Assuntos
Imunodeficiência de Variável Comum/diagnóstico , Dermatoses Faciais/patologia , Granuloma/patologia , Xantomatose/patologia , Idoso , Bochecha , Imunodeficiência de Variável Comum/complicações , Pálpebras , Dermatoses Faciais/complicações , Feminino , Granuloma/complicações , Humanos , Pescoço , Xantomatose/complicaçõesRESUMO
OBJECTIVES: To evaluate the toxic effects and maximum tolerated dose of topical carmustine [1,3-bis(2-chloroethyl)-1-nitrosourea] following intravenous O6-benzylguanine in the treatment of cutaneous T-cell lymphoma (CTCL), and to determine pharmacodynamics of O6-alkylguanine DNA alkyltransferase activity in treated CTCL lesions. DESIGN: Open-label, dose-escalation, phase I trial. SETTING: Dermatology outpatient clinic and clinical research unit at a university teaching hospital. PATIENTS: A total of 21 adult patients (11 male, 10 female)with early-stage (IA-IIA) refractory CTCL, mycosis fungoides type, treated with topical carmustine following intravenous O6-benzylguanine. INTERVENTION: Treatment once every 2 weeks with 120 mg/m(2) intravenous O6-benzylguanine followed 1 hour later by whole-body, low-dose topical carmustine starting at 10 mg, with 10-mg incremental dose-escalation in 3 patient cohorts. Cutaneous T-cell lymphoma lesional skin biopsy specimens were taken at baseline and 6 hours, 24 hours, and 1 week after the first O6-benzylguanine infusion for analysis of O6-alkylguanine-DNA alkyltransferase activity. MAIN OUTCOME MEASURES: Clinical response measured by physical examination and severity-weighted assessment tool measurements, safety data acquired by review of adverse events at study visits, and O6-alkylguanine-DNA alkyltransferase activity in treated lesion skin biopsy specimens. RESULTS: A minimal toxic effect was observed through the 40-mg carmustine dose level with 76% of adverse events being grade 1 based on the National Cancer Institute Common Terminology Criteria for Adverse Events. Mean baseline O6-alkylguanine-DNA alkyltransferase activity in CTCL lesions was 3 times greater than in normal controls and was diminished by a median of 100% at 6 and 24 hours following O6-benzylguanine with recovery at 1 week. Clinical disease reduction correlated positively with O6-alkylguanine-DNA alkyltransferase activity at 168 hours (P=.02) and inversely with area under the curve of O6-alkylguanine-DNA alkyltransferase over 1 week (P=.01). Twelve partial responses and 4 complete responses were observed (overall response, 76% [95% CI, 0.55-0.89]). Five patients discontinued therapy owing to adverse events with a possible, probable, or definite relationship to the study drug. CONCLUSION: O6-benzylguanine significantly depletes O6-alkylguanine-DNA alkyltransferase in CTCL lesions and in combination with topical carmustine is well tolerated and shows meaningful clinical responses in CTCL at markedly reduced total carmustine treatment doses.
