Assuntos
Doenças Palpebrais/cirurgia , Granuloma de Corpo Estranho/induzido quimicamente , Transtornos Necrobióticos/induzido quimicamente , Platina/efeitos adversos , Complicações Pós-Operatórias , Idoso , Blefaroplastia/métodos , Remoção de Dispositivo , Feminino , Granuloma de Corpo Estranho/diagnóstico , Granuloma de Corpo Estranho/cirurgia , Humanos , Transtornos Necrobióticos/diagnóstico , Transtornos Necrobióticos/cirurgia , Próteses e ImplantesRESUMO
BACKGROUND: Uterine fibroids are the most common uterine tumours in females of reproductive age. During pregnancy, uterine fibroids may be complicated by aseptic necrobiosis. We herein report an ambiguous clinical presentation of uterine fibroids in pregnancy and discuss the diagnostic challenges encountered in our resource-constraint setting. CASE PRESENTATION: A term pregnant Cameroonian woman was admitted to our maternity unit with clinical findings suggestive of a strangulated umbilical hernia. She underwent an emergency caesarean section which fortuitously revealed aseptic necrobiosis of a uterine fibroid, managed within the same surgical intervention by myomectomy. Her post-operative course was uneventful. CONCLUSION: The authors highlight the need for a high index of suspicion by healthcare providers, as well as the need for a multidisciplinary approach for a favourable maternal and foetal outcome.
Assuntos
Leiomioma/diagnóstico , Transtornos Necrobióticos/diagnóstico , Complicações Neoplásicas na Gravidez/diagnóstico , Neoplasias Uterinas/diagnóstico , Adulto , Camarões , Cesárea , Diagnóstico Diferencial , Feminino , Humanos , Gravidez , Nascimento a Termo , Miomectomia Uterina , Útero/patologia , Útero/cirurgiaAssuntos
Anticorpos Monoclonais Humanizados/uso terapêutico , Doença de Crohn/tratamento farmacológico , Pneumopatias/etiologia , Nódulos Pulmonares Múltiplos/etiologia , Transtornos Necrobióticos/etiologia , Adulto , Anticorpos Monoclonais Humanizados/efeitos adversos , Doença de Crohn/complicações , Doença de Crohn/patologia , Diagnóstico Diferencial , Humanos , Pulmão/diagnóstico por imagem , Pulmão/patologia , Pneumopatias/diagnóstico , Pneumopatias/diagnóstico por imagem , Pneumopatias/patologia , Masculino , Nódulos Pulmonares Múltiplos/diagnóstico , Nódulos Pulmonares Múltiplos/diagnóstico por imagem , Nódulos Pulmonares Múltiplos/patologia , Transtornos Necrobióticos/diagnóstico , Transtornos Necrobióticos/diagnóstico por imagem , Transtornos Necrobióticos/patologia , Tomografia Computadorizada por Raios XAssuntos
Doença de Crohn/complicações , Granulomatose com Poliangiite/diagnóstico , Pulmão/patologia , Transtornos Necrobióticos/diagnóstico , Criança , Diagnóstico Diferencial , Feminino , Fluordesoxiglucose F18 , Granulomatose com Poliangiite/complicações , Humanos , Infecções/diagnóstico , Nódulos Pulmonares Múltiplos/etiologia , Transtornos Necrobióticos/complicações , Tomografia por Emissão de PósitronsRESUMO
A well appearing 73-year-old Caucasian lady presented with a long-standing history of yellowish atrophic lesions on her limbs and trunk. The lesions were asymptomatic. These were found to be consistent with the diagnosis of Necrobiotic Xanthogranuloma (NXG) on histology. The reported patient did not have the characteristic skin changes and instead had unusual lesions reminiscent of morphoea not described in the literature. She reported no associated systemic symptoms.
Assuntos
Clobetasol/uso terapêutico , Granuloma/diagnóstico , Transtornos Necrobióticos/diagnóstico , Paraproteinemias/diagnóstico , Xantomatose/diagnóstico , Idoso , Anti-Inflamatórios/uso terapêutico , Feminino , Glucocorticoides/uso terapêutico , Granuloma/tratamento farmacológico , Granuