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1.
Medicentro (Villa Clara) ; 27(1)mar. 2023.
Article in Spanish | LILACS | ID: biblio-1440507

ABSTRACT

La morfea es una rara enfermedad fibrosante de piel y tejidos subyacentes que aparece a cualquier edad, con más frecuencia en mujeres. Es de etiología autoinmune, benigna, con remisión espontánea o recidivante crónica; y se caracteriza por la aparición de áreas de piel esclerosadas, únicas o múltiples, redondeadas o lineales, asintomáticas, de evolución crónica y sin afección sistémica. Se presentó un paciente en edad pediátrica con esta enfermedad, cuya clínica, examen diagnóstico e histología fueron compatibles con la enfermedad de morfea lineal que, a pesar del retraso en su diagnóstico una vez iniciado el tratamiento inmunosupresor presentó una evolución favorable, simultáneamente se le realizó fisioterapia.


Morphea is a rare fibrosing disease of the skin and underlying tissues which is more common in women and can appear at any age. It is an autoimmune and benign disease with spontaneous remission or chronic relapsing course; it is characterized by the appearance of single or multiple, rounded or linear, asymptomatic, chronically evolving sclerosed skin areas without systemic involvement. We present a female pediatric patient with this disease, whose symptoms, diagnostic examination and histology were compatible with linear morphea that, despite the delay in its diagnosis, she had a favorable evolution once immunosuppressive treatment was started, and physiotherapy was simultaneously performed.


Subject(s)
Scleroderma, Localized , Child Health
2.
Surg. cosmet. dermatol. (Impr.) ; 14: e20220093, jan.-dez. 2022.
Article in Portuguese | LILACS-Express | LILACS | ID: biblio-1391094

ABSTRACT

Introdução: a hemiatrofia facial progressiva, também conhecida como síndrome de Parry Romberg, é uma forma rara de esclerodermia, cujo manejo de sequelas é desafiador. Objetivo: diante desse desafio, o objetivo deste estudo foi avaliar pela primeira vez na literatura a eficácia do preenchimento da hidroxiapatita de cálcio (CaHa) na reconstrução da mandíbula de um paciente com síndrome de Parry Romberg. Relato do caso: paciente do sexo feminino, 15 anos, com atrofia progressiva do lado direito da face por síndrome de Parry Romberg. Após o controle da doença, a principal queixa da paciente era assimetria facial, principalmente na região mandibular. Discussão: a CaHa é um preenchedor injetável biocompatível, que é gradualmente reabsorvido e substituído por estroma fibrovascular, formado principalmente por novo colágeno, em um processo que ocorre sem qualquer reação imunológica. Este perfil de segurança torna a CaHa uma boa escolha para a correção de sequelas de esclerodermia estável. Conclusão: este relato permite concluir que a biocompatibilidade do preenchimento de CaHa e a adequação para correção da mandíbula também são aplicáveis em procedimentos reconstrutivos para esclerodermia estável, de forma segura e minimamente invasiva, com ótimos resultados estéticos. O procedimento deve ser programado caso a caso, e um acompanhamento regular também é recomendado.


Introduction: Progressive Facial Hemiatrophy, also known as Parry Romberg Syndrome, is a rare form of linear scleroderma. The management of facial atrophy sequelae is challenging. Objective: This study aims to evaluate for the first time in literature the effectiveness of the calcium hydroxylapatite (CaHa) filler in the jawline reshaping of a patient with Parry Romberg Syndrome. Case report: A 15-year-old woman with progressive atrophy of the right side of the face due to Parry Romberg Syndrome. After disease control, the patient's main complaint was facial asymmetry, mainly in the jawline region. Discussion: CaHa is a biocompatible injectable filler that is gradually resorbed and replaced by fibrovascular stroma, mainly formed for new collagen, in a process that occurs without any immunological reaction. This safety profile makes CaHa a good choice for correcting stable scleroderma defects. Conclusion: This report concludes that CaHa filler biocompatibility and suitability for the jawline correction are also applicable in reconstructive procedures for stable scleroderma, safely and minimally invasively, with optimal aesthetic results. The method must be programmed case-by-case, and a regular follow-up is also recommended.

