RESUMEN
Dengue virus (DENV) is a global health problem. Severe dengue can manifest with hemorrhage and signs of organ dysfunction, including the kidneys. The innate immune system is an important barrier against arbovirus infection and, specifically in dengue, the cytokines IL1ß and IL18 and caspase-1 activation make up a set of host immune strategies. Cell death mechanisms include pyroptosis, necroptosis and autophagy, each with peculiar markers: gasdermin, RIPK3/MLKL, LC3, respectively. In DENV infection, necrosis and apoptosis are involved and, when infecting monocytes and macrophages in vitro, DENV is capable of inducing pyroptosis. Our objective was to explore the presence of markers of necroptosis, pyroptosis and autophagy in renal lesions caused by DENV. MATERIAL AND METHODS: twenty specimens of lesions from patients who died due to DENV infection, from the pathology department of Hospital Guilherme Álvaro, Santos, SP, were subjected to histological and immunohistochemical studies. Histological sections were stained with hematoxylin-eosin to evaluate tissue changes or collected for research with antibodies: anti-DENV (Instituto Evandro Chagas-PA), RIPK3 (NBP2-45592), MLKL (ab184718), gasdermin D (#36425), LC3 (14600-AP), caspase 1 (#98033), IL1ß (AF201-NA) and IL18 (SC6178). Semi-quantitative analysis was performed on 20 glomeruli and evaluation on tubules and mononuclear cells. This study was approved by the ethics committee of the USP Faculty of Medicine. RESULTS: histological analysis demonstrated glomerular congestion, glomerulitis (medium to severe), acute kidney injury and hyalinization of the glomeruli. Viral antigens were visualized on mononuclear cells. LC3 (autophagy) expression ranged from moderate to intense (++/+++) in glomeruli, tubules and mononuclear cells. The expression of gasdermin (pyroptosis) was mild (+) in most cases in the glomeruli and moderate (++) in the tubules. RIPK3 and MLKL (necroptosis) mild in tubules and mononuclear cells (+). The expression of the cytokines IL1ß and IL18 and caspase 1 was moderate (++). Statistical analysis showed greater expression of LC3 over the others. CONCLUSIONS: Our results contribute to the understanding of the pathogenesis of renal involvement in severe dengue, considering the likely anti-viral mechanism of autophagy. To a lesser extent, pyroptosis is also present, corroborating previous data.
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Virus del Dengue , Riñón , Necroptosis , Piroptosis , Dengue Grave , Humanos , Riñón/patología , Riñón/virología , Virus del Dengue/patogenicidad , Dengue Grave/patología , Autofagia , Interleucina-1beta/metabolismo , Interleucina-18/metabolismo , Caspasa 1/metabolismo , Proteína Serina-Treonina Quinasas de Interacción con Receptores/metabolismo , Masculino , Apoptosis , Femenino , Adulto , Biomarcadores , Persona de Mediana Edad , Proteínas Quinasas/metabolismo , Proteínas de Unión a Fosfato/metabolismo , GasderminasAsunto(s)
Células Dendríticas , Eritema Multiforme , Subunidad alfa del Receptor de Interleucina-3 , Transducción de Señal , Receptor Toll-Like 7 , Receptor Toll-Like 9 , Humanos , Eritema Multiforme/patología , Subunidad alfa del Receptor de Interleucina-3/metabolismo , Células Dendríticas/metabolismo , Receptor Toll-Like 7/metabolismo , Receptor Toll-Like 9/metabolismo , Recurrencia , Femenino , Masculino , Adulto , Persona de Mediana EdadRESUMEN
BACKGROUND: Renal transplant recipients (RTRs) are at increased risk of developing skin cancer; however, the role of immunosuppression is not yet fully understood. In this study, we evaluated the immunohistochemical changes in the skin of RTRs under three different immunosuppression regimens: mTOR inhibitors (mTORi), sirolimus or everolimus, mycophenolic acid (MPA) precursors such as mycophenolate sodium or mofetil, or azathioprine (AZA). METHODS: We evaluated biopsies of sun-exposed and sun-protected skin for immunohistochemical quantification of B lymphocytes (CD20+ ), T lymphocytes (CD3+ , CD4+ , and CD8+ ), and Langerhans cells (LCs) (CD1a+ ) in 30 RTRs and 10 healthy controls. The RTRs were divided into three groups: mTORi (n = 10), MPA (n = 10), and AZA (n = 10). RESULTS: No differences were observed in the number of B lymphocytes. However, a significant decrease in the number of T lymphocytes and LCs was observed in both sun-protected and sun-exposed skin in the AZA and MPA groups, although to a lesser degree in the latter group. The skin of the mTORi group did not differ from that of the control group in terms of the number of B and T lymphocytes and LCs. CONCLUSIONS: Patients treated with mTORi exhibit preserved cellular elements related to cutaneous immune surveillance. The use of AZA induced a greater degree of skin immunosuppression than in the control group, as demonstrated by the decrease in T lymphocytes and LCs.