Assuntos
Carmustina/administração & dosagem , Guanina/análogos & derivados , Micose Fungoide/tratamento farmacológico , Neoplasias Cutâneas/tratamento farmacológico , Administração Tópica , Adulto , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos Alquilantes/administração & dosagem , Biomarcadores Tumorais/metabolismo , Biópsia , Relação Dose-Resposta a Droga , Quimioterapia Combinada , Inibidores Enzimáticos/administração & dosagem , Feminino , Seguimentos , Guanina/administração & dosagem , Humanos , Infusões Intravenosas , Masculino , Pessoa de Meia-Idade , Micose Fungoide/enzimologia , Micose Fungoide/patologia , O(6)-Metilguanina-DNA Metiltransferase/antagonistas & inibidores , O(6)-Metilguanina-DNA Metiltransferase/metabolismo , Neoplasias Cutâneas/enzimologia , Neoplasias Cutâneas/patologia , Resultado do TratamentoRESUMO
BACKGROUND: Localized scleroderma or morphea is a connective tissue disorder characterized by fibrosis of the skin and subcutaneous tissue. Excessive accumulation of collagen underlies the fibrosis, yet the pathogenesis is unknown. A subset of localized scleroderma/morphea, juvenile localized scleroderma (JLS), affects children and adolescents. OBJECTIVES: The clinical and microscopic features of JLS have not been fully characterized. The goal is to better characterize the microscopic features of JLS. METHODS: We collected a distinctive data set of 35 children with JLS, 19 (54%) of whom presented with hypopigmented lesions, and performed a retrospective chart and pathology review. We had adequate tissue for immunostaining studies on 8 of these individuals. RESULTS: We found that: (1) CD34 and factor XIIIa immunostaining, reported previously in adult morphea and scleroderma, when used with clinical information, is valuable for confirming a diagnosis of JLS; and (2) presence of hypopigmented lesions in JLS correlates with immunostaining studies. Decreased numbers of MelanA(+) melanocytes were present at the dermoepidermal junction in lesional skin in two of 3 children with hypopigmented JLS and in two of 4 children with nonhypopigmented JLS. LIMITATIONS: The number of cases is small, a function of the small number of children who have biopsy specimens with material sufficient for multiple immunostaining procedures. CONCLUSIONS: These results provide a useful immunostaining method for confirmation of the diagnosis of JLS. They suggest a complex autoimmune phenotype in some children with JLS.
Assuntos
Epiderme/ultraestrutura , Hipopigmentação/patologia , Esclerodermia Localizada/patologia , Adolescente , Distribuição por Idade , Antígenos CD34/metabolismo , Biópsia por Agulha , Estudos de Casos e Controles , Criança , Pré-Escolar , Epiderme/patologia , Fator XIII/metabolismo , Feminino , Seguimentos , Humanos , Hipopigmentação/diagnóstico , Hipopigmentação/epidemiologia , Imuno-Histoquímica , Incidência , Masculino , Melanócitos/metabolismo , Microscopia Eletrônica , Valores de Referência , Estudos Retrospectivos , Medição de Risco , Esclerodermia Localizada/diagnóstico , Esclerodermia Localizada/epidemiologia , Escleroderma Sistêmico/diagnóstico , Escleroderma Sistêmico/epidemiologia , Escleroderma Sistêmico/patologia , Índice de Gravidade de Doença , Distribuição por SexoRESUMO
BACKGROUND: The statin medications for lowering of blood cholesterol can be associated with cutaneous lichenoid reactions but also a variety of other adverse cutaneous eruptions, including Stevens-Johnson syndrome, toxic epidermolytic necrolysis, porphyria cutanea tarda, linear IgA bullous dermatosis, and reaction patterns (lupus and dermatomyositis-like and pustular). Cutaneous eruptions ("eczema" in the product literature) owing to simvastatin are reported in approximately 1.5% individuals compared with placebo. OBJECTIVE: To correlate the clinical and microscopic features of an unusual vesiculobullous reaction to simvastatin. METHODS: Retrospective analysis of clinical information and skin biopsies. RESULTS: We present the case of a 70-year-old man with chronic vesiculobullous and pustular annular lesions on distal arms, legs, hands, and feet for 2 years. The eruption was recalcitrant to potent topical corticosteroids. Multiple biopsies at different times showed a spongiotic and lichenoid hypersensitivity reaction resembling contact dermatitis, purpuric drug eruption, and pustular folliculitis. The common themes in the histopathology were spongiosis with microvesiculation, focal lichenoid infiltrates, dermal hemorrhage, and chronic superficial inflammatory cell infiltrates with eosinophils. The eruption began when simvastatin was started, improved when it was stopped, recurred with rechallenge, and cleared when simvastatin was discontinued. CONCLUSIONS: Acral cutaneous vesiculobullous eruption is an uncommon adverse drug eruption due to simvastatin, one of many different patterns possible. A high level of suspicion for an unexplained cutaneous eruption in an older individual on statins is important to identification of the disorder and discontinuation of the offending medication.
