Assuntos
Fatores Imunológicos , Pênfigo , Rituximab , Pênfigo/tratamento farmacológico , Pênfigo/patologia , Humanos , Rituximab/administração & dosagem , Rituximab/uso terapêutico , Resultado do Tratamento , Fatores Imunológicos/administração & dosagem , Fatores Imunológicos/uso terapêutico , Feminino , Masculino , Pessoa de Meia-Idade , AdultoRESUMO
BACKGROUND: Among the autoimmune diseases causing erosive lesions and blisters on skin and mucous membranes is pemphigus. Within this is a rare subtype known as seborrheic pemphigus or Senear-usher syndrome which is characterized by broken blisters and crusts involving the seborrheic areas. CASE REPORT: A 40-year-old female patient, initially treated in a first level unit for a condition of 45 days of evolution, characterized by thick scabby lesions with an erythematous base, pruritic and painful, located in the center of the face, with posterior extension towards the abdomen, thorax, and extremities. Treatment consisted of prednisolone, with favorable evolution. The biopsy of the lesions with the diagnosis of seborrheic pemphigus. CONCLUSIONS: Senear-usher syndrome is a rare disease of multifactorial origin. Early diagnosis and adequate treatment are decisive factors to avoid the evolution and advanced forms of the disease.
ANTECEDENTES: Dentro de las enfermedades autoinmunes que provocan lesiones erosivas y ampollas en la piel y las mucosas se encuentra el pénfigo. Un subtipo raro de esta enfermedad es el pénfigo seborreico, o síndrome de Senar-Usher, caracterizado por ampollas rotas y costras que afectan las áreas corporales que secretan grasa. REPORTE DE CASO: Paciente femenina de 40 años, atendida inicialmente en una unidad de primer nivel por un cuadro de 45 días de evolución, caracterizado por lesiones costrosas gruesas de base eritematosa, pruriginosas y dolorosas, de localización centro-facial, con posterior extensión hacia el abdomen, tórax y extremidades. El tratamiento consistió en prednisolona, con evolución favorable. La biopsia de las lesiones confirmó el diagnóstico de pénfigo seborreico. CONCLUSIONES: El síndrome de Senear-Usher, o pénfigo seborreico, es una enfermedad excepcional, de origen multifactorial. El diagnóstico oportuno y tratamiento adecuado son factores decisivos para evitar la evolución de la enfermedad a formas avanzadas.
Assuntos
Pênfigo , Síndromes de Usher , Adulto , Humanos , Vesícula , Pênfigo/diagnóstico , Pênfigo/tratamento farmacológico , Pênfigo/patologia , Pele , FemininoRESUMO
BACKGROUND: Pemphigus vulgaris (PV) and pemphigus foliaceus (PF) are subtypes of pemphigus with distinct clinical and laboratory features. The transition between these two subtypes has rarely been reported previously. METHODS: The data of PV patients who exhibited clinical and immunoserological transition to PF during the follow-up period were retrospectively evaluated regarding their demographical, clinical, and laboratory characteristics. RESULTS: Among 453 patients diagnosed with PV, 13 (2.9%) patients exhibited clinical and immunoserological transition from PV to PF. The mean age of PV patients at the time of diagnosis was 39.8 ± 14.7 (19â62) years and 7 (53.8%) of them were female. These patients showed clinical and immunoserological transition from PV to PF after a period ranging from 4 months to 13 years (mean 36.2 ± 41 months). In addition to typical clinical features of PF, all patients had positive anti-desmoglein-1 and negative anti-desmoglein-3 antibody levels after the clinical transition had occurred without any mucosal involvement. During a mean 7.8 ± 5.8 (2â21) years of follow-up period after the transition from PV to PF, only one female patient had experienced a re-transition to PV characterized by a relapse of disease involving mucosal surfaces with positive anti-desmoglein-3 antibody levels following a 5-year period of remission period without treatment. STUDY LIMITATIONS: Single-center study with a retrospective study design. CONCLUSION: Our series is the largest group of patients reported to show the transition from PV to PF to date with a long follow-up period. The reason behind the disappearance of anti-desmoglein-3 antibodies and the pathogenesis of this phenomenon is not yet elucidated.
