RESUMEN
BACKGROUND: Evidence regarding long-term therapeutic outcomes and disease-specific survival (DSS) in Extramammary Paget's disease (EMPD) is limited. OBJECTIVES: To assess the DSS and outcomes of surgical and nonsurgical therapeutic modalities in a large cohort of EMPD patients. METHODS: Retrospective chart review of EMPD patients from 20 Spanish tertiary care hospitals. RESULTS: Data on 249 patients with a median follow-up of 60 months were analyzed. The estimated 5-, 10-, and 15-year DSS was 95.9%, 92.9%, and 88.5%, respectively. A significantly lower DSS was observed in patients showing deep dermal invasion (≥1 mm) or metastatic disease (P < .05). A ≥50% reduction in EMPD lesion size was achieved in 100% and 75.3% of patients treated with surgery and topical therapies, respectively. Tumor-free resection margins were obtained in 42.4% of the patients after wide local excision (WLE). The 5-year recurrence-free survival after Mohs micrographic surgery (MMS), WLE with tumor-free margins, WLE with positive margins, radiotherapy, and topical treatments was 63.0%, 51.4%, 20.4%, 30.1%, and 20.8%, respectively. LIMITATIONS: Retrospective design. CONCLUSIONS: EMPD is usually a chronic condition with favorable prognosis. MMS represents the therapeutic alternative with the greatest efficacy for the disease. Recurrence rates in patients with positive margins after WLE are similar to the ones observed in patients treated with topical agents.
Asunto(s)
Enfermedad de Paget Extramamaria , Humanos , Estudios Retrospectivos , Enfermedad de Paget Extramamaria/cirugía , Cirugía de Mohs , Análisis de Supervivencia , Márgenes de Escisión , Resultado del Tratamiento , Recurrencia Local de Neoplasia/epidemiología , Recurrencia Local de Neoplasia/terapia , Recurrencia Local de Neoplasia/patologíaRESUMEN
BACKGROUND: Topical imiquimod has shown to be an effective treatment for EMPD, although available evidence supporting its use is based on case reports and small series of patients. OBJECTIVES: To investigate the therapeutic outcomes and analyze potential clinico-pathological factors associated with imiquimod response in a large cohort of EMPD patients. METHODS: Retrospective chart review of 125 EMPD patients treated with imiquimod at 20 Spanish tertiary-care hospitals. RESULTS: During the study period, patients received 134 treatment regimens with imiquimod, with 70 (52.2%) cases achieving complete response (CR), 41 (30.6%) partial response and 23 (17.2%) no response. The cumulative CR rates at 24 and 48 weeks of treatment were 46.3% and 71.8%, respectively, without significant differences between first-time and previously treated EMPD. Larger lesions (≥6â cm; p = 0.038) and EMPD affecting >1 anatomical site (p = 0.002) were significantly associated with a worse treatment response. However, the CR rate did not differ significantly by the number of treatment applications (≤4 vs. > 4 times/week; p = 0.112). Among patients who achieved CR, 30 (42.9%) developed local recurrences during a mean follow-up period of 36â months, with an estimated 3 and 5-year recurrence free-survival of 55.7% and 36.4%, respectively. CONCLUSIONS: Imiquimod appears as an effective therapeutic alternative for both first-line and previously treated EMPD lesions. However, a less favorable therapeutic response could be expected in larger lesions and those affecting >1 anatomical site. Based on our results, a 3-4 times weekly regimen of imiquimod with a treatment duration of at least 6â months could be considered an appropriate therapeutic strategy for EMPD patients.
RESUMEN
Cutaneous granulomatous reactions are diverse, both from the clinical and the pathological perspective. Most underlying pathophysiological aspects remain elusive. Interstitial granulomatous dermatitis and palisaded neutrophilic and granulomatous dermatitis have been claimed to be reactions to systemic disorders, such as infectious, inflammatory, or neoplastic conditions. Recently, the overarching term "reactive granulomatous dermatitis" has been coined to unify both entities. We herein report two cases of reactive granulomatous dermatitis presenting with the widely known, albeit infrequent "rope sign" and provide clinicopathological correlation. The two patients included a 53-year-old woman with enlarging erythematous plaques and underlying palpable cords on both sides of trunk near axillae (rope sign), and a 51-year-old woman with personal history of rheumatoid arthritis and a palpable cord on the left aspect of the trunk. Pathological findings were compatible with reactive granulomatous dermatitis in both cases. In conclusion, the rope sign represents a strikingly infrequent but decisive diagnostic clue of reactive granulomatous dermatitis.
Asunto(s)
Granuloma , Humanos , Femenino , Persona de Mediana Edad , Granuloma/patología , Granuloma/diagnóstico , Dermatitis/patología , Dermatitis/diagnósticoRESUMEN
Palmoplantar epidermoid cysts can range in clinical presentation from an asymptomatic slowly enlarging mass to a painful nodule. We report two cases: an epidermoid cyst on the sole and another on the palm. This article reviews the possible etiology, diagnosis, and prognosis of palmoplantar epidermoid cysts.
