RESUMO
Linear atrophoderma of Moulin is an acquired rare and self-limited skin condition. It is characterized by atrophic bandlike skin lesions that often show hyperpigmentation and always follow the lines of Blaschko. Usually it begins in childhood or adolescence and there is no evidence of any long term progression. We describe a case of a 21-year-old woman with clinical and histological features of linear atrophoderma of Moulin. The patient was successfully treated with methotrexate 20 mg/week during 6 months with an improvement of skin pigmentation and atrophy. Approximately, 30 cases of linear atrophoderma of Moulin were described in the literature. There is not a proven effective treatment of this dermatosis. High dose penicillin, topical corticosteroids, heparin, and oral potassium aminobenzoate have been used but found to be uneffective. To our knowledge, this is the first case of extensive linear atrophoderma of Moulin treated with methotrexate.
Assuntos
Fármacos Dermatológicos/uso terapêutico , Hiperpigmentação/tratamento farmacológico , Metotrexato/uso terapêutico , Dermatopatias/tratamento farmacológico , Pigmentação da Pele/efeitos dos fármacos , Pele/efeitos dos fármacos , Atrofia , Feminino , Humanos , Hiperpigmentação/diagnóstico , Pele/patologia , Dermatopatias/diagnóstico , Fatores de Tempo , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Labial mucosa is an atypical site of basal cell carcinoma. The involvement of the vermilion lip, devoid of hair follicles and sweat glands, contrasts with the concept of its origin from pilar structures. We report a case of basal cell carcinoma developed on the vermilion upper lip. CASE REPORT: A 49-year-old woman, presented with an asymptomatic, 1-cm-diameter, erythematous, telangiectatic and crusted nodule on the upper lip evolving for 9 months and having once interested the vermilion border. There were no cervical lymph nodes. Diagnosis of infiltrative basal cell carcinoma was made by histological study, which showed a tumoral proliferation of epithelial basal cells infiltrating the dermis with perineural and muscular infiltration. DISCUSSION: Our report illustrates a rare but not exceptional site of basal cell carcinoma. The nodule, initially confined to the vermilion border, has then developed onto the mucosal and the cutaneous areas. Histopathological study revealed, as previously reported, infiltarative features. Basal cell carcinoma of the lip should be rapidly managed since its invasion to deeper structures occurs early.
Assuntos
Carcinoma Basocelular/patologia , Neoplasias Labiais/patologia , Derme/patologia , Epiderme/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Mucosa/patologia , Músculo Liso/patologia , Invasividade Neoplásica , Fibras Nervosas/patologiaAssuntos
Doença de Crohn/diagnóstico , Fissura Anal/etiologia , Granuloma de Células Gigantes/etiologia , Dermatopatias Vesiculobolhosas/etiologia , Úlcera Cutânea/etiologia , Doenças da Vulva/diagnóstico , Biópsia , Doença de Crohn/complicações , Doença de Crohn/patologia , Edema/etiologia , Feminino , Fissura Anal/patologia , Tecido de Granulação/patologia , Granuloma de Células Gigantes/patologia , Humanos , Pessoa de Meia-Idade , Períneo/patologia , Dermatopatias Vesiculobolhosas/patologia , Úlcera Cutânea/patologia , Coloração e Rotulagem , Doenças da Vulva/patologiaRESUMO
BACKGROUND: Dermatomyositis (DM) is a rare but serious disease. The aim was to evaluate the epidemiology, presenting clinical manifestations, therapeutic features and outcome of patients with DM. METHODS: From January 1986 to December 2003, we collected retrospectively cases of DM identified at the dermatology department of Habib Thameur hospital. We included only patients with definitive diagnosis of DM. RESULTS: We included 13 patients, mean aged 32.85 years: 9 adults and 4 children. We identified 9 cases of adult DM among them 2 cases of wiihich were of paraneoplastic DM and 4 cases of juvenile DM among them 1 case of amyopathic DM and 1 case of DM associated with connective tissue disease. The evolution of DM followed the course of the neoplasm for the 2 patients with paraneoplastic DM. All patients received corticotherapy initially. Patients were followed-up for an average of 3 years (2 months to 10 years). We found no serious repercussions on the growth of children with juvenile DM. CONCLUSION: DM is rare in Tunisia. In our study, its frequency is about 0.72 patients per year. Age of onset of DM is markedly smaller than the one reported in the relevant litterature. Our 2 cases of DM associated with cancer are considered like really paraneoplastic. Juvenile DM seems to have a relatively good prognosis.