3.
Chinese Journal of Dermatology ; (12): 308-315, 2022.
Article in Chinese | WPRIM | ID: wpr-933549

ABSTRACT

Objective:To determine classification and clinical features of morphea.Methods:A retrospective analysis was conducted on epidemiological information about clinical manifestations of and laboratory data from 180 patients with morphea, who visited Zhongshan Hospital, Fudan University from January 2010 to July 2021. Two-independent-sample t test was used to compare the age at onset between genders, and chi-square test to analyze differences in clinical characteristics between different genders and subtypes. Results:Among the 180 patients, 123 were females and 57 were males, with a male-to-female ratio of 1∶2.16. The age at onset of morphea was 28.69 ± 17.97 years for female patients, and 29.90 ± 20.67 years for male patients. Among them, linear morphea was the most common type in this study (68 cases, 37.78%), followed by plaque morphea (63 cases, 35.00%), mixed morphea (28 cases, 15.56%) and deep morphea (21 cases, 11.67%). The disease occurred in all age groups, but the age at onset significantly varied among different clinical subtypes ( F = 5.95, P < 0.001). No significant difference was observed in the age at onset or proportion of clinical subtypes between genders ( F = 0.15, P = 0.696; χ2 =2.88, P = 0.410). Atrophoderma of Pasini and Pierini (APP) was very common (62 cases, 34.44%) in the 180 patients, which mainly manifested as plaques or linear lesions, and 26 out of 45 patients with plaque APP and 11 out of 17 with linear APP were both accompanied by other subtypes of morphea. Among the 75 patients tested for autoantibody profiles, 34 (45.33%) presented with positive results. More diverse types of autoantibodies were found in female patients compared with male patients, and antinuclear antibodies, anti-SSA and anti-SSB antibodies were the most common types. There were various types of comorbidities in female patients, but lichen sclerosus et atrophicus and vitiligo were the most common comorbidities in both genders. Conclusion:High incidence and frequent co-occurrence with other subtypes of APP may be the characteristics of Chinese patients with morphea, and it is recommended to classify morphea into plaque, linear, deep and mixed subtypes.

4.
Rev. méd. Chile ; 149(5): 807-809, mayo 2021. ilus
Article in English | LILACS | ID: biblio-1389503

ABSTRACT

ABSTRACT Taxanes are a class of chemotherapeutic agents with common associated dermatologic adverse events, such as skin hyperpigmentation, hand-foot skin syndrome, paronychia and onycholysis. Taxane-induced scleroderma is rare. Few cases with skin findings resembling systemic sclerosis, have been reported after the administration of these agents. We report two cases with stage IV breast cancer, aged 66 and 71 years, who developed sclerodermic skin lesions in their extremities after starting treatment with placlitaxel and nabplaclitaxel respectively.


Los taxanos son agentes quimioterapéuticos cuyo uso se asocia a problemas dermatológicos tales como hiperpigmentación, síndrome manos-pies, paroniquia y onicolisis. La esclerodermia inducida por taxanos es rara, con pocos casos informados en la literatura. Informamos los casos de dos pacientes con cáncer de mama en estado IV, de 66 y 71 años, que desarrollaron lesiones esclerodérmicas en las extremidades después de ser tratadas con placlitaxel y nabplaclitaxel, respectivamente.