Assuntos
Pênfigo , Humanos , Feminino , Adulto , Pessoa de Meia-Idade , Masculino , Pênfigo/patologia , Estudos Retrospectivos , Autoanticorpos , Desmogleína 1 , Desmogleína 3RESUMO
BACKGROUND: A new variant of endemic pemphigus foliaceus is present in El Bagre, Colombia, and surrounding municipalities (El Bagre-EPF) that affects the skin and in some presentations affects other organs with autoantibodies directed against cell junctions. METHODS: We studied 200 El Bagre-EPF patient perilesional skin biopsies, as well as 200 skin biopsies from normal controls in the endemic area. RESULTS: We observed blister extrusions of sebaceous glands or entire pilosebaceous units via the isthmus in 23% of the patients and not in the controls. CONCLUSIONS: The extrusion of hair follicular unit contents is consistent with our previous pathologic findings of autoreactivity to these units, and their observed clinical decrease in patients affected by El Bagre-EPF.
Assuntos
Pênfigo , Autoanticorpos , Vesícula/epidemiologia , Colômbia/epidemiologia , Doenças Endêmicas , Humanos , Pênfigo/patologia , América do SulRESUMO
Background: Pemphigus vulgaris comprises a group of heterogeneous blistering autoimmune diseases of the skin and mucosa. Esophageal involvement within pemphigus vulgaris is rare with an uncertain prevalence that requires a detailed diagnostic and a therapeutic approach. Clinical case: 37-year-old female, with a history of treatment with Cox-2 inhibitors due to herniated disc. She is sent to the Gastroenterology Service for weight loss of approximately 5 kilos in a month, with the presence of dysphagia, odynophagia and retrosternal pain with poor tolerance to the oral route. Endoscopy was performed, which reported esophagitis dissecans superficialis (EDS), erythematous gastropathy of the antrum and normal duodenum. Findings were correlated with the diagnosis of pemphigus vulgaris with exclusive involvement of the esophagus. The evaluation did not identify lesions on the skin, oral cavity or other mucous membranes. A new endoscopy was performed as a control and it was found immunofluorescence of the esophageal biopsy reactive to IgG 2. Initial management was given with glucocorticoids, anti-inflammatories and immunosuppressants. Conclusions: The importance of the study of pemphigus lies not only in the high associated morbidity and mortality, but also in its intrinsic rarity and the complexity of its detection, given that patients usually take several months to have an accurate diagnosis and even more time to achieve therapeutic goals. It is a priority the dissemination of the study of pemphigus among health professionals involved in its detection.
Introducción: el pénfigo vulgar comprende un grupo de enfermedades heterogéneas autoinmunes ampollosas de la piel y las mucosas. La afectación esofágica en el pénfigo vulgar es rara, con una prevalencia incierta que requiere un abordaje diagnóstico y terapéutico detallado. Caso clínico: mujer de 37 años, con antecedentes de tratamiento con inhibidores de la Cox-2 debido a hernia discal. Se envió a Gastroenterología por pérdida de peso de aprox. 5 kg en un mes. La paciente tuvo presencia de disfagia, odinofagia y dolor retroesternal con pobre tolerancia a la vía oral. Se hizo endoscopía que reportó esofagitis disecante superficial y gastropatía eritematosa de antro; el duodeno estaba en estado normal. Los hallazgos se correlacionaron con el diagnóstico de pénfigo vulgar con afectación exclusiva a esófago. En la valoración no se identificaron lesiones en piel, cavidad oral u otras mucosas. Se hizo nueva endoscopía como control y se encontró inmunofluorescencia de biopsia esofágica reactiva a IgG 2. Se dio manejo inicial con glucocorticoides, antiinflamatorios e inmunosupresores. Conclusiones: la importancia del estudio del pénfigo radica no solo en la alta morbimortalidad asociada, sino en lo raro y complejo de su detección, pues los pacientes suelen tardar varios meses en tener un diagnóstico certero y aún más en conseguir las metas terapéuticas. Es prioritaria la difusión del estudio del pénfigo entre los profesionales de la salud involucrados en su detección.
