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1.
Ann Dermatol Venereol ; 139(6-7): 481-5, 2012 Jun.
Artigo em Francês | MEDLINE | ID: mdl-22721482

RESUMO

BACKGROUND: Reactive perforating collagenosis (RPC) belongs to the group of perforating dermatoses, which comprises elastosis perforans serpiginosa, RPC, perforating folliculitis and Kyrle's disease. RPC was initially described as a distinctive form of transepithelial elimination of altered collagen related to superficial trauma. Two types are distinguished: a hereditary type (MIM 216700), which is rare and begins during early childhood, and a second type, called acquired RPC, which is more frequent, appears in adults and is associated with other diseases, diabetes mellitus, renal insufficiency, solid tumors, lymphomas and AIDS. We report the case of a young man whose illness began during infancy, militating in favor of a diagnosis of a hereditary form of RPC. The description of similar lesions in the patient's brother confirmed our diagnosis. PATIENTS AND METHODS: A 26-year-old man, the child of consanguinous parents, presented crusted papular lesions on his hands. The cutaneous lesions, located on the external side of the limbs, had been present since childhood, with flares during winter. Histologic analysis showed a cup-shaped depression in the epidermis containing keratinous material with extruded degenerated collagen towards the cutaneous surface. Treatment with topic retinoids did not result in any real resolution of the disease. The patient reported the presence of similar lesions in his brother, which was consistent with our diagnosis. DISCUSSION: The pathogenesis of hereditary RPC is still unknown, even if superficial trauma is suspected as the cause of RPC. In contrast, in diabetes, acquired RPC pathogenesis has recently been related to advanced glycation end-products of collagen.


Assuntos
Doenças do Colágeno/diagnóstico , Dermatoses do Pé/diagnóstico , Dermatoses da Mão/diagnóstico , Dermatopatias Genéticas/diagnóstico , Adulto , Membrana Basal/patologia , Biópsia , Colágeno/ultraestrutura , Doenças do Colágeno/genética , Doenças do Colágeno/patologia , Consanguinidade , Diagnóstico Diferencial , Dermatoses do Pé/genética , Dermatoses do Pé/patologia , Dermatoses da Mão/genética , Dermatoses da Mão/patologia , Humanos , Masculino , Pele/patologia , Dermatopatias Genéticas/genética , Dermatopatias Genéticas/patologia
3.
Ann Dermatol Venereol ; 137(8-9): 536-40, 2010.
Artigo em Francês | MEDLINE | ID: mdl-20804898

RESUMO

BACKGROUND: Annular elastolytic giant cell granuloma (AEGCG) is a rare form of granulomatous dermatosis. It is characterised histologically by phagocytosis of elastic fibres by multinucleated cells. We report a favourable outcome in a case of AEGCG following PUVA therapy and treatment with synthetic antimalarials. PATIENTS AND METHODS: A 67-year-old retired wine grower presented with highly pruritic annular lesions with raised borders on the shoulders and trunk that had been present for several months. Histological examination of a biopsy sample from the erythematous border was characteristic of AEGCG. Various topical treatments proved ineffective and systemic corticosteroids attenuated the patient's pruritus but had no effect on the skin lesions. PUVA therapy resulted in regression of lesions on the trunk, but the rash spread to the patient's arms and was covered with epidermal microcysts. PUVA therapy was discontinued and treatment with a synthetic antimalarial (hydroxychloroquine 400mg/d) was initiated, resulting in complete regression of the lesions. DISCUSSION: AEGC was isolated in 1979 by Hanke et al. on the basis of five cases seen in females. This is a rare form of dermatosis with some 30 cases being reported in the English literature. The clinical aspect is fairly evocative, with erythematous papular lesions, either alone or in groups, with a raised border and a lighter centre tending towards atrophy. In most cases, the lesions are found predominantly in areas exposed to sunlight. The histological appearance is characteristic, with an image of giant cell elastophagic granuloma from which the name of the disease is taken. This appearance allows the disease to be differentiated from a number of other granulomatous diseases. The aetiology is unknown and treatment is empirical. Spontaneous cure can occur and consistent results have not been obtained with any treatments. In our case, PUVA was partly successful, and the synthetic antimalarials resulted in complete regression of residual lesions.


