Surgical outcomes and psychosocial impact of giant congenital melanocytic nevus surgery: A single-center case series of 136 patients.

PURPOSE: The aim of this study was to evaluate the outcomes, complications and psychosocial impact of surgical treatment of giant congenital melanocytic nevus (GCMN). METHODS: Patients with surgically treated GCMN who attended our clinic between May 2014 and May 2018 were included. Patient demographics and data on the characteristics of the nevus, surgical treatment, and the psychosocial impact (including C-DLQI/DLQI questionnaires) were collected. RESULTS: One hundred thirty-six patients were included (median age 9 years). Mean age at first surgery was 34 (+/- 61.45) months; 5.53 (+/- 3.69) surgical interventions were necessary to completely excise the nevus. The expanded skin flap was the preferred surgical technique in most locations. Complications were common but not severe. Of the patients studied, 70.4% reported that the surgery had a minor impact on their quality of life (QoL). Patients and caregivers stated that surgical treatment should begin as soon as possible, even in cases where early treatment did not have an impact on their QoL nor on their satisfaction with the surgery (p < 0.05). The lower the patient age at first surgery, the higher the surgeon's satisfaction (p < 0.01). CONCLUSIONS: Surgical treatment is a safe option for management of GCMN, and has a low impact on QoL. Patients, caregivers, and surgeons agree that the treatment should begin as soon as possible. This is the largest single-center study evaluating surgical treatment in GCMN patients and its psychosocial impact, and the first to take into account the patient, caregivers and dermatologists opinion of surgical results.

Large Cells With CD30 Expression and Hodgkin-like Features in Primary Cutaneous Marginal Zone B-Cell Lymphoma: A Study of 13 Cases.

The presence of CD30 cells in cutaneous lymphomas has come to prominence in recent years as a potential diagnostic and therapeutic marker. In primary cutaneous marginal zone B-cell lymphomas, the presence of large CD30 cells with Hodgkin-like features and their significance have not yet been studied. Here we describe the main clinical, histologic, immunophenotypic, and molecular characteristics of 13 cases of primary cutaneous marginal zone lymphomas featuring >10% of CD30 large cells, and analyze their relationship with histologic and clinical progression of the disease and with other morphologic and immunophenotypic features. We report 10 male and 3 female patients, 4 with early-local disease and 8 with locoregional advanced disease without extracutaneous involvement but with a high relapse rate of 69%. We describe an association between a high level of CD30 expression and disease progression, with increased clinical recurrence in cases with >15% of CD30 cells. We also discuss the differential diagnosis with other cutaneous and systemic lymphomas, especially Hodgkin lymphoma.

Mycosis Fungoides Associated With Lesions in the Spectrum of Primary Cutaneous CD30+ Lymphoproliferative Disorders: The Same Process or 3 Coexisting Lymphomas?

Mycosis fungoides (MF) is the most common type of cutaneous T-cell lymphoma, primary cutaneous CD30 lymphoproliferative disorders (pc CD30 LPD) being the second most prevalent. There is evidence that MF and pc CD30 LPD may coexist and share T-cell clonality, suggesting a common origin. These findings were supported by a T-cell receptor clonality assessment by the polymerase chain reaction coupled with capillary electrophoresis, although results produced by this method may be ambiguous. We describe an otherwise healthy 46-year-old man who developed, over the course of 5 months, a tumor consisting of primary cutaneous anaplastic large cell lymphoma and, subsequently, several papules of lymphomatoid papulosis (LyP). Both lymphomas appeared on a single patch of MF, which had been present on the patient's right buttock for at least 2 years. T-cell receptor clonality of the 3 types of neoplastic lesions and apparently non-involved skin were assessed by a next-generation sequencing-based method. We found that MF, primary cutaneous anaplastic large cell lymphoma and LyP harbored the same top 2 clones. Non-involved skin harbored other T-cell clones. In this patient, these findings suggest that MF, LyP and pc CD30 LPD were different clinicopathological manifestations arising from the neoplastic proliferation of the same T-cell clone.