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Trasplante de Riñón , Células de Langerhans , Humanos , Células de Langerhans/patología , Trasplante de Riñón/efectos adversos , Inmunosupresores/efectos adversos , Ácido Micofenólico/uso terapéutico , Azatioprina/uso terapéutico , Subgrupos LinfocitariosRESUMEN
BACKGROUND: Atopic dermatitis (AD) is an itchy, chronic and inflammatory skin condition, with dysfunctional immune response and skin barrier defects. Reduction of filaggrin (FLG) and tight junctions (TJ) proteins, such as claudin-1 (CLDN-1), expression in cutaneous epithelial barrier is remarkable in AD pathogenesis. Ocular involvement occurs in approximately 40% of AD patients leading to changes in the structure of the conjunctiva. OBJECTIVES: We aimed to evaluate the expression of FLG and CLDN-1 in the ocular surface of adults with AD, analysing bulbar conjunctival cells collected by a novel non-invasive cellular imprint. METHODS: Bulbar conjunctival epithelial cells were collected by cellular imprint technique, and FLG and CLDN-1 expression were assessed by immunofluorescence (IF) and real-time polymerase chain reaction (RT-PCR). RESULTS: We detected increased expression of FLG and CLDN-1, as well as their transcript levels in AD patients compared with healthy controls (HC). There was a positive correlation between tear film break-up time (TBUT) and FLG expression. Fluorescein staining was inversely associated with FLG expression. CONCLUSIONS: Our results may reflect a reactive response of the ocular surface to AD-related ocular inflammation and associated dry eye disease. Further investigations focusing on the role of FLG and TJ expression in the ocular surface of AD patients may increment the understanding of the pathophysiology of extracutaneous AD and developing future targeted therapies.