Asunto(s)
Quiste Epidérmico , Enfermedades del Pie , Mano , Anciano de 80 o más Años , Quiste Epidérmico/diagnóstico por imagen , Quiste Epidérmico/patología , Pie/diagnóstico por imagen , Enfermedades del Pie/diagnóstico por imagen , Enfermedades del Pie/patología , Mano/diagnóstico por imagen , Humanos , Persona de Mediana EdadAsunto(s)
Carcinoma Basocelular , Carcinoma de Células Renales , Neoplasias Renales , Neoplasias Cutáneas , Neoplasias de los Tejidos Blandos , Humanos , Proteínas Hedgehog , Carcinoma Basocelular/tratamiento farmacológico , Carcinoma Basocelular/patología , Neoplasias Cutáneas/tratamiento farmacológico , Neoplasias Cutáneas/patologíaAsunto(s)
Queratodermia Palmoplantar , Psoriasis , Humanos , Psoriasis/patología , Vacunación/efectos adversosAsunto(s)
Cicatriz , Cicatrización de Heridas , Humanos , Cicatriz/patología , Estética , Estudios ProspectivosRESUMEN
Pseudoxanthoma elasticum-like papillary dermal elastolysis is an acquired elastic tissue disorder clinically similar to pseudoxanthoma elasticum in the absence of systemic involvement. Histopathologically, special staining of elastic fibers demonstrates a total or partial band-like loss of elastic fibers in the papillary dermis. Although ultraviolet radiation seems to be one of the main etiological factors in this entity, we report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck of a woman who wore hijab.
Asunto(s)
Dermis/patología , Tejido Elástico/patología , Seudoxantoma Elástico/patología , Anciano , Biopsia , Dermoscopía , Femenino , HumanosRESUMEN
Erythroderma as the first manifestation of a solid organ malignancy is rare. The underlying cancer is a challenging condition to diagnose. There are a few cases of erythroderma in cancer patients reported in the literature. We here describe the case of a 70-year-old man who presented with asthenia, weight loss, dry cough and total body erythema with desquamation over the past month. A chest computed tomography scan showed a nodular lesion, which was finally diagnosed as a squamous cell lung carcinoma. To our knowledge, as an erythroderma presentation, only 13 cases have been reported in the literature. This case report demonstrates the need to search for a neoplasm in patients presenting with erythroderma, particularly in the presence of accompanying debilitating symptoms.
Asunto(s)
Dermatitis Exfoliativa/patología , Neoplasias Pulmonares/patología , Neoplasias de Células Escamosas/patología , Anciano , Biopsia , Dermatitis Exfoliativa/etiología , Eritema/patología , Humanos , Neoplasias Pulmonares/complicaciones , Masculino , Neoplasias de Células Escamosas/complicaciones , Síndromes Paraneoplásicos/patología , Tomografía Computarizada por Rayos XAsunto(s)
Antiinflamatorios/administración & dosificación , Clobetasol/administración & dosificación , Dermatosis de la Mano/diagnóstico , Crema para la Piel/administración & dosificación , Síndrome de Sweet/diagnóstico , Anciano de 80 o más Años , Biopsia , Femenino , Dermatosis de la Mano/tratamiento farmacológico , Dermatosis de la Mano/inmunología , Dermatosis de la Mano/patología , Humanos , Piel/inmunología , Piel/patología , Síndrome de Sweet/tratamiento farmacológico , Síndrome de Sweet/inmunología , Síndrome de Sweet/patología , Resultado del TratamientoRESUMEN
Abstract Erythroderma as the first manifestation of a solid organ malignancy is rare. The underlying cancer is a challenging condition to diagnose. There are a few cases of erythroderma in cancer patients reported in the literature. We here describe the case of a 70-year-old man who presented with asthenia, weight loss, dry cough and total body erythema with desquamation over the past month. A chest computed tomography scan showed a nodular lesion, which was finally diagnosed as a squamous cell lung carcinoma. To our knowledge, as an erythroderma presentation, only 13 cases have been reported in the literature. This case report demonstrates the need to search for a neoplasm in patients presenting with erythroderma, particularly in the presence of accompanying debilitating symptoms.
Asunto(s)
Humanos , Masculino , Anciano , Dermatitis Exfoliativa/patología , Neoplasias de Células Escamosas/patología , Neoplasias Pulmonares/patología , Síndromes Paraneoplásicos/patología , Biopsia , Tomografía Computarizada por Rayos X , Dermatitis Exfoliativa/etiología , Neoplasias de Células Escamosas/complicaciones , Eritema/patología , Neoplasias Pulmonares/complicacionesRESUMEN
Abstract Pseudoxanthoma elasticum-like papillary dermal elastolysis is an acquired elastic tissue disorder clinically similar to pseudoxanthoma elasticum in the absence of systemic involvement. Histopathologically, special staining of elastic fibers demonstrates a total or partial band-like loss of elastic fibers in the papillary dermis. Although ultraviolet radiation seems to be one of the main etiological factors in this entity, we report a case of pseudoxanthoma elasticum-like papillary dermal elastolysis on the neck of a woman who wore hijab.