ABSTRACT
La morfea es una enfermedad de la piel que se manifiesta en forma de inflamación y fibrosis. En niños y jóvenes, también se conoce como esclerodermia juvenil localizada. En edad infantil, afecta con mayor frecuencia al sexo femenino y la edad de comienzo se ha establecido en torno a los 5-7 años. Una clasificación reciente divide la morfea en: circunscrita (en placas), lineal, generalizada, panesclerótica y mixta. Alrededor de un 40% de los pacientes presentan manifestaciones extracutáneas. Los tratamientos empleados en morfea infantil son: fototerapia, calcitriol oral, calcipotriol tópico, tacrolimus 0,1% tópico, metotrexato, glucocorticoides tópicos y sistémicos, mofetil micofenolato, bosentán e imiquimod 5% tópico. Diversas medidas de resultado pueden ayudar a monitorizar el tratamiento. Los estudios pronósticos son escasos, pero apuntan hacia una enfermedad con tendencia a un curso crónico o intermitente-recurrente y una frecuencia considerable de secuelas
Morphea is an inflammatory, fibrosing skin disorder. When it occurs in childhood, it is also known as localized juvenile scleroderma. It is more common in girls and typically appears around the age of 5 to 7 years. According to a recent classification system, morphea is divided into 5 types: circumscribed (plaque), linear, generalized, pansclerotic, and mixed. Approximately 40% of patients present extracutaneous manifestations. Childhood morphea is treated with phototherapy, oral or topical calcitriol, topical tacrolimus 0.1%, methotrexate, topical or systemic corticosteroids, mycophenolate mofetil, bosentán, and topical imiquimod 5%. A variety of measuring tools are used to monitor response to treatment. Few prognostic studies have been conducted, but findings to date suggest that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae
Subject(s)
Humans , Male , Female , Infant , Child, Preschool , Child , Adolescent , Scleroderma, Localized/epidemiology , Scleroderma, Localized/drug therapy , Scleroderma, Localized/diagnosis , Phototherapy/methods , Prognosis , Scleroderma, Localized/etiology , Scleroderma, Localized/pathology , Vitamin D/therapeutic use , Immunologic Factors/therapeutic useABSTRACT
Morphea is an inflammatory, fibrosing skin disorder. When it occurs in childhood, it is also known as localized juvenile scleroderma. It is more common in girls and typically appears around the age of 5 to 7 years. According to a recent classification system, morphea is divided into 5 types: circumscribed (plaque), linear, generalized, pansclerotic, and mixed. Approximately 40% of patients present extracutaneous manifestations. Childhood morphea is treated with phototherapy, oral or topical calcitriol, topical tacrolimus 0.1%, methotrexate, topical or systemic corticosteroids, mycophenolate mofetil, bosentán, and topical imiquimod 5%. A variety of measuring tools are used to monitor response to treatment. Few prognostic studies have been conducted, but findings to date suggest that the disease tends to run a chronic or intermittent-recurrent course and frequently causes sequelae.
Subject(s)
Scleroderma, Localized , Acute-Phase Proteins/analysis , Bosentan/therapeutic use , Calcitriol/therapeutic use , Child , Child, Preschool , Female , Humans , Imiquimod/therapeutic use , Immunosuppressive Agents/therapeutic use , Incidence , Male , Phototherapy , Prevalence , Prognosis , Quality of Life , Scleroderma, Localized/classification , Scleroderma, Localized/epidemiology , Scleroderma, Localized/pathology , Scleroderma, Localized/therapy , Vitamin D/therapeutic useABSTRACT
No disponible
Subject(s)
Humans , Female , Adult , Melanoma/diagnosis , Skin Neoplasms/diagnosis , Facial Neoplasms/diagnosis , Asymptomatic DiseasesSubject(s)
Facial Neoplasms/diagnosis , Melanoma/diagnosis , Skin Neoplasms/diagnosis , Adult , Asymptomatic Diseases , Biomarkers, Tumor/analysis , Chemotherapy, Adjuvant , Facial Neoplasms/chemistry , Facial Neoplasms/drug therapy , Facial Neoplasms/surgery , Female , Humans , Interferons/therapeutic use , Melanoma/chemistry , Melanoma/drug therapy , Melanoma/surgery , Prognosis , Remission Induction , Skin Neoplasms/chemistry , Skin Neoplasms/drug therapy , Skin Neoplasms/surgeryABSTRACT
