PubMed Health⌕ Search

Biomedical subjects

E Baselga

Publications and source records attributed to E Baselga.

At least 19 recordsLinked to original sources
PubMed1998

Dyskeratosis congenita with linear areas of severe cutaneous involvement.

Dyskeratosis congenita (DC) is a rare hereditary disorder of skin which may be associated with aplastic anemia. The pattern of inheritance is X-linked recessive in most instances, but autosomal dominant and autosomal recessive types have been documented. Reticulated hyperpigmentation usually is the first manifestation. The pigmentary changes may be limited to neck, upper chest, and proximal parts of the limbs initially but within affected areas the involvement is always diffuse. We report on a patient with typical diffuse cutaneous signs of dyskeratosis congenita superimposed with hyperpigmentation that was more pronounced along Blaschko's lines. To explain this phenomenon, we assume that the patient has the autosomal dominant type and that loss of heterozygosity occurred in a somatic cell giving rise to a population of cells that migrated along these lines during embryogenesis.

Adolescent↗
PubMed1998

[Mucocutaneous manifestations in acute HIV infection. 3 case reports].

We report three patients who developed a generalized rash with oral, genital or perianal ulcerations as a result of acute infection due to HIV. The primary infection was diagnosed by seroconversion (by means of EIA and Western blot techniques). Definitive diagnosis was established on days 52, 85 and 97 after the appearance of the rash. The p24 protein of the HIV was only detected in the early phase of the disorder in the two cases in which this study was carried out.

Acute Disease↗
PubMed1997

Preauricular skin defects. A consequence of a persistent ectodermal groove.

BACKGROUND: The term aplasia cutis is used to describe congenital localized defects of the skin. This affliction is the end result of various in utero events. Aplasia cutis of the face, although rare, has been associated with numerous dysmorphic features and described under many clinical terms. OBSERVATIONS: We studied 10 patients with oval, atrophic patches distributed in a linear pattern on the preauricular region of the face. Most of the defects were bilateral, and all consistently fell in an oblique line extending from the preauricular region to the angle of the mouth. This line corresponds to the region of fusion between the maxillary and mandibular facial prominences during embryonic development. CONCLUSIONS: This type of facial aplasia cutis may be the result of incomplete fusion of the ectodermal groove between the maxillary and mandibular facial prominences. Although other types of facial skin defects may share a similar pathogenic mechanism, they are distinct in that they occur in different regions and may have other abnormal facial features.

Biopsy↗
PubMed1997

Systemic lupus erythematosus presenting with red lunulae.

Red lunulae have been observed in association with a large spectrum of cutaneous and systemic diseases. We describe a 13-year-old girl with painful red lunulae of the fingernails who subsequently developed systemic lupus erythematosus. The appearance of Beau's lines suggested inflammation of the nail matrix. Oral prednisone produced significant improvement in the nail changes. To our knowledge, red lunulae have not been previously recognized as the presenting sign of systemic lupus erythematosus.

Adolescent↗
PubMed1997

Purpura in infants and children.

Hemorrhage into the skin (purpura) may result from abnormalities in any of the three components of hemostasis: platelets, plasma coagulation factors, and blood vessels. The morphology, size, and distribution of the hemorrhagic lesions are helpful diagnostic features. The main causes of purpura in the newborn and the more common hemorrhagic disorders in children are reviewed.

Adolescent↗
PubMed1997

Juvenile xanthogranuloma.

Juvenile xanthogranuloma (JXG) is a benign, self-healing disorder characterized by solitary or multiple yellow-red nodules on the skin and, occasionally, in other organs. It is predominantly a disease of infancy or early childhood, although adults may also be affected. Histologically, JXG represents an accumulation of histiocytes lacking Birbeck granules (non-Langerhans cells), which can be differentiated from Langerhans cells by specific staining techniques. Affected persons have normal lipid metabolism. JXG is therefore classified as a normolipemic non-Langerhans cell histiocytosis. The patient's general health is not impaired and, in the absence of associated conditions, the prognosis is excellent. Diagnosis is readily made in typical cases, but may be more difficult in unusual variants.

Adult↗
PubMed1997

Histopathological changes of primary HIV infection. Description of three cases and review of the literature.

The histopathological changes observed in the cutaneous rash of three patients who suffered the acute phase of HIV infection are described. In all three patients a perivascular and interstitial inflammatory infiltrate was present in the upper and mid-reticular dermis. In one biopsy isolated areas of epidermal necrosis were observed and in the two other biopsies a perifollicular inflammatory infiltrate was detected with perforation in one case. Furthermore, a periductal infiltrate was observed in one of these biopsies.

