[Management of giant congenital nevi].
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Biomedical subjects
Publications and source records attributed to R Ruiz-Maldonado.
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BACKGROUND: Extensive epidermal necrosis in newborn infants is an unusual event of heterogeneous cause. OBJECTIVE: The objective of this article is to describe what seems to be a previously unrecognized lethal disease. METHODS: The clinical and histopathologic features of three premature infants, two of them nonidentical twins, and the autopsy findings of one of them were analyzed. RESULTS: Intrauterine lethal epidermal necrosis with hair follicle calcification, except for the face, hands, feet, elbows, and knees, was present in all three patients. Some histopathologic features were suggestive of epidermal apoptosis. CONCLUSION: We propose that the clinicopathologic alterations in our patients represent a new condition that may be caused by massive epidermal apoptosis.
Oral retinoids are molecules derived from vitamin A that represent one of the most important steps forward in dermatologic therapeutics in the present century. The treatment of acne, severe psoriasis, and severe disorders of keratinization, prevalent diseases in children and adolescents, have radically changed since the advent of oral retinoids. Like most highly-effective medications, oral retinoids also have important untoward effects. Specialists, and in particular, dermatologists and pediatricians should be prepared to maneuver the delicate balance between therapeutic efficacy and side effects in order to give the pediatric patient the maximum benefit with the lowest possible risk.
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Postinflammatory hypopigmentation and hyperpigmentation are frequently encountered problems and represent the sequelae of various cutaneous disorders as well as therapeutic interventions. However, the underlying mechanisms and the variability individuals show for developing hypopigmentation or hyperpigmentation are not well understood. The authors propose an inherited individual chromatic tendency that is based on "weak" or "strong" melanocytes and their tendency to respond to trauma or inflammation with either hypopigmentation or hyperpigmentation. Clinical examples and management of both hypopigmentation and hyperpigmentation are discussed.
BACKGROUND: Dermabrasion is a painful procedure that requires the use of anesthetics, which, in their application, adds to the patient's pain. OBJECTIVE: To describe the use of saturated phenol as a transient local anesthetic for manual dermabrasion. METHODS: Manual dermabrasion of small areas (4 cm2) was performed in 12 patients using saturated 88% phenol as a local anesthetic. RESULTS: Cosmetic results were good or excellent in 11 patients. Pain was minimal in eight patients, moderate in three, and absent in one. CONCLUSION: Saturated phenol is an effective and safe anesthetic alternative for manual dermabrasion of small areas.
BACKGROUND: Magnetic resonance imaging (MRI) findings suggestive of neurocutaneous melanosis (NCM) have been reported in asymptomatic patients with giant congenital melanocytic nevi (GCMN). OBJECTIVE: To investigate the presence of NCM and the clinical neurologic status of patients with GCMN involving the head an neck. METHODS: Thirteen patients with GCMN involving the head and neck were clinically examined by pediatric specialists in dermatology, ophthalmology and neurology. Electroencephalograms, noncontrasted and contrasted computerized tomography (CT) scans and MRI were performed. RESULTS: Eleven of 13 patients with GCMN of the head and neck previously considered asymptomatic were found to present mild but evident neurologic alterations. No signs of NCM were found in the CT scans or in the MRI. CONCLUSIONS: Patients with GCMN of the head and neck may have associated neurologic alterations not related to the presence of neurocutaneous melanosis.
The increasing incidence during the last decades and the projection for the future places malignant melanoma (MM) among the most important public health problems world-wide. Given the limited success of therapy for MM, for the time being the fighting strategy against MM should focus mainly on prevention and early detection.
BACKGROUND: Because of their size and the possibility of complications, giant hemangiomas represent a therapeutic challenge. Various forms of treatment have been used, with variable results, including surgery, embolization, lasers, pentoxifylline, and corticosteroids. Interferon alfa has been used successfully to treat life-threatening hemangiomas, possibly by means of its antiangiogenic activity. OBSERVATIONS: We treated 7 infants with organ-interfering and/or life-threatening giant hemangiomas with subcutaneous injections of 3 million U/m2 per day of interferon alfa-2b during the first month and subsequently every 48 to 72 hours, depending on the evolution in each case. The treatment lasted from 3 to 12 months. In 2 patients, interferon alfa-2b was administered while prednisone therapy was being tapered. In all 7 patients, there was considerable reduction of the volume of the hemangiomas and remission of their complications. All patients presented with fever, neutropenia, and an increase in serum aminotransferase levels. The patients who received interferon alfa-2b and prednisone seemed to improve at a faster rate. CONCLUSIONS: Interferon alfa-2b is a good option for the treatment of patients with steroid-resistant, organ-interfering and/or life-threatening giant hemangiomas. In our experience, the adverse effects were transient and minor and did not require the interruption of the treatment.
