Asunto(s)

RESUMEN

INTRODUCTION: Lymphomatoid papulosis (LyP) is a CD30+ T-cell lymphoproliferative disorder (LPD) presenting as a recurrent eruption of papules and nodules which resolve spontaneously. CD30+ LPD prevalence in African American (AA)/Black patients is lower compared to White patients. CD30+ LPD has been recently reported to have worse outcomes in AA patients compared to White patients. METHODS: A retrospective chart review identified eight AA patients with LyP. We describe our experience with these eight patients and review the literature on similar cases. RESULTS: In half of the eight included patients, lesions occurred 1-4 years before they were diagnosed. In six patients (75%), resolution of the lesions resulted in hyperpigmented macules and scars. Five patients (63%) had also mycosis fungoides. Most of the patients who were followed (4/7, 57%) did not have complete resolution at their last visit, despite different treatment approaches. Discussion: Our results highlight that although LyP has an indolent course in AA/Black patients, residual hyperpigmentation and scars frequently occur, highlighting the need for better treatments of this lymphoproliferative disorder in this specific population. J Drugs Dermatol. 2020;19(1):89-91. doi:10.36849/JDD.2020.4602

Asunto(s)

Asunto(s)

Asunto(s)

Asunto(s)

Asunto(s)

RESUMEN

Rare associations of immunological disorders can often tell more than mice and rats about the pathogenesis of immunologically mediated human kidney disease. Cases of glomerular disease with thyroiditis and Graves' disease and of minimal change disease with lymphoepithelioma-like thymic carcinoma and lymphomatoid papulosis were recently reported in Pediatric Nephrology. These rare associations can contribute to the unraveling of the pathogenesis of membranous nephropathy (MN) and minimal change disease (MCD) and lead to the testing of novel research hypotheses. In MN, the target antigen may be thyroglobulin or another thyroid-released antigen that becomes planted in the glomerulus, but other scenarios can be envisaged, including epitope spreading, polyreactivity of pathogenic antibodies, and dysregulation of T regulatory cells, leading to the production of a variety of auto-antibodies with different specificities [immune dysregulation, polyendocrinopathy, enteropathy, X-linked (IPEX syndrome)]. The occurrence of MCD with hemopathies supports the role of T cells in the pathogenesis of proteinuria, although the characteristics of those T cells remain to be established and the glomerular permeability factor(s) identified.

Asunto(s)

RESUMEN

Thyroid hormones play an important role in the growth of the kidney and maintenance of its functions. Prolonged hypothyroidism is known to be accompanied by changes in renal morphology such as thickening of the glomerular and tubular basement membranes as well as increased mesangial matrix. Increased transcapillary leakage of plasma proteins leading to proteinuria and generalized edema is also a known complication of hypothyroidism. In particular, autoimmune thyroiditis is associated with proteinuria. Most previous reports of autoimmune thyroiditis with nephrotic syndrome have demonstrated mixed pathological morphology marked by predominant membranous glomerulopathy. Here we present a patient whose initial presentation with profound hypothyroidism and autoimmune thyroiditis was dominated by nephrotic syndrome secondary to type 1 membranoproliferative glomerulonephritis (MPGN). The association of MPGN and autoimmune thyroiditis is very rare.

Asunto(s)

Asunto(s)

RESUMEN

BACKGROUND: Histologically, diffuse dermal infiltrates of large atypical lymphocytes can be seen in lesions as indolent as type C lymphomatoid papulosis (LyP) to ones as aggressive as NK/T-cell lymphoma. While lesions of lymphomatoid papulosis are definitionally positive for CD30, their ability to express CD56 has not been formally studied. The objective of the current study was to determine whether or not the large atypical cells of LyP express the natural killer cell marker, CD56. METHODS: Biopsies from 18 patients with LyP were studied with monoclonal antibodies to CD30, CD56, CD8, and TIA-1. These included four type C LyP lesions. Clinical information was obtained by chart review and included extent of LyP lesions, presence/absence of disease at follow-up, and any associated hematologic malignancies,. RESULTS: None of the biopsies exhibited CD56 positivity within the large atypical cells of LyP. While some biopsies demonstrated CD56-positive, small, presumably reactive, lymphocytes within the infiltrate, their presence did not correlate with extent of disease, persistence of disease, or propensity for an associated non-LyP hematologic malignancy. CONCLUSIONS: The large atypical cells of types A and C LyP do not exhibit positivity for CD56, and thus a panel of antibodies that includes CD30 and CD56 can readily distinguish between the benign end of the spectrum of CD30-positive lymphoproliferations and aggressive NK/T-cell lymphoma.

Asunto(s)

Asunto(s)

RESUMEN

Mucho antes que los hongos fueran considerados responsables de causar enfermedades, Alibert, en 1806, describió un desorden severo en el cual aparecían en la piel grandes tumores necróticos parecidos a los hongos. A causa de este parecido él denominó a la enfermedad Micosis Fungoide. Su alumno Bazin, en 1876, describió las tres fases evolutivas de la enfermedad y en 1938 Sézary and Bouvrain descubrió lo que más tarde fue reconocido como una variante eritrodérmica leucémica llamada "Síndrome de Sézary". Rappaport trabajando con Thomas, demostró que las Micosis Fungoide eran un Linfoma Cutáneo especificado, con aspectos distintivos histopatológicos extracutáneos similares a los hallados en la piel; Long and Mihm, casi al mismo tiempo, llegaron a conclusiones similares. Luego de esto, las Micosis Fungoides llegaron a ser el foco de intenso interés de oncólogos y dermatólogos. Ahora el nombre preferido es: Linfoma Cutáneo de Células T (CTCL). En esta presentación se mostró la correlación clínica-histopatológica de los linfomas cutáneos a células T y sus variantes. (AU)

Asunto(s)

RESUMEN

Mucho antes que los hongos fueran considerados responsables de causar enfermedades, Alibert, en 1806, describió un desorden severo en el cual aparecían en la piel grandes tumores necróticos parecidos a los hongos. A causa de este parecido él denominó a la enfermedad Micosis Fungoide. Su alumno Bazin, en 1876, describió las tres fases evolutivas de la enfermedad y en 1938 Sézary and Bouvrain descubrió lo que más tarde fue reconocido como una variante eritrodérmica leucémica llamada "Síndrome de Sézary". Rappaport trabajando con Thomas, demostró que las Micosis Fungoide eran un Linfoma Cutáneo especificado, con aspectos distintivos histopatológicos extracutáneos similares a los hallados en la piel; Long and Mihm, casi al mismo tiempo, llegaron a conclusiones similares. Luego de esto, las Micosis Fungoides llegaron a ser el foco de intenso interés de oncólogos y dermatólogos. Ahora el nombre preferido es: Linfoma Cutáneo de Células T (CTCL). En esta presentación se mostró la correlación clínica-histopatológica de los linfomas cutáneos a células T y sus variantes.

Asunto(s)

RESUMEN

Forty-one patients with lymphomatoid papulosis have been followed from 1 to 22 years (mean 11.4 years, median 10 years). Six patients developed malignant lymphoma, 3 cutaneous T-cell lymphoma, 2 Ki-1 large cell lymphoma, and 1 Hodgkin's disease. A clinical malignant presentation combined with the finding of aneuploidy in skin lesions seem to be indications of a malignant potential. Treatment with methotrexate in low dosage is an efficient treatment of lymphomatoid papulosis and probably diminishes the risk of malignancy.

Asunto(s)