Subject(s)
Humans , Female , Scleroderma, Systemic/chemically induced , Scleroderma, Systemic/drug therapy , Breast Neoplasms/drug therapy , Antineoplastic Agents/adverse effects , Bridged-Ring Compounds/adverse effects , Taxoids/adverse effects
5.
An. bras. dermatol ; 96(1): 72-75, Jan.-Feb. 2021. tab, graf
Article in English | LILACS | ID: biblio-1152792

ABSTRACT

Abstract Intravascular large B-cell lymphoma is a rare, non-mass-forming, extranodal large B-cell lymphoma subtype characterized by the presence of tumor cells in the lumens of vessels. It is divided into two major types: classical and Asian. Patients presenting only with skin involvement are mostly female, at a younger age than classical intravascular large B-cell lymphoma patients, and have a better prognosis. Since the diagnosis of cases with isolated skin involvement is difficult, keeping this entity in mind, performing a careful microscopic examination, and applying new, effective treatment regimens will make it possible to achieve better clinical outcomes in these cases.


Subject(s)
Humans , Female , Skin Neoplasms/diagnosis , Panniculitis/diagnosis , Lymphoma, Large B-Cell, Diffuse/diagnosis , Lymphoma, Large B-Cell, Diffuse/drug therapy , Prognosis , Skin
6.
J. bras. nefrol ; 41(4): 580-584, Out.-Dec. 2019. graf
Article in English | LILACS | ID: biblio-1056602

ABSTRACT

Abstract In kidney biopsies reviews, scleroderma renal crisis (SRC) is characterized by vascular endothelial injuries, C4d deposits on peritubular vessels, and acute and chronic injuries coexisting on the same biopsy. The clinical signs of thrombotic microangiopathy (TMA) are described in systemic sclerosis (SSc), nevertheless, it has not been related to acute injuries described on kidney biopsies. We report a case of SRC in a patient with scleroderma-dermatomyositis overlap syndrome, which also showed clinical and histopathological data of TMA. On fundus examination, a severe acute hypertensive retinopathy was found. The kidney biopsy showed severe endothelial damage with widening of mucoid cells at the level of the intima, focal concentric proliferation on most small arterioles, and C3, C4d, and IgM deposits along the capillary walls. The genetic study of complement only showed the presence of membrane cofactor protein (MCP) risk haplotypes, without other genetic complement disorders. We understand that in a patient with TMA and SSc, the kidney damage would be fundamentally endothelial and of an acute type; moreover, we would observe clear evidence of complement activation. Once further studies correlate clinical-analytical data with anatomopathological studies, it is likely that we will be forced to redefine the SRC concept, focusing on the relationship between acute endothelial damage and complement activation.


Resumo Nas revisões de biópsias renais, a crise renal esclerodérmica (CRE) é caracterizada por lesões endoteliais vasculares, depósitos de C4d em vasos peritubulares e lesões agudas e crônicas que coexistem na mesma biópsia. Os sinais clínicos de microangiopatia trombótica (MAT) são descritos na esclerose sistêmica (ES); no entanto, não foram relacionados às lesões agudas descritas nas biópsias renais. Relatamos um caso de CRE em um paciente com síndrome de superposição de esclerodermia-dermatomiosite, que também apresentou dados clínicos e histopatológicos de MAT. No exame de fundo do olho, foi encontrada uma retinopatia hipertensiva aguda grave. A biópsia renal mostrou lesão endotelial grave com alargamento das células mucoides ao nível da íntima, proliferação concêntrica focal na maioria das pequenas arteríolas e depósitos de C3, C4d e IgM ao longo das paredes dos capilares. O estudo genético do complemento mostrou apenas a presença de haplótipos de risco da proteína cofator de membrana (PCM), sem outros distúrbios genéticos do complemento. Entendemos que em um paciente com MAT e ES, o dano renal seria fundamentalmente endotelial e do tipo agudo; além disso, observaríamos evidências claras de ativação do complemento. Uma vez que novos estudos correlacionam dados clínico-analíticos com estudos anatomopatológicos, é provável que sejamos forçados a redefinir o conceito de CRE, enfocando a relação entre dano endotelial agudo e ativação do complemento.