BACKGROUND: Hydroa vacciniforme (HV) is a disease of unknown origin characterized by erythema, vesicles, necrosis, and varicelliform scars in light-exposed skin. Systemic involvement is absent. A few patients have been reported with "severe HV" with systemic involvement, development of non-Hodgkin's lymphoma, and a poor prognosis. OBJECTIVE: Our purpose was to characterize and differentiate our patients' disease from HV. METHODS: We performed a retrospective clinicopathologic study of 14 children previously diagnosed as having "severe HV." RESULTS: The extension and severity of the cutaneous lesions, fever, wasting, failure to thrive, hepatosplenomegaly, vasculitis, panniculitis, and potential development of lymphoma are features that clearly differentiate edematous scarring vasculitic panniculitis from HV. CONCLUSION: Edematous scarring vasculitic panniculitis is a novel multisystemic disease with malignant potential that is not related to classic HV.
We describe a 2 1/2-year-old girl with encephalocraniocutaneous lipomatosis. The dysmorphologic manifestations in the skull, brain, skin, and eyes associated with a normal karyotype suggested the diagnosis of this rare neurocutaneous syndrome.
We examined the prevalence of IgE and IgG4 class antibodies to the saliva of Aedes communis and Aedes aegypti mosquitoes in the sera of three groups of exposed children using a sensitive immunoblot method. The frequencies of IgE antibodies to the major 36-kD A. communis and A. aegypti saliva antigens ranged from 82 to 90% in the 20 Finnish, 17 Kenyan, and 20 Mexican children. The corresponding IgG4 antibody frequencies were 85, 41, and 20%, respectively. The nonexposed 20 Icelandic children did not show IgE or IgG4 antisaliva antibodies. Several of the Finnish children showed also IgE and IgG4 antibodies to a 22-kD A. communis saliva antigen. The Finnish children abnormally sensitive to mosquito bites had frequently IgE and IgG4 antibodies to the 22-kD A. communis saliva antigen, suggesting that these antibodies play a role in the pathogenesis of immediate cutaneous mosquito bite reactions. In contrast to this, no increase was found in the A. aegypti antibody frequencies in the Kenyan and Mexican children with papular urticaria, suggesting that humoral immune response to A. aegypti saliva is not involved in the development of this disorder. The present results show that humoral IgE and IgG4 immune responses to Aedes mosquito saliva antigens is common in children living both in temperature and tropical zones. The IgE antibodies seem to be involved in the immediate mosquito bite whealing, and the occurrence of the IgG4 subclass antisaliva antibodies might be an indicator of intense mosquito bite exposure.
The frequency of different malignant cutaneous tumors (MCTs), primary and metastatic, in children is not known. We reviewed all MCTs, primary and metastatic, seen during a 20-year period in a large general pediatric hospital. Fifty-three MCTs, 36 primary and 17 metastatic, were diagnosed in 36,207 pediatric dermatology patients. The incidence was 1.4 per 1000 patients. The relative frequency of occurrence of the different tumors was as follows: rhabdomyosarcoma, 25%; lymphomas, 19%; basal cell carcinoma, 13%; leukemia, 13%; neuroblastoma, 10%; malignant melanoma, 6%; squamous cell carcinoma, 6%; unclassified sarcomas, 4%; epithelioid schwannoma, 2%; ependymoma, 2%. The mean follow-up was 3 years; 48% died, 27% were lost to follow-up, and 25% are under control. We conclude that primary and metastatic MCTs in children are rare. Their types differ from MCTs in an older age population. MCTs in children are associated with a high mortality rate, often related to late recognition.
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An infant had a 3 x 1.5-cm congenital, slow-growing, lobulated tumor below the right inferior eyelid. The mass transilluminated, and needle aspiration yielded tears. Dacryocystography showed a large cystic area connected with the lower lacrimal canaliculum. Histologically, the tumor proved to be a dermoid cyst. This association has not been reported previously. Surgical excision and suture of the pedicule resulted in permanent cure.
Two children with acute lymphoblastic leukemia undergoing chemotherapy had a special form of scabies characterized by fine scaling of the scalp simulating seborrheic dermatitis. Pruritus was mild or absent. Immunocompromised children and adults should receive whole-body and scalp antiscabietic treatment.