Assuntos
Hipersensibilidade a Drogas/etiologia , Inibidores de Hidroximetilglutaril-CoA Redutases/efeitos adversos , Sinvastatina/efeitos adversos , Dermatopatias Vesiculobolhosas/induzido quimicamente , Idoso , Hipersensibilidade a Drogas/diagnóstico , Humanos , Masculino , Dermatopatias Vesiculobolhosas/patologiaRESUMO
Disseminated superficial actinic porokeratosis (DSAP) is the most common form of porokeratosis, occurring mainly in women on the extremities as atrophic patches rimmed by a ridge of keratin (the cornoid lamella that is diagnostic of porokeratosis histologically and is thought to be a clonal keratinocyte proliferation). DSAP can sometimes coexist with other forms of porokeratosis (Mibelli, linear porokeratosis, porokeratosis palmaris et plantaris disseminata, and punctate porokeratosis). Rare variants of linear porokeratosis are the hyperkeratotic and verrucous forms which usually occur on the buttocks or perianal area. We present clinical and histopathologic findings from biopsy specimens of a 73-year-old woman with DSAP on the distal extremities, linear/segmental porokeratosis on thighs, and verrucous porokeratosis on buttocks and mons pubis. The verrucous lesions had been present for 30+ years, the DSAP and linear lesions for 10+ years. Biopsy specimens from distal extremity showed classic features of DSAP with infrequent cornoid lamellae separated by atrophic epidermis. Biopsy specimens from the mons pubis and thigh showed epidermal hyperplasia with multiple, almost contiguous, broad cornoid lamellae. Coexisting variants of porokeratosis are rare and our conclusions are drawn from a few cases in the literature. The rare variants of porokeratosis are of interest because the clinical morphology correlates with the histopathology.
Assuntos
Epiderme/patologia , Genes Dominantes , Poroceratose , Idoso , Biópsia , Feminino , Humanos , Poroceratose/classificação , Poroceratose/genética , Poroceratose/patologiaRESUMO
Donor CD4+ T cells are thought to be essential for inducing delayed host tissue injury in chronic graft-versus-host disease (GVHD). However, the relative contributions of distinct effector CD4+ T cell subpopulations and the molecular pathways influencing their generation are not known. We investigated the role of the STAT3 pathway in a murine model of chronic sclerodermatous GVHD. This pathway integrates multiple signaling events during the differentiation of naive CD4+ T cells and impacts their homeostasis. We report that chimeras receiving an allograft containing STAT3-ablated donor CD4+ T cells do not develop classic clinical and pathological manifestations of alloimmune tissue injury. Analysis of chimeras showed that abrogation of STAT3 signaling reduced the in vivo expansion of donor-derived CD4+ T cells and their accumulation in GVHD target tissues without abolishing antihost alloreactivity. STAT3 ablation did not significantly affect Th1 differentiation while enhancing CD4+CD25+Foxp3+ T cell reconstitution through thymus-dependent and -independent pathways. Transient depletion of CD25+ T cells in chimeras receiving STAT3-deficient T cells resulted in delayed development of alloimmune gut and liver injury. This delayed de novo GVHD was associated with the emergence of donor hematopoietic stem cell-derived Th1 and Th17 cells. These results suggest that STAT3 signaling in graft CD4+ T cells links the alloimmune tissue injury of donor graft T cells and the emergence of donor hematopoietic stem cell-derived pathogenic effector cells and that both populations contribute, albeit in different ways, to the genesis of chronic GVHD after allogenic bone marrow transplantation in a murine model.
