RESUMO
Childhood sarcoidosis is a rare granulomatous disorder that affects several systems including the lungs, skin, eyes, and bones. On histopathology, non-caseating granuloma is the classic feature. We present a case of late childhood sarcoidosis of the skin, lungs, and bones demonstrating granulomas with fibrinoid necrosis, making distinction from tuberculosis difficult.
Assuntos
Sarcoidose/patologia , Tuberculose/patologia , Adolescente , Diagnóstico Diferencial , Feminino , Proteínas de Choque Térmico/genética , Humanos , Mycobacterium tuberculosis/genética , Mycobacterium tuberculosis/isolamento & purificação , Peptidil Dipeptidase A/análise , Reação em Cadeia da Polimerase , Prednisona/administração & dosagem , Sarcoidose/tratamento farmacológicoRESUMO
Self-healing juvenile cutaneous mucinosis (SHJCM) is a rare disorder of unknown origin, which occurs in children in good health. It is characterized by the multiplication of transient cutaneous papules and nodules, mainly located on the head and periarticular areas that spontaneously resolve. Histological features of SHJCM have been well described; therefore, the diagnosis is usually made easily when papules are biopsied. We report a series of 3 new cases of SHJCM presenting mainly with nodular lesions. Histological examination of these nodules showed either lesions consistent with nodular or proliferative fasciitis or nonspecific panniculitis. Mucinous deposits were present but often inconspicuous, so could be disregarded. We wanted to emphasize this misleading presentation because a biopsy for histological examination is always mandatory in cases of proliferating nodules to rule out malignant tumors. Therefore, the diagnosis always requires discussion between pathologists and clinicians to rapidly reassure the parents and avoid inappropriate therapy.
Assuntos
Proliferação de Células , Erros de Diagnóstico/prevenção & controle , Mucinoses/patologia , Mucinas/análise , Neoplasias Cutâneas/patologia , Pele/patologia , Biomarcadores/análise , Biópsia , Pré-Escolar , Fasciite/etiologia , Fasciite/patologia , Humanos , Imuno-Histoquímica , Lactente , Masculino , Mucinoses/complicações , Mucinoses/metabolismo , Paniculite/etiologia , Paniculite/patologia , Valor Preditivo dos Testes , Remissão Espontânea , Pele/química , Fatores de TempoRESUMO
BACKGROUND: Angiolymphoid hyperplasia with eosinophilia (ALHE) is a benign but potentially disfiguring vascular lesion. It is usually characterized by dermal and subcutaneous nodules, primarily in the head and neck region. Spontaneous regression is common, but persistent or recurrent lesions may require treatment. Several treatments have been reported but surgery is the most efficient one. METHODS AND RESULTS: We report a 32-year-old man presenting with multiple nodules on the cheeks, preauricular region and the scalp and who received treatment with isotretinoin (0.5 mg/kg/day) for 1 year with complete resolution of one of his scalp nodules. The rest of the lesions remained stable and were treated with surgical excision without recurrence. CONCLUSION: Isotretinoin may play a role in the treatment of ALHE due to its antiangiogenic properties via a reduction of vascular endothelial growth factor (VEGF) production by keratinocytes.
Assuntos
Hiperplasia do Linfonodo Gigante/tratamento farmacológico , Fármacos Dermatológicos/uso terapêutico , Eosinofilia/tratamento farmacológico , Isotretinoína/uso terapêutico , Dermatopatias/tratamento farmacológico , Adulto , Hiperplasia do Linfonodo Gigante/complicações , Hiperplasia do Linfonodo Gigante/diagnóstico , Fármacos Dermatológicos/administração & dosagem , Relação Dose-Resposta a Droga , Eosinofilia/complicações , Eosinofilia/diagnóstico , Seguimentos , Humanos , Isotretinoína/administração & dosagem , Masculino , Dermatopatias/complicações , Dermatopatias/diagnósticoRESUMO
The influence of bone marrow biopsy (BMB) histology on prognosis and management of follicular lymphomas (FL) remains controversial. A total of 390 patients with grade 1 or 2 FL were prospectively included in the multicentric Groupe d'Etude des Lymphomes Folliculaires trial and their BMB reviewed in order (i) to quantify the ratio of lymphomatous foci (LFo) area to that of BMB size (LFo/BMB), (ii) to determine the BMB patterns for a practical grading of marrow infiltration, (iii) to assess the intra- and inter-observer reproducibility of this grading and (iv) to analyse this grading on event-free (EFS) and overall survival (OS), using univariate and multivariate analyses. A total of 267 patients (68%) had BMB involvement, with inter- and intra-observer reproducibility for classifying the patterns of involvement of 91 and 96%, respectively. Uni- and multivariate analyses demonstrated the adverse influence of (i) a ratio of LFo/BMB > or = 0.1, i.e. three or four nodules/medullary space or > or = 1 nodule + foci of diffuse involvement on EFS (P = 0.03) and (ii) two different histological patterns in the same BMB on EFS (P = 0.004) and OS (P = 0.001). This latter finding was only significant in patients with a high tumour burden and remained significant in multivariate analysis. These results indicate that BMB histology can predict survival of FL patients with a high tumour burden, and may help in defining their treatment.