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Ramón Ruiz-Maldonado

Publications and source records attributed to Ramón Ruiz-Maldonado.

12 recordsLinked to original sources
PubMed2004

Medallion-like dermal dendrocyte hamartoma: a new clinically and histopathologically distinct lesion.

Dermal dendrocyte hamartomas are extremely rare; only two examples have been described with clinical features different from our cases and with incomplete immunohistochemical characterization. We report three female patients presenting a medallion-shaped, well-defined, slightly atrophic and asymptomatic congenital lesion. All 3 patients showed a fusiform-cell proliferation. Immunohistochemistry was positive for CD34, factor XIIIa, and fascin. Electron microscopy showed typical features of dermal dendrocytes. We believe that the lesions described represent a new, clinically and histopathologically distinct lesion originating in dermal dendrocytes. We propose to name it medallion-like dermal dendrocyte hamartoma.

Antigens, CD34↗
PubMed2003

Phakomatosis pigmentovascularis II A and II B: clinical findings in 24 patients.

Nearly 200 cases of phakomatosis pigmentovascularis (PPV) have been reported worldwide, most of them of Japanese origin. There are 5 types and 10 subtypes of PPV. Its etiology might be explained by the twin spotting phenomenon. The relative frequency of PPV at the National Institute of Pediatrics was 5.8 per 100,000 pediatric patients and 0.634 per 100,000 dermatological patients. We report 24 cases of PPV with an average follow up of 5 years and the following findings: PPV type II A in 4 male and 2 female patients with melanosis bulbi in 3 and glaucoma in 1. PPV type II B in 7 male and 11 female patients, with melanosis bulbi in 9, glaucoma in 9, iris mammillations in 2, Sturge Weber syndrome in 6 female patients, and Klippel-Trenaunay syndrome in 2 males, hemifacial, hemicorporal, or limb hypertrophy without venous insufficiency in 6 female and 4 male patients. During the follow-up time of 60 months, progressive fading of melanotic and vascular macules were observed in 7 patients. No other types of PPV were found. Systemic involvement in PPV was related to the body surface area affected by the vascular macules. Ectodermal and mesodermal migration disorders might be involved in the pathogenesis of PPV.

Child↗
PubMed2002

Exanthems in hospitalized pediatric patients: concordance between pediatric and dermatological diagnoses.

BACKGROUND: Exanthems in children often represent a diagnostic challenge. PURPOSE: To determine the concordance between pediatric and dermatological diagnoses of exanthems. DESIGN: Prospective study. PROCEDURE: Exanthems that appeared in hospitalized pediatric patients were diagnosed by pediatricians. Pediatric dermatologists, by consensus, either confirmed or modified the diagnoses. Whenever possible, laboratory tests were used to confirm the final clinical diagnoses. Age and evolution were compared with the Kruskal-Wallis test; the kappa coefficient was used to determine concordance. SETTING: Institutional tertiary referral pediatric care center. RESULTS: Concordance between pediatric and dermatological diagnoses was found in only 44 patients. When pediatric and dermatological diagnoses of exanthems were classified, the concordance between both diagnoses was very low (kappa = 0.165). CONCLUSIONS: Pediatric specialists, other than dermatologists, failed to diagnose common skin diseases. This may be a consequence of insufficient training in dermatology by medical schools and pediatric residency.

Adolescent↗
PubMed2002

Oral desensitization in papular urticaria in children.

Papular urticaria (PU) is among the commonest skin ailments in children. Induced specific desensitization to insect bites is theoretically an effective means of prevention of PU. In this double blind placebo controlled study, an oral vaccine prepared from insect saliva was compared with placebo (stable vaccine solvent). Vaccine and placebo effectiveness were tested by counting active PU lesions, serum eosinophils, and IgE, before and after 4 months of treatment. Statistically significant differences between oral vaccine and placebo were not found in the clinical or the immunological variables tested. We conclude that, although a lack of oral vaccine efficacy was suspected, larger study samples are needed to strengthen our conclusion.

Child, Preschool↗
PubMedDate not supplied

Relapsing polychondritis: a pediatric case.