Assuntos
Transplante de Medula Óssea/imunologia , Linfócitos T CD4-Positivos/metabolismo , Doença Enxerto-Hospedeiro/etiologia , Fator de Transcrição STAT3/metabolismo , Escleroderma Sistêmico/etiologia , Animais , Transplante de Medula Óssea/efeitos adversos , Linfócitos T CD4-Positivos/imunologia , Doença Crônica , Modelos Animais de Doenças , Camundongos , Fator de Transcrição STAT3/imunologia , Transdução de Sinais/imunologia , Transplante HomólogoRESUMO
Pseudolymphomatous folliculitis is a rare entity. We present a 62-year-old man with a recurrent solitary nodule on his nose requiring multiple excisions. Microscopic examination of the excisions showed a dense lymphocytic infiltrate containing numerous histiocytes and S100+, CD1a+ dendritic cells that surrounded and infiltrated hypertrophic hair follicles. Diffuse sheets of CD3+ T cells and nodular clusters of CD20+ B cells were also seen. There was normal reactive pattern of follicular centers. Light chain restriction was not detected. T-cell receptor and immunoglobulin heavy chain gene rearrangements by polymerase chain reaction revealed negative findings. A diagnosis of pseudolymphomatous folliculitis was made based on the hypertrophic hair follicles, periadnexal S100+ and CD1a+ dendritic cells, and negative clonal gene rearrangement study findings. This case of recurrent pseudolymphomatous folliculitis is instructive because of the resemblance to cutaneous lymphomas and cutaneous lymphoid hyperplasias, and the need for correct diagnosis to avoid overtreatment of this indolent condition.
Assuntos
Foliculite/patologia , Linfoma Cutâneo de Células T/patologia , Neoplasias Cutâneas/patologia , Linfócitos B/patologia , Complexo CD3/análise , Diagnóstico Diferencial , Rearranjo Gênico , Humanos , Masculino , Pessoa de Meia-Idade , Proteínas de Neoplasias/análise , Neoplasias Nasais/patologia , Reação em Cadeia da Polimerase , Linfócitos T/patologiaRESUMO
Psoriasis is initiated and maintained through a multifaceted interplay between keratinocytes, blood vessels, gene expression, and the immune system. One previous psoriasis model demonstrated that overexpression of the angiopoietin receptor Tie2 in endothelial cells and keratinocytes led to the development of a psoriasiform phenotype; however, the etiological significance of overexpression in each cell type alone was unclear. We have now engineered two new mouse models whereby Tie2 expression is confined to either endothelial cells or keratinocytes. Both lines of mice have significant increases in dermal vasculature but only the KC-Tie2-overexpressing mice developed a cutaneous psoriasiform phenotype. These mice spontaneously developed characteristic hallmarks of human psoriasis, including extensive acanthosis, increases in dermal CD4(+) T cells, infiltrating epidermal CD8(+) T cells, dermal dendritic cells and macrophages, and increased expression of cytokines and chemokines associated with psoriasis, including interferon-gamma, tumor necrosis factor-alpha, and interleukins 1alpha, 6, 12, 22, 23, and 17. Host-defense molecules, cathelicidin, beta-defensin, and S100A8/A9, were also up-regulated in the hyperproliferative skin. All of the phenotypic traits were completely reversed without any scarring following repression of the transgene and were significantly improved following treatment with the anti-psoriasis systemic therapeutic, cyclosporin A. Therefore, confining Tie2 overexpression solely to keratinocytes results in a mouse model that meets the clinical, histological, immunophenotypic, biochemical, and pharmacological criteria required for an animal model of human psoriasis.
