RESUMEN

Advanced-stage primary cutaneous T-cell lymphoma has an unfavorable prognosis and low survival rates. Aggressive treatment with chemotherapy is not curative and causes considerable side effects. The combination of bexarotene and denileukin diftitox is associated with an acceptable safety profile and a likely synergistic effect because bexarotene is capable of modulating expression of IL-2 receptor and enhance the susceptibility of T-cell leukemia cells to denileukin diftitox. In the case reported here, the response to this combined treatment was satisfactory and well tolerated. The patient showed a complete regression of pruritus, restlessness, and insomnia. Skin lesions improved partially, and lymphadenopathy was reduced and finally disappeared completely.

RESUMEN

Buerger disease or thromboangiitis obliterans (TAO) is a thrombotic occlusive, non-atherosclerotic segmental inflammatory disease that affects the small and medium-sized arteries and veins in the extremities of the limbs, frequently requiring amputation. Cessation of tobacco use is the only known effective treatment, though preliminary results from the use of pharmacological therapy implicated in pathogenesis of TAO have demonstrated noticeable clinical improvement of patients. We report the case of a 35-year-old woman with active TAO, refractory to smoking cessation and conventional therapy, who exhibited a favorable clinical response to treatment with bosentan, an oral dual endothelin receptor antagonist, administered on a compassionate-use basis. Six months after starting bosentan therapy, the pain and trophic lesions in the patient's toes had completely disappeared. Bosentan was well tolerated, without any observed adverse reaction. The findings of this case report suggest that bosentan may be considered a therapeutic option for patients with active disease, despite quitting smoking, or for those who fail in absolute abstention from smoking.

Asunto(s)

Asunto(s)

RESUMEN

Epithelial mucinosis (follicular mucinosis) is a reactive histological pattern characterized by the accumulation of mucin in the infundibular, follicular, and sebaceous epithelium, that may occur in multiple skin conditions and cannot be considered a specific disease. Alopecia mucinosa is a clinicopathological entity with distinctive skin lesions, mucin deposits in the hair follicles and periadnexal lymphoid infiltrates. Three types of mucinous alopecia have been described: idiopathic, persistent or benign chronic, and associated to lymphoma. For some authors, these types of mucinous alopecia are not independent diseases, but rather represent variants of mycosis fungoides with epithelial mucinosis.

Asunto(s)

RESUMEN

La mucinosis epitelial (mucinosis folicular) es un patrón histológico reactivo caracterizado por la acumulación de mucina en el epitelio infundibular, folicular o sebáceo, que puede ocurrir en múltiples procesos cutáneos, y que no puede ser considerada una enfermedad específica. La alopecia mucinosa es una entidad clinicopatológica con lesiones cutáneas distintivas, depósitos de mucina en los folículos pilosos e infiltrados linfoides perianexiales. Se han descrito tres tipos de alopecia mucinosa: idiopática, persistente o crónica benigna y asociada a linfoma. Para algunos autores, estos tipos de alopecia mucinosa no son enfermedades independientes, sino que representan variantes de micosis fungoides con mucinosis epitelial

Epithelial mucinosis (follicular mucinosis) is a reactive histological pattern characterized by the accumulation of mucin in the infundibular, follicular, and sebaceous epithelium, that may occur in multiple skin conditions and cannot be considered a specific disease. Alopecia mucinosa is a clinicopathological entity with distinctive skin lesions, mucin deposits in the hair follicles and periadnexal lymphoid infiltrates. Three types of mucinous alopecia have been described: idiopathic, persistent or benign chronic, and associated to lymphoma. For some authors, these types of mucinous alopecia are not independent diseases, but rather represent variants of mycosis fungoides with epithelial mucinosis

Asunto(s)

RESUMEN

El síndrome de DRESS es una toxicodermia grave caracterizada por exantema, fiebre, adenopatías, alteraciones hematológicas (eosinofilia, linfocitos atípicos) y afectación de órganos internos. Los anticonvulsivantes aromáticos y las sulfonamidas son los fármacos que con mayor frecuencia se asocian a esta entidad clínica. El diagnóstico se establece por la exposición al fármaco, los hallazgos clínicos y de laboratorio y el diagnóstico diferencial con otras enfermedades. El manejo terapéutico incluye la rápida retirada del fármaco responsable, el tratamiento de soporte y la prevención de la sepsis. El uso de corticoesteroides sistémicos resulta controvertido. Presentamos un caso de síndrome de DRESS por sulfonamidas con grave afectación multiorgánica. Con la corticoterapia sistémica se obtuvo una importante mejoría clínica y analítica, por lo que recomendamos su uso cuando existe afectación importante de órganos internos

DRESS síndrome is a severe drug reaction characterised by rash, fever, lymph nodes enlargement, hematologic abnormalities (eosinophilia, atipical lymphocytes) and internal organ involvement. Aromatic anticonvulsivants and sulphonamides are the most common drugs associated with this clinical entity. The diagnosis is established by the drug exposition, the clinical and laboratory features and the differential diagnosis with another diseases. The therapeutic management includes the prompt withdrawal of the offending drug, the supportive therapy and the prevention of sepsis. The use of systemic corticosteroids remains controversial. We repport a sulphonamides DRESS syndrome with severe multiorganic involvement. Clinical and analytic improvement has been obtained after systemic corticotherapy. We suggest their use when internal organs are severely affected

Asunto(s)

RESUMEN

La dermatomiositis juvenil es la miopatía inflamatoria idiopática juvenil más frecuente, que se caracteriza por debilidad muscular proximal simétrica, eritema en heliotropo y pápulas y placas en superficies extensoras. El electromiograma y la biopsia muscular confirman el diagnóstico, pero son técnicas invasivas; la resonancia magnética es una alternativa útil en el diagnóstico y el seguimiento, asociado a 105 datos clínicos y analíticos. El tratamiento precoz y agresivo puede, en muchos casos, controlar las manifestaciones agudas y las secuelas a largo plazo de la dermatomiositis juvenil, incluida la calcinosis. Presentamos un caso clínico de dermatomiositis juvenil con buena evolución inicial con dosis esteroideas bajas, que fue preciso aumentar posteriormente. Comentamos las aportaciones de la resonancia magnética en nuestro caso y en la literatura

Juvenile dermatomyositis (JOM) is the most common pediatric inflammatory myopathy characterized by progressive proximal muscle weakness, heliotrope rash, and erythematous papules and plaques on extensor surfaces. Electromyography and muscular biopsy confirm the clinical diagnosis, but they are invasive techniques. Magnetic resonance findings in this disease may be useful in establishing its diagnosis and follow-up within the clinical contexto Often, aggressive and rapid treatment directed can control the acute manifestations and long-range sequelae of JOM, including calcinosis cutis. We report a case of juvenile dermatomyositis with good initial response to low esteroids dose, that we must increased lately. We comment the usefull magnetic resonance findings in our case and in the literature

Asunto(s)