04, 0.34 between onefold and twofold, twofold and threefold, and threefold and fourfold, respectively). CONCLUSION: Baseline UAS7 was an independent factor associated with antihistamine-refractory isolated CSU.
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Urticária Crônica , Urticária , Adulto , Humanos , Urticária Crônica/tratamento farmacológico , Qualidade de Vida , Estudos Retrospectivos , Doença Crônica , Urticária/diagnóstico , Antagonistas dos Receptores Histamínicos H1/uso terapêutico , Antagonistas dos Receptores Histamínicos/uso terapêutico , Resultado do TratamentoRESUMO
Bullous systemic lupus erythematosus (BSLE) is an uncommon cutaneous presentation that occurs even less frequent in the pediatric population. A retrospective review was performed from January 2012 to December 2021 in all pediatric patients (aged <18 years) who fulfilled the diagnostic criteria for BSLE to evaluate the clinical characteristics, extracutaneous involvement, histopathologic features, immunofluorescence patterns, serological abnormalities, internal organ involvement, treatments, and outcomes. Among 1,415 patients with SLE, five patients were validated for the diagnosis of BSLE, accounting for 0.35%. The mean age at diagnosis was 12.2 years (standard deviation, 1.92). The clinical features of BSLE in the study population were generalized tense bullae and large extensive vesicles on the lips and perioral and mucosal areas. Pediatric BSLE in the study population revealed high SLE disease activity with multiple organ involvement. Hematologic abnormalities, serositis, and renal involvement were found in all patients, while polyarthritis (40%) and neurological abnormalities (40%) were less frequently observed. Systemic corticosteroids, intravenous immunoglobulin, immunosuppressants, antimalarials, and dapsone were prescribed in the study population. The cutaneous lesions subsided in all patients with a median clearance duration of 14 days (range, 5-56 days). BSLE in the pediatric population has auxiliary manifestations with high disease activity. Multiple organ involvement, especially hematologic abnormalities, serositis, and renal involvement, was frequently found in the study population. Although cutaneous lesions in BSLE subsided in all patients, involvement of other organs, especially renal impairment, required aggressive treatment, and long-term follow-up.
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Background: Prurigo nodularis (PN) is a chronic pruritic skin disease which can greatly impact patients' quality of life. Moreover, the pathogenesis remains unclear, making it a difficult-to-treat condition. Aims: To investigate the expression of interleukin-31 (IL-31) in serum and skin biopsy specimens of PN patients and healthy subjects and identify its possible correlation to disease severity and itch intensity. Methods: Patients with PN and healthy volunteers were recruited for the study. Expression levels of IL-31 were measured by enzyme-linked immunosorbent assay and immunohistochemistry. Baseline characteristics, disease activity, itch intensity, and related laboratory results were collected. Results: Forty-three PN patients and 31 healthy subjects participated in our study. The PN patients had significantly higher mean serum IL-31 levels than the healthy subjects (52.9 ± 18.2 versus 36.3 ± 10.7 pg/ml, p < 0.001). Epidermal and dermal PN lesions also exhibited significantly higher IL-31 expression compared with the healthy skin (p < 0.001 and p = 0.01, respectively). However, there was no significant difference in serum or lesional expression of IL-31 by disease severity or itch intensity. Conclusion: Increased IL-31 expression in serum and PN lesions suggests that IL-31 has a potential role in the pathogenesis of PN.
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BACKGROUND: Evidence demonstrates that parenteral administration of methotrexate (MTX) has a higher drug bioavailability than oral administration. This difference is even more pronounced for medium to high dosages. AIM: To compare the efficacy, safety, and tolerability of oral and subcutaneous (SC) MTX for treatment of psoriasis. METHODS: A randomized, comparative, single-blind, 32-week study was conducted. The clinical response was evaluated using the Psoriasis Area Severity Index (PASI) and patient global satisfaction was assessed using a visual analogue scale (VAS). RESULTS: In total, 77 completed the study: 38 in the SC and 39 in the oral MTX group. No significant between-group differences were found in the number attaining PASI improvement of 75% (PASI75), 90% (PASI90) and 100% (PASI100) at Weeks 16 and 32: PASI75 (P = 0.14 and P = 0.21, respectively), PASI90 (P = 0.23 and P = 0.18) and PASI100 (P = 0.62 and P = 0.22). According to the mean VAS, no significant differences between the groups were found at any time points except at Week 32 that the mean VAS was significantly higher in the SC group (P = 0.03). Adverse events were comparable in both groups. CONCLUSION: SC and oral administration of MTX had similar efficacies in improving the PASI score even at the highest tolerable dose; however, the SC MTX group had higher overall patient satisfaction than the oral MTX group. No difference in tolerability was found.