Assuntos
Modelos Animais de Doenças , Células Endoteliais/metabolismo , Queratinócitos/metabolismo , Psoríase/genética , Receptor TIE-2/genética , Animais , Western Blotting , Expressão Gênica , Perfilação da Expressão Gênica , Humanos , Camundongos , Camundongos Transgênicos , Neovascularização Patológica/genética , Neovascularização Patológica/metabolismo , Neovascularização Patológica/patologia , Análise de Sequência com Séries de Oligonucleotídeos , Fenótipo , Psoríase/metabolismo , Psoríase/patologia , Receptor TIE-2/metabolismo , Reação em Cadeia da Polimerase Via Transcriptase Reversa , Pele/irrigação sanguínea , Pele/metabolismo , Pele/patologia , Fator A de Crescimento do Endotélio Vascular/biossínteseRESUMO
BACKGROUND: We report a case of granulomatous periorificial dermatitis (GPD). A 13-year-old Jamaican boy presented with pink to flesh-colored perioral and periocular papules that erupted during a summer visit to Jamaica. The child was initially diagnosed with sarcoidosis and treated with oral corticosteroids, but the eruption recurred 2 years later. He was referred to Dermatology. Biopsy of one of the facial papules revealed a dense granulomatous infiltrate with surrounding prominent lymphocytes. A diagnosis of GPD was made. OBJECTIVE AND CONCLUSION: Also known as facial Afro-Caribbean childhood eruption (FACE), GPD occurs in prepubertal children. It can be confused with sarcoidosis, infection, and granulomatous rosacea but contains lymphocytes around the granulomas and lacks the systemic involvement seen in sarcoidosis. Cultures are invariably negative. The histologic features of GPD and granulomatous rosacea can be identical, and certain treatments may exacerbate the condition, highlighting the importance of clinical correlation. The correct diagnosis is important to minimize treatment as GPD is ultimately self-limited.
Assuntos
Dermatite/patologia , Granuloma/patologia , Rosácea/patologia , Dermatopatias Papuloescamosas/patologia , Pele/patologia , Adolescente , Antibacterianos/administração & dosagem , Fármacos Dermatológicos/administração & dosagem , Diagnóstico Diferencial , Eritromicina/administração & dosagem , Face , Granuloma/tratamento farmacológico , Humanos , Masculino , Dermatopatias Papuloescamosas/tratamento farmacológico , Tacrolimo/administração & dosagem , Tacrolimo/análogos & derivadosRESUMO
BACKGROUND: Plasma cells are normally found in bone marrow and the intestinal tract. They appear in the skin in malignant conditions, autoimmune diseases, infection, and idiopathic and poorly understood disorders such as primary nodular amyloidosis. It is uncommon to find collections of plasma cells in the skin in the absence of these conditions. OBSERVATIONS: We present 2 cases of cutaneous plasmacytosis, one in a white, female adolescent aged 15 years with an 11-year history of a solitary, asymptomatic, violaceous plaque on the left anterior tibia and the other in a white, male child aged 7 years with a 2-year history of a solitary erythematous plaque on the right anterior tibia. In both patients, infiltration of mature polyclonal plasma cells was confined to an area on the skin with papulonodules. There was no history of previous trauma, malignant conditions, autoimmune disease, or infection in either child. CONCLUSION: Although incipient or occult systemic disease cannot be definitively ruled out, the course of these 2 individuals suggests that isolated primary cutaneous plasmacytosis in children is a benign chronic process with no adverse sequelae.
Assuntos
Plasmócitos/patologia , Dermatopatias/patologia , Pele/patologia , Adolescente , Criança , Feminino , Humanos , Dermatoses da Perna/patologia , MasculinoRESUMO
Primary cicatricial or scarring alopecias (CA) are a group of inflammatory hair disorders of unknown pathogenesis characterized by the permanent destruction of the hair follicle. The current treatment options are ineffective in controlling disease progression largely because the molecular basis for CA is not understood. Microarray analysis of the lymphocytic CA, Lichen planopilaris (LPP), compared to normal scalp biopsies identified decreased expression of genes required for lipid metabolism and peroxisome biogenesis. Immunohistochemical analysis showed progressive loss of peroxisomes, proinflammatory lipid accumulation, and infiltration of inflammatory cells followed by destruction of the pilosebaceous unit. The expression of peroxisome proliferator-activated receptor (PPAR) gamma, a transcription factor that regulates these processes, is significantly decreased in LPP. Specific agonists of PPARgamma are effective in inducing peroxisomal and lipid metabolic gene expression in human keratinocytes. Finally, targeted deletion of PPARgamma in follicular stem cells in mice causes a skin and hair phenotype that emulates scarring alopecia. These studies suggest that PPARgamma is crucial for healthy pilosebaceous units and it is the loss of this function that triggers the pathogenesis of LPP. We propose that PPARgamma-targeted therapy may represent a new strategy in the treatment of these disorders.