Subject(s)
Humans , Male , Middle Aged , Raynaud Disease/complications , Vision Disorders/etiology , Acute Kidney Injury/etiology , Kidney/blood supply , Capillaries/metabolism , Angiotensin-Converting Enzyme Inhibitors/therapeutic use , Immunohistochemistry , Papilledema/pathology , Dermatomyositis/complications , Dermatomyositis/immunology , Hypertensive Retinopathy/diagnosis , Hypertensive Retinopathy/pathology , Hypertensive Retinopathy/drug therapy , Acute Kidney Injury/diagnosis , Anemia, Hemolytic/diagnosis , Anemia, Hemolytic/etiology , Kidney/pathology , Kidney/diagnostic imaging
7.
An. bras. dermatol ; 94(4): 473-475, July-Aug. 2019. graf
Article in English | LILACS | ID: biblio-1038301

ABSTRACT

Abstract: Atrophoderma of Pasini and Pierini is a skin disorder affecting dermal collagen and is clinically characterized by well-defined plaques of depressed skin. Histopathological changes are subtle, and in most cases, the diagnosis requires a comparative study with healthy skin from the same anatomical site. High frequency ultrasound is a useful imaging method for diagnosis of atrophic skin changes. A case is presented in which ultrasound can support the clinical and the histopathological diagnosis of atrophoderma of Pasini and Pierini.


Subject(s)
Humans , Female , Adult , Skin Diseases/pathology , Skin Diseases/diagnostic imaging , Ultrasonography, Doppler, Color/methods , Dermis/pathology , Dermis/diagnostic imaging , Atrophy/pathology , Atrophy/diagnostic imaging , Biopsy , Early Diagnosis
8.
An. bras. dermatol ; 94(4): 479-481, July-Aug. 2019. graf
Article in English | LILACS | ID: biblio-1038315

ABSTRACT

Abstract: A 63-year-old black female patient with blisters and exulcerations on the face, neck, upper limbs, and subsequent evolution with hypochromic sclerotic areas and alopecia, is reported. Chronic hepatitis C and presence of high levels of porphyrins in urine were demonstrated. There was complete remission with the use of hydroxychloroquine, photoprotection, and treatment of hepatitis. Significant sclerodermoid involvement of the skin as a manifestation of porphyria cutanea tarda secondary to hepatitis C emphasizes the importance of diagnostic suspicion regarding skin manifestation in order to indicate the appropriate therapy, and to minimize the hepatic morbidity.


Subject(s)
Humans , Female , Middle Aged , Scleroderma, Localized/etiology , Porphyria Cutanea Tarda/etiology , Porphyria Cutanea Tarda/pathology , Hepatitis C, Chronic/complications , Hepatitis C, Chronic/pathology , Scleroderma, Localized/pathology , Scleroderma, Localized/therapy , Treatment Outcome , Porphyria Cutanea Tarda/therapy , Hepatitis C, Chronic/therapy , Alopecia/etiology
9.
São Paulo med. j ; 137(1): 96-99, Jan.-Feb. 2019. tab, graf
Article in English | LILACS | ID: biblio-1004742

ABSTRACT

ABSTRACT CONTEXT: Localized scleroderma (morphea) is characterized by fibrosis of skin and subcutaneous tissue. Granuloma annulare is a relatively common disease that is characterized by dermal papules and arciform plaques. CASE REPORT: Here, we present the case of a 42-year-old woman who developed granuloma annulare on the dorsum of her feet and abdominal region, and morphea on the anterior side of her lower limbs. We also discuss the etiological and pathogenetic processes that may cause the rare coexistence of these two diseases. CONCLUSION: Only a few cases in the literature have described coexistence of morphea and granuloma annulare.