Assuntos
Metotrexato , Psoríase , Administração Oral , Humanos , Psoríase/induzido quimicamente , Psoríase/tratamento farmacológico , Índice de Gravidade de Doença , Método Simples-Cego , Resultado do TratamentoRESUMO
BACKGROUND: Chronic spontaneous urticaria (CSU) is a common pruritic skin condition, the pathogenesis of which remains unclear. Interleukin-31 (IL-31) is a major pruritogenic cytokine that plays a role in inducing pruritus in various skin diseases. AIM: To 1) compare serum IL-31 levels among CSU patients, psoriasis patients with pruritic symptoms, and healthy subjects, 2) examine the correlations between serum IL-31 levels and disease severity, and 3) compare IL-31 levels in patients with and without CSU-associated auto-antibodies. METHODS: Patients with CSU, psoriasis with pruritic symptoms, and healthy volunteers were recruited in the study. Serum IL-31 levels were measured with commercial kits. Baseline characteristics, urticaria activity score, psoriasis area severity index, pruritic intensity score, and related laboratory results were collected. RESULTS: Sixty-five CSU patients, 30 psoriasis patients who had pruritus, and 31 healthy subjects participated in our study. The CSU patients had significantly higher mean serum IL-31 levels than the psoriasis patients (252.4 ± 115.5 vs 121.4 ± 16.6 pg/mL, P < 0.001). Both CSU and psoriasis patients also had significantly higher mean serum IL-31 when compared with the healthy subjects. Serum IL-31 levels of CSU and psoriasis patients did not differ significantly according to disease or itching severity. Thyroid antibodies and antinuclear antibodies were positive in 22 (33.8%) and 28 (43.1%) CSU patients, respectively. The CSU patients with ANA titers ≥1:160 had significantly higher mean serum IL-31 levels than in those who were negative for ANA and those with titers of 1:80 (P < 0.003 and P < 0.008, respectively). CONCLUSION: Higher serum IL-31 levels were found in patients with CSU and psoriasis with pruritic symptoms. This suggests that IL-31 has a possible role in the pathogenesis of CSU and psoriasis with pruritic symptoms.
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Chronic pruritus of unknown origin (CPUO) is a refractory condition. The expression of Interleukin-31 (IL-31), a major pruritogenic cytokine, in CPUO patients has not been investigated. This study aimed to investigate the potential association of IL-31 with CPUO. This was a cross-sectional, analytical study. Patients diagnosed with CPUO and healthy subjects were included at a ratio of 1:2. Serum IL-31 levels were measured in both groups and compared. There were 10 CPUO and 20 healthy subjects who participated in this study. The median IL-31 level in the CPUO group was significantly higher than in the healthy group (127.3 vs 34.4 pg/mL; P < .001). The presence of CPUO was independently associated with IL-31 levels with a coefficient of 89.678 (P < .001). The serum IL-31 cutoff point for CPUO was 56.8 pg/mL, with an area under the receiver operating characteristic curve (ROC) of 100%. Chronic pruritus of unknown origin was significantly and independently associated with higher IL-31 levels. Further clinical trials of IL-31-related treatment may be justified in CPUO patients.
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Rapidly progressive interstitial lung disease (RP-ILD) and its distinctive cutaneous features are highly associated with the presence of anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody in patients with dermatomyositis (DM), leading to a poor prognosis. We describe the case of a 25-year-old man who developed progressive proximal muscle weakness with RP-ILD and had unusual cutaneous findings (cutaneous ulcerations and livedo reticularis) accompanied by classical cutaneous features (heliotrope rash, Gottron's papules, Gottron's sign, and flagellate erythema). Blood test was positive for anti-MDA5 antibody. He was treated with intravenous corticosteroids and immunoglobulin, but passed away due to respiratory failure within 1 month after admission. Our case highlights that the presence of cutaneous ulcerations and livedo reticularis, in addition to RP-ILD, are useful clinical clues that may aid in the detection of anti-MDA5 antibody, early initiation of combined immunosuppressants, and prognosis prediction in patients with classical DM.
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BACKGROUND: An increase in dosages up to fourfold of second generation antihistamines is recommended for recalcitrant chronic spontaneous urticaria (CSU). No regimen guidelines about dose de-escalation, however, are mentioned once the disease is controlled. OBJECTIVE: To demonstrate the treatment outcomes and dose reduction in desloratadine assessed using the urticarial activity score over 7 consecutive days. METHODS: Medical records of all patients with CSU treated with desloratadine were collected retrospectively during a period from January 2010 to December 2013. RESULTS: Sixty-seven (94.4%) patients had remission of the disease with variable doses of desloratadine. The patients who had CSU concomitant with antithyroid antibodies or high erythrocyte sedimentation rates had a greater tendency not to respond to the standard dose. Once the disease was completely controlled, 67 patients finished the treatment, but 63 (94%) patients had recurrent symptoms. Sixty-three patients took the same dose that induced the response for a further 4 weeks before stopping or reducing the dose, 41/63 (65.1%), however developed urticaria again. Forty-one patients took the same dose for a further 8 weeks; only 2/41 (4.9%) patients developed the rash again. The mean follow-up period after the disease was controlled was 7.5 months. CONCLUSION: Most patients need higher than the standard doses to obtain remission. Once the disease is completely controlled, however, maintenance of the same dosage for at least 8 weeks before dose reduction is suggested in order to avoid recurrence of the symptoms.