Assuntos
Alopecia/genética , Cicatriz/genética , Deleção de Genes , Folículo Piloso/metabolismo , PPAR gama/genética , PPAR gama/metabolismo , Células-Tronco/metabolismo , Alopecia/metabolismo , Animais , Estudos de Casos e Controles , Cicatriz/metabolismo , Modelos Animais de Doenças , Humanos , Líquen Plano/genética , Líquen Plano/metabolismo , Metabolismo dos Lipídeos/fisiologia , Camundongos , Camundongos Knockout , Análise em Microsséries , Peroxissomos/metabolismoAssuntos
Anticorpos Monoclonais/efeitos adversos , Antirreumáticos/efeitos adversos , Artrite Reumatoide/tratamento farmacológico , Dermatite/etiologia , Toxidermias/etiologia , Adalimumab , Anticorpos Monoclonais Humanizados , Dermatite/patologia , Toxidermias/patologia , Epiderme/patologia , Feminino , Humanos , Pessoa de Meia-IdadeRESUMO
1,25-Dihydroxyvitamin D(3) (1,25(OH)(2)D(3)) is the biologically active ligand for the vitamin D receptor (VDR). VDR(-/-) mice have a hair follicle-cycling defect resulting in alopecia. However, mice lacking 25-hydroxyvitamin D(3) 1alpha-hydroxylase (CYP27B1(-/-)), and having no circulating 1,25(OH)(2)D(3), have normal follicular function. These mouse models indicate that VDR functions independently of 1,25(OH)(2)D(3) in regulating hair-follicle cycling. Here, we show that VDR(-/-) mice rapidly develop chemically induced skin tumors, whereas CYP27B1(-/-) and wild-type mice do not, indicating that VDR, and not the 1,25(OH)(2)D(3) ligand, is essential for protection against skin tumorigenesis. Because the majority of human skin cancer results from exposure to UV, the susceptibility of VDR(-/-) mice to this carcinogen was also evaluated. VDR(-/-) mice developed UV-induced tumors more rapidly and with greater penetrance than did VDR(+/+) mice. p53 protein levels were upregulated at similar rates in UV-treated keratinocytes of VDR(-/-) and VDR(+/+) mice. However, rates of thymine-dimer repair and UV-induced apoptosis were significantly lower in VDR(-/-) epidermis compared with the wild type epidermis. UV-induced epidermal thickening was also attenuated in VDR(-/-) skin, indicating that VDR plays a critical role in the repair and removal of severely damaged keratinocytes and adaptation of the skin to chronic UV exposure.
Assuntos
Neoplasias Induzidas por Radiação , Receptores de Calcitriol/deficiência , Neoplasias Cutâneas/etiologia , Neoplasias Cutâneas/metabolismo , Raios Ultravioleta , Adaptação Fisiológica , Animais , Apoptose , Suscetibilidade a Doenças , Epiderme/fisiopatologia , Epiderme/efeitos da radiação , Queratinócitos/patologia , Camundongos , Camundongos Knockout , Penetrância , Dímeros de Pirimidina/química , Dímeros de Pirimidina/metabolismo , Receptores de Calcitriol/metabolismo , Pele/patologia , Pele/fisiopatologia , Pele/efeitos da radiação , Neoplasias Cutâneas/patologia , Neoplasias Cutâneas/fisiopatologia , Vitamina D/análogos & derivados , Vitamina D/genéticaRESUMO
The prototypic autoimmune diseases involving skin (lupus, dermatomyositis) typically result in epithelial injury and autoantibodies to characteristic cellular antigens. Disease-specific autoantibodies are also found in scleroderma, but scleroderma is different from other cutaneous autoimmune diseases because epithelial injury does not occur. Multiple factors and combinations of factors (immune system, vascular and extracellular matrix abnormalities) are the most likely triggers in an individual with a genetic predisposition to scleroderma. These lead to increased synthesis of normal collagen in skin, lungs and gut in the systemic form of scleroderma, systemic sclerosis. The hypotheses for the pathophysiology of scleroderma are diverse and include abnormal immunologic processes such as cytokine and chemokine dysregulation, abnormal T cell signaling, B cell dysfunction, injury due to autoantibodies to endothelial cells, persistent wound healing condition due to dysregulation of matrix homeostasis, abnormalities in the fibrinolytic system, polymorphisms in critical molecules of the immune system and matrix homeostasis, and microchimerism due to fetal/maternal placental exchange of HLAcompatible cells. Systemic sclerosis/scleroderma is chronic and progressive. To date, no definitive therapy is effective for any of the scleroderma variants, although agents for the vascular dysfunction have some utility. Hematopoietic bone marrow or stem cell transplantation before significant tissue fibrosis has occurred may be the most effective treatment.
