Ichthyosis bullosa of Siemens: a topical therapy option.
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Administration, Topical↗
Biomedical subjects
R Falabella
Publications and source records attributed to R Falabella.
Eosinophilic, polymorphic, and pruritic eruption associated with radiotherapy.
OBJECTIVE: To characterize the epidemiological, clinical, and histopathological features of patients with cancer who develop widespread polymorphic and pruritic skin lesions following radiotherapy. PATIENTS, DESIGN, AND INTERVENTIONS: During phase 1, epidemiological and clinical features of 103 patients with cancer, 83 treated with radiotherapy (71 women and 12 men) and 20 controls who did not undergo radiotherapy (16 women and 4 men), were explored during 3 months (October 1995 to January 1996). During phase 2, in 30 additional patients with cancer who were treated with telecobalt or linear accelerator, 18 with skin lesions (15 women and 3 men) and 12 without lesions (10 women and 2 men), the following were investigated: (1) hematoxylin-eosin-stained sections for routine histopathological examination and direct immunofluorescence, and lymphocytic markers; (2) blood, skin, and primary tumor eosinophilia; and (3) the presence of antiepidermal autoantibodies. Patients were examined during 5 months (February 1996 to June 1996). SETTING: A dermatology department at a university hospital. RESULTS: During phase 1, 14 (17%) of the 83 patients undergoing radiotherapy developed an eruption. Acral excoriations, erythematous papules, vesicles, and bullae were the most frequent lesions. During phase 2, in 18 patients, a superficial and deep lymphocytic perivascular infiltrate with numerous eosinophils, intraepidermal and interstitial eosinophilic infiltrates, eosinophilic panniculitis, IgM and C3 perivascular deposits, and slightly predominant CD4+ cells were observed. No antiepidermal autoantibodies were found. CONCLUSIONS: The clinical, histopathological, and immunopathologic features in patients with cancer undergoing radiotherapy are described. To our knowledge, this condition has not been well characterized. Because of its unique presentation, the denomination "eosinophilic, polymorphic, and pruritic eruption associated with radiotherapy" is suggested.
Adult↗
Changes in expression of signal transduction proteins in T lymphocytes of patients with leprosy.
Advanced stages of mycobacterial diseases such as leprosy and tuberculosis are characterized by a loss of T-cell function. The basis of this T-cell dysfunction is not well understood. The present report demonstrates major alterations in the expression of signal transduction molecules in T cells of leprosy patients. These alterations were most frequently observed in lepromatous leprosy (LL) patients. Of 29 LL patients, 69% had decreased T-cell receptor zeta-chain expression, 48% had decreased p56(lck) tyrosine kinase, and 63% had a loss of nuclear transcription factor NF-kappaB p65. An electrophoretic mobility shift assay with the gamma interferon core promoter region revealed a loss of the Th1 DNA-binding pattern in LL patients. In contrast, tuberculoid leprosy patients had only minor signal transduction alterations. These novel findings might improve our understanding of the T-cell dysfunction observed in leprosy and other infectious diseases and consequently might lead to better immunologic evaluation of patients.
Adult↗
Lepromatous and tuberculoid leprosy: clinical presentation and cytokine responses.