«Tinea incognito» en un paciente con una infección por el virus de la inmunodeficiencia humana / Tinea incognito in a patient with human immunodeficiency virus infection

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Erythema multiforme in a newborn associated with acute acquired cytomegalovirus infection.

Erythema multiforme is exceptional in newborns, and none of the few available reports has revealed a clear etiologic agent, not even herpes simplex virus. Immunocompetent patients rarely present with cutaneous cytomegalovirus involvement, and few cases of cytomegalovirus-associated erythema multiforme have been described, none of them in newborns. We report the first case of erythema multiforme in a newborn associated with cytomegalovirus infection.

Características clínicas, perfil hormonal y marcadores de autoinmunidad de pacientes con enfermedad de Graves / Clinical characteristics, thyroid function profile and autoimmune markers in patients with Graves' disease

Objetivo: Describir las características clínicas, el perfil hormonal y los marcadores de autoinmunidad de pacientes con enfermedad de Graves (EG), atendidos en el servicio de endocrinología de un hospital general. Material y métodos: Estudio descriptivo, retrospectivo de pacientes con diagnóstico de EG atendidos en el servicio de endocrinología del HospitalNacional Cayetano Heredia durante el año 2005. Los pacientes fueron identificados utilizando el Registro Diario de Atención y Otras Actividades del consultorio externo. Para el análisis estadístico se utilizó el programa SPSS versión 12,0. Resultados: Se revisaron 111 historias clínicas. La edad promedio fue de 40,6 ± 14 años. El 71,2% eran mujeres. El 20,7% tenía antecedentefamiliar de otra enfermedad autoinmune. El tiempo de enfermedad promedio antes de la consulta fue 18,9 ± 30,4 meses. Los síntomas más frecuentes fueron: tremor, piel delgada, palpitaciones y baja de peso. El peso promedio de la glándula tiroides estimado por palpación fue 69 ± 27,5 gr, 38,7% presentaba exoftalmos y 18,9% mixedema pretibial. El perfil hormonal mostró TSH suprimida, T4 libre y T3 total elevados. El 86,7% de los casos tenía autoanticuerpos anti-peroxidasa tiroidea (anti-TPO) positivo. En 95,5% el tratamiento incluyó el uso de tionamidas. El análisis bivariado mostró que las mujeres tuvieron antecedente familiar de enfermedad tiroidea autoinmune en mayor frecuencia que los varones (27,8 vs. 9,4%) (p=0,04). Conclusión: La población estudiada con EG fueron con mayor frecuencia mujeres entre la tercera y sexta década de la vida.El peso estimado de la glándula tiroides fue tres veces lo normal, con alta frecuencia de oftalmopatía y mixedema. La mayoría tiene marcadores humorales de autoinmunidad y el tratamiento inicial se basa en el uso de tionamidas.

Objective: To describe the clinical characteristics, thyroid function profile and autoimmune markers in patients with Graves’ disease (GD) treated at the endocrine service of a general hospital. Material and methods: Descriptive and retrospective study in patients with GD treated at the Hospital Nacional Cayetano Heredia during 2005, identified in the Daily Registry of Attention and Other Activities of the Endocrine Service. For statistical analysis, SPSS software was used. Results: We reviewed 111 medical charts. The mean age was 40.6 ± 14 years. 71.2% were women. Twenty three patients (20.7%) reported familial history of autoinmune disease. The mean time of disease was 18.9 ± 30.4 months before diagnosis. The most frequent symptoms were: tremor, thin skin, palpitations and weight loss. The mean thyroid weight was 69 ± 27.5 grams. In 38.7% exophthalmos was present and 18.9% had myxedema. Hormonal profile showed suppressed TSH, elevated levels of free T4 and total T3. In 86.7% autoantibodies against thyroid peroxidase (anti-TPO) were positive. The initial treatment included thionamides in 95.5% of patients. Further statistical analysis showed that women had more frequent familiar history of autoinmune thyroid disease than men (27.8% vs. 9.4%) (p = 0.04). Conclusions: Most of our patients with GD were women between third and sixth decade of life. The estimated weight of the thyroid gland was three times the normal weight and ophthalmopathy and myxedema were frequent. Most of our patients had positive autoimmune markers and theinitial treatment included thionamides.