RESUMO
ABSTRACT: We present the histopathology of 12 skin biopsies from 6 patients with vacuoles, enzyme E1, X-linked, autoinflammatory, somatic syndrome and review the literature. The age of these 6 men ranges from 62 to 83 years (median of 70 years). UBA1 mutation was documented in all 6 patients. Multiple organ systems were involved with constitutional symptoms noted in 4 of 6 patients (67%), cutaneous involvement in 6 of 6 patients (100%), hematologic abnormalities in 6 of 6 patients (100%), pulmonary involvement in 4 of 6 patients (67%), musculoskeletal abnormalities in 3 of 6 patients (50%), vascular thrombosis in 2 of 6 patients (33%), ocular involvement in 2 of 6 patients (33%), and gastrointestinal involvement in 5 of 6 patients (83%). Of the 6 presented patients, neutrophilic dermatosis was seen in 3 biopsies, histiocytoid neutrophilic dermatosis in 1 biopsy, neutrophilic dermatosis with vasculitis in 1 biopsy, neutrophilic and granulomatous dermatitis in 2 biopsies, septal panniculitis consistent with erythema nodosum in 2 biopsies, and nonspecific patterns in 3 biopsies. In summary, neutrophilic dermatosis, small-vessel vasculitis, and panniculitis are frequent histopathologic patterns noted in decreasing frequency in skin biopsies of the patients with vacuoles, enzyme E1, X-linked, autoinflammatory, somatic syndrome. However, the histopathologic findings can be diverse, nonspecific in some instances, and varied among different biopsies obtained from the same patient.
Assuntos
Vacúolos , Humanos , Masculino , Pessoa de Meia-Idade , Idoso , Biópsia , Idoso de 80 Anos ou mais , Vacúolos/patologia , Enzimas Ativadoras de Ubiquitina/genética , Pele/patologia , Mutação , Síndrome , Doenças Genéticas Ligadas ao Cromossomo X/patologia , Doenças Genéticas Ligadas ao Cromossomo X/genéticaAssuntos
Hospitalização , Cobertura do Seguro , Penfigoide Bolhoso , Humanos , Penfigoide Bolhoso/economia , Penfigoide Bolhoso/epidemiologia , Estudos Retrospectivos , Estudos Transversais , Hospitalização/estatística & dados numéricos , Feminino , Masculino , Idoso , Cobertura do Seguro/estatística & dados numéricos , Idoso de 80 Anos ou mais , Pessoa de Meia-IdadeRESUMO
Intravascular lymphomas are rare disease conditions that exhibit neoplastic lymphoid cells that are confined mainly to the lumens of small capillaries and medium-sized vessels. The majority of the intravascular lymphomas are of B-cell origin, but they can include NK/T-cell and CD30+ immunophenotypes. In the histologic differential diagnosis are benign proliferations such as intralymphatic histiocytosis and intravascular atypical CD30+ T-cell proliferation. In this review, we discuss the clinical, histopathologic, and molecular findings of intravascular B-cell lymphoma, intravascular NK/T-cell lymphoma, intralymphatic histiocytosis, and benign atypical intravascular CD30+ T-cell proliferation.
RESUMO
Child and adolescent psychiatry involves simultaneously balancing duties to various vulnerable parties. Balancing autonomy and protection for adolescents is complex; state laws governing these situations often add confusion. Common prescribing patterns in child psychiatry lack robust evidence, and utilization of stimulants, atypical antipsychotics, and polypharmacy has skyrocketed. Significant concerns about distributive justice arise from alarming patterns in psychiatric treatment of vulnerable populations, like those affected by poverty, racism, adverse childhood experiences, and certain legal statuses. Principles of justice and respect for persons support the need for safe, adequate, and appropriate psychiatric treatment, including psychosocial interventions and resources, for all children.