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Biomedical subjects

E Marinho

Publications and source records attributed to E Marinho.

At least 19 recordsLinked to original sources

A paraneoplastic mixed bullous skin disease: breakdown in tolerance to multiple epidermal antigens.

We report a new type of paraneoplastic mixed bullous skin disease in a patient with a B-cell lymphoma associated with monoclonal IgM kappa paraproteinaemia. The patient's clinical and histological features were reminiscent of bullous pemphigoid. Characterization of antiepidermal antibodies by immunoblotting and indirect immunoelectron microscopy demonstrated a novel pattern of reactivity: IgG antibodies were directed against desmoplakins I-II and BPAG2, and were associated with antidesmoglein 3 polyclonal IgM antibodies. Such an autoreactive pattern involving antidesmoplakins, anti-BPAG2 and antidesmoglein 3 antibodies has not been previously reported. It reflects the breakdown of tolerance to multiple epidermal antigens observed in some patients with malignancy.

Autoantibodies↗

[Langerhans cell histiocytosis and myelomonocytic leukemia: a non-fortuitous association].

BACKGROUND: Langerhans cell histiocytosis is an uncommon clonal disorder. Its reactional or genetic nature is debated. CASE REPORT: Three patients aged 71, 75 and 73 years with Langerhans cell histiocytosis also developed myelomoncytic leukemia (AML4, AML5, and chronic myelomonocytic leukemia). In two cases, there was no prior treatment which could potentially induce leukemia. In the third case, AML4 occurred shortly (10 months) after initiation of a vinblastin treatment. DISCUSSION: This pathogenic association suggests a common origin for these two conditions, corresponding to an anomalous pluripotent stem cell line. These cases provide further evidence favoring the hypothesis of a genetic origin rather than a reactional origin for Langerhans cell histiocytosis.

Aged↗

[Epithelioid sarcoma manifesting as chronic plantar arch ulceration].

BACKGROUND: Epitheloid sarcoma is an uncommon malignant soft tissue tumor observed in the distal extremities of young men. We report a case of long standing ulceration of the sole which was found to be an epithelioid sarcoma. CASE REPORT: A 78-year-old woman had an indolent ulceration of the left sole for several months. Physical examination disclosed a well demarcated 3-cm ulcerated lesion with a red center and flat edges. Skin sections confirmed the diagnosis of epithelioid sarcoma. Cells stained positively for anti-vimentin, anti-cytokeratin and anti-epithelial membrane antigen, but not for anti-S100 protein and anti-actin antibodies. Wide local excision was performed. DISCUSSION: Epithelioid sarcoma is an uncommon malignant tumor which apparently differentiates from mesenchymatous cells. It is usually observed in the distal extremities in young males, predominantly the hands and forearms. The tumor presents a firm, flesh-colored indolent nodule. Ulceration usually develops and involves the subcutis and deeper soft tissue, particularly fascial planes, aponeuroses and tendon sheaths. Treatment is wide surgical excision with or without radiotherapy. The case reported here on the sole of the foot in a 78-year-old women is unusual. Clinicians should be aware that the initial biopsy may not be contributive and that repeated biopsies may be necessary for positive diagnosis.

Aged↗

[Neutrophilic eccrine hidradenitis associated with relapse of acute myeloblastic leukemia].

BACKGROUND: Neutrophilic eccrine hidradentitis is a recently described clinical entity. Most reported cases have occurred in patients given chemotherapy for acute myelogenous leukemia, suggesting a drug induced mechanism. Some authors have considered however that neutrophilic eccrine hidradenitis belongs to the group of neutrophilic dermatoses. CASE REPORT: We observed neutrophilic eccrine hidradentitis in a 48-year-old man which developed when he suffered a relapse of acute leukemia. He had not been given chemotherapy in the preceding months. DISCUSSION: This case favors the hypothesis that neutrophilic eccrine hidradenitis is associated with myeloid hemotology disorders and not a complications secondary to treatment.

Hidradenitis↗

[Toxicoderma caused by prednisone and prednisolone. Value of skin tests in the screening of cross sensitivity].

INTRODUCTION: Allergic reactions to general corticosteroid therapy are uncommon. CASE REPORT: We report a patient with systemic lupus erythematousus who developed skin rash after initiation of prednisone then prednisolone therapy. Histology evidence suggested leukocytoclastic vasculitis. The skin tests (prick tests, intradermoreactions and patch-tests) using a battery of injectable corticosteroids showed a highly positive reaction to prednisolone, methylprednisolone and dexamethasone on the intradermo-reactions 24 hours later. Histology examination of a positive-response showed leukocytoclastic vasculitis associated with eczematiform alterations of the epidermis compatible with a drug reaction. The skin tests however were negative for betamethasone, triamcinolone, paramethasone and hydrocorticose. The patient was treated with betamethasone and no skin reaction was observed. DISCUSSION: Skin tests, particularly intradermo-reactions read 24 hours later would appear to be useful in identifying possible cross-sensitivity.

Aged↗

Cutaneous vasculitis and IgA glomerulonephritis in ankylosing spondylitis.

Two patients with ankylosing spondylitis were found to have IgA nephropathy and leucocytoclastic cutaneous vasculitis. Immunofluorescence showed perivascular deposition of IgA in the skin of one patient and in the mesangium of both patients. Such an association has been reported only once before. This supports the concept of abnormal IgA immune stimulation in the pathogenesis of ankylosing spondylitis.

Glomerulonephritis, IGA↗

[Smooth muscle hamartoma: anatomoclinical characteristics and nosological limits].

Smooth muscle hamartoma is an uncommon cutaneous dysembryoplasia usually diagnosed in infancy. Among the 61 cases published since 1923, 56 were congenital and 3 appeared in young adults. We report a case in which the lesions started at the age of 15 years as a papular plaque in the right mammary region of a young woman. A review of the literature showed that the usual clinical presentation is a frequently pigmented plaque made of often follicular papules and measuring 1 to 10 centimeters on average. Excessive hairiness is the most frequent sign, being observed in more than two-thirds of the cases, and Darier's pseudo-sign is present in about 53 p. 100 of the patients. The disease is electively located on the lumbar region, the back and the root of the limbs. In 3 cases the lesions were generalized and the patients looked like fatty "Michelin-Tire Babies". The course of the disease is always favourable, and associated pathologies remain exceptional: urticaria pigmentosa and psychomotor retardation have been reported in two cases of the generalized form. Histology is characterized by the presence of numerous smooth muscle fibres disseminated in the dermis and diversely oriented, sometimes in contact with hair follicles which retain their normal morphology. The differential clinical diagnosis is with naevocytic naevus, café-au-lait spots, mastocytosis and connective tissue hamartoma. Belatedly revealed forms of the disease must be distinguished from Becker's hamartoma, but it must be known that in certain cases the classification is so difficult that some authors have suggested that smooth muscle hamartoma and Becker's hamartoma are only two poles of a single spectrum of dysembryoplastic lesions involving to varying degrees the epidermic and hair structures. Finally, the distinction between the localized forms of late onset smooth muscle hamartoma and multiple leiomyomas "en plaques" remains difficult both anatomico-clinically and nosologically.

Adolescent↗