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Dermatitis Atópica , Proteínas Filagrina , Claudina-1/genética , Dermatitis Atópica/genética , Humanos , Proteínas de Filamentos Intermediarios/genética , Proteínas de Filamentos Intermediarios/metabolismo , Mutación , Piel/metabolismoAsunto(s)
Foliculitis , Dermatosis del Cuero Cabelludo , Alopecia , Humanos , Inflamación , Cuero CabelludoRESUMEN
BACKGROUND: Pemphigus herpetiformis (PH) is a rare clinical subtype of pemphigus with the presence of urticarial plaques, severe pruritus, rare acantholysis and eosinophilic spongiosis. OBJECTIVES: The aim of this study was to investigate the influence of IL-31 and pro-inflammatory cytokines/chemokines in the pathogenesis of PH. METHODS: Twenty-five patients with PH and three groups: pemphigus foliaceus (PF = 14), pemphigus vulgaris (PV = 15) and healthy controls (HC = 20) were selected for this study. The groups were analysed by immunohistochemistry utilizing IL-31, IL-31RA, IL-4, IL-17 and TNF-α antibodies. Serum levels of IL-4, IL-13, TNF, CXCL8, CCL5 and CCL2 were evaluated by cytometric bead array. RESULTS: Analysis of IL-31 family of PH patients revealed the following findings: (i) Enhanced in situ expression of IL-31 in PH samples, compared to PF and to PV (epidermis); (ii) Cutaneous IL-31RA expression in PH samples was higher than in PF, PV and HC groups (epidermis and dermis); (iii) PF patients that evolved to PH showed significant increased IL-31RA epidermal expression during the PH phase. Profile of pro-inflammatory cytokines (IL-4, IL-17 and TNF-α) in PH patients' skin exhibited: (i) Enhanced IL-4 expression, when compared to patients with PF (epidermis and dermis) and with PV (epidermis); (ii) Augmented IL-17 expression than PF and PV patients (epidermis); (iii) Augmented expression of TNF-α when compared to PF at the epidermal level. Evaluation of circulating cytokines and chemokines showed higher levels of CXCL8 and CCL2 in PH sera compared to HC group. CONCLUSIONS: IL-31 and IL-31RA, cytokines related to pruritus, and pro-inflammatory chemokines (CXCL8 and CCL2) seem to exert a role in the pathogenesis of PH. These findings support future studies to clarify the role of IL-31 pathway as a potential therapeutic target for patients with PH.
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Enfermedades Autoinmunes , Pénfigo , Acantólisis , Quimiocina CCL2 , Citocinas , Humanos , Interleucina-13Asunto(s)
Alopecia , Dioxinas , Liquen Plano , Receptores de Hidrocarburo de Aril , Fibrosis , HumanosRESUMEN
BACKGROUND: Lichen planus (LP) is an inflammatory skin disease with unknown aetiology. Activation by pathogen-associated molecular patterns or environmental stimuli may activate some components of inflammasomes that contribute to the inflammatory process in LP lesions. AIM: To characterize the inflammasomes in skin lesions and peripheral blood mononuclear cells (PBMCs) of patients with LP under Toll-like receptor (TLR) activation. METHODS: In total, 15 patients with LP and 14 healthy controls (HCs) were enrolled in the study. Inflammasome expression in skin was evaluated by real-time PCR and immunohistochemistry, while ELISA was used to assess the production of interleukin (IL)-1ß by PBMCs under stimulation with TLR4 and TLR7/TLR8 agonists and adenosine triphosphate (ATP). RESULTS: Compared with the levels in HC samples, increased expression of the inflammasome AIM2 was verified in both epidermal and dermal sections of LP skin lesions, whereas NLRP1 and IL-ß expression levels were enhanced in the dermis. LP skin lesion samples exhibited higher AIM2 transcript levels, similar NLRP1 levels and lower pro-IL-1ß mRNA levels compared with HC samples. We verified that, compared with PBMCs from HC subjects, PBMCs from patients with LP produced similar amounts of IL-1ß after induction by TLR4 agonists but lower IL-1ß levels after induction by TLR7/TLR8 agonists, regardless of the addition of ATP. CONCLUSION: Alterations in innate immunity, such as inflammasome component expression in skin lesions and PBMCs, were observed in patients with LP. Further investigations of dysfunctional inflammasome activation and the chronic inflammatory status of LP are required.