Subject(s)
Humans , Female , Adult , Scleroderma, Localized/diagnosis , Granuloma Annulare/diagnosis , Scleroderma, Localized/complications , Scleroderma, Localized/pathology , Granuloma Annulare/complications , Granuloma Annulare/pathology , Rare Diseases
10.
Chinese Journal of Dermatology ; (12): 561-563, 2018.
Article in Chinese | WPRIM | ID: wpr-710427

ABSTRACT

Objective To investigate clinical and pathological features of 6 cases of disabling pansclerotic morphea (DPM).Methods Clinical and pathological manifestations of and follow-up results in 6 patients,who were clinically and histopathologically diagnosed with DPM in the Department of Pathology,Hospital for Skin Diseases,Chinese Academy of Medical Sciences and Peking Union Medical College from 2007 to 2017,were retrospectively analyzed.Results Among the 6 patients,4 were male and 2 were female.The age of onset ranged from 3 to 10 years,with an average age of 6.5 years.The average duration from the occurrence to the confirmation of the diagnosis was 6.2 years (range,2-10 years).At all the lesional sites,skin atrophy,thining and tightness occurred,and the limbs became thin.Additionally,there were muscular atrophy and visible deep thick veins on the surface of the limbs.The contracture,deformity and dysfunction of the adjacent joints occurred in 4 cases,and the lower limbs were obviously shortened in 2 cases.Peripheral blood examination showed no increase of eosinophils or hypergammaglobulinemia.Imaging examination revealed smooth cortical bone and clear trabecular bone,and no osseous abnormality was observed.Histopathological examination of contracted skin lesions of the lower limbs revealed atrophic and thinned epidermis,hyperpigmentation in the basal layer,hyperplastic,thickened,hardened and partly homogenized collagen fibers in the middle to deep dermis,subcutaneous adipose tissue region and deep tissue of the skin.Conclusions DPM usually does not affect viscera,but often involves deep tissue of the limbs.Histopathologically,DPM is mainly characterized by obviously hyperplastic and hardened collagen fibers in the dermis and subcutaneous tissue.

11.
An. bras. dermatol ; 92(5): 635-637, Sept.-Oct. 2017. tab
Article in English | LILACS | ID: biblio-887051

ABSTRACT

Abstract: Background: Localized scleroderma is a chronic inflammatory skin disease characterized by sclerosis of the dermis and subcutaneous tissue. Platelets play an important role in inflammation. Following activation, platelets rapidly release numerous mediators and cytokines, which contribute to inflammation. Objectives: To evaluate whether there was any relation between localized scleroderma and platelet parameters. Methods: Forty-one patients with localized scleroderma were enrolled in the study. The control group consisted of 30 healthy subjects. Results: The mean platelet volume level in the patient group was 9.9 ± 1.3 fl and in the control group was 7.6 ± 1.1 fl. This difference was statistically significant (p< 0.001). The plateletcrit values are minimally higher in the patient group as compared to the control group. It was statistically significant (p<0.001). There was no significant difference in the platelet counts between the two groups (p= 0.560) In the patient group, there was no significant relation between the mean platelet volume levels and clinical signs of disease (p=0.09). However, plateletcrit values are higher in generalized than localized forms of disease (p=0.01). Study Limitations: The limited number of patients and the retrospective nature of the study were our limitations. Conclusions: This study suggests that platelets might play a role in the pathogenesis of scleroderma. Platelet parameters may be used as markers for evaluating disease severity and inflammatory processes. Thus, there is a need for more detailed and prospective studies.