No disponible
Subject(s)
Humans , Male , Aged , Erythema/chemically induced , Erythema/complications , Erythema/therapy , Drug-Related Side Effects and Adverse Reactions/complications , Drug-Related Side Effects and Adverse Reactions/diagnosis , Drug-Related Side Effects and Adverse Reactions/therapy , Antineoplastic Agents/adverse effects , Emollients/metabolism , Emollients/therapeutic use , Keratosis/therapy , Keratoderma, Palmoplantar/complications , Keratoderma, Palmoplantar/therapyABSTRACT
PResentamos un caso de dermatofibroma epitelioide rico en células granulares. Solamente se han descrito 11 casos de dermatofibroma de células granulares, de los cuales sólo tres tienen rasgos epitelioides. Es importante reconocer este subtipo de dermatofibroma, ya que puede confundirse con un gran número de procesos benignos o malignos, de diferente aplicación pronóstica y terapeútica (AU)
Subject(s)
Adult , Male , Humans , Granular Cell Tumor/complications , Granular Cell Tumor/diagnosis , Granular Cell Tumor/etiology , Granular Cell Tumor/pathology , Leiomyoma, Epithelioid/diagnosis , Leiomyoma, Epithelioid/etiology , Leiomyoma, Epithelioid/surgery , Skin Neoplasms/surgery , Skin Neoplasms/diagnosis , Skin Neoplasms/etiology , Skin Neoplasms/pathology , Histiocytoma, Benign Fibrous/diagnosis , Histiocytoma, Benign Fibrous/surgery , Histiocytoma, Benign Fibrous/etiology , Histiocytoma, Benign Fibrous/pathology , Histiocytoma, Benign Fibrous/chemistry , Prognosis , Granuloma, Pyogenic/diagnosis , Granuloma, Pyogenic/pathology , Adenocarcinoma/diagnosis , Adenocarcinoma/etiology , Biomarkers, Tumor/chemistry , Biomarkers, Tumor , Immunohistochemistry/methodsABSTRACT
BACKGROUND: Toxic epidermal necrolysis (TEN) is a severe skin disorder characterized by separation of the dermal-epidermal junction, as is observed in second-degree superficial burns. It has been proposed that immunosuppressive treatment may improve prognosis of patients with TEN. METHODS: We report here a case series of patients with TEN treated with cyclosporin A (CSA) without other concomitant immunosuppressive agent. These patients (n = 11) were consecutively admitted to our Intensive Care Burn Unit because of severe TEN, involving a large body surface area (83 +/- 17% [mean +/- SD], median, 90%; range, 35-96%) and were treated with CSA 3 mg/kg per day enterally every 12 hours. We compared the series of patients treated with CSA with a historical series of patients admitted to our Intensive Care Burn Unit before CSA was introduced as part of the treatment protocol These patients (n = 6) were treated with cyclophosphamide (150 mg i.v. every 12 hours) and different doses of corticosteroids (> or =1 mg/kg per day of 6-methyl-prednisolone). Both groups of patients were similar in regard to age, delay from onset of disease to Intensive Care Burn Unit admission, and body surface area involved. RESULTS: Time from the onset of skin signs to arrest of the disease progression (1.4 +/- 0.3 days, vs. 3.6 +/- 1.5 days) and to complete reepithelialization (12.0 +/- 3.6 days, vs. 17.6 +/- 3.1 days) was significantly shorter in patients treated with CSA compared with those treated with cyclophosphamide and corticosteroids (p = 0.0002, and p = 0.0058, respectively). Significantly fewer patients in the CSA group had > or =4 organs failing (2 of 11 vs. 3 of 6, respectively, p = 0.029), had severe leukopenia (<1,000 cells/microL) (0 of 11 vs. 4 of 6, respectively, p = 0.006), or died (3 of 6 vs. 0 of 11, respectively, p = 0.0029). CONCLUSION: We conclude that immunosuppressive treatment with CSA is safe and is associated with a rapid reepithelialization rate and a low mortality rate in patients with severe TEN. Our data suggest that this regimen could be more effective than treatment with cyclophosphamide and corticosteroids. Prospective controlled trials are required to test the hypothesis that CSA is more effective than cyclophosphamide or other immunosuppressive regimens for the treatment of TEN.