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Inflamasomas/genética , Leucocitos Mononucleares/metabolismo , Liquen Plano/metabolismo , Enfermedades de la Piel/metabolismo , Proteínas Adaptadoras Transductoras de Señales , Adulto , Proteínas Reguladoras de la Apoptosis , Proteínas de Unión al ADN , Femenino , Humanos , Inmunidad Innata/inmunología , Interleucina-1beta/metabolismo , Liquen Plano/patología , Masculino , Persona de Mediana Edad , Proteínas NLR , ARN Mensajero/genética , Enfermedades de la Piel/patología , Receptor Toll-Like 7 , Receptor Toll-Like 8 , Receptores Toll-Like , Regulación hacia Arriba/genéticaRESUMEN
Mucosal lesions of paracoccidioidomycosis (PCM) are frequently described and clinically important. Macrophages are classified as M1 or M2. M1 are proinflammatory and M2 are related to chronicity. Dectin-1 recognizes ß-glucan and plays an important role against fungal cells. The objective was to verify the presence of M1, M2, and dectin-1 and a possible correlation with Th1/Th2 cytokines in mucosal PCM lesions. In sum, 33 biopsies of oral PCM were submitted to histological and immunohistochemistry analysis, and positive cells were quantified. Eleven biopsies were characterized by compact granulomas (G1), 12 with loose granulomas (G2), and 10 with both kind of granulomas (G3). pSTAT-1 was equally increased in the three groups. G1 was characterized by an increased number of CD163+ macrophages. G2 presented similar number of arginase 1, iNOS, and CD163 expressing cells. G3 presented an increased number of cells expressing arginase 1 and CD163 over iNOS. G1 and G3 presented high number of cells expressing interferon (IFN)-γ; interleukin (IL) 5 was increased in G2 and G3; the expression of IL10 was similar among the three groups, and the expression of tumor necrosis factor (TNF)-α was higher in G3. G1 correlates to Th1 cytokines and pSTAT-1 and G2 correlates to Th2 cytokines. G3 presents both kinds of cytokines. We could not associate the expression of arginase-1, CD163, iNOS, and dectin-1 with the pattern of cytokines or kind of granuloma.
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Citocinas/inmunología , Macrófagos/inmunología , Mucosa Bucal/inmunología , Paracoccidioidomicosis/inmunología , Biopsia , Citocinas/clasificación , Granuloma/inmunología , Granuloma/microbiología , Granuloma/patología , Humanos , Inmunohistoquímica , Lectinas Tipo C/genética , Lectinas Tipo C/inmunología , Macrófagos/clasificación , Boca/inmunología , Boca/microbiología , Boca/patología , Mucosa Bucal/microbiología , Mucosa Bucal/patología , Paracoccidioides/inmunología , Fenotipo , Piel/inmunología , Piel/microbiología , Piel/patología , Células TH1/inmunología , Células Th2/inmunologíaAsunto(s)
Antígenos HLA-G/metabolismo , Interleucina-10/metabolismo , Lentigo/patología , Melanoma/patología , Neoplasias Cutáneas/patología , Adulto , Anciano , Biopsia , Progresión de la Enfermedad , Femenino , Antígenos HLA-G/inmunología , Humanos , Interleucina-10/inmunología , Lentigo/inmunología , Masculino , Melanoma/inmunología , Persona de Mediana Edad , Pronóstico , Piel/inmunología , Piel/patología , Neoplasias Cutáneas/inmunologíaRESUMEN
BACKGROUND: Erythroderma is a clinical skin syndrome shared by patients with cutaneous disorders of distinct aetiologies as a result of the combined actions of chemokines, adhesion molecules, and cytokines, such as vascular endothelial growth factor (VEGF). OBJECTIVE: To evaluate the profile of serum levels of VEGF and soluble vascular endothelial growth factor receptor 1 (sVEGFR-1) in pemphigus foliaceus (PF) patients with erythroderma. METHODS: We conducted a retrospective study, which included (i) a chart review of all PF patients from the Autoimmune Blistering Clinic, University of Sao Paulo, Brazil, from January 1991 to December 2014, together with an evaluation of demographic variables, hospitalization duration and complications and (ii) analysis of the circulating VEGF and sVEGFR-1 levels in PF patients with erythroderma by ELISA. The controls included patients with pemphigus vulgaris or psoriasis. RESULTS: We observed higher serum VEGF levels in PF patients during erythroderma than during the non-erythrodermic phase. PF patients showed increased serum levels of sVEGFR-1 during the erythrodermic phase in comparison to controls. Interestingly, the sVEGFR-1 and antidesmoglein-1 levels were positively correlated during the non-erythrodermic period. CONCLUSION: Erythroderma, which represents one clinical form of PF, implies more severe outcomes. The circulating levels of VEGF, a potent endothelial activator, are increased in PF patients with erythroderma; this result suggests the contribution of the blood vessel endothelium to the pathogenesis of this clinical syndrome. Interestingly, our findings showed a positive correlation between the sVEGFR-1 and antidesmoglein-1 antibody levels, indicating a suppressive response to VEGF augmentation during the erythrodermic phase of PF.