A pediatric case of relapsing polychondritis is reported. Diagnostic features were recurrent episodes of painful inflammation of ear cartilage, episcleritis, and atopic disease. A good therapeutic response was obtained with systemic corticosteroids, dapsone, and methotrexate.

Child↗
PubMedDate not supplied

Streptococcal exanthem in a blaschkolinear pattern: clinical evidence for genetic mosaicism in hypomelanosis of ito.

Due to the presence of two different clones of cells in early embryogenesis, numerous congenital and acquired dermatoses have a linear distribution following the lines of Blaschko. Acquired inflammatory skin diseases are rarely observed in linear patterns. Our patient was born with macrocephaly, left eye glaucoma, and a left facial and contralateral corporal hemihypertrophy, cerebral dysgenesis, and skeletal abnormalities. Hypopigmented S-shaped linear macules on the trunk and linear streaks on the arms and legs were compatible with hypomelanosis of Ito. At 5 years of age the patient presented with an erythematous follicular exanthem compatible with scarlet fever exclusively in the lines of Blaschko. This fact suggests a genetic mosaicism.

Child, Preschool↗
PubMedDate not supplied

Infantile myofibromatosis: a cause of leg length discrepancy.

An infant with self-healing, multifocal cutaneous infantile myofibromatosis with leg-length discrepancy as a sequela is reported. This condition should be suspected in infants with one or more firm or hard nodules in the skin, subcutaneous tissue, bone, muscle, or viscera. The histopathologic picture is diagnostic. Treatment and prognosis depend on the extension and location of the tumors.

Diagnosis, Differential↗
PubMedDate not supplied

Discoid lupus erythematosus in children: clinical, histopathologic, and follow-up features in 27 cases.

Among 27 pediatric patients with a clinicopathologic diagnosis of discoid lupus erythematosus (DLE), 15 had localized cutaneous lesions and 12 had disseminated lesions. During a mean follow-up period of 36 months, seven patients (26%) developed systemic lupus erythematosus (SLE). Four of these patients were less than 10 years of age. No correlation was found between localized and disseminated lesions and evolution to SLE. Three of four patients with a positive family history for rheumatoid disease developed SLE (p < 0.05). Hyperpigmentation was significantly more frequent (p < 0.04) in children less than 10 years of age. There was a female predominance of 5:1 among patients less than 10 years of age. Our findings suggest that onset of DLE prior to 10 years of age does not indicate a greater risk of developing SLE. The occurrence of localized or disseminated lesions does not seem to influence the outcome.

Adolescent↗
PubMedDate not supplied

Epidermal nevus syndromes: clinical findings in 35 patients.

Of the patients with epidermal nevi, 10-18% may have disorders of the eye, nervous, and musculoskeletal systems. A predisposition to malignant neoplasms in ectodermal and mesodermal structures may also be found. There are six different epidermal nevus syndromes described so far: Proteus, congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome, phakomatosis pigmentokeratotica, sebaceous nevus, Becker nevus, and nevus comedonicus. Thirty-five patients with epidermal nevus syndrome seen at the National Institute of Pediatrics in Mexico City during a 31-year period are described. This syndrome represented 7.9% of 443 patients with epidermal nevi; its relative frequency was 1 case per 11,928 pediatric patients and 1 case per 1080 dermatologic patients. Nine epidermal nevus syndrome patients (26%) had Proteus syndrome. Sebaceous nevus syndrome was found in six patients (17%), while the nevus comedonicus syndrome was found in three (8%). Two patients were diagnosed with phakomatosis pigmentokeratotica and one patient with congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome. This is the first report of phakomatosis pigmentokeratotica and congenital hemidysplasia with ichthyosiform nevus and limb defect syndrome in Mexican patients. One patient had an inflammatory linear verrucous epidermal nevus with systemic involvement. Thirteen patients (37%) had keratinocytic nevi with systemic involvement. We propose the keratinocytic nevus syndrome to be defined as the association of a keratinocytic nevus with neuronal migration and/or musculoskeletal disorders in addition to a higher risk for mesodermal neoplasms.

Abnormalities, Multiple↗