Enfermedad de Paget extramamaria

Background and objective: Extramammary Paget disease (EMPD) has seldom been studied in Mediterranean populations. We aimed to review the characteristics of our patients with EMPD, the presence of a neoplasm in continuity, and the long-term course of the disease. Patients and methods: Retrospective observational study of 27 patients diagnosed with EMPD between 1990 and 2015. All clinical and pathology findings related to clinical course and outcomes were retrieved for analysis. Results: Twenty patients were women and 7 were men. Ages ranged from 42 to 88 years (median, 76 years). Lesions were in the following locations: vulva (16 cases), pubis-groin (5), perianal region (4), and axilla (2). Time from onset to diagnosis ranged from 1 to 60 months (median, 12 months) and maximum lesion diameter from 20 to 140mm (median, 55mm). In 3 cases (11.1%) EMPD was a secondary condition. None of the lesions developed on a previous cutaneous adnexal adenocarcinoma. Ten of the 24 primary EMPDs (41.7%) invaded the dermis. Eight of the 27 patients (29.6%) experienced local recurrence after the initial surgical treatment.Three patients (11.1%) died as a consequence of metastasis from the EMPD. Conclusions: The presence of an underlying cutaneous adnexal adenocarcinoma is uncommon, but it is not unusual to find an extracutaneous adenocarcinoma in continuity. Although EMPD is a slow-growing tumor, dermal invasion is frequent and metastasis is not uncommon. Local recurrence is common even after excision with wide margins and may be delated, so long term follow-up is essential

"Clown nose" as first manifestation of squamous cell carcinoma of the lung

Background: Skin metastases occur in 0.7% to 9% of all patients with cancer and are usually considered a late event in the evolution of most visceral carcinomas. The development of a nodular metastatic lesion on the nasal tip is known as clown nose sign. Main observation: We report a 64-year-old man that developed a nodular lesion on his nasal tip as first manifestation of squamous lung carcinoma. Conclusion: The biopsy of the cutaneous metastasis may be helpful to histopathologically confirm the suspected primary tumour avoiding invasive diagnostic procedures

Edema angioneurótico hereditario: a propósito de un caso

Los autores exponen el caso de una paciente diagnosticada de Edema Angioneurótico Hereditario y plantean los problemas y la profilaxis necesaria para un tratamiento dental. Remarcan la importancia de trabajo en equipo con otras especialidades médicas, para evitar complicaciones, en este caso muy graves, en el transcurso del tratamiento dental

Granuloma Annulare: a case-control study of possible associated diseases

Introduction: Granuloma annulare (GA) has been reported as associated with multiple diseases, mainly diabetes mellitus (DM), thyroid diseases, and dyslipidemia. However, the high prevalence of some of these illnesses makes it difficult to assess whether the association is real or fortuitous. Objectives: Our objective was to analyze the clinical features of GA patients and the possible associations. Methods: This is a retrospective observational study of 225 patients with biopsy-proven GA diagnosed between 2009 and 2019 in a referral university hospital in Barcelona, Spain. Clinical charts were reviewed to obtain clinical data. As a control group we used a random list of 225 patients diagnosed in the hospital traumatology department in the same period, matched by age and sex. Results: Diabetes was diagnosed in 40 GA patients (18%) (34 in the control group, 15%) and hypothyroidism in 33 (15%) (22 in the control group 9.8%); the differences were not significant. We also did not detect any association with uveitis, sarcoidosis, necrobiosis lipoidica, Sweet syndrome, HIV infection, hepatitis B, or hematological malignancies. We only detected a possible association with hepatitis C (6 GA patients, 2.7%, versus 0 controls, P = 0.03), and hypercholesterolemia (108 GA patients, 48%, versus 79 controls, 35%, P = 0.007). Conclusions: The possible pathogenic explanations for the association with hepatitis C and hypercholesterolemia seem unlikely. We consider that the association of GA with other diseases, including hypercholesterolemia and hepatitis C, is doubtful and that it there is no justification rule out possible associated diseases in patients with GA

Multidisciplinary approach and long-term follow-up in a series of 640 consecutive patients with sarcoidosis: cohort study of a 40-year clinical experience at a tertiary referral center in Barcelona

Cohort studies of large series of patients with sarcoidosis over a long period of time are scarce. The aim of this study is to report a 40-year clinical experience of a large series of patients at Bellvitge University Hospital, a tertiary university hospital in Barcelona, Spain. Diagnosis of sarcoidosis required histological confirmation except in certain specific situations. All patients underwent a prospective study protocol. Clinical assessment and follow-up of patients were performed by a multidisciplinary team.From 1976 to 2015, 640 patients were diagnosed with sarcoidosis, 438 of them (68.4%) were female (sex ratio F/M 2:1). The mean age at diagnosis was 43.3 ± 13.8 years (range, 14-86 years), and 613 patients (95.8%) were Caucasian. At diagnosis, 584 patients (91.2%) showed intrathoracic involvement at chest radiograph, and most of the patients had normal pulmonary function. Erythema nodosum (39.8%) and specific cutaneous lesions (20.8%) were the most frequent extrapulmonary manifestations, but there was a wide range of organ involvement. A total of 492 patients (76.8%) had positive histology. Follow-up was carried out in 587 patients (91.7%), over a mean of 112.4 ± 98.3 months (range, 6.4-475 months). Corticosteroid treatment was administered in 255 patients (43.4%), and steroid-sparing agents in 49 patients (7.7%). Outcomes were as follows: 111 patients (18.9%) showed active disease at the time of closing this study, 250 (42.6%) presented spontaneous remission, 61 (10.4%) had remission under treatment, and 165 (28.1%) evolved to chronic sarcoidosis; among them, 115 (19.6%) with mild disease and 50 (8.5%) with moderate to severe organ damage. A multivariate analysis showed that at diagnosis, age more than 40 years, the presence of pulmonary involvement on chest radiograph, splenic involvement, and the need of treatment, was associated with chronic sarcoidosis, whereas Löfgren syndrome and mediastinal lymphadenopathy on chest radiograph were indicators of good outcome.Sarcoidosis is a multisystem disease with protean clinical-radiographic manifestations. Although almost half of patients follow a spontaneous resolution or under treatment, a significant number of them may have several degrees of organ damage. This study emphasizes the value of a multidisciplinary approach and long-term follow-up by specialized teams in sarcoidosis