Assuntos
Dermatomiosite/epidemiologia , Adolescente , Adulto , Criança , Pré-Escolar , Dermatomiosite/tratamento farmacológico , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , TunísiaRESUMO
BACKGROUND: Digital necrosis is a frequent vascular disease. Its very often reflects arterial disorders of macro or mirocirculation. Paraneoplastic digital necrosis is rare, however. AIM: Report a new case of paraneoplastic digital necrosis CASE: Our patient was a 62-year old diabetic female patient with a history of total thyroid ablation at 50 year old. She presented with bilateral digital necrosis of the second, third and fourth fingers. During her recovery, we have found a biological inflammatory syndrome and abdominal echography revealed a metastatic liver. Radiological investigations in search for the primitive tumour were negative. The patient was referred to a specialized center for a palliative chemotherapy. CONCLUSION: Etiologies of digital necrosis are various, mainly iatrogen, professional, toxic, atheroma or systemic disease. In case of no evident cause, explorations should be gone to detect underlying malignancies.
Assuntos
Dedos/patologia , Síndromes Paraneoplásicas/diagnóstico , Diabetes Mellitus Tipo 1/complicações , Feminino , Humanos , Neoplasias Hepáticas/secundário , Pessoa de Meia-Idade , Necrose , Neoplasias Primárias Desconhecidas/patologia , Cuidados Paliativos , TireoidectomiaRESUMO
The clinical features of childhood discoid lupus erythematosus (DLE) are similar to those of adult DLE in presentation and chronic course. However, children have a particularly high levels of transition to systemic disease. Systemic lupus erythematosus (SLE) is the most common rheumatic disease associated with significant morbidity and mortality in children. This is a retrospective study reporting all cases of childhood lupus erythematosus observed in the dermatology department of Habib Thameur Hospital over a 14-year period. From 1989 to 2003, six cases of childhood lupus erythematosus are included, three patients with discoid lupus erythematosus (2 girls, 1 boy), and three patients with systemic lupus erythematosus (2 boys, 1 girl). The mean age of onset was 12 years (range 10-16 years). Skin manifestations were localized in sun exposed areas in both discoid and systemic lupus erythematosus. Photosensitivity was noted in all cases. The diagnosis was confirmed by histopathologic examination, direct immunofluorescence, and immunologic findings. Treatment included sun avoidance, oral hydroxychloroquine, and topical and systemic steroids. An average follow-up time was 18.1 months (1-96 months). The severity of onset of SLE is usually greater in children than adults. We note that lupus erythematosus is not a static disease and progression from DLE to SLE is possible.
Assuntos
Lúpus Eritematoso Discoide/patologia , Lúpus Eritematoso Sistêmico/patologia , Adolescente , Criança , Feminino , Humanos , Masculino , Estudos RetrospectivosRESUMO
This is a retrospective study about 49 cases of xeroderma pigmentosum (XP). The goal was to determine the clinical features of XP in Tunisia. Our study revealed the predominance of a moderate form. Malignant skin tumors showed early. Squamous cell carcinoma (SCC) remainsed the most frequent skin malignancy in all clinical forms. Neurological abnormalities were more frequent in the moderate form. Mental retardation and peripheral neuropathy were the most common signs.
Assuntos
Xeroderma Pigmentoso/complicações , Adolescente , Adulto , Criança , Pré-Escolar , Oftalmopatias/etiologia , Feminino , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Doenças do Sistema Nervoso/etiologia , Estudos Retrospectivos , Dermatopatias/etiologiaRESUMO
INTRODUCTION: - The induction of malignant diseases is one of the most concerning late effects of ionizing radiation. The topic of this study deals with skin tumors developed in the irradiated areas in children given X-ray therapy for tinea capitis. MATERIAL AND METHODS: - All patients with malignant tumors of the scalp referred to Salah Azaiz Institute between 1970 and 2001 have been questioned in order to determine if there had been a prior X-ray irradiation for tinea capitis, its modality, and its consequences. The first scalp irradiation goes back to 1922 and the last was performed in 1963. RESULTS: - Ninety-eight patients with 150 radio-induced cancers of the scalp following irradiation for tinea capitis are reported (1.5 lesion per patient). The patients were irradiated in various hospitals and dispensaries throughout the country. Eighty-one patients (82%) had only one session of radiation. The average age at irradiation was 12 (+/-6) years, the latent period for radiation-induced skin cancers was 36 (+/-14) years. In 61 patients (62%), the scalp appeared normal and in 38% radiodermatitis was noted. Patient age at diagnosis of malignancy varied from 20 to 83 years with an average of 47 years. Basal cell carcinomas (125 cases) and spinocellular carcinomas (16 cases) were the most common, three other cases of annexial tumors, two malignant non-Hodgkin's lymphomas and four melanoma lesions are also present. Radiotherapy was used for the treatment of 74 patients (alone in 42 and associated with surgery in 32 patients); 14 patients had exclusive surgical excision. CONCLUSIONS: - Basal cell carcinomas are the most frequent tumors arising on chronic radiodermatitis. In spite of the long latency period, patients' young age at irradiation explained the occurrence of these cancers at a relatively young age. Literature review is suggesting recessive mutation of tumor-suppressor genes as the characteristic abnormality in radio-induced cancer.
