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A Guerra Tapia

Publications and source records attributed to A Guerra Tapia.

16 recordsLinked to original sources
PubMed2003

[Acromelanosis].

Acromelanosis is an independent disease entity, characterized by increased skin pigmentation, usually located on the acral areas of the fingers and toes. It is mostly seen in newborns or during the first years of life. Only a few cases of this entity have previously been described in the medical literature. In some of these cases, the cutaneous lesions spread to affect large parts of the skin surface. A possible association with other benign and malignant diseases has been proposed. Differential diagnosis must be made with a wide variety of systemic and dermatologic conditions, especially dermatoses with acral distribution of macular hyperpigmentation, including acropigmentation. In this article, we report a new case of acromelanosis in a 5-week-old girl showing two peculiar clinical features: associated melanosis of the genital mucosa and stabilization of the lesions after an initial phase of progression and proximal spread. In addition, the most important features of this rare cutaneous disease are discussed.

Female↗
PubMed2003

Pustular generalized perforating granuloma annulare.

We report a 84-year-old man with a 13-year history of recurrent generalized asymptomatic pustular lesions. Histology revealed areas of necrobiosis surrounded by palisading granulomas and transepidermal and follicular elimination of the necrobiotic material. A dense infiltrate of neutrophils was also found. Although 26% of patients with generalized perforating granuloma annulare have some yellow pustule-like papules, these correspond histologically to the yellow viscous necrobiotic material extruding through the epidermis and not to a real neutrophilic infiltrate. This is the first case report of perforating granuloma annulare with recurrent generalized pustular lesions with a dense infiltrate of neutrophils.

Aged↗
PubMed2002

[Hypohidrotic ectodermal dysplasia: A cause of fever of unknown origin].

The term ectodermal dysplasias includes many disorders that share some clinical features such as involvement of one or several ectodermal structures and congenital origin. Currently, 154 different types divided into 11 clinical subgroups (Freire Maia classification 1994) have been described. The most frequent entity is hypo- or anhidrotic ectodermal dysplasia (Christ-Siemens-Touraine syndrome). This is a rare hereditary disease whose main characteristic is the absence, or more often the reduction, of sweat glands, leading to an increase in body temperature together with anomalies of the epidermis and its appendages (hair and nails). We present a case of hypohidrotic ectodermal dysplasia in a premature 18-month-old boy who was referred to our department because of markedly dry skin since birth and recurrent eczematous and lichenification lesions that had been successfully treated with topical corticosteroids. Physical examination revealed mild alopecia with sparse and fine blonde hair and the absence of dental alveoli. The boy's mother had noticed slight sweating and episodes of fever without clinical symptoms, which were more frequent in summer. Hypohidrotic ectodermal dysplasia should be included in the differential diagnosis of fever of unknown origin.

Child, Preschool↗
PubMed1998

[Validation of the Spanish version of the diagnostic questionnaire of the Working Group on Atopic Dermatitis of the United Kingdom].

BACKGROUND: Although there are several diagnostic questionnaires for atopic dermatitis (AD), they have not yet been validated or cannot be used in population studies. Our objective was to validate the questionnaire developed by United Kingdom Working Party's Diagnostic criteria for Atopic Dermatitis (UKWPDCAD). METHODS: Validation was developed in two steps: first, the questionnaire was adapted by means of translation and back-translation. Second, validation was conducted with 237 patients referred to the hospital with diagnoses unknown to the investigators. Patients were independently assessed by the investigators who were blinded to the questionnaire. RESULTS: A total of 102 cases of AD were diagnosed among the 237 patients. The questionnaire showed a sensitivity of 76.5%, with a 95% confidence interval (CI) of 66.8%-84.1%. The specificity was 90.4% (CI = 83.8%-94.6%); the positive predictive value was 85.7% (CI = 76.4%-91.8%), and the negative predictive value 83.6% (CI = 76.3%-89%). CONCLUSIONS: The spanish version of the questionnaire has shown a high diagnostic accuracy, similar to the original written in english. We consider it a useful tool to be used in frequency studies of AD in large general populations.

Adolescent↗
PubMed1997

Lichen myxoedematosus associated with human immunodeficiency virus infection--report of two cases and review of the literature.

Lichen myxoedematosus (LM), or papular mucinosis, is an uncommon papular eruption caused by the dermal deposition of mucin. Three clinical forms can be distinguished, namely localised, disseminated (involving more than one site), and generalised LM, the last is called scleromyxoedema, and demonstrates erythema and skin sclerosis. Paraproteinaemia, often consisting of an abnormal IgG with lambda light chains, is usually present in patients with LM. Visceral involvement has also been documented. An association between LM and human immunodeficiency virus (HIV) infection has been reported recently. We now describe two further HIV-positive patients with LM and present a review of the literature regarding this association.

Adult↗
PubMed1996

Hepatoerythropoietic porphyria: relationship with familial porphyria cutanea tarda.

Hepatoerythropoietic porphyria is characterized by an early beginning of severe photosensitivity, with an increase in urinary uroporphyrin excretion and other porphyrins, high isocoproporphyrin fecal levels and an accumulation of protoporphyrin in erythrocytes. It is caused by a dramatic decrease in the activity of the uroporphyrinogen decarboxylase. We report a clinical, biochemical and enzymatic study in a family, where a 2-year-old girl suffers from a hepatoerythropoietic porphyria, and the patient's maternal uncle from a porphyria cutanea tarda. We discuss the relationship between these diseases and their known mutations.

Child, Preschool↗
PubMed1991

Cutaneous lesions in severe combined immunodeficiency: two case reports and a review of the literature.

Two patients with severe combined immunodeficiency (SCID) in whom cutaneous lesions were the first clinical feature were studied. Neither the morphology nor the histology of the lesions was uniform, although we have noted some common findings that can, in subsequent cases, lead us to suspect SCID. The immunologic defects were not uniform, representing the two poles of the spectrum of SCID. We believe that early recognition of the skin lesions is very important, since the patient's life expectancy can be increased by a bone marrow transplantation (1).

Female↗
PubMedDate not supplied

Ichthyosis: the skin manifestation of multiple sulfatase deficiency.

Juvenile sulfatidosis (Austin type) or multiple sulfatase deficiency is an extremely rare autosomal recessive disorder affecting the activity of many sulfatases: arylsulfatase A, several mucopolysaccharide sulfatases, and steroid sulfatase. Certain aspects of the clinical phenotype can be attributed mainly to a deficiency of one specific sulfatase. Most patients develop metachromatic leukodystrophy caused by arylsulfatase A deficiency, dysostosis multiplex by mucopolysaccharide sulfatase deficiency, and ichthyotic skin by steroid sulfatase deficiency. We describe a 7-year-old boy with developmental delay from 7 months of age, progressive spastic quadriparesis, and coarse facial features. By 27 months of age, an ichthyotic rash had developed on the limbs, trunk, and scalp. A skin biopsy specimen revealed hyperkeratosis with a normal granular layer. The diagnosis of multiple sulfatase deficiency was demonstrated by measuring sulfatase activities in fresh leukocytes: there were large deficiencies of arylsulfatase A and B plus reduced arylsulfatase C. The ichthyosis associated with multiple sulfatase deficiency has an autosomal recessive inheritance, is caused by steroid sulfatase deficiency, and the scaling is sometimes milder than in X-linked recessive ichthyosis. This could reflect the residual activity of steroid sulfatase in some cases.

Child↗