Specific (granulomatous) oral lesions of sarcoidosis: Report of two cases

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology that usually presents with bilateral hiliar adenopathies, pulmonary infiltrations, and ocular and cutaneous lesions. The specific cutaneous lesions, or granulomatous, are produced in from 9% to 37% of patients with systemic sarcoidosis. Nevertheless, few patients have been described having specific lesions from sarcoidosis in the oral mucous membrane. We present 2 patients with systemic sarcoidosis who developed specific sarcoidosis lesions in the oral mucous membrane. The first patient presented a plaque on the tongue of a chronic nature associated with facial lupus pernio. The second patient presented a rapidly growing nodular lesion on the lower lip that was in fact the presentation sign of the disease. Although infrequent, oral lesions may constitute the first sign of systemic sarcoidosis. For this reason the oral mucous membrane needs to be explored when there is suspicion of sarcoidosis, and systemic sarcoidosis must be included in the differential diagnosis of oral granulomatous lesions

Lesiones específicas (granulomatosas) de sarcoidosis en mucosa oral

La sarcoidosis es una enfermedad granulomatosa multisistémica de etiología desconocida que suele presentarse con adenopatías hiliares bilaterales, infiltrados pulmonares y lesiones oculares y cutáneas. Las lesiones cutáneas específicas o granulomatosas se producen entre el 9% y 37% de pacientes con sarcoidosis sistémica. Sin embargo, se han descrito pocos pacientes con lesiones específicas de sarcoidosis en la mucosa oral. Presentamos 2 pacientes con sarcoidosis sistémica que desarrollaron lesiones específicas de sarcoidosis en la mucosa oral. El primer paciente presentó una placa en la lengua de curso crónico asociada a lupus pernio facial. El segundo paciente presentó una lesión nodular en labio inferior de rápido crecimiento que constituyó el signo de presentación de la enfermedad. Aunque poco frecuentes, las lesiones orales pueden constituir el primer signo de sarcoidosis sistémica. Por dicho motivo hay que explorar la mucosa oral cuando se sospeche una sarcoidosis y la sarcoidosis sistémica debe ser incluida en el diagnóstico diferencial de lesiones granulomatosas orales

Specific (granulomatous) oral lesions of sarcoidosis: Report of two cases

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology that usually presents with bilateral hiliar adenopathies, pulmonary infiltrations, and ocular and cutaneous lesions. The specific cutaneous lesions, or granulomatous, are produced in from 9% to 37% of patients with systemic sarcoidosis. Nevertheless, few patients have been described having specific lesions from sarcoidosis in the oral mucous membrane. We present 2 patients with systemic sarcoidosis who developed specific sarcoidosis lesions in the oral mucous membrane. The first patient presented a plaque on the tongue of a chronic nature associated with facial lupus pernio. The second patient presented a rapidly growing nodular lesion on the lower lip that was in fact the presentation sign of the disease. Although infrequent, oral lesions may constitute the first sign of systemic sarcoidosis. For this reason the oral mucous membrane needs to be explored when there is suspicion of sarcoidosis, and systemic sarcoidosis must be included in the differential diagnosis of oral granulomatous lesions

Lesiones específicas (granulomatosas) de sarcoidosis en mucosa oral

La sarcoidosis es una enfermedad granulomatosa multisistémica de etiología desconocida que suele presentarse con adenopatías hiliares bilaterales, infiltrados pulmonares y lesiones oculares y cutáneas. Las lesiones cutáneas específicas o granulomatosas se producen entre el 9% y 37% de pacientes con sarcoidosis sistémica. Sin embargo, se han descrito pocos pacientes con lesiones específicas de sarcoidosis en la mucosa oral. Presentamos 2 pacientes con sarcoidosis sistémica que desarrollaron lesiones específicas de sarcoidosis en la mucosa oral. El primer paciente presentó una placa en la lengua de curso crónico asociada a lupus pernio facial. El segundo paciente presentó una lesión nodular en labio inferior de rápido crecimiento que constituyó el signo de presentación de la enfermedad. Aunque poco frecuentes, las lesiones orales pueden constituir el primer signo de sarcoidosis sistémica. Por dicho motivo hay que explorar la mucosa oral cuando se sospeche una sarcoidosis y la sarcoidosis sistémica debe ser incluida en el diagnóstico diferencial de lesiones granulomatosas orales

Edema angioneurótico hereditario: a propósito de un caso

Los autores exponen el caso de una paciente diagnosticada de Edema Angioneurótico Hereditario y plantean los problemas y la profilaxis necesaria para un tratamiento dental. Remarcan la importancia de trabajo en equipo con otras especialidades médicas, para evitar complicaciones, en este caso muy graves, en el transcurso del tratamiento dental