OBJECTIVE: This study analyzes the major clinical characteristics of patients with active leprosy in relation to the in vitro immune response to the T-lymphocyte activator anti-CD3. METHODS: Thirty-eight patients with an established diagnosis of leprosy were classified according to the Ridley and Jopling table. Peripheral blood mononuclear cells from both lepromatous leprosy (LL) and tuberculoid leprosy (TL) patients and healthy controls were used to evaluate lymphocyte proliferation; immunoenzymatic assays were used to evaluate cytokine production (IL-1, IL-2, IL-4, IL-6, IL-10, IFN-gamma). RESULTS: Peripheral blood mononuclear cells from both LL and TL patients displayed blastogenic responses to anti-CD3. The cytokines IL-1 beta, IL-6, IL-10, and IFN-gamma were detected in culture supernatants. Endogenous production of IL-1 beta was significantly higher in cell cultures from patients with the lepromatous form of the disease compared to those with tuberculoid leprosy. Production of IL-6 in response to anti-CD3 was observed in a significantly higher proportion of LL than TL patients (P = 0.0025). Gamma-interferon production did not differ between TL and LL, but a direct correlation was observed between time of multidrug treatment and IFN production in vitro (P = 0.016). Interleukin-10 was detected in culture supernatants of lymphocytes activated by anti-CD3 from both patient groups, but not from healthy controls. CONCLUSIONS: The findings of this study suggest that patients with the two distinct forms of leprosy are capable of responding to a polyclonal T-lymphocyte stimulus such as anti-CD3 and provide evidence suggestive of alterations in the immune responses mediated by cytokines that may contribute to the spectrum of disease and response to treatment.
Adult↗
The minigrafting test for vitiligo: detection of stable lesions for melanocyte transplantation.
BACKGROUND: Selected patients with stable and refractory vitiligo may consider melanocyte transplantation as a therapeutic alternative. A method to anticipate the response to surgical repair is not available. OBJECTIVE: We evaluated the pigment spread of minigrafts when implanted within achromic lesions of stable vitiligo as a test to identify good candidates for surgical repigmentation. METHODS: Four to six minigrafts of 1.0 to 1.2 mm were implanted within lesions of patients with unilateral (localized) and bilateral (generalized) vitiligo. Pigment spread was assessed 3 months later. RESULTS: Forty-seven subjects were examined. In unilateral vitiligo 19 of 20 patients (95%) had a positive test result in comparison with only 13 of 27 patients (48%) with bilateral vitiligo (p = 0.002). CONCLUSION: The minigrafting test is a reliable tool to identify patients with stable vitiligo who may respond to melanocyte transplantation. Unilateral (localized) vitiligo is the best indication for surgical repigmentation.
Adult↗
An epidemiologic case-control study in a population with vitiligo.
BACKGROUND: Previous epidemiologic studies of vitiligo have not included a sex- and age-matched population. OBJECTIVE: Our purpose was to attempt to determine possible risk factors and clinical differences associated with unilateral and bilateral vitiligo. METHODS: Two hundred thirty-four patients and 234 normal control subjects were examined and questioned. RESULTS: Patients with unilateral vitiligo were younger and had an earlier age at onset. Koebner's phenomenon and halo nevus were infrequent in the total vitiligo group, but no difference between vitiligo types was observed. Subjects with bilateral vitiligo more frequently had light skin (types I, II, and III) and more commonly had an associated autoimmune disease. CONCLUSION: Unilateral and bilateral vitiligo differ substantially in several clinical aspects, which suggests a different pathogenic mechanism for each condition.
Adolescent↗
Surgical combination therapy for vitiligo and piebaldism.
BACKGROUND: Refractory and stable defects of vitiligo and piebaldism may be unresponsive to medical therapy. Melanocyte transplantation can restore the normal pigmentation in some selected patients. OBJECTIVES: To evaluate the efficacy of additional mini-grafting with 1.0-1.2-mm punch grafts to complete the restoration of achromic defects when performing surgical correction of leukoderma. METHODS: Eight patients with refractory stable leukoderma were treated with melanocyte transplantation; three with segmental vitiligo had epidermal shave, by removing the hyperpigmented macules at the periphery of achromic lesions; two others received suction epidermal grafts; and three subjects were treated by in vitro cultured epidermal autografts. All patients received additional mini-grafts in areas of residual achromia. RESULTS: The depigmented defects were 100% restored in seven patients, and in one subject 80% improvement was observed. CONCLUSION: Surgical methods, followed by additional mini-grafting, may be helpful to restore completely the depigmented defects when residual achromia, after treatment with the methods described above, is still present.