Assuntos
Tecido Elástico/patologia , Granuloma Anular/diagnóstico , Granuloma de Células Gigantes/diagnóstico , Corticosteroides/uso terapêutico , Idoso , Antimaláricos/uso terapêutico , Antipruriginosos/uso terapêutico , Diagnóstico Diferencial , Quimioterapia Combinada , Granuloma Anular/complicações , Granuloma Anular/tratamento farmacológico , Granuloma Anular/patologia , Granuloma de Células Gigantes/complicações , Granuloma de Células Gigantes/tratamento farmacológico , Granuloma de Células Gigantes/patologia , Hemangioma/complicações , Humanos , Hidroxicloroquina/uso terapêutico , Achados Incidentais , Neoplasias Hepáticas/complicações , Masculino , Terapia PUVA , Prurido/tratamento farmacológico , Prurido/etiologia
5.
Ann Dermatol Venereol ; 135(5): 380-3, 2008 May.
Artigo em Francês | MEDLINE | ID: mdl-18457724

RESUMO

BACKGROUND: Leishmaniasis covers three well-individualized clinical variants, each due to individual species found in different geographic areas. Herein we report the first case of cutaneous leishmaniasis due to Leishmania major involving bone marrow in an AIDS patient in Burkina Faso. CASE REPORT: A 38-year-old HIV-positive man presented with generalized, copper-coloured, painless, infiltrated, itching, papulonodular lesions present over the previous 10 months. Skin biopsy confirmed the diagnosis of diffuse cutaneous leishmaniasis. The bone-marrow smear showed numerous leishmania. The culture was positive and L. major was identified. The patient was being treated with antiretroviral medication and a pentavalent antimonial compound. The disease progression consisted of attacks and remissions separated by an average of three weeks. DISCUSSION: L. major is the Leishmania species identified in Burkina Faso. It is responsible for typical cutaneous leishmaniasis but particular clinical forms have been described in immunodeficient patients, especially with diffuse cutaneous involvement. The spread of L. major infection to bone marrow could represent a public health problem in our country, where the HIV epidemic is still not under control, and particular vigilance is thus called for.


Assuntos
Medula Óssea/parasitologia , Infecções por HIV/complicações , Leishmaniose Cutânea/complicações , Adulto , Animais , Fármacos Anti-HIV/uso terapêutico , Antiprotozoários/uso terapêutico , Burkina Faso , Infecções por HIV/tratamento farmacológico , Infecções por HIV/parasitologia , Humanos , Leishmania major/efeitos dos fármacos , Leishmania major/isolamento & purificação , Leishmaniose Cutânea/tratamento farmacológico , Masculino , Resultado do Tratamento
6.
Dakar Med ; 52(2): 90-4, 2007.
Artigo em Francês | MEDLINE | ID: mdl-19102100

RESUMO

INTRODUCTION: The epidermodysplasia verruciformis is a rare, autosomic, recessive, genodermatose characterized by a chronic, disseminated, cutaneous infection with human papillomavirus. The majority of these patients have a genetic or acquired immunodeficiency. PATIENTS AND METHODS: This retrospective study was conducted on the records of all patients who presented in our dermatology department with an epidermodysplasia verruciformis in a 13 years and 6 months period, from January 1st, 1992 to June 30th, 2005. RESULTS: We have collected 45 cases of epidermodysplasia verruciformis. They were aged from 3 to 57 years, with a mean of 24.6 years. The most concerned age bracket was that from zero to 9 years. They were 29 women (64.4%) and 16 men (35.6%). The eruption presented as papules of 2 to 3 mm size, associated with hypochromic, finely squamous macules with the same size. We noted three cases of itching. We found 37.7% of family cases. We observed 14 cases of HIV positive patients and one case of cancer. CONCLUSION: This study confirmed that the epidermodysplasia verruciformis was rare. Genetic factors or immunodeficiency would support the appearance of the disease.


Assuntos
Infecções Oportunistas Relacionadas com a AIDS/epidemiologia , Epidermodisplasia Verruciforme , Soropositividade para HIV/epidemiologia , Adolescente , Adulto , Fatores Etários , Burkina Faso/epidemiologia , Criança , Pré-Escolar , Epidermodisplasia Verruciforme/diagnóstico , Epidermodisplasia Verruciforme/epidemiologia , Epidermodisplasia Verruciforme/genética , Epidermodisplasia Verruciforme/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Fatores Sexuais , Pele/patologia
7.
Hippokratia ; 11(2): 89-91, 2007 Apr.
Artigo em Inglês | MEDLINE | ID: mdl-19582185

RESUMO

Adnexal tumors of the hair follicle can be regarded as lesions that show similar differentiation to one or more portions of the hair follicle. Trichoblastic carcinoma is a rare malignant adnexal tumor, which usually occurs on the scalp. There have been reported cases with regional lymph node metastasis. We report a case of a 65 years old man with a painless irregular mass of the posterior surface of the right pinna, which was slowly growing over a 5-year period. He had a history of a similar lesion on the same site, which was removed 8 years before he presented to us. We excised the lesion and the defect was covered with an advancing flap. Because of the histology result the patient was re-operated and reviewed for over 3 years and there wasn't any sign of recurrence.

8.
Ann Dermatol Venereol ; 133(5 Pt 1): 435-8, 2006 May.
Artigo em Francês | MEDLINE | ID: mdl-16760829

RESUMO

BACKGROUND: In common intradermal or compound nevi, nevocytes may be associated with other kinds of cells, tissues or lesions. In rare cases, they are associated with notable hyperplasia of the elastic fibers. CASE-REPORTS AND RESULTS: In 11 nevi (10 intradermal or compound nevi and 1 blue nevus), we observed striking hyperplasia of the elastic fibers strictly limited to the nevi, with normal aspect of the elastic fibers in the surrounding reticular dermis. These fibers were thicker than normal elastic fibers and showed tight connexions with nevus cells. This hyperplasia was not clinically relevant. It was correlated neither with the age and sex of patients nor with the site of the excised nevi. It was seen in 0.1% of excised nevi. DISCUSSION: Hyperplasia of elastic fibers in some common nevi is a curiosity which may be classified together with other vascular, nervous, epithelial or connective twin lesions occasionally associated with the nevocytic nevi. It has to be added to the list of so-called "twin nevi".


Assuntos
Nevo/patologia , Neoplasias Cutâneas/patologia , Pele/patologia , Adolescente , Adulto , Idoso , Feminino , Humanos , Hiperplasia/patologia , Masculino , Pessoa de Meia-Idade
9.
Ann Dermatol Venereol ; 132(5): 462-5, 2005 May.
Artigo em Francês | MEDLINE | ID: mdl-15988359

RESUMO

INTRODUCTION: Cutaneous actinomycosis of the buttocks is a rare granulomatous bacterial infection that usually starts in the perianal area. We present an exceptional case in the form of a pseudo-tumor. CASE REPORT: A 69 year-old woman, in general good health, developed an indurate mass on the supra-external quadrant of the right buttock. The tumor was centered by an ulcerated nodule with a diameter of around 10 centimeters. Imaging showed invasion of the soft tissue of the skin in the internal psoas muscle, the adipose tonality of which was compatible with a liposarcoma. The skin biopsy revealed characteristic bacterial grain in the center of a cholesterol granuloma. Subsequent culture in aerobic milieu identified Actinomyces gerencseriae. Cure was obtained following complete exeresis of the fibrous tissue and 8 months of antibiotic amoxicillin-clavulanic acid therapy. DISCUSSION: Other than the most unusual clinical aspect, the originality of this case of actinomycosis of the buttocks is based on its potential appendix origin, 4 years after acute appendicitis, with slow posterior fistulation. Other cases of actinomycosis of appendix origin have been reported and its delayed onset following the intervention has been documented. The pseudo-sarcomatous aspect was responsible for diagnostic wandering. The histological image and, subsequently, the results of the bacteriological culture confirmed the diagnosis.


Assuntos
Actinomyces/patogenicidade , Actinomicose/patologia , Actinomyces/isolamento & purificação , Actinomicose/tratamento farmacológico , Actinomicose/cirurgia , Idoso , Combinação Amoxicilina e Clavulanato de Potássio/uso terapêutico , Biópsia , Nádegas/microbiologia , Nádegas/patologia , Quimioterapia Combinada/uso terapêutico , Feminino , Humanos
12.
Ann Dermatol Venereol ; 131(8-9): 795-8, 2004.
Artigo em Francês | MEDLINE | ID: mdl-15505547