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Antagonistas não Sedativos dos Receptores H1 da Histamina/administração & dosagem , Loratadina/análogos & derivados , Urticária/tratamento farmacológico , Adulto , Doença Crônica , Feminino , Antagonistas não Sedativos dos Receptores H1 da Histamina/efeitos adversos , Humanos , Loratadina/administração & dosagem , Loratadina/efeitos adversos , Quimioterapia de Manutenção/métodos , Masculino , Pessoa de Meia-Idade , Recidiva , Indução de Remissão/métodos , Retratamento , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
Systemic scleroderma-also known as systemic sclerosis (SSc)-is a chronic systemic connective tissue disease characterized by collagen deposition in cutaneous and internal organs, leading to skin sclerosis and multiple organ fibrosis. The pathogenesis is complex and remains poorly understood. Treatment is based on organ involvement and requires a multidisciplinary approach. Skin sclerosis can cause disability, leading to decreasing quality of life. Various systemic antifibrotic therapies have been used; however, most have unsatisfactory results. Recently, phototherapy and in particular ultraviolet A (UVA) has been used to treat skin sclerosis in SSc patients with satisfactory results. The main mechanisms include lymphocyte apoptosis, cytokine alteration, inhibition of collagen synthesis and increased collagenase production, and neovascularization, leading to the breakdown of collagen fibrils resulting in skin softening or even healing digital ulcers. Most studies reported that psoralen plus UVA (PUVA) and UVA1 phototherapy improved clinical outcomes vis-à-vis skin sclerosis, joint mobility, ulcers, and histopathology. PUVA and UVA1 phototherapy therefore have potential as an alternative or adjunctive therapy for patients with SSc.
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Terapia PUVA/métodos , Escleroderma Sistêmico/tratamento farmacológico , Apoptose , Colágeno/metabolismo , Colagenases/metabolismo , Citocinas/metabolismo , Humanos , Linfócitos/metabolismo , Linfócitos/patologia , Escleroderma Sistêmico/metabolismo , Escleroderma Sistêmico/patologia , Pele/metabolismo , Pele/patologiaRESUMO
Sweet's syndrome (SS) is associated with various diseases including non-tuberculous mycobacterial infection (NTM). Recent reports have shown that SS associated with NTM is increasing. Clinical features of SS associated with NTM may be different from SS associated with other associated diseases. The aim of the present study was to compare clinical parameters and treatment outcomes of SS associated with NTM and other associated diseases. Patients from January 2004 to April 2014 diagnosed with SS were retrospectively enrolled. Clinical variables were compared between SS patients with and without NTM infection. There were 51 SS patients during the study period; 36 patients (70.59%) had NTM. Clinical variables between the NTM and other associated diseases were comparable: age, sex, and pattern and locations of skin lesions. Five laboratory factors were significantly different between the groups including white blood cell counts (NTM 25 800 vs 12 850 cells/mm(3) ), lymphocyte percentages (13.0% vs 18.7%), monocytes (3.0% vs 7.2%), blood urea nitrogen (BUN) (11.7 vs 8.1 mg/dL) and serum creatinine (Cr) (1.0 vs 0.7 mg/dL). The presence of markedly high white blood cell counts, a low percentage of mononuclear cells and high BUN/Cr levels in SS may be a clinical clue to recognize the association with NTM infections; particularly in dissemination.