Subject(s)
Humans , Male , Female , Child , Adolescent , Adult , Middle Aged , Young Adult , Scleroderma, Localized/blood , Mean Platelet Volume , Biomarkers , Case-Control Studies
12.
Medisur ; 15(3): 414-419, may.-jun. 2017.
Article in Spanish | LILACS | ID: biblio-894735

ABSTRACT

La esclerodermia localizada es la forma localizada cutánea de esclerodermia, caracterizada por una fibrosis que se manifiesta en forma de placas o bandas cutáneas escleróticas infiltradas al tacto. La prevalencia se estima en aproximadamente 1-9/100.000, por tal razón se presenta el caso de una paciente con manifestaciones de una esclerodermia cutánea localizada cuya lesión apareció en una mama dos meses antes de asistir a consulta en su área de salud. La principal lesión que presentaba era una placa áspera, que no se dejaba pellizcar, de color carmelita, bordes irregulares, de aproximadamente seis centímetros de diámetro, localizada en mitad inferior de la mama derecha, incluyendo pezón y areola. Se detectaron las alteraciones histológicas propias de la enfermedad de base lo que corroboró el diagnóstico planteado y se descartó mediante otros exámenes cualquier presencia de proceso maligno.


Located scleroderma in the located for of cutaneous scleroderma, characterized by a fibrosis which shows in plaque form of cutaneous sclerotic bands infiltrated to touch. The prevalence is estimated in approximately 1-9/100.000, this is the reason why a case of a patient is presented with signs of a located cutaneous scleroderma. The lesion presented in a breast two months before visiting the doctor in her area. The main lesion was a tough plaque, which was not possible to pinch, brown colored, irregular edges, of approximately six centimeters, located in the mid-inferior right breast, including the nipple and areola. Histological changes which characterize the base disease were found, which corroborated the diagnosis and any malignant process was ruled out by other performed exams.

13.
Rev. argent. dermatol ; 98(1): 42-45, mar. 2017. ilus
Article in Spanish | LILACS | ID: biblio-843112

ABSTRACT

La esclerodermia pertenece a un grupo de enfermedades autoinmunes del tejido conectivo, que produce: inflamación, disfunción vascular y fibrosis excesiva del tejido conectivo de soporte de la piel y los órganos viscerales. A nivel bucal se evidencian afecciones como xerostomía, microstomía, caries y enfermedad periodontal. El tratamiento odontológico requiere un manejo integral por parte de los profesionales, que comprenda las patologías orales presentes y los síntomas permanentes característicos de la enfermedad sistémica de base. El objetivo de este artículo es presentar dos casos de esclerodermia resaltando sus manifestaciones orales presentes.


Scleroderma belongs to a group of autoimmune connective tissue diseases that causes inflammation, vascular dysfunction and excessive fibrosis of the connective tissue supporting the skin and visceral organs. A level oral conditions such as xerostomia, microstomia, caries and periodontal disease are evident. Dental treatment requires a comprehensive management by professionals who understand oral pathologies and permanent characteristic symptoms of systemic disease. The aim of this paper is to present two cases of scleroderma highlighting present oral manifestations.

14.
An. bras. dermatol ; 92(2): 271-272, Mar.-Apr. 2017. tab, graf
Article in English | LILACS | ID: biblio-838064

ABSTRACT

Abstract: Werner syndrome is a rare autosomal recessive disorder, caused by mutations in the WRN gene. Clinical findings include: senile appearance, short stature, grey hair, alopecia, bird-like face, scleroderma-like skin changes, skin ulcers, voice abnormalities, cataracts, osteoporosis, type 2 diabetes mellitus, ischemic heart disease and hypogonadism. The syndrome begins to become apparent in adolescence but it is usually diagnosed in the third or fourth decade of life. Since the patients usually die by the age of 40-50 years related to malignant neoplasms or atherosclerotic complications, they should be closely followed and treated for complications


Subject(s)
Humans , Male , Adult , Werner Syndrome/diagnosis , Scleroderma, Localized , Werner Syndrome/complications , Diagnosis, Differential , Leg Ulcer/etiology
15.
Chinese Journal of Rheumatology ; (12): 833-836, 2017.
Article in Chinese | WPRIM | ID: wpr-666300