Assuntos
Doenças Autoimunes/genética , Doenças Autoimunes/imunologia , Escleroderma Sistêmico/genética , Escleroderma Sistêmico/imunologia , Animais , Doenças Autoimunes/patologia , Doenças Autoimunes/terapia , Linfócitos B/imunologia , Linfócitos B/patologia , Quimiocinas/genética , Quimiocinas/imunologia , Quimerismo , Doença Crônica , Células Endoteliais/imunologia , Células Endoteliais/patologia , Matriz Extracelular/genética , Matriz Extracelular/imunologia , Matriz Extracelular/patologia , Feminino , Transfusão Feto-Materna/genética , Transfusão Feto-Materna/imunologia , Transfusão Feto-Materna/patologia , Fibrinólise/genética , Fibrinólise/imunologia , Predisposição Genética para Doença , Transplante de Células-Tronco Hematopoéticas , Humanos , Masculino , Polimorfismo Genético/imunologia , Gravidez , Escleroderma Sistêmico/patologia , Escleroderma Sistêmico/terapia , Transdução de Sinais/genética , Transdução de Sinais/imunologia , Linfócitos T/imunologia , Linfócitos T/patologia , Cicatrização/genética , Cicatrização/imunologiaAssuntos
Equimose/patologia , Neoplasias Pulmonares/patologia , Neoplasias Pulmonares/secundário , Melanoma/patologia , Melanoma/secundário , Recidiva Local de Neoplasia/patologia , Neoplasias Primárias Múltiplas/patologia , Neoplasias Cutâneas/patologia , Idoso de 80 Anos ou mais , Antineoplásicos Alquilantes/uso terapêutico , Biópsia , Dacarbazina/análogos & derivados , Dacarbazina/uso terapêutico , Equimose/tratamento farmacológico , Feminino , Humanos , Neoplasias Pulmonares/tratamento farmacológico , Melanoma/tratamento farmacológico , Recidiva Local de Neoplasia/tratamento farmacológico , Neoplasias Primárias Múltiplas/tratamento farmacológico , Pele/patologia , Neoplasias Cutâneas/tratamento farmacológico , Temozolomida , Resultado do TratamentoRESUMO
Erythema nodosum is an inflammatory reaction of the skin characterized by tender erythematous patches or nodules, usually located on the lower extremities. This report illustrates an association of erythema nodosum with a rare malignancy and an uncommon infectious agent in humans. There are many diseases associated with erythema nodosum; we propose that hairy cell leukemia and group C streptococcus be considered among this list.
