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Biomedical subjects

J M De Moragas

Publications and source records attributed to J M De Moragas.

At least 19 recordsLinked to original sources
PubMed1996

Reiter's syndrome-like pattern in AIDS-associated psoriasiform dermatitis.

BACKGROUND: The prevalence of psoriasiform dermatitis in HIV-infected patients is similar to that in the general population, but its clinical severity and the immunosuppression in these patients pose special problems of therapeutic management. Furthermore, a distinctive clinical pattern has been reported in some cases. In order to assess these features in our clinical setting, we have done a retrospective study on the clinical records of all human immunodeficiency virus (HIV)-positive patients with psoriasiform dermatitis requiring systemic treatment. METHODS: The clinical records were reviewed of seven HIV-positive patients who were referred between 1988 and 1994 to a University Hospital Dermatology Department from an HIV-clinic because of psoriasiform dermatitis, resistant to topical treatment. RESULTS: The clinical appearance was rather uniform, with the following common features: facial seborrhea, flexural and acral involvement, with pustulosis of the palms and soles, and frequent arthritis. Lesions appeared in nonterminal stages of acquired immunodeficiency syndrome (AIDS). Three patients developed cutaneous lesions after the diagnosis of HIV infection was made and showed the most severe clinical involvement and arthritis. Etretinate, followed by RePUVA, proved to be the most effective systemic therapy prescribed, with only rare adverse effects. Methotrexate was shown to be effective, but it's use was accompanied by hematologic toxicity. Cyclosporine A treatment was moderately effective and was not associated with progression of AIDS. CONCLUSIONS: A characteristic Reiter-like clinical picture was observed in AIDS-related psoriasiform dermatitis. Etretinate and RePUVA were effective and safe in controlling the lesions. Physiopathologic mechanisms involved in the development of AIDS-related psoriasis might provide an explanation for the outstanding similarity of the clinical pattern in those patients.

Acquired Immunodeficiency Syndrome↗
PubMed1996

Expression of the p53 protein in sun-damaged skin.

The expression of the p53 protein in photodamaged skin from the face, forearm and neck of 18 subjects was compared to non-exposed skin taken at autopsy from the abdomens of 9 subjects of the same age. Paraffin sections and the polyclonal antibody CM1 were used with the ABC technique. The p53 protein was expressed in the nuclei of keratinocytes in 7 of the 18 samples obtained from sun-exposed skin and in 1 of 9 samples from non-exposed skin. The expression in sun-exposed skin can be due either to its overexpression due to DNA damage or to the appearance of mutant forms.

Adult↗
PubMed1996

Mycosis fungoides associated with unusual epidermal hyperplasia.

A 58-year-old white woman presented with widespread pruritic brownish plaques and hyperpigmented flexural lesions with a velvety appearance. On histopathological examination, the macules were diagnostic of mycosis fungoides, plaque stage, and the flexural lesions showed epidermal hyperplasia with a seborrhoeic keratosis-like appearance. There was intense mucin deposition and marked reduction of elastic fibres in the papillary dermis, as well as a moderately dense dermal lymphoid infiltrate composed of CD4+ T cells with occasional atypia and focal epidermotropism. The clinical and pathological manifestations of cutaneous T-cell lymphomas, including mycosis fungoides (MF), may show considerable variation. Apart from the three classic stages, a number of unusual clinical presentations and a broad spectrum of histopathological findings have been reported in the literature. In this report, a case of MF, plaque stage, with flexural lesions clinically reminiscent of (pseudo)acanthosis nigricans is presented. On histopathological examination, there was epidermal hyperplasia with a seborrhoeic keratosis-like appearance, with intense deposition of mucin and marked reduction of elastic fibres in the papillary dermis, together with a lymphoid infiltrate composed mainly of CD4+ cells with occasional atypia and epidermotropism. Cytokines produced by the lymphocytes in the infiltrate might account for the epithelial and dermal changes that characterize this peculiar variant of MF.

Female↗
PubMed1996

Generalized acquired cutis laxa associated with coeliac disease: evidence of immunoglobulin A deposits on the dermal elastic fibres.