Assuntos
Esofagite , Pênfigo , Adulto , Biópsia , Esofagite/diagnóstico , Feminino , Humanos , Pênfigo/diagnóstico , Pênfigo/patologia , Pele/patologiaRESUMO
Fogo selvagem (FS) is a blistering skin disease caused by pathogenic IgG4 autoantibodies to desmoglein 1 (DSG1). Preclinical FS and leishmaniasis are endemic to certain regions of Brazil and exhibit nonpathogenic anti-DSG1 antibodies. Recurring bites from Lutzomyia longipalpis, the sand fly vector of leishmaniasis, immunize individuals with L. longipalpis salivary antigens LJM17 and LJM11. We measured the antibody responses to LJM17, LJM11, and DSG1 in normal settlers and patients with FS from an endemic focus of FS and nonendemic control populations. We also immunized mice with these antigens and assessed the IgG response. Healthy individuals and patients with FS from endemic areas had significantly higher values of IgG4 anti-LJM17 antibodies than nonendemic controls (P < 0.001 for both). The levels of IgG anti-DSG1 and IgG4 anti-LJM17 and anti-LJM11 antibodies correlated positively in normal settlers and patients with FS. Mice immunized with recombinant LJM17 produced IgG1 antibodies (human IgG4 homolog) that strongly cross-reacted with recombinant DSG1; these IgG1 antibodies were inhibited by LJM17, LJM11, and DSG1 in a dose-dependent manner. However, they did not bind human or mouse epidermis by indirect immunofluorescence. Lastly, we identified short-sequence homologies of surface-exposed residues within the human DSG1 ectodomain and LJM17. Inoculation by LJM17 from L. longipalpis-elicited DSG1-cross-reactive IgG4 antibodies may lead to FS in genetically predisposed individuals.
Assuntos
Mordeduras e Picadas/imunologia , Desmogleína 1/imunologia , Proteínas de Insetos/imunologia , Pênfigo/imunologia , Psychodidae/imunologia , Animais , Autoanticorpos/imunologia , Autoantígenos/imunologia , Mordeduras e Picadas/epidemiologia , Mordeduras e Picadas/patologia , Brasil/epidemiologia , Reações Cruzadas , Modelos Animais de Doenças , Doenças Endêmicas , Epiderme/imunologia , Epiderme/patologia , Humanos , Insetos Vetores/imunologia , Insetos Vetores/parasitologia , Leishmaniose Cutânea/epidemiologia , Leishmaniose Cutânea/imunologia , Leishmaniose Cutânea/parasitologia , Camundongos , Pênfigo/epidemiologia , Pênfigo/patologia , Psychodidae/parasitologia , Proteínas Recombinantes/imunologia , Proteínas e Peptídeos Salivares/imunologiaRESUMO
The authors have successfully treated and monitored a case of paraneoplastic pemphigus in association with follicular dendritic cell sarcoma aggravated by hyaline-vascular Castleman's disease. The patient was a 56-year-old female who presented with recalcitrant erosive lichen planus of the oral cavity, tongue, and genital mucosa, along with polymorphous eruptions throughout her body. Histological examination of the cutaneous lesions, indirect immunofluorescence on rat bladder epithelium, and western blot of human keratinocyte proteins identified anti-epidermal antibodies in the patient's serum. Positron emission tomography and computed tomography scans found a mass in her retroperitoneal region. Pathology and immunohistochemistry investigation further corroborated the diagnosis of follicular dendritic cell sarcoma originated from hyaline-vascular Castleman's disease. Complete remission was achieved and the patient has been monitored for four years.