Assuntos
Eritema Nodoso/patologia , Leucemia de Células Pilosas/patologia , Paniculite/microbiologia , Paniculite/patologia , Neoplasias Cutâneas/patologia , Infecções Estreptocócicas/diagnóstico , Streptococcus/classificação , Adulto , Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Biópsia por Agulha , Eritema Nodoso/complicações , Eritema Nodoso/diagnóstico , Seguimentos , Humanos , Imuno-Histoquímica , Dermatoses da Perna/complicações , Dermatoses da Perna/diagnóstico , Dermatoses da Perna/patologia , Leucemia de Células Pilosas/complicações , Leucemia de Células Pilosas/tratamento farmacológico , Masculino , Índice de Gravidade de Doença , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/tratamento farmacológico , Infecções Estreptocócicas/complicações , Streptococcus/isolamento & purificação , Resultado do TratamentoRESUMO
We present a rare case of pagetoid reticulosis arising in a 5-year-old white boy. He had a history of a large chronic erythematous, scaly patch on his left buttock that had shown intermittent partial response to a topical antifungal medication. A punch biopsy specimen revealed dramatic epidermal hyperplasia, with parakeratosis and prominent exocytosis of single and clustered mononuclear cells (Pautrier's microabscesses) into the epidermis. Some of these exhibited hyperchromatic nuclei with irregular contours. They stained prominently for CD3, CD4, and CD8, with a predominance of CD8(+) cells. T-cell receptor gene rearrangement by polymerase chain reaction was negative for a clonal process on a second biopsy specimen that was nondiagnostic on routine sections. Pagetoid reticulosis is an indolent, unilesional variant of mycosis fungoides, in which the atypical T cells may express a CD4(-)/CD8(+) phenotype. This is in contrast to primary cutaneous epidermotropic CD8(+) cytotoxic T-cell lymphoma, which is often very aggressive with a poor outcome. Pagetoid reticulosis is exceedingly rare in children and adolescents. Two features predict a benign course in this 5-year-old child: the unilesional clinical presentation and the CD8 predominance of the epidermal lymphocytes.
Assuntos
Micose Fungoide/patologia , Neoplasias Cutâneas/patologia , Antígenos CD8/análise , Pré-Escolar , Elétrons , Humanos , Masculino , Micose Fungoide/radioterapia , Neoplasias Cutâneas/radioterapiaRESUMO
BACKGROUND: Sarcoidosis is a systemic disease that may present as tattoo granulomas. OBJECTIVE: A patient with systemic sarcoidosis who developed a granulomatous reaction within a tattoo is presented to stimulate interest in this unusual phenomenon. METHODS AND RESULTS: A patient with a 6-year history of pulmonary sarcoidosis developed sarcoidal granulomas restricted to one pigment of a tattoo. Previous reports of sarcoidal granulomas within tattoos are reviewed, and information about the pathogenesis of this process is explored. CONCLUSION: Sarcoid granulomas may develop in tattoos as an isolated local reaction or as the presenting sign of systemic sarcoidosis. The reaction itself may provide insight into further understanding the pathogenesis of sarcoidosis.
Assuntos
Sarcoidose/diagnóstico , Sarcoidose/etiologia , Dermatopatias/diagnóstico , Dermatopatias/etiologia , Tatuagem/efeitos adversos , Abdome , Adulto , Corantes/efeitos adversos , Diagnóstico Diferencial , Feminino , Humanos , Sarcoidose/patologia , Dermatopatias/patologiaRESUMO
Patients with anhidrotic ectodermal dysplasia and immunodeficiency (EDA-ID) have mutations in the gene on the X chromosome encoding nuclear factor kappaB (NF-kappaB) essential modulator (NEMO), resulting in conical teeth, sparse hair, anhidrosis or hypohydrosis, and recurrent bacterial infections. The same gene is mutated in incontinentia pigmenti (IP), and mutations that do not completely abolish NF-kappaB activity allow survival of male fetuses. We present a case of a 1-year-old boy with a history of EDA-ID who was evaluated for an eruption that intermittently affected his scalp, upper back, cheeks, legs, and arms. A biopsy specimen taken from the back showed the presence of compact dyskeratotic cells with fragmented nuclei and numerous apoptotic keratinocytes scattered throughout the spinous and granular layer. The diagnosis of EDA-ID with IP was made. This case illustrates the complexity and overlapping features of the genodermatoses involving signaling pathways of the cell.