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Dermatitis Exfoliativa/complicaciones , Pénfigo/sangre , Factor A de Crecimiento Endotelial Vascular/sangre , Adulto , Femenino , Humanos , Masculino , Persona de Mediana Edad , Pénfigo/complicacionesRESUMEN
Livedoid vasculopathy is a chronic disorder characterised by recurrent reticulated purpura on lower extremities, associated with painful purpuric or necrotic macules and ulcerations. Current knowledge indicates LV to be a thrombo-occlusive vasculopathy of cutaneous blood vessels; exact pathogenesis is yet to be understood. Elevated levels of lipoprotein(a) have been found in LV patients. To date, elevated plasma levels of lipoprotein(a) are considered an independent and causal genetic risk factor for the development of cardiovascular disease, as well as a relevant factor in hypercoagulable states. Because of its structural homology with plasminogen, Lp(a) might have important anti-fibrinolytic properties. Altered endothelial function and participation in immune and autoimmune processes, such as antiphospholipid syndrome, are also potential mechanisms of Lp(a) involvement in LV pathogenesis. Lp(a) is part of the wound healing process; the possibility of Lp(a) serum elevation to reflect an acute-phase reagent in LV scenario is also considered. The objective of this review is to examine the possible association of lipoprotein(a) with LV pathogenesis, based on its effects on thrombogenesis, fibrinolysis and autoimmunity.
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Lipoproteína(a)/fisiología , Trombofilia/fisiopatología , Enfermedades Vasculares/fisiopatología , Humanos , Modelos TeóricosRESUMEN
OBJECTIVE: Corticotropin-releasing hormone (CRH) and pro-opiomelanocortin (POMC) axis activation leads to the production of hormones, such as adrenocorticotrophic hormone (ACTH) and the α-melanocyte stimulating hormone (α-MSH). Data regarding the role of these hormones in systemic lupus erythematosus (SLE) are scarce. In the present study we aim to evaluate the participation of this axis in the cutaneous involvement of SLE. METHODS: Seventeen SLE patients were clinically evaluated, and biopsies from affected and unaffected skin of these patients were compared with 17 healthy control individuals. Immunohistochemical analyses for CRH, ACTH, α-MSH, and MC-1R were performed, and the serum levels of α-MSH, IL-1, IL-1ra, IL-6, IL-10, IL-12p70, IL-17, TNF-α, and IFN-γ were measured. RESULTS: The affected skin of the SLE patients exhibited higher CRH expression in the deep dermis compared to the skin of the controls (p = 0.024), whereas the tissue expression of ACTH, cortisol, α-MSH and its receptor MC-1R were comparable in SLE patients and controls. Higher serum levels of IFN-γ (p = 0.041), TNF-α (p = 0.001) and IL-6 (p = 0.049) were observed in SLE patients compared with controls, while α-MSH levels were similar in both groups. CONCLUSION: The novel finding of elevated CRH expression solely in the affected skin deep dermis supports the notion of a cutaneous local dysfunction of the CRH-POMC axis in the pathogenesis of cutaneous SLE lesions.