Cells, Cultured↗
Novel mutations and deletions of the KIT (steel factor receptor) gene in human piebaldism.
Piebaldism is an autosomal dominant genetic disorder of pigmentation characterized by white patches of skin and hair. Melanocytes are lacking in these hypopigmented regions, the result of mutations of the KIT gene, which encodes the cell surface receptor for steel factor (SLF). We describe the analysis of 26 unrelated patients with piebaldism-like hypopigmentation--17 typical patients, 5 with atypical clinical features or family histories, and 4 with other disorders that involve white spotting. We identified novel pathologic mutations or deletions of the KIT gene in 10 (59%) of the typical patients, and in 2 (40%) of the atypical patients. Overall, we have identified pathologic KIT gene mutations in 21 (75%) of 28 unrelated patients with typical piebaldism we have studied. Of the patients without apparent KIT mutations, none have apparent abnormalities of the gene encoding SLF itself (MGF), and genetic linkage analyses in two of these families are suggestive of linkage of the piebald phenotype to KIT. Thus, most patients with typical piebaldism appear to have abnormalities of the KIT gene.
Adolescent↗
Nonvenereal treponematoses: yaws, endemic syphilis, and pinta.
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Developing Countries↗
Melanocyte reservoir in vitiligo.
BACKGROUND: Dopa-negative, inactive melanocytes, present in the middle portion of the hair follicle, but also in hair bulbs, have been reported as a source of pigment cells, when repopulation of epidermal melanocytes occurs. A melanocyte reservoir in these anatomical sites has been suggested. Our objective was to investigate the ability of the lower third of the hair follicle (hair bulb) to repigment achromic skin in vitiligo. METHODS: Scalp hair bulbs were transplanted within leukodermic areas in 10 patients with vitiligo. RESULTS: Repigmentation around the grafts was suitable for evaluation in four cases. Dopa-positive (+) cells were seen in the epidermal basal cell layer of the repigmented areas. CONCLUSIONS: Although these findings were observed only in a few patients, they suggest that melanocytes from the implanted lower third portion of the hair follicle (hair bulb) act as a reservoir in this anatomic location and are able to migrate and repigment achromic areas in vitiligo.
Adult↗
Treatment of refractory and stable vitiligo by transplantation of in vitro cultured epidermal autografts bearing melanocytes.
BACKGROUND: Previous reports demonstrated the usefulness of in vitro cultured epidermis for repigmenting vitiligo. OBJECTIVE: Our purpose was to determine the potential of in vitro cultured epidermal sheets to treat extensive areas of intractable vitiligo. METHODS: In nine patients with long-standing, stable, and refractory vitiligo, autologous epidermis was cultured in vitro in H-MEM but without growth enhancers or hormones and transplanted onto previously denuded achromic lesions. RESULTS: Repigmentation was achieved to almost 100% in three subjects, 60% improvement was seen in two patients, and 30% to 40% in three additional cases. Only one patient had almost no response. Long-term observations in these patients indicate that repigmentation obtained by this method is permanent. CONCLUSION: Transplantation of in vitro cultured epidermis bearing melanocytes is potentially effective to treat extensive areas of vitiligo, but this method is presently at an experimental stage.
Adult↗
Transplantation of in vitro-cultured epidermis bearing melanocytes for repigmenting vitiligo.
A new method for repigmenting vitiligo by transplantation of in vitro-cultured epidermis bearing melanocytes is described. The artificially grown epidermis was implanted after vitiliginous skin was denuded with liquid nitrogen. Satisfactory repigmentation was attained. This technique offers a new approach for treating refractory vitiligo.
Adolescent↗
[In vitro culture of melanocyte-bearing epidermis and its application in the treatment of vitiligo and the stable leukodermas].