ABSTRACT

Objective Systemic sclerosis sine scleroderma (ssSSc) is a rare type of systemic sclerosis,comparison between ssSSc and other types of systemic sclerosis (SSc),ssSSc is carried out in this study.Methods We reported a patient with Raynand's phenomenon,esophagus,intestine and lung involvement,positive ANA,anti-Scl-70 antibody,but had no skin sclerosis.Results For visceral involvement,ssSSc was not different from limited cutaneous systemic sclerosis (lcSSc),The incidence of pulmonary hypertension was higher in patients with ssSSc,lower with calcinosis,finger ulcer and acral dissolution,and the incidence of ssSSc and lcSSc interstitial lung disease was higher than that of diffuse cutaneous systemic sclerosis,(dcSSc).Conclusion Although ssSSc do not have skin sclerosis,however,visceral involvement is more extensive.Early diagnosis and treatmeat is extremely important.

16.
An. bras. dermatol ; 91(4): 534-536, July-Aug. 2016. graf
Article in English | LILACS | ID: lil-792449

ABSTRACT

Abstract: Lichen sclerosus is an uncommon inflammatory dermatosis with preferential involvement of the urogenital region. The extragenital involvement is uncommon and is characterized by small rounded macules or papules, pearly white in color. The coexistence of lichen sclerosus and scleroderma plaques in most cases with extragenital location has been reported in the literature. We report a case of lichen sclerosus associated with scleroderma in children, highlighting the importance of dermoscopy in diagnosis.


Subject(s)
Humans , Female , Child , Scleroderma, Localized/pathology , Scleroderma, Localized/diagnostic imaging , Lichen Sclerosus et Atrophicus/pathology , Lichen Sclerosus et Atrophicus/diagnostic imaging , Dermoscopy/methods , Scleroderma, Localized/complications , Skin/pathology , Skin/diagnostic imaging , Lichen Sclerosus et Atrophicus/complications , Erythema/pathology , Erythema/diagnostic imaging
17.
An. bras. dermatol ; 91(2): 209-211, Mar.-Apr. 2016. graf
Article in English | LILACS | ID: lil-781359

ABSTRACT

Abstract The scleroderma en coup de sabre is a variant of localized scleroderma that occurs preferentially in children. The disease progresses with a proliferative and inflammatory phase and later atrophy and residual deformity, which are treated with surgical techniques such as injectable fillers, transplanted or autologous fat grafting and resection of the lesion. Among the most widely used fillers is hyaluronic acid. However, there are limitations that motivate the search for alternatives, such as polymethylmethacrylate, a permanent filler that is biocompatible, non-toxic, non-mutagenic and immunologically inert. In order to illustrate its application, a case of scleroderma en coup de sabre in a 17-year-old patient, who was treated with polymethylmethacrylate with excellent aesthetic results, is reported.


Subject(s)
Humans , Male , Adolescent , Scalp Dermatoses/drug therapy , Scleroderma, Localized/drug therapy , Antimutagenic Agents/therapeutic use , Polymethyl Methacrylate/therapeutic use , Scalp Dermatoses/pathology , Scleroderma, Localized/pathology , Reproducibility of Results , Treatment Outcome , Forehead
18.
Chinese Journal of Dermatology ; (12): 248-251, 2015.
Article in Chinese | WPRIM | ID: wpr-468688

ABSTRACT

Morphea complicated by Hashimoto's thyroiditis is reported in two sisters.Case 1:a 64-year-old female presented with skin rashes on the anterior neck,trunk and bilateral anterior shins for 5 years,itching skin rashes on the perineum for 4 years,and Hashimoto's thyroiditis for 9 years.Physical examination revealed grade 1 enlargement of firm thyroid gland without exophthalmos or pretibial myxedema.Dermatological examination showed pink patches on the neck and breast,sclerosis and atrophy of skin over the back,porcelain-white patches on the perineum.Histopathological findings suggested the diagnosis of morphea on the breast and lichen sclerosus et atrophicus on the perineum.Case 2:a 55-year-old female,who was the younger sister of case 1,suffered from gradual sclerosis and atrophy of skin in the left inframammary region and abdominal region for 4 years,as well as Hashimoto's thyroiditis for 3 years.Similarly,physical examination revealed grade 1 enlargement of firm thyroid gland without exophthalmos or pretibial myxedema.Hypopigmentation,sclerosis and atrophy of skin were observed in the left inframammary region,abdominal region and central back region.Histopathological examination suggested a diagnosis of morphea.According to the clinical and histopathological manifestations,periodic acid-Schiff staining and thyroid gland function test results,the 2 cases were both diagnosed as morphea complicated by Hashimoto's thyroiditis.