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Hormona Adrenocorticotrópica/análisis , Hormona Liberadora de Corticotropina/análisis , Lupus Eritematoso Sistémico/patología , Piel/patología , alfa-MSH/análisis , Adulto , Autoanticuerpos/sangre , Estudios de Casos y Controles , Citocinas/sangre , Femenino , Humanos , Hidrocortisona/sangre , Persona de Mediana EdadRESUMEN
BACKGROUND: Atopic dermatitis (AD) in adults and profile of skin barrier proteins and inflammatory cytokines. OBJECTIVE: Evaluation of the expression of skin barrier proteins such as filaggrin, claudins 1 and 4 and of circulating inflammatory cytokines (Th1/Th2/Th17) in adults with AD. METHODS: Thirty-three adult patients with AD diagnosed according to the Hanifin & Rajkacriteria, and 25 healthy controls were enrolled in the study. AD severity was measured by Eczema Area and Severity Index (EASI). Laboratory assays included immunohistochemistry analysis of skin barrier proteins, such as filaggrin, claudins 1 and 4 and interleukin-17 (IL-17) from skin samples and determination of circulating cytokine levels (IL-2, 4, 5, 6, 10, 17A, TNF and IFN-γ) by flow cytometry (Cytometric Bead Array). RESULTS: We observed a reduced expression of filaggrin and claudin 1 in lesional skin of AD patients, when compared to controls. There was an inverse correlation of filaggrin expression and disease severity. In addition, IL-17 expression was enhanced in AD patients. Similarly, higher levels of inflammatory cytokines (IL-2, 5, 6, 10, 17A and IFN-γ) were found in AD patients. CONCLUSION: Our data reinforce the role of an altered skin barrier in the pathogenesis of AD. Our results show not only reduced expression of filaggrin and claudin 1 in lesional atopic skin but also inverse correlation of filaggrin expression and disease severity. Moreover, elevation of in situ IL-17 and of circulating interleukin levels in AD emphasize the systemic, inflammatory profile of this defective skin barrier dermatosis.
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Dermatitis Atópica/metabolismo , Interleucinas/sangre , Fenómenos Fisiológicos de la Piel , Piel/química , Adolescente , Adulto , Anciano , Estudios de Casos y Controles , Claudina-1/análisis , Claudina-1/metabolismo , Claudina-4/análisis , Claudina-4/metabolismo , Femenino , Proteínas Filagrina , Humanos , Interferón gamma/sangre , Interleucina-17/metabolismo , Proteínas de Filamentos Intermediarios/análisis , Proteínas de Filamentos Intermediarios/metabolismo , Masculino , Persona de Mediana Edad , Índice de Severidad de la Enfermedad , Factor de Necrosis Tumoral alfa/sangre , Adulto JovenRESUMEN
The frequency of histoplasmosis among solid organ transplant (SOT) recipients appears to be low where there are only a few case series, mostly among renal and liver transplant recipients. Herein we report a case of a 44-year-old woman who underwent a living-related renal transplant 18 years prior to evaluation, developed a nodule after followed by ulceration upon her posterior right leg and a second one upon her left leg 3 months and 2 months before her hospitalisation, respectively. The biopsy of lesion revealed the presence of Histoplasma spp. Bone marrow aspiration was performed and also revealed the same organism. She had initially received itraconazole without improvement of lesions, while a new lesion appeared on her left arm. Healing of all lesions could be observed after 40 days of liposomal amphotericin B when she was submitted to skin grafts on the legs and a surgical treatment on the arms, and the myelosuppression improved simultaneously. Histoplasmosis seems to be very uncommon among patients who underwent to organ solid transplantation. Most cases occur within 12-18 months after transplantation, although unusual cases have been presented many years post-transplant. There are cases reported in the literature, occurring from 84 days to 18 years after organ transplantation, but without cutaneous involvement. Our patient developed lesions on limbs and myelosuppression after 18 years of chronic immunosuppression medication. This case suggests that besides cutaneous histoplasmosis is an uncommon infection following iatrogenic immunosuppression and even rarer over a long period after the transplantation. Clinicians who care SOT recipient patients must bear in mind histoplasmosis infection as differential diagnosis in any case of cutaneous injury with prolonged fever and try to use as many tools as possible to make the diagnosis, once this disease presents a good prognosis if it is diagnosed and treated promptly.