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Infecções por Mycobacterium não Tuberculosas/sangue , Infecções por Mycobacterium não Tuberculosas/complicações , Síndrome de Sweet/diagnóstico , Síndrome de Sweet/etiologia , Adulto , Idoso , Contagem de Células Sanguíneas , Nitrogênio da Ureia Sanguínea , Creatinina/sangue , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Infecções por Mycobacterium não Tuberculosas/microbiologia , Complexo Mycobacterium avium/isolamento & purificação , Mycobacterium fortuitum/isolamento & purificação , Estudos Retrospectivos , Síndrome de Sweet/sangue , Resultado do TratamentoRESUMO
BACKGROUND: Lipodermatosclerosis (LDS) is a chronic fibrosing panniculitis associated with venous insufficiency. Although LDS is often a clinical diagnosis, it can be confused with other panniculitides. Microscopic examination is therefore essential to support the diagnosis in this condition. Histopathologic changes, however, have not been extensively defined. The purpose of this study was to characterize the histopathologic spectrum of this condition correlated with clinical manifestation. METHODS: A total of 25 cases were collected retrospectively, and the clinical information and histopathologic findings were reviewed. RESULTS: Of 25 patients, the female to male ratio was 4 : 1. The mean age was 54 years (range, 31-74 years). Clinical features were acute in eight (32%), subacute in 12 (48%), and chronic in five (20%). The microscopic study mostly demonstrated vascular stasis changes of varying degrees depending on the age of the lesion. Adipocyte necrosis with thickened septa, extravasation of erythrocytes, and lymphocytic infiltration were major findings in the early lesions. In the chronic lesion, lipomembranous fat necrosis with microcyst formation, vascular stasis changes in subcutaneous tissue, and septal fibrosis were predominant features. Iron deposition or hemosiderin extending to the subcutaneous layer was always seen in all specimens at the subacute and chronic stages. CONCLUSION: The diagnosis of LDS still needs clinicopathologic correlation. The constellation of findings including septal fibrosis, lipomembranous fat necrosis, prominent vascular changes of stasis, and erythrocytic extravasation can be used to define LDS histopathologically. Interestingly, iron deposition in the subcutaneous tissue is a useful finding for this chronic condition.
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Adipócitos/patologia , Tecido Adiposo/patologia , Dermatite/diagnóstico por imagem , Dermatite/patologia , Esclerodermia Localizada/diagnóstico por imagem , Esclerodermia Localizada/patologia , Doença Aguda , Adulto , Idoso , Doença Crônica , Cistos/patologia , Dermatite/metabolismo , Eritrócitos , Feminino , Hemossiderina/metabolismo , Humanos , Ferro/metabolismo , Masculino , Microscopia , Pessoa de Meia-Idade , Necrose/patologia , Estudos Retrospectivos , Esclerodermia Localizada/metabolismo , Dermatopatias Vasculares/etiologia , Dermatopatias Vasculares/patologiaRESUMO
Toxic epidermal necrolysis (TEN) is an uncommon severe cutaneous adverse reaction. Although controversies remain in the pathophysiology and management of this condition, improvements in survival and morbidity have been observed over the past decade. The aim of the present study was to demonstrate the clinical profiles of TEN in Thai patients and the treatment outcome with dexamethasone pulse therapy assessed by using the Severity of Illness Score for Toxic Epidermal Necrolysis (SCORTEN). Medical records of all patients with TEN were collected retrospectively from January 2002 to December 2012. Epidemiological features, etiologies, treatments and clinical outcomes were reviewed. Of 18 patients, the female to male ratio was 1:1 and the mean age was 49.7 years. Cephalosporins (27.8%), phenytoin (16.7%), carbamazepine, sulfonamide drugs and allopurinol (11.1% each) were implicated as leading causes of TEN. Hepatitis was the most frequent complication (77.8%). Pulsed high doses of dexamethasone 1-1.5 mg/kg per day for a short period were administrated in all cases. Two of the 18 patients receiving corticosteroids (SCORTEN 5 and 6) died. The mortality rate was 11% (2/18 patients), however, no patient receiving systemic corticosteroids died if the patients had less than 4 points on SCORTEN. The clinical features of Thai patients with TEN were similar to other reports. In conclusion, in addition to withdrawal of the suspected agent and intensive supportive care, the administration of short-term dexamethasone pulse therapy, particularly during the initial phase, may be beneficial in reducing the mortality rate.
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Corticosteroides/uso terapêutico , Síndrome de Stevens-Johnson/tratamento farmacológico , Corticosteroides/administração & dosagem , Adulto , Idoso , Idoso de 80 Anos ou mais , Dexametasona/administração & dosagem , Dexametasona/uso terapêutico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Resultado do Tratamento , Adulto JovemRESUMO
The objective of this study was to demonstrate the clinical profiles of Stevens-Johnson syndrome (SJS) in Thai patients, and to compare those clinical features between younger and older patients. Medical records of all patients with SJS who were admitted to Srinagarind Hospital Medical School, Khon Kaen, Thailand, from January 2002 to December 2014 were reviewed. Epidemiological features, etiologies, treatment and clinical outcomes were collected. There were 45 patients with SJS during the 10-year period. Females were the majority (57.8%) and the median age was 49 years. Hepatitis was the most frequent complication (67.5%). Phenytoin (15.6%), sulfonamide drugs (15.6%) and allopurinol (13.3%) were implicated as leading causes of SJS. Steroids were prescribed in 37 cases (82.2%). The mortality rate was 4.4%. Comparing older patients to younger patients, allopurinol appeared to be the main instigating drug to develop SJS with an odds ratio of 5.6 (95% confidence interval, 2.8-10.6). In conclusion, clinical features of Thai patients with SJS were similar to other reports. Allopurinol had the strongest association with SJS in older patients as compared to the younger ones.