Interferon alpha-2B in juvenile hyaline fibromatosis.
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Biomedical subjects
Publications and source records attributed to R Ruiz-Maldonado.
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OBJECTIVE: To characterize the clinical features, response to therapy, evolution and prognosis of cutaneous mastocytosis in children. BACKGROUND: Mastocytosis in children, instead of being induced by a potentially oncogenic c-kit mutation, is probably a clonal disease with benign prognosis. METHODS: The clinicopathological features, evolution and response to treatment were analysed in 71 children with mastocytosis. RESULTS: There were 53 (75%) cases of urticaria pigmentosa, 12 (17%) cases of mastocytoma, and six (8%) cases of diffuse cutaneous mastocytosis. In 92% of cases disease onset was in the first year of life. There was a male predominance 1.8 : 1. Treatment did not modify the disease evolution. Eighty per cent of patients improved or had spontaneous resolution of the disease. CONCLUSION: The most frequent clinical form of mastocytosis was urticaria pigmentosa followed by mastocytoma and diffuse cutaneous mastocytosis. Darier's sign was present in 94% of cases. A negative Darier's sign does not rule out mastocytosis. In contrast to adults, mastocytosis in children usually has a benign course making sophisticated or invasive diagnostic tests unnecessary. A classification of paediatric cutaneous mastocytosis is proposed.
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BACKGROUND: Leprosy is an infectious disease with two polar forms, tuberculoid leprosy (TL) and lepromatous leprosy (LL), which are dominated by T-helper (Th) 1 and Th2 cells, respectively. High concentrations of prostaglandin E2 produced by the inducible enzyme cyclooxygenase type 2 (COX-2) in LL could inhibit Th1 cytokine production, contributing to T-cell anergy. OBJECTIVES: To compare the COX-2 expression in LL and TL. METHODS: Skin biopsies from 40 leprosy patients (LL, n = 20; TL, n = 20) were used to determine by immunohistochemistry and automated morphometry the percentage of COX-2 immunostained cells. RESULTS: Most COX-2-positive cells were macrophages; their percentages in the inflammatory infiltrate located in the papillary dermis, reticular dermis and periadnexally were significantly higher in LL than TL (P < 0.001 by Student's t-test). CONCLUSIONS: The high expression of COX-2 in LL may be related to high prostaglandin production contributing to T-cell anergy.
Desmoplasia has been described in melanoma and Spitz naevus but not in giant congenital melanocytic naevus (GCMN). In melanoma desmoplasia is associated with a better survival. Four paediatric patients with hard, ligneous, progressively hypopigmented and alopecic GCMN were seen among 143 cases of GCMN at the Department of Dermatology of the National Institute of Paediatrics, Mexico City. Clinically, induration was progressive in three patients and regressive in one. Pigmentation was regressive in all. Histopathologically, all four patients showed intense dermal fibrosis, scarce naevus cells, and hypotrophic or absent hair follicles. Follow-up and serial biopsies in three patients documented the progressive nature of fibrosis and naevus cell depletion. No evidence of malignant transformation was found. Naevus cell depletion resulted in pigment loss and may have reduced the risk of malignant transformation. Although the cause of fibrosis is unknown, the possibility of an immune reaction to naevus cells is postulated.
A 13-month-old male infant with 14 hair whorls in the scalp, sparse frontal hair, wide forehead, ectropion, abnormal implantation of eyelashes, peculiar face and depigmented nipples is reported. Other aspects of his physical and mental development were within normal limits. The constellation of clinical features in this patient appear to represent a previously undescribed syndrome.
In addition to onchocerciasis and other filarial diseases, ivermectin has been used for the treatment of scabies, head lice, larva migrans and gnathostomiasis. However, there is concern regarding the safety of its use in children under 5 years of age or weighing less than 15 kg. We present our experience in 18 children (aged 14 months to 17 years), with scabies or cutaneous larva migrans successfully treated with ivermectin. They included four cases of crusted scabies associated with immunosuppression and seven cases of common scabies four of whom had associated clinical mental retardation, immunosuppression or hypomobility. A further seven patients had cutaneous larva migrans. Fifteen patients were cured with a single dose of ivermectin, and three patients with crusted scabies required a second dose. None of our patients suffered significant adverse effects. We believe that ivermectin is a safe and effective alternative treatment of cutaneous parasitosis in children.