Skin specimens were cultured in vitro in a attempt to reproduce epidermal sheets bearing melanocytes. After exploring different parameters and culture conditions, 16 out of 45 samples yielded pigmented epidermal membranes with melanocytes. This epidermis bearing melanocytes was transplanted to a patient with segmental vitiligo and a satisfactory repigmentation was achieved. The factors contributing to the development of this pigmented in vitro cultured epidermis, and the potential of this technology for treating refractory but stable forms of vitiligo, are discussed.
Adult↗
Leukoderma punctata.
Thirteen patients with vitiligo (1 segmental, 4 focal, 8 generalized) aged 7 to 38, most of them female children, developed numerous punctate hypopigmented and achromic spots. The spots measured 0.5 to 1.5 mm and were located primarily on the sun-exposed areas of the extremities; they appeared following treatment with PUVASOL. Two of these patients experienced a reduction of this leukodermic defect, whereas the remaining patients showed a stable clinical course. Dopa and Fontana stains disclosed, in most cases, decreased but not absent functional melanocytes and a marked reduction of melanin. Ultrastructural studies demonstrated slight to severe damage of keratinocytes and melanocytes similar to that previously reported in vitiligo patients. The phototoxic effect of PUVASOL therapy is suggested as a possible etiologic factor in these patients. A probable relationship among idiopathic guttate hypomelanosis, leukoderma punctata, and vitiligo is discussed.
Adolescent↗
Treatment of localized vitiligo by autologous minigrafting.
Autologous minigrafting has been reported as an effective method for repigmenting diverse types of stable leukoderma. A group of 22 patients with localized vitiligo, 17 segmental and five focal, who are under treatment with this method, are described. Thirteen patients attained a 90% to 100% repigmentation, two others achieved a partial improvement, and five patients had a positive test area indicating the possibility of repigmentation by means of this procedure. Only two patients had a negative test with minigrafts and, consequently, they were left untreated. Autologous minigrafting is suggested as an alternative for treating localized vitiligo, particularly when other medical therapeutic attempts have failed in repigmenting this often refractory condition.
Adolescent↗
Idiopathic guttate hypomelanosis.
Idiopathic guttate hypomelanosis is a common and frequently ignored dermatosis. It appears late in life and increases with aging. The studies so far reported do not support the hypothesis of a residual leukoderma following trauma, and the relation of IGH to chronic solar exposure has not yet been documented. However, the role of ultraviolet A and ultraviolet B in the pathogenesis of this dermatosis should be explored by more refined methods. Although it may be genetically modulated, a multifactorial etiology rather than a single cause is likely. An active depigmenting mechanism may underlie the melanocyte disturbance instead of a simple residual defect. Intralesional triamcinolone in very low concentrations with or without minigrafts of normally pigmented skin could be of some therapeutic value.
Humans↗
On the pathogenesis of idiopathic guttate hypomelanosis.
Idiopathic guttate hypomelanosis is a common leukodermic dermatosis of obscure origin, consisting of small 2- to 5-mm achromic or hypopigmented macules, mainly affecting the exposed upper and lower extremities. In a group of 400 consecutive dermatologic patients, idiopathic guttate hypomelanosis was much more prevalent in women than in men. However, in both sexes this prevalence became more common with advancing age. In another group of fifteen patients with idiopathic guttate hypomelanosis and fifteen normal controls matched by age, sex, and skin type, the following was found: A cause-effect relationship between chronic actinic exposure and the development of idiopathic guttate hypomelanosis could not be established by statistical studies. A family aggregation survey disclosed a higher prevalence of idiopathic guttate hypomelanosis in the family of patients with idiopathic guttate hypomelanosis than in the control group. Epithelial atrophy, patchy absence of melanocytes and melanin, flattening of the rete pegs, and basket weave hyperkeratosis were the most prominent histologic findings of idiopathic guttate hypomelanosis. Minigrafts of normal skin implanted in idiopathic guttate hypomelanosis lesions did not modify the achromic defects, whereas intralesional triamcinolone with or without grafts improved the appearance of these lesions.
Adult↗