RESUMO
OBJECTIVE: The authors investigated the expression of IgG4 and IgG in cutaneous Rosai-Dorfman Disease (CRDD) to further improve the understanding of this disease. METHODS: The authors retrospectively reviewed the clinicopathological features of 23 CRDD patients. The authors diagnosed CRDD by the presence of emperipolesis and immunohistochemical (IHC) staining of histiocytes consisting of S-100(+)/CD68(+)/CD1a(-) cells. The expressions of IgG and IgG4 in cutaneous specimens were assessed by IHC (EnVision) and quantitatively calculated by a medical image analysis system. RESULTS: All 23 patients, including 14 males and 9 females, were confirmed to have CRDD. Their ages ranged from 17 to 68 years (mean 47.91 ± 14.16). The most frequently affected skin regions were the face, followed by the trunk, ears, neck, limbs, and genitals. In 16 of these cases, the disease presented as a single lesion. IHC staining of sections showed that IgG was positive (≥ 10 cells/High-Power Field [HPF]) in 22 cases, while IgG4 was positive (≥ 10 cells/HPF) in 18 cases. Moreover, the IgG4/IgG proportion ranged from 1.7% to 85.7% (mean 29.50 ± 24.67%, median 18.4%) in the 18 cases. STUDY LIMITATIONS: In the majority of studies, as well as in the current study, the design. RDD is a rare disease, so the sample size is small. In the next studies to come, the authors will expand the sample for multi-center verification and in-depth study. CONCLUSION: The positive rates of IgG4 and IgG and the IgG4/IgG ratio assessed through IHC staining may be important in understanding the pathogenesis of CRDD.
Assuntos
Histiocitose Sinusal , Dermatopatias , Masculino , Feminino , Humanos , Adolescente , Adulto Jovem , Adulto , Pessoa de Meia-Idade , Idoso , Histiocitose Sinusal/diagnóstico , Imunoglobulina G/metabolismo , Estudos Retrospectivos , Dermatopatias/patologia , Histiócitos , Proteínas S100RESUMO
The cutaneous form of Rosai-Dorfman disease is very rare in childhood. The clinical spectrum is highly variable and histopathological study with immunohistochemistry is essential for the diagnosis. We present the case of a 3-year-old boy with the diagnosis of cutaneous Rosai-Dorfman disease and review the pediatric cases published in the literature.
Assuntos
Histiocitose Sinusal , Dermatopatias , Masculino , Humanos , Criança , Pré-Escolar , Histiocitose Sinusal/diagnóstico , Histiocitose Sinusal/patologia , Dermatopatias/diagnóstico , Dermatopatias/patologia , Pele/patologia , Imuno-HistoquímicaAssuntos
Doenças Mamárias/diagnóstico , Mama/patologia , Histiocitose Sinusal , Biópsia Guiada por Imagem/métodos , Ultrassonografia Mamária/métodos , Neoplasias da Mama/diagnóstico , Diagnóstico Diferencial , Feminino , Histiócitos/patologia , Histiocitose Sinusal/diagnóstico , Histiocitose Sinusal/imunologia , Humanos , Imuno-Histoquímica , Pessoa de Meia-IdadeRESUMO
RESUMEN La enfermedad de Rosai-Dorfman (ERD) o histiocitosis sinusal con linfadenopatía masiva es una enfermedad infrecuente, de etiología desconocida caracterizada por linfadenopatías cervicales masivas bilaterales. El compromiso extraganglionar puede ocurrir en diferentes sitios, incluida la vía aérea, en la cual la localización nasosinusal es la más frecuente, pero puede comprometer también otros sitios de la vía aérea superior. Dentro de éstos, el compromiso laríngeo es muy poco frecuente. Se presenta el caso de una paciente de 82 años con antecedentes de ERD localizada en los ganglios linfáticos cervicales en remisión espontánea, que desarrolló posteriormente una obstrucción subaguda de las vías respiratorias. La tomografía computarizada y la nasofaringolaringoscopía mostraron lesiones subglóticas que obstruían severamente las vías respiratorias. Se realizó una traqueostomía de emergencia y biopsia de las lesiones, confirmando el diagnóstico de ERD extranodal.
ABSTRACT Rosai-Dorfman disease or sinus histiocytosis with massive lymphadenopathy is a rare disease of unknown etiology characterized by bilateral massive cervical lymphadenopathy. Extranodal involvement can occur in different sites including airway where nasosinusal involvement is common but this disease may occur in other sites of the upper airway. Laryngeal involvement is rare. We present the case of an 82-year-old female patient with a history of Rosai-Dorfman disease located in cervical lymph nodes in spontaneous remission that subsequently develops airway obstruction. Computed tomographic scan and nasopharyngolaryngoscopy showed subglottic lessons that severely obstructed the airway. Emergency tracheotomy and biopsy were performed, confirming the diagnosis of extranodal RDD. Patient was observed and tracheostomy was maintained with good tolerance.
Assuntos
Humanos , Feminino , Idoso de 80 Anos ou mais , Histiocitose Sinusal/diagnóstico , Doenças da Laringe/diagnóstico , Obstrução das Vias Respiratórias/cirurgia , Traqueostomia , Tomografia Computadorizada por Raios X , Histiocitose Sinusal/cirurgia , Doenças da Laringe/cirurgia , LaringoestenoseRESUMO
Sinus histiocytosis with massive lymphadenopathy, generally known by the name of Rosai-Dorfman disease is a rare benign condition principally affecting cervical lymph nodes. Concurrent extra-nodal disease frequently occurs, however, solitary extra-nodal disease involving the mandible is exceedingly rare with less than five reported cases in the English literature. We describe a case of primary involvement of the mandible in a 27-year-old female, and discuss the differential diagnosis of this disease with other histiocytic lesions.
Assuntos
Humanos , Feminino , Adulto , Histiocitose Sinusal/diagnóstico , Mandíbula/patologia , Diagnóstico Diferencial , Doença de Erdheim-Chester , Histiocitose de Células de Langerhans , Histiocitose Sinusal/complicações , Doenças RarasRESUMO
A 75-year-old male presented with generalized erythematous, scaly plaques and painless lymphadenopathy. Rosai-Dorfman disease was suspected based on clinical manifestations and confirmed by histopathologic and immune reactivity studies performed on the biopsy obtained from the left supraclavicular lymph node. The patient was also diagnosed with multiple myeloma according to urine electrophoresis, serum light chain assay, and bone marrow biopsy, which were initially performed for evaluation of anemia. This report highlights the dermatological manifestations of Rosai-Dorfman disease with generalized painless lymphadenopathy.
Assuntos
Histiocitose Sinusal/patologia , Mieloma Múltiplo/patologia , Idoso , Biópsia , Histiocitose Sinusal/diagnóstico , Humanos , Masculino , Mieloma Múltiplo/diagnóstico , Pele/patologiaRESUMO
Abstract: A 75-year-old male presented with generalized erythematous, scaly plaques and painless lymphadenopathy. Rosai-Dorfman disease was suspected based on clinical manifestations and confirmed by histopathologic and immune reactivity studies performed on the biopsy obtained from the left supraclavicular lymph node. The patient was also diagnosed with multiple myeloma according to urine electrophoresis, serum light chain assay, and bone marrow biopsy, which were initially performed for evaluation of anemia. This report highlights the dermatological manifestations of Rosai-Dorfman disease with generalized painless lymphadenopathy.