Acquired cutis laxa (ACL) is an uncommon elastolytic disorder of unknown aetiology. In rare instances, ACL has been reported in association with autoimmune diseases and dermal deposit of immunoglobulins, suggesting that destruction of elastic tissue may be immunologically mediated. We report a 35-year-old man with generalized acquired cutis laxa (GACL) associated with a persistent papular erythematous eruption that histopathologically showed some resemblance to dermatitis herpetiformis. A marked reduction and degeneration of dermal elastic fibres was noted in biopsies from loose-hanging skin. Direct immunofluorescence from non-inflammatory loose skin revealed granular immunoglobulin A (IgA) deposits at the basement membrane zone and fibrillar IgA deposits in the dermal papillae. IgA deposits were also observed on the elastic fibres of the reticular dermis. Electron microscopy of skin from the submammary fold revealed fragmented elastic fibres, partial absence of peripheral microfibrils and abundant neutrophils, some of which were degranulated and adjacent to elastic fibres. Immunoelectron microscopy of an erythematous papule revealed IgA deposits around dermal elastic fibres. Antigliadin, antireticulin and antiendomysium antibodies were present. Jejunal biopsies showed a gluten-sensitive enteropathy. A possible IgA-mediated immune mechanism for the development of GACL in our patient is suggested.

Adult↗
PubMed1995

[Congenital spontaneously regressive cutaneous Langerhans cell histiocytosis with bone involvement (eosinophilic granuloma)].

INTRODUCTION: The classification of Langerhans' cell histiocytosis into 5 forms does not cover all types of clinical presentations. We observed a patient with inborn Langerhans' cell histiocytosis involving the skin and bone tissue. The clinical course was benign during the first two years of the patient's life. CASE REPORT: Skin lesions noted at birth resolved spontaneously but recurred twice in a more benign form. Histology examination showed "band" infiltration of the papillary derma composed of Langerhans' cells in direct contact with the basal epidermal layer and a few eosinophils. Immunolabelling was intensely positive for protein S100 confirming the diagnosis of Langerhans' cell histiocytosis. Extension was limited to a lytic lesion in the lower part of the left tibia which was treated by curettage. DISCUSSION: In our opinion, Langerhans' cell histiocytosis covers a wide range of clinical presentations. Our case was remarkable because of the benign course despite its congenital nature and dissemination to skin and bone. Prolonged surveillance of Langerhans' cell histiocytosis is required for patients without any apparent prognosis factors.

Bone Diseases↗
PubMed1993

Acute febrile neutrophilic dermatosis (Sweet's syndrome).

BACKGROUND: Sweet's syndrome is well recognized and not infrequently diagnosed in Spain; however, the range of clinical and pathologic expression may not have been fully realized. METHODS: We reviewed 30 consecutive Spanish cases of Sweet's syndrome diagnosed in our department from 1979 to 1990, with special attention to clinical and histopathologic findings. RESULTS: Distinctive clinical features in our series included oral mucosa lesions in four patients (13%), development of pathergy phenomenon in one case, concurrent nodular lesions resembling erythema nodosum on the limbs in nine cases (30%), and lung involvement in two patients. Infectious disease and drug treatment were recorded as possible triggering factors of Sweet's syndrome in eight and seven patients respectively. Associated underlying systemic disorders were present in 15 (50%) of our patients. The most frequent associations were hematologic neoplasia in four patients, solid neoplasia in two, and chronic idiopathic inflammatory bowel disease in three patients. Dressler's syndrome and sicca syndrome were found in one patient each. Histopathologic studies of skin biopsy specimens obtained at presentation disclosed typical features of Sweet's syndrome in all cases. Epidermal involvement, with variable degrees of spongiosis, exocytosis of polymorphonuclear leukocytes and keratinocyte necrosis, was a prominent feature in 83% of biopsy specimens. CONCLUSIONS: Further characterization of the clinicopathologic spectrum of Sweet's syndrome is necessary as the recognition of the full spectrum of this syndrome will improve our diagnostic abilities and provide a solid clinical basis for prospective studies that allow dissection of the intricate patho-mechanisms involved in this fascinating disorder.

Adult↗
PubMed1990

[Effect of minoxidil on DNA synthesis in cultured fibroblasts from healthy skin or keloids].

A potential therapeutic role of minoxidil in fibrotic or sclerotic conditions, such as keloids, has been suggested on the basis of its reported ability to inhibit the proliferation of human dermal fibroblasts. We have studied the effect of minoxidil on 3H-deoxythymidine uptake in cultures of human dermal fibroblasts derived from lesional and non-lesional skin from patients with keloids. The effect of the addition of fetal bovine serum, plasma and growth factors (insulin, EGF, PDGF, FGF and beta-TGF) to the medium has also been studied. Minoxidil, at concentrations ranging from 500 to 1,000 microM., caused an increase in DNA synthesis, which was proportional to the initial degree of fibroblast activation. Concentrations higher than 1,000 microM. caused a cytotoxic effect. Some reservations arise on the potential therapeutic use of minoxidil in conditions characterized by increased fibroblast proliferation, given the narrow margin between activation of DNA synthesis and cytotoxicity we have found in our experimental model.