The aim was to be able to evaluate the diagnosis of two diseases by a consensus of clinical opinion used in the Department of Dermatology of the National Institute of Paediatrics in Mexico City. To differentiate between scleroderma 'en coup de sabre' (SCS) and progressive facial hemiatrophy (PFH), colour slides of 13 patients diagnosed as SCS and nine as PFH were examined by two dermatologists and microscopic slides by two pathologists. In both cases, the slides were randomly presented and no clinical information was given. The clinical and histopathological findings were statistically compared with two-tailed tests and alpha = 0.05. Kappa coefficients were obtained to evaluate the concordance between dermatologists, pathologists, and in terms of the consensus diagnosis. The usefulness of photographic assessment is limited by the inability to palpate the consistency of lesions. The most important clinical feature that differentiated both conditions was cutaneous sclerosis present in eight of 13 patients with SCS and in none of the PFH patients (P < 0.005). Other clinical features more frequently found in SCS were cutaneous hyperpigmentation and alopecia. The more frequent clinical features in PFH were total hemifacial involvement and ocular changes. Statistically significant histopathological features were: connective tissue fibrosis present in all cases with SCS and two of nine patients with PFH (P < 0.0002); adnexal atrophy present in 11 of 13 patients with SCS, and in three of nine with PFH (P < 0.02), and mononuclear cell infiltrates in all patients with SCS cf. six with PFH (P < 0.05). Our results suggest that in most cases it is possible to differentiate SCS from PFH based on clinicopathological findings.
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A case of circle hairs is reported and the literature on the subject reviewed. Differential diagnoses are discussed as well as some theories on the nature of circle hairs.
Benign cephalic histiocytosis (BCH) is best understood as a form of non-Langerhans cell histiocytosis, specifically as an early mononuclear variant of juvenile xanthogranuloma (JXG). However, the progression of BCH into JXG in the same patient has only been reported once before. We describe the case of a 2-year-old girl with asymptomatic, large, ill-defined infiltrated flat plaques over both cheeks, in addition to isolated papules. A punch biopsy of a plaque revealed dermal infiltration by vacuolated and scalloped histiocytes positive for CD68 KP-1, and that lacked expression of CD1a and S-100 protein, favoring macrophages over Langerhans cells. Electron microscopy study showed comma-shaped intracytoplasmic bodies in the histiocytic cells leading to the diagnosis of BCH. One year later, after an episode of varicella-zoster infection, the flat plaques over the cheeks became large reddish-yellow nodules, and in a second biopsy appeared to progress to JXG. Virus-related mechanisms of progression are discussed.
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The purpose of this study is to compare the esthetics of scars resulting from small surgical facial wounds sutured either with simple interrupted percutaneous suture (SIPS) or with running intradermal suture (RIS). 93 small (0.6-1.9 cm) benign, facial new growths, mostly (86%) intradermal nevi were surgically removed. Forty-seven surgical wounds were sutured with SIPS and 46 with RIS. All surgical procedures were performed by the same dermatologic surgeon. For comparison, lesions were grouped in five facial areas. Other variables such as the age of the patient and type and size of lesions were similar in both groups. Evaluation of each scar was made blindly by two independent observers and by the patients. Judged by independent observers 90 days after surgery, excellent (45 %) to good (45%) results were obtained in similar proportion with either SIPS or RIS suturing in 90% of the patients. In the patients' self evaluation, excellent (85%) to good (14%) results were obtained in 99% of the cases. Suture marks and tracks present 30 days after surgery in 28% of scars sutured with SIPS disappeared 90 days after surgery. The esthetic results obtained by suturing small surgical facial wounds with SIPS or RIS were similarly good to excellent when observed 90 days after surgery. There was no advantage in using RIS over SIPS in the type of wounds described.