19.
Chinese Journal of Dermatology ; (12): 559-563, 2015.
Article in Chinese | WPRIM | ID: wpr-468395

ABSTRACT

Objective To investigate the effects of ciprofloxacin on dermal collagen synthesis and profibrotic gene expressions in an experimental mouse model of scleroderma induced by bleomycin. Methods Experimental mouse models of scleroderma were established by subcutaneous injection of bleomycin into the dorsal skin of 15 BALB/c mice for 4 consecutive weeks. Then, the mouse models were randomly and equally divided into 3 groups to be topically treated with 1% ciprofloxacin cream (ciprofloxacin group), 2.5% asiaticoside cream (asiaticoside group)and cream vehicle (model group)respectively for 5 consecutive weeks. Five mice firstly injected with sterile phosphate buffered saline (PBS)for 4 weeks then topically treated with cream vehicle for 5 weeks served as the blank control group. After the 5-week topical treatment, all the mice were sacrificed, skin specimens were resected from the dorsal skin of them, and subjected to HE staining and Masson staining. Further more, an immunohistochemical assay was performed to measure the expressions of type I collagen (COL-1), matrix metalloproteinase-1 (MMP1) and tissue inhibitor of matrix metalloproteinase-1 (TIMP1), semi-quantitative reverse transcription PCR to quantify the expressions of connective tissue growth factor (CTGF), transforming growth factor-β1 (TGFβ1)and Smad3 genes, and alkaline hydrolysis-spectrophotometry to determine the level of hydroxyproline in skin. Statistical analysis was carried out by one-way analysis of variance and the least significant difference(LSD)test with the SPSS 17.0 software. Results Compared with the blank control group, the model group showed increased dermal thickness at injection sites (432.76 ± 93.74 μm vs. 301.69 ± 79.47 μm, P 0.05). Moreover, compared with cream vehicle, asiaticoside down-regulated the expressions of the three profibrotic genes(CTGF, TGFβ1 and Smad3)to different extents (all P 0.05). Conclusion Ciprofloxacin may counteract dermal fibrosis by inhibiting the TGFβ1/Smad3 pathway and modulating the unbalanced expressions of MMP1 and TIMP1.

20.
Chinese Journal of Dermatology ; (12): 346-347, 2015.
Article in Chinese | WPRIM | ID: wpr-463860

ABSTRACT

Objective To evaluate the performance of surgical approaches in the treatment of linear scleroderma of the head and face. Methods Forty-three patients with stable linear scleroderma of the head and face were included in this study. Surgical approaches, which included direct suture,skin flap translocation, soft-tissue augmentation and filling operation with dermal tissues, were selected and utilized alone or in combination according to the size, location and degree of atrophy and depression of lesions. Results After surgical treatment, wound dehiscence developed in one of these patients, but the wound healed after two sessions of suture. During one year of follow up, all the patients achieved favorite color, feeling and blood supply in the filling areas as well as good facial profile and visual appearance with no obvious convexity and concavity. Skin flaps matched well with surrounding tissues, incision lines were inconspicuous, and hairline looked naturally with well-distributed hairs in these patients after treatment. Patients were satisfied with treatment results. Conclusion Satisfied cosmetic outcomes can be achieved by using surgical approaches in patients with stable linear scleroderma of the head and face.

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