Adult↗
PubMed1997

Detection of Mycobacterium tuberculosis DNA in lobular granulomatous panniculitis (erythema induratum-nodular vasculitis).

OBJECTIVE: To determine, using polymerase chain reaction (PCR) amplification, if Mycobacterium tuberculosis complex DNA is present in the skin biopsy specimens of lobular granulomatous panniculitis. DESIGN: A retrospective descriptive study. SETTING: A university-based hospital. PATIENTS: From the 65 patients included in the study, we examined 72 paraffin-embedded skin biopsy specimens with a histologic diagnosis of erythema induratum or nodular vasculitis. The biopsy specimens were from the histopathological archives of the Departments of Dermatology and Pathology of the Hospital de la Santa Creu i Sant Pau, Barcelona, Spain, from 1976 to 1994. Twenty-two biopsy specimens were excluded from the final analysis because we could not amplify the internal control. MAIN OUTCOME MEASURES: Detection of a 123-base pair fragment of the IS6110 insertion sequence specific for M tuberculosis complex. RESULTS: The results of PCR amplification were positive for M tuberculosis complex DNA in 77% of the skin biopsy specimens. No significant difference could be detected with respect to the age of the patients, ulceration of the nodules, reactivity to purified protein derivative, abnormal results of a chest x-ray examination, personal and family history of tuberculosis, and PCR results. The presence and degree of necrosis on histologic examination were significantly higher in the PCR-positive group (P = .04). None of the following variables were associated with PCR results: presence of vasculitis, degree of granulomatous infiltrates, number of giant cells, and presence of well-organized granulomas. CONCLUSIONS: The DNA of M tuberculosis can be detected in a considerable number of skin biopsy specimens of lobular granulomatous panniculitis. None of the clinical and histologic variables evaluated could accurately predict the results of PCR amplification.

Adult↗
PubMed1996

[Usefulness of polymerase chain reaction for the diagnosis of Bazin erythema induratum].

BACKGROUND: Erythema induratum of Bazin (BEI), is included in the group of cutaneous granulomatous lobulillar panniculitis. The aethiopathogenic association between EI and tuberculosis can not rely on the clinicohistological features of these panniculitis and M. tuberculosis has never been isolated from BEI lesions. Detection of the mycobacterial DNA by PCR on cutaneous biopsy samples would allow to confirm this association. PATIENTS AND METHODS: Fourteen patients with clinical BEI were chosen retrospectively. Seventeen lesional biopsy samples were obtained, stained with the Kinyoun carbolfuchsin acid-fast technique and haematoxylin and eosin and tested by PCR. A fragment of the IS6110 insertion sequence specific of M. tuberculosis was amplified and confirmed by digestion with Sal I restriction endonuclease. The efficiency of the procedure, the presence of inhibitory substances and the preservation of DNA were checked by PCR of the beta-actin gene. RESULTS: M. tuberculosis DNA was detected in 12 of the 17 samples tested (70.5%) which corresponded to 10 of the 14 patients (71.4%). According to beta-actin PCR results, the rate of extracted DNA was inadequate on four of the five negative biopsies. CONCLUSIONS: The results of these series suggest the probable involvement of M. tuberculosis on the BEI pathogenesis and give support to the usefulness of the PCR in the diagnosis of this pathology concerning the need of specific treatment.

Adult↗
PubMed1996

Detection of M. tuberculosis complex DNA in a lesion resembling sarcoidosis.

We describe a tuberculin test (PPD) negative patient with a chronic cutaneous lesion with histological features resembling sarcoidosis, in whom M. tuberculosis complex DNA was detected in formalin-fixed paraffin-embedded tissue by polymerase chain reaction (PCR) amplification. The lesion cleared with antituberculous treatment.

DNA, Bacterial↗
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

Dermatomal lichenoid chronic graft-vs-host disease following varicella-zoster infection despite absence of viral genome.

Localized cutaneous graft-versus-host disease (GVHD) following a dermatomal distribution or in a pattern of Blaschko's lines. Some authors have postulated that dermatomal GVHD is triggered by a varicella-zoster virus infection, although in reported cases, there was no history of a preceding herpes zoster. We describe a case of GVHD localized to the exact dermatome of a culture-proven varicella-zoster virus infection. PCR analysis failed to detect persistence of viral genome in the affected skin.

Adolescent↗