Cell Division↗
PubMed1990

[Tubular apocrine adenoma. Description of 4 cases].

We present four new cases of tubular apocrine adenoma. Clinical histopathological and ultrastructural findings are discussed. The presence of carcinoembryonic antigen (CEA) has been studied in three cases of tubular apocrine adenoma. This antigen was found predominantly in the lumen of the ducts and in the apical portion of the luminal cells. This pattern is similar to the distribution described in normal sweat glands and in other adnexal tumors.

Adenoma↗
PubMed1989

[Woolly hair nevus associated with systemic epidermal nevus].

A 22-year-old female with two woolly hair nevus of the scalp and a systematized epidermal nevus is reported. This association has been rarely described. Scanning electron microscopy of the woolly hair demonstrates oval and triangular hair shaft sections and longitudinal grooves. The structure of the cuticle was not disturbed.

Adult↗
PubMed1989

[Kinetics of eicosanoid formation in human epidermal cells in suspension].

Increased levels of eicosanoids in lesions of psoriasis suggest activation of arachidonic acid metabolism in different cell types play a physiopathologic role. The contribution of each cell type present in psoriasis has been the subject of some controversy, which has led us to study the metabolism of arachidonic acid in human epidermal cells suspensions. 12-HETE was found to be the main product, followed by PGE2 and PGF2 alpha; no 5-lypoxygenase products were found. Thus epidermal cell contribution to LTB4 levels present in plaques of psoriasis appears to be irrelevant. Conversely, increased levels of 12-HETE probably do result from arachidonic acid metabolism by epidermal cells.

Arachidonic Acids↗
PubMed1988

[Pigmented dermatofibrosarcoma protuberans (Bednar's tumor)].

Pigmented dermatofibrosarcoma protuberans, first described by Bednar under the term storiform neurofibroma, is an infrequent neoplasm accounting for 1 to 5% of cases of dermatofibrosarcoma protuberans. Bednar's tumor is composed of spindle shaped cells arranged in a distinctive storiform or carthwheel pattern, and melanin-containing dendritic cells scattered within the tumor. The differential diagnosis with (non-pigmented) dermatofibrosarcoma protuberans is based on the presence of this population of pigmented cells. Two cases of pigmented dermatofibrosarcoma protuberans (Bednar's tumor) are reported here in, and a discussion follows on the clinicopathological features of this neoplasm and the different hypotheses on its histogenesis.

Adult↗
PubMed1988

[Actinic reticuloid. Immunohistochemical study].

A patient that fulfilled the clinical and histological features of actinic reticuloid has been studied. He presented a positive patch test for fragance mix. Typical cutaneous lesions were reproduced with a solar simulator. The immunochemical studies of the inflammatory infiltrates of these induced lesions gave a higher percentage of T8 in relationship to T4 lymphocytes. Most of the cells were HLA-DR positive. No clonality of these lymphocytes was detected. In the peripheral blood lymphocytes, an increased percentage of T8 lymphocytes was also observed.

Exudates and Transudates↗
PubMed1987

[KID syndrome (keratitis-ichthyosis-deafness)].

The KID syndrome is characterized by congenital ichthyosis, vascular keratitis and neurosensorial deafness. We report a 17 year old female patient, the first case of KID syndrome in Spanish literature. Red, dry, scaling skin was present at birth with sparse hair. At the age of six, malar erythema was prominent, with perioral ragades and onset of progressive neurosensory deafness. At the age of ten, vascularizing keratitis developed. At 12, treatment with etretinate failed to improve the ichthyosis. We review the clinical, pathological and analytical features of KID syndrome and discuss its relationship to other ichthyoses.

Adolescent↗
PubMed1987

[Linear sebaceous nevus syndrome].

We report a patient that fulfills the clinical features of the linear nevus sebaceous syndrome. This syndrome was defined by the triad: Linear nevus sebaceous, seizures and mental retardation. Further descriptions suggested an association with hamartomas of mesenchymal structures and increased risk of neoplasm transformation. Previously reported cases are reviewed. The complexity of classification of this neurocutaneous syndrome is discussed.

Facial Neoplasms↗