Subject(s)
Cyclosporine/therapeutic use , Immunosuppressive Agents/therapeutic use , Stevens-Johnson Syndrome/drug therapy , Adult , Aged , Aged, 80 and over , Burns/drug therapy , Critical Care , Cyclophosphamide/adverse effects , Cyclophosphamide/therapeutic use , Cyclosporine/adverse effects , Drug Therapy, Combination , Female , Humans , Immunosuppressive Agents/adverse effects , Male , Methylprednisolone/adverse effects , Methylprednisolone/therapeutic use , Middle Aged , Treatment OutcomeABSTRACT
Objetivos: Los leiomiosarcomas superficiales (de la piel y tejido subcutáneo) son neoplasias poco frecuentes, de ditinto comportamiento biológico según se localicen en la dermis o hipodermmis. Recientemente se ha propuesto su subclasificación en dos patrones histológicos: nodular y difuso. Material y Métodos: Presentamos cinco casos de leiomiosarcomas cutáneos primarios diagnosticados en nuestro Departamento entre 1991 y 1998 y revisamos la literatura, centrándonos en los hallazgos inmunohistoquímicos y en el diagnóstico diferencial. Resultados Cuatro pacientes eran varones y uno, mujer; sus edades oscilaban entre los 34 y 85 años (media 56,6). En cuatro casos las lesiones eran solitarias, y un paciente presentó lesiones múltiples; se localizaron en las extremidades (tres en las superiores y uno en las inferiores) y en el tronco. Desde el punto de vista histológico dos tumoraciones se localizaban exclusivamente en la dermis y tres afectaban además al tejido celular subcutáneo. Se identificó un patrón de crecimiento nodular en tres casos, difuso en uno y mixto en otro. La inmunohistoquímica mostró en todos los casos positividad para al menos uno de los tres marcadores de diferenciación muscular utilizados (desmina, SMA y HHF35), siendo la actina de músculo liso (SMA) positiva en el 100 por ciento de los casos. Las queratinas y la proteína S100 fueron negativas en todos los casos, encontrándose células dendítricas S100 positivas atrapadas por el crecimiento tumoral en dos casos. En cuanto a la evolución, actualmente tres pacientes se encuentran libres de enfermedad, sin datos e recidiva o metástasis. Dos fallecieron, pero sólo uno de ellos a consecuencia de la evolución de un leiomiosarcoma cuatro años después del diagnóstico inicial. Conclusiones: Consideramos que es necesario utilizar un panel de anticuerpos amplioi en el diagnóstico de estos tumores (SMA, HHF35, desmina, proteína S100 y citoqueratinas) dada la distinta expresión de estos anticuerpos en los leiomiosarcomas. Además, en todos los casos está indicado reaizar un estrecho seguimiento clínico (AU)
Subject(s)
Adult , Aged , Male , Middle Aged , Humans , Leiomyosarcoma/diagnosis , Leiomyosarcoma/etiology , Leiomyosarcoma/pathology , Immunohistochemistry/methods , Sarcoma/diagnosis , Sarcoma/pathology , Skin Neoplasms/diagnosis , Skin Neoplasms/pathology , Skin Neoplasms/therapy , Diagnosis, Differential , Mitotic Index , Nevus, Intradermal/diagnosis , Nevus, Intradermal/pathology , Epidermal Cyst/diagnosis , Epidermal Cyst/pathology , Carcinoma, Squamous Cell/diagnosis , Carcinoma, Squamous Cell/pathology , Keratins/analysis , Keratins , Histiocytoma, Benign Fibrous/diagnosis , Histiocytoma, Benign Fibrous/pathologyABSTRACT
Two cases of penile squamous cell carcinoma with distinctive clinicopathologic characteristics are presented. The tumors appeared in patients infected with HIV and were located in the glans of the penis. Histologically, the neoplasms were well-differentiated, infiltrating, squamous cell carcinomas. The entire spectrum from benign condyloma to infiltrative squamous cell carcinoma was present in the two patients. In both cases, human papillomavirus (HPV) could be demonstrated using polymerase chain reaction analysis. The reported cases suggest a synergic interaction of HPV and HIV in the carcinogenic process of some penile carcinomas.
Subject(s)
Carcinoma, Squamous Cell/complications , Carcinoma, Squamous Cell/virology , HIV Seropositivity/complications , Papillomaviridae/isolation & purification , Papillomavirus Infections/complications , Penile Neoplasms/complications , Penile Neoplasms/virology , Tumor Virus Infections/complications , Aged , Humans , Male , Middle AgedABSTRACT
We report the first case of metastatic involvement of the skin by a soft tissue mesenchymal chondrosarcoma (MS). A 64-year-old man presented 15 months after resection of a 7.0 cm MS from his left forearm with a rapidly growing, erythematous nodule on the left side of the upper lip. The lesion was clinically interpreted as a keratoacanthoma. The histologic appearance was identical to that of the soft tissue MS; an immunohistochemical stain for CD99 was positive. Lung and bone metastases were subsequently documented. Our case expands the differential diagnosis of malignancies with cartilaginous differentiation that can involve the skin.
Subject(s)
Chondrosarcoma, Mesenchymal/secondary , Keratoacanthoma/diagnosis , Skin Neoplasms/secondary , Soft Tissue Neoplasms/pathology , 12E7 Antigen , Antigens, CD/analysis , Cell Adhesion Molecules/analysis , Chondrosarcoma, Mesenchymal/chemistry , Chondrosarcoma, Mesenchymal/therapy , Combined Modality Therapy , Diagnosis, Differential , Humans , Male , Middle Aged , Skin Neoplasms/chemistry , Soft Tissue Neoplasms/chemistry , Soft Tissue Neoplasms/therapyABSTRACT
Trichilemmal keratosis (TK) is an uncommon epidermal tumor that exhibits a keratinizing surface with the formation of a cutaneous horn and that clinically resembles a hyperkeratotic actinic keratosis. Histologically, there is verrucous hyperplasia of the epidermis with orthokeratotic hyperkeratosis. TK is characterized by abrupt keratinization without formation of a granular cell layer, in the same manner as that in which the outer root sheath keratinizes (trichilemmal keratinization). The epidermis is acanthotic and contains pale-staining keratinocytes. Epithelial lobules and small trichilemmal cysts are connected to the thickened epidermis. We describe the clinical, histologic, and immunohistochemical findings of two cases of TK.