Assuntos
Carcinoma Basocelular/etiologia , Carcinoma de Células Escamosas/etiologia , Neoplasias de Cabeça e Pescoço/etiologia , Neoplasias Induzidas por Radiação/etiologia , Radiodermite/etiologia , Radioterapia/efeitos adversos , Couro Cabeludo , Neoplasias Cutâneas/etiologia , Tinha do Couro Cabeludo/radioterapia , Adulto , Fatores Etários , Idoso , Idoso de 80 Anos ou mais , Braquiterapia , Carcinoma Basocelular/radioterapia , Carcinoma Basocelular/cirurgia , Carcinoma de Células Escamosas/radioterapia , Carcinoma de Células Escamosas/cirurgia , Criança , Terapia Combinada , Humanos , Pessoa de Meia-Idade , Neoplasias Induzidas por Radiação/radioterapia , Neoplasias Induzidas por Radiação/cirurgia , Fatores de TempoRESUMO
Various treatment modalities are used to treat warts and this indicates that none is uniformly effective. Cimetidine, an H2-receptor antagonist, has been tried to treat warts because of its immunomodulatory effects. Sixty children with multiple and recalcitrant warts were included in a placebo-controlled, double-blind study. Patients were randomly allocated to treatment groups equally. The groups received cimetidine, 25 to 30 mg/kg daily, or placebo for 2 months. At the end of the therapy, only 10 cimetidine-treated and 9 placebo-treated were examined. Cure rates obtained were 60% in the cimetidie-treated group and 33% in the placebo-treated group. Among our data and the review of the literature, we believe that cimetidine is not appropriate as standard therapy for warts, but it may be an interested therapy for multiple and recalcitrant warts of children.
Assuntos
Adjuvantes Imunológicos/farmacologia , Cimetidina/farmacologia , Verrugas/tratamento farmacológico , Adjuvantes Imunológicos/administração & dosagem , Administração Oral , Criança , Cimetidina/administração & dosagem , Método Duplo-Cego , Feminino , Humanos , Masculino , Placebos , Resultado do TratamentoRESUMO
Dermatofibrosarcoma (DFS) protuberans is a rare mesenchyma tumor in childhood characterised by slow growth. We report the result of a retrospective study of 7 childhood (4 males) cases of through a fifteen year period. The mean age was 13 years. 4 Tumors were (DFS) located on the limbs: 2 on the trunk: and on the scalp. Clinical types were multinodular and infiltrated in 3 cases, nodular in two cases and ulcerated with budding also in two cases. The mean delay between diagnostic and the appearance of the tumor was about 24 months for 4 initial tumors. Histological features were typical in all cases. We report 3 recurrences of dermatofibrosarcoma arising few months after surgical excision of an initial tumor, in one case the tumor was diagnosed histocytofibroma. Six patient underwent surgical excision with wide margins, and in one case the margins were considered borderline. No recurrences were reported, the follow-up was between 17 months to 9 years. It's a rare tumor in childhood, 160 cases were reported. The clinical and evolutive mode were entirely comparable to the adult cases. However the initial appearance as benign atrophic or indurated plaque is more frequent in childhood, but this appearance wasn't noted in our series.
Assuntos
Dermatofibrossarcoma/cirurgia , Recidiva Local de Neoplasia , Neoplasias Cutâneas/cirurgia , Adolescente , Criança , Dermatofibrossarcoma/diagnóstico , Dermatofibrossarcoma/patologia , Feminino , Humanos , Masculino , Prognóstico , Neoplasias Cutâneas/diagnóstico , Neoplasias Cutâneas/patologia , Resultado do TratamentoRESUMO
Angiomatoid fibrous histiocytoma is a rare tumour affecting young adults. Unlike conventional malignant fibrous histiocytoma, its extension is only local thus giving a good prognosis. We report the cases of a 9 year-old girl and a 16 year-old boy presenting respectively, with an axillary tumour 5 cm of diameter and a paravertebral subcutaneous tumour 1.5 cm of diameter. In both cases, the diagnosis was not initially suspected. The treatment consisted in surgical resection.