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Enfermedades de la Médula Ósea/diagnóstico , Enfermedades de la Médula Ósea/microbiología , Dermatomicosis/diagnóstico , Dermatomicosis/microbiología , Histoplasma/aislamiento & purificación , Histoplasmosis/diagnóstico , Histoplasmosis/microbiología , Adulto , Aloinjertos , Anfotericina B/uso terapéutico , Antifúngicos/uso terapéutico , Médula Ósea/microbiología , Médula Ósea/patología , Enfermedades de la Médula Ósea/patología , Enfermedades de la Médula Ósea/terapia , Dermatomicosis/patología , Dermatomicosis/terapia , Femenino , Histoplasmosis/patología , Histoplasmosis/terapia , Humanos , Huésped Inmunocomprometido , Trasplante de Riñón , Trasplante de Piel , Úlcera Cutánea/microbiología , Úlcera Cutánea/patología , Receptores de Trasplantes , Resultado del TratamientoRESUMEN
BACKGROUND: Few authors have been attempting between mast cells and dermal dendrocytes interactions on urticaria. OBJECTIVE: To describe the extruded mast cell granules and dermal dendrocytes in drug-induced acute urticaria. METHODS: Seven patients with drug-induced acute urticaria were enrolled in the study. We token skin biopsies of urticaria lesion and perilesional skin. The 14 fragments collected were processed to immunogold electron microscopy using single stains to tryptase and FXIIIa, besides double immunogold labeling with both. RESULTS: Some sections demonstrated mast cells in degranulation process, both in anaphylactic and piecemeal degranulation types. After double immunogold staining, 10 nm (FXIIIa) and 15 nm (Tryptase) gold particles were present together over the granules in mast cells indicating that tryptase and FXIIIa are each localized within the granules of these cells. Interestingly, we found a strong evidence of than the exocytosed mast cell granules contents both FXIIIa and tryptase immunolabeled are phagocytized by dermal dendrocytes. CONCLUSIONS: The current observations provide morphological evidence that the exocytosis-phagocytosis mechanisms of mast cell granules represents one pathophysiological example of mast cells-dermal dendrocytes interactions in urticaria.
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Comunicación Celular , Fagocitosis/fisiología , Urticaria/inducido químicamente , Urticaria/patología , Adulto , Gránulos Citoplasmáticos/patología , Dermis/citología , Dermis/patología , Factor XIIIa/metabolismo , Femenino , Humanos , Inmunohistoquímica , Mastocitos/citología , Mastocitos/patología , Microscopía Electrónica de Transmisión/métodos , Microscopía Inmunoelectrónica/métodos , Persona de Mediana Edad , MuestreoRESUMEN
Fibroblastic rheumatism (FR) was first described in 1980 by Chaouat et al., and there have been few cases reported to date. The cause remains unknown. We report the first Latin-American patient with FR, to our knowledge, who is also the patient with the most striking dermatological features described in the literature. The diagnosis was based on the presence of a number of typical features. Clinically, the patient presented skin nodules and polyarthropathy with flexion contractures of the fingers. The histological findings compressed fibroblastic proliferation, thickened collagen fibres, dermal fibrosis and a decreased number of elastic fibres. Immunoreactivity for beta-catenin, alpha-smooth muscle actin and the monoclonal antibody HHF-35 showed myofibroblastic differentiation. Treatment with prednisone slightly reduced the number of nodules but did not improve the rheumatological symptoms. This condition has shown a poor response to many treatments proposed by previous authors. Further study will be necessary to identify effective treatment.