Carlos de la Torre

Acrochordons are not a component of the Birt-Hogg-Dubé syndrome: does this syndrome exist? Case reports and review of the literature.

Multiple fibrofolliculomas, trichodiscomas, and acrochordons compose the triad of cutaneous lesions characterizing the Birt-Hogg-Dubé syndrome, inherited in an autosomal dominant fashion. We report a case of a family who had the triad of tumors of the Birt-Hogg-Dubé syndrome. Two members were observed clinically and histologically. Biopsies of the facial papules disclosed features of the fibrofolliculoma/trichodiscoma spectrum. Lesions that were clinically acrochordon-like proved to correspond to the same histopathologic spectrum. The characterization of the nature of the acrochordon-like lesions in our patients and the review of the literature allow us to question if acrochordons (skin tags) should be maintained as a component of this association. In light of our conclusion that fibrofolliculoma, trichodiscoma, and the acrochordon-like lesions are histologic variations of a single lesion, we further question whether the term syndrome is valid.

Acquired cutaneous lymphangiectasia in a patient with cirrhotic ascites

Lymphangiectasia results from acquired dilation of lymphatic vessels. Areas of skin affected by obstruction or destruction of lymphatic drainage are said to be prone to the development of lymphangiectasia. Cirrhosis is a cause of alterations of lymph flow.

Cowden's disease and Down syndrome. An exceptional association.

Cowdens disease with typical mucocutaneous features developed in a 24-year-old woman who had Down syndrome. To our knowledge this association has not been reported previously. The interest lies in the overlap of some characteristic features of both diseases.

A cutaneous multilobated B-cell lymphoma

A patient with multilobated B-cell malignant lymphoma with lesions limited to the skin is described. The light, electron microscopic, and immunohistochemical features of this unusual morphologic variant of non-Hodgkins lymphoma are described. The nosologic position of this histologic subtype is discussed. The clinical course, with lesions confined to the skin, and the response to treatment suggest a favorable prognosis.

Abdominal nodules as a presentation of obstruction of the inferior vena cava and factor V Leiden mutation

Abdominal nodular lesions are not usually considered a sign of deep venous thrombosis. However, we have seen a patient with abdominal nodular lesions due to venous thrombosis, that led to the diagnosis of factor V Leiden mutation, a recently described cause of hypercoagulability.

Regressive Lichenoid Eruption during an Acute Episode of Q Fever

Q fever is a worldwide distributed human rickettsiosis that was described by Derrik in 1935. Q fever is a zoonotic disease, endemic in the Iberian Peninsula, caused by Coxiella burnetii , a strict intracellular bacterium [1] . Infection has been reported in a wide variety of animals (mammals, birds and ticks), including domestic pets. The human transmission occurs mainly by inhalation of contaminated airborne particles, not by a tick bite, as in other rickettsial diseases. Q fever also differs from other rickettsial diseases by the absence of typical exanthema. We report a case of a 73-year-old Spanish man, resident in a rural area, who presented with fever, general malaise, myalgia, headache and cutaneous lesions. During the last month, he referred constitutional symptoms accompanied by fever (39 ° C) and chills and in the week prior to admission acute onset of a pruritic cutaneous eruption on the hands, neck and face. Cutaneous examination showed red-violaceous papules grouped in well-defined plaques on the dorsum of the hands, scalp and neck ( fig. 1 ). The general clinical examination revealed a hypoventilation and a pericardial rub. The rest of the physical examination was unremarkable. Laboratory tests showed a white blood cell count of 2.5 ! 10 9 l –1 (range 4.5–11) with a normal formula and mild elevated liver enzymes (liver function tests): aspartate aminotransferase 34 IU/l (normal level 10–37), alanine aminotransferase 45 IU/l (normal level 7–40). The rest of the biochemistry parameters were within normal limits. The chest radiogram revealed bilateral pleural effusion, and the transthoracic echocardiogram showed a mild pericardial effusion. The patient was admitted to the hospital, and levofloxacine therapy was started. The blood and urine cultures as well as the serology for Cytomegalovirus , Epstein-Barr virus, and hepatitis A, B, and C virus were negative. The diagnosis of acute infection with C. burnetii was confirmed by a positive serology by indirect immunofluorescent antibody testing with titers of 1: 2,560 for IgG in phase II and titers of 1: 2,048 for IgG in phase I. The biopsy of the skin lesion was consistent with the diagnosis of lichenoid reaction: presence of irregular acanthosis and liquefactive degeneration of the basal layer with presence of colloid bodies and a band-like infiltrate composed mainly by lymphocytes and histiocytes in the superficial dermis ( fig. 2 ). A diagnosis of regressive lichenoid eruption during an acute episode of Q fever was established. The treatment was modified, and a schedule of oral doxycycline, 100 mg twice a day for 14 days, was initiated. Two weeks later the patient showed great improvement of the systemic symptoms, with resolution of the pleural efDermatology 2006;213:251–252

Mucinosis pretibial como manifestación de dermatitis de estasis

Pretibial mucin deposits may be a sign of Graves disease (pretibial myxedema) or may appear as a result of a disorder in venous or lymphatic return. It is possible to distinguish the etiology through histopathological findings. We present a case of pretibial mucinosis as a manifestation of statis dermatitis.

Acquired ichthyosis associated with Sézary's syndrome

Abstract We report a case of a 70-year-old woman affected by acquired ichthyosis. Morphologic studies of mononuclear cells in peripheral blood revealed that 50% were Sezary cells, and analyses of surface phenotype disclosed a CD4 predominance. Reports of association with cutaneous T cell lymphoma, mycosis fungoides type, have been described, but we have found no reports with Sezarys syndrome.