Assuntos
Histiocitoma Fibroso Benigno/patologia , Adolescente , Criança , Feminino , Humanos , MasculinoRESUMO
Mycosis fungoides is an epidermotropic cutaneous T lymphoma. It's a non Hodgkinian lymphoma. We report the results of a retrospective review of 11 mycosis fungoide seen during 22 years. The frequency of MF was about 39.3% among all cutaneous lymphoma. Six patients were male and 5 were female; the mean age was about 56 years. Mean delay between diagnostic and the first manifestation was about 25 months. All patients had the progressive form: 4 had infiltrate plaques and 7 were at the tumoral phase. Lymph nodes and medullar metastases were noted respectively in 1 and 2 cases. Treatment was mono or polychemotherapy associated in 6 cases with topical drug. Three patients died of their diseases According to our experience and after reviewed the literature, we notice that our patients are slightly younger without male predominance. The diagnostic was done tardily and this may explain the pejorative prognostic.
Assuntos
Protocolos de Quimioterapia Combinada Antineoplásica/uso terapêutico , Metástase Linfática , Micose Fungoide/patologia , Administração Tópica , Idade de Início , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Diagnóstico Diferencial , Progressão da Doença , Evolução Fatal , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Micose Fungoide/epidemiologia , Micose Fungoide/etiologia , Prognóstico , Tunísia/epidemiologiaRESUMO
BACKGROUND: Bathing suit ichthyosis (BSI) is an uncommon phenotype classified as a minor variant of autosomal recessive congenital ichthyosis (ARCI). OBJECTIVES: We report a case of BSI in a 3-year-old Tunisian girl with a novel mutation of the transglutaminase 1 gene (TGM1). CASE REPORT: This infant had been born with a collodion membrane encasing her entire body. From the age of three months, brownish scaling was noted on the bathing suit area. Histology showed orthohyperkeratosis with acanthosis of the epidermis. The granular layer was normal, and the superficial dermis was mildly inflammatory, confirming a diagnosis of proliferating ichthyosis. Molecular analysis in the patient and her parents revealed the mutation I304F of TGM1. Treatment with emollients and keratolytics partially improved the patient's skin condition. CONCLUSIONS: Bathing suit ichthyosis is an uncommon phenotype unique in its topography, which involves the trunk but spares the face and extremities. Previous studies using molecular analysis have shown that BSI is caused mainly by mutations in TGM1. Twenty missense mutations have been reported in BSI. Of these 20 missense mutations, nine occurred only in patients with the BSI phenotype and 11 were common to BSI and other types of ARCI. Until recently, there has been no genotype-phenotype correlation. Therefore, the same mutation of the transglutaminase 1 could result in either generalized ARCI or BSI. The present case demonstrates this phenotype in a White Tunisian patient with a novel mutation of TGM1 (I304F) not previously reported in BSI.
Assuntos
Ictiose Lamelar/genética , Transglutaminases/genética , Pré-Escolar , Consanguinidade , Análise Mutacional de DNA , Emolientes/uso terapêutico , Feminino , Humanos , Ictiose Lamelar/patologia , Ictiose Lamelar/terapia , Fenótipo , TunísiaRESUMO
INTRODUCTION: Docetaxel belongs to the taxane group of chemotherapeutic agents used in the management of various malignant diseases. Nail changes as a complication of such treatment are observed in about 44%. Subungual haemorrhages (SH), are very rare following docetaxel therapy and only a few cases have been previously reported. OBSERVATION: An 80-year-old man suffering from prostate adenocarcinoma was treated with a 3-weekly cure of docetaxel started 3 months earlier. Nail changes occurred after the 5th cycle of docetaxel. Clinical examination revealed orange discoloration of the nail plates, subungueal haemorrhages (SH) and onycholysis involving nails of all the digits and toenails of both hands and feet. These features were highly suggestive of nail toxicity following docetaxel therapy. DISCUSSION: Nail changes secondary to Taxane chemotherapy includes nail bed dyschromia, onycholysis, red or orange Beau's lines and subungueal hyperkeratosis. SH, as reported in this case, is related to the cumulative dose of docetaxel and should not be attributed to other systemic diseases. Clinicians should recognize this complication to avoid abusive treatment or investigations and inform the patients about the possibility of nail changes secondary to taxane drugs.