RESUMO

INTRODUCTION: Superficial granulomatous pyoderma is a rare variant of pyoderma gangrenosum, which is often diagnosed at a late stage because of misleading clinical-histopathological features. We report a new case of this rare disease. CASE REPORT: A 67 year-old man presented with inflammatory lesions that had become ulcerated over the past 4 months despite prolonged antibiotic therapy. The histopathological aspect was initially suggestive of palisading granuloma annulare and subsequently a necrotizing granuloma compatible with a mycobacteriosis. The various supplementary examinations eliminated the possibility of an infectious or systemic disease. The lesions became painful and ulcerated. A second biopsy showed a neutrophilic dermatosis with dermal necrosis and a granuloma leading to the diagnosis of superficial granulomatous pyoderma. The lesions healed after 3 months of systemic corticosteroids at the dose of 1 mg/kg/day. DISCUSSION: The clinical features of superficial granulomatous pyoderma are similar to those of classical pyoderma gangrenosum. However, its histological profile is more superficial and is associated with dermal granulomas. The latter are unusual in neutrophilic dermatosis and would suggest an infectious disease. This explains the frequent diagnostic errors, with inappropriate anti-infectious treatments and notably unnecessary surgical exeresis.


Assuntos
Granuloma Piogênico/patologia , Corticosteroides/uso terapêutico , Idoso , Diagnóstico Diferencial , Granuloma Piogênico/tratamento farmacológico , Humanos , Inflamação , Masculino , Dermatopatias Bacterianas/diagnóstico , Úlcera Cutânea/patologia , Resultado do Tratamento
13.
Genet Couns ; 15(2): 175-82, 2004.
Artigo em Inglês | MEDLINE | ID: mdl-15287417

RESUMO

Familial cylindromatosis: we report a daughter with turban tumor and her mother with cylindromatosis. The dermal eccrine cylindroma arose as small, solitary lesions on the head of the mother when she was 28 years old. The following years other tumors became apparent. She was operated on several times. The first lesions appeared on the frontal part of the scalp of the daughter when she was 23 years old. Other tumors grew on the scalp. Histopathological examination of the excised tumors showed the same lesions in both the mother and the daughter: dermal eccrine cylindromata. Family history showed that the daughter's maternal aunt had a few tumors. Dermal eccrine cylindroma should be differentiated from malignant syndromes such as basal naevoid carcinoma or metastases and from neurofibromata. The gene of familial cylindromatosis was localised to chromosome 16q12-q13 and it was proposed that this gene is a tumor supressor gene.


Assuntos
Carcinoma Adenoide Cístico/genética , Neoplasias Primárias Múltiplas/genética , Síndromes Neoplásicas Hereditárias , Neoplasias Cutâneas/genética , Adulto , Carcinoma Adenoide Cístico/patologia , Feminino , Humanos , Neoplasias Primárias Múltiplas/patologia , Síndromes Neoplásicas Hereditárias/patologia , Linhagem , Neoplasias Cutâneas/patologia
14.
Ann Dermatol Venereol ; 131(3): 279-82, 2004 Mar.
Artigo em Francês | MEDLINE | ID: mdl-15107748

RESUMO

INTRODUCTION: Non T non B CD4+ CD56+ leukemia is often revealed by cutaneous lesions. We report 2 patients with this disorder who had characteristic anatomoclinical findings. CASE REPORTS: An 81 year-old female and a 75 year-old man presented with erythematous macules which increased in number and progressed to infiltrated plaques and nodules. The lesions became ecchymotic, but the patients remained in good general condition and blood count as well as bone marrow examination were unremarkable. A cutaneous biopsy revealed a lymphomatous mononuclear cell infiltrate. The cells expressed CD4 and CD56, but not CD3. The cutaneous lesions preceded for 10 and 9 months respectively the appearance of overt leukemia and medullar involvement. At this stage, the patients deceased rapidly from their leukemia. DISCUSSION: This is an original anatomoclinical syndrome. The histopathologist must pay attention to the unusual CD4+ and CD3- immunophenotype and search for CD56 expression. The malignant cell responsible for this type of leukemia is now individualized and corresponds to a type II dendritic cell precursor.


Assuntos
Antígenos CD/análise , Antígenos CD4/análise , Leucemia/complicações , Leucemia/patologia , Glicoproteínas de Membrana/análise , Neoplasias Cutâneas/etiologia , Idoso , Biópsia , Evolução Fatal , Feminino , Humanos , Imuno-Histoquímica , Proteína Cofatora de Membrana
16.
Ann Dermatol Venereol ; 131(12): 1051-4, 2004 Dec.
Artigo em Francês | MEDLINE | ID: mdl-15692437

RESUMO

BACKGROUND: Granuloma have already been described in the context of immunosuppression. We report six cases of widespread granuloma annulare occurring in patients with drug-induced immunosuppression. CASE REPORTS: There were four women and two men, with mean age of 61 years (35-76). Three patients were treated with chemotherapy for breast (2 cases) and liver cancer. One woman was given chemotherapy for Hodgkin's disease. One had undergone liver transplantation and was given cyclosporine, another was treated with systemic steroids for polyarthritis. All had generalized non-photoexposed granuloma annulare, composed of multiple erythematous papules, sometimes with annular pattern. The lesions were localized on the trunk, legs and arms. Histopathology revealed granuloma annulare in each patient. DISCUSSION: These six cases suggest a relationship between drug-induced immunodeficiency and generalized granuloma annulare. The immune dysregulation induced by the drugs may have been responsible for the formation of granuloma annulare.


Assuntos
Granuloma Anular/induzido quimicamente , Síndromes de Imunodeficiência/induzido quimicamente , Adulto , Idoso , Feminino , Humanos , Doença Iatrogênica , Masculino , Pessoa de Meia-Idade
18.
Ann Dermatol Venereol ; 130(6-7): 631-4, 2003.
Artigo em Francês | MEDLINE | ID: mdl-13679701

RESUMO

INTRODUCTION: Melanoma of the soft tissue is a rare tumor that develops in most cases in the deep structures, only rarely involving the skin surface. We report an exceptional case of melanoma of the superficial soft tissue, exclusively involving the skin surface. OBSERVATION: A 35-year-old man presented with a superficial, pink, soft, mobile, lesion on the thigh. The histological examination revealed a tumoral proliferation of the reticular dermis and hypodermis, composed of pale giant cells grouped in nests or in lobules separated by connective ducts. Immunostaining was positive for HMB45, protein S-100, vimentine and NKIC3. It was negative for EMA, CD34, smooth muscle actin, cytokeratine and desmine. The search for a metastatic localization was negative. Extensive exeresis was performed. Three years later, the patient was still in complete remission. The macroscopic, histological and immunohistochemical examinations concluded in the diagnosis of a strictly dermohypodermic melanoma of the soft tissues. DISCUSSION: Our case report of a melanoma of the soft tissues is original because of the superficial localization of the tumor that, to our knowledge, has never been reported. It underlines the interest of performing systematic biopsies of any fast growing cutaneous lesion of recent discovery.


Assuntos
Melanoma , Neoplasias de Tecidos Moles , Adulto , Biópsia , Derme/patologia , Seguimentos , Humanos , Imuno-Histoquímica , Masculino , Melanoma/diagnóstico , Melanoma/patologia , Melanoma/cirurgia , Neoplasias de Tecidos Moles/diagnóstico , Neoplasias de Tecidos Moles/patologia , Neoplasias de Tecidos Moles/cirurgia , Tela Subcutânea/patologia , Coxa da Perna , Fatores de Tempo , Resultado do Tratamento
20.
Ann Dermatol Venereol ; 130(5): 542-5, 2003 May.
Artigo em Francês | MEDLINE | ID: mdl-12843833

RESUMO

INTRODUCTION: Increased level of homocysteine has been shown to be associated with atherosclerotic disease and venous thrombosis. There are only exceptional reports of cutaneous disease due hyperhomocysteinemia. CASE REPORT: A 71-year-old man presented with an acral purpura mainly located under the nail plates and resulting in onycholysis. Histologic examination of a skin biopsy specimen showed thromboses of dermal vessels without vasculitis. Laboratory tests revealed highly elevated homocysteinemia due to a mutation in the methylene-tetra-hydrofolate-reductase gene. No other cause of purpura or thrombophilia was found. When oral folic acid was given, both homocysteinemia and cutaneous lesions were controlled. However, a biological and clinical recurrence occurred when therapy was discontinued. DISCUSSION: Hyperhomocysteinemia has probably a causal role in this original case of acral purpura. Since treatment is effective, the detection of hyperhomocysteinemia should be proposed in patients with cutaneous lesions secondary to distal vascular thromboses.


Assuntos
Hiper-Homocisteinemia/complicações , Púrpura/etiologia , Idoso , Biópsia , Ácido Fólico/administração & dosagem , Ácido Fólico/farmacologia , Hematínicos/administração & dosagem , Hematínicos/farmacologia , Humanos , Masculino , Unhas/patologia , Púrpura/patologia , Recidiva
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