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ABSTRACT: Leukemia cutis corresponds to skin infiltration by malignant hematopoietic cells. It is most commonly reported in acute myeloid leukemia, particularly in subtypes with a monocytic component. Its clinical manifestations are extremely variable, and histopathologic diagnosis of cutaneous leukemic infiltrates may be challenging. We report the first case of cutaneous, that is, extramedullary, aleukemic relapse of acute myeloid leukemia within an unusual chilblain-like eruption that imposed a challenging clinical and histopathologic diagnosis. Primary chilblains are uncommon in the elderly, and a systemic underlying cause should be thoroughly investigated. In patients presenting with atypical chilblains (ie, persistent chilblains developing even without exposure to cold temperatures and/or refractory to therapy) and with a history of hematologic disorders such as leukemias, histopathologic examination is crucial to identify leukemic or aleukemic phases of relapse of underlying leukemia and initiate timely treatment.
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Pérnio , Exantema , Leucemia Mieloide Aguda , Neoplasias Cutâneas , Idoso , Humanos , Pérnio/diagnóstico , Temperatura BaixaRESUMO
Myopericytoma is an uncommon benign neoplasm that arises from the perivascular myoid cells. It typically presents as a painless well-circumscribed cutaneous or soft-tissue nodule, most commonly on the extremities of adults. Histologically, it is characterized by spindle-shaped myoid-appearing cells with a concentric arrangement in vessel walls, that are immunoreactive to alpha-smooth muscle actin and often for h-caldesmon, but negative for other smooth muscle markers. Herein, we present an unusual case of a painful subungual myopericytoma presenting as a dark subungual discoloration.
Assuntos
Hemangiopericitoma , Miopericitoma , Doenças da Unha , Adulto , Hemangiopericitoma/patologia , Humanos , Miopericitoma/patologia , Doenças da Unha/diagnóstico , Doenças da Unha/patologia , Pericitos/patologia , Pele/patologiaRESUMO
Becker nevus, first described by Samuel William Becker in 1949, is a focal epidermal hypermelanotic disorder. It commonly presents as a unilateral hyperpigmented patch that is predominantly distributed on the upper trunk and proximal extremities and frequently associated with hypertrichosis. There have been few reports in the literature of Becker nevus with bilateral involvement; multiple Becker nevi is also unusual. Herein, we report a young man with two bilateral symmetrical giant Becker nevi, one on the trunk with extension to both arms and the second on the abdomen.
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Neoplasias Primárias Múltiplas/patologia , Nevo/patologia , Neoplasias Cutâneas/patologia , Abdome , Humanos , Masculino , Tronco , Adulto JovemRESUMO
Onychomatricoma is a rare benign tumor of the nail matrix, characterized by finger-like projections that invade the nail plate. The fingernails of Caucasian women are most commonly affected. Because this tumor can easily mimic other more prevalent ungual diseases, it is crucial to be acquainted with its characteristic clinical and histopathologic features. The authors present a case of a 40-year-old man with an onychomatrichoma in the fourth left toenail, which was initially misdiagnosed and treated as onychomycosis.
Assuntos
Doenças da Unha , Onicomicose , Neoplasias Cutâneas , Masculino , Humanos , Feminino , Adulto , Unhas/patologia , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/cirurgia , Neoplasias Cutâneas/patologia , Doenças da Unha/diagnóstico , Doenças da Unha/cirurgia , Doenças da Unha/patologia , Onicomicose/diagnóstico , Onicomicose/cirurgia , Onicomicose/patologia , Dedos/patologiaRESUMO
Background: Idiopathic inflammatory myopathies (IIM) are a rare heterogeneous group of diseases characterised by chronic skeletal muscle inflammation, but other organs are also frequently involved. IMM represent a diagnostic challenge and a multidisciplinary approach is important to ensure successful diagnosis and adequate follow-up of these patients. Objective: To describe the general functioning of our multidisciplinary myositis clinic, highlighting the benefits of multidisciplinary team management in patients with confirmed or suspected IIM and to characterise our clinical experience. Methods: Description of the organization of a dedicated multidisciplinary myositis outpatient clinic, supported by IMM specific electronic assessment tools and protocols based on our Portuguese Register - Reuma.pt. In addition, an overview of our activity between 2017 and 2022 is provided. Results: An IIM multidisciplinary care clinic, based on a close collaboration between Rheumatologists, Dermatologists and Physiatrist is detailed in this paper. One hundred and eighty-five patients were assessed in our myositis clinic; 138 (75%) of those were female, with a median age of 58 [45-70] years. At the last appointment, 130 patients had a confirmed IIM diagnosis, and the mean disease duration was 4 [2-6] years. The most frequent diagnosis was dermatomyositis (n = 34, 26.2%), followed by antisynthetase syndrome (n = 27, 20.8%) and clinically amyopathic/paucimyopathic dermatomyositis (n = 18, 13.8%). Twenty-four patients (18.5%) were on monotherapy and 94 (72.3%) were on combination therapy. Conclusion: A multidisciplinary approach is important to ensure the correct diagnosis and follow-up of these patients. A myositis clinic, with a standardised practice at a tertiary hospital level, contributes to a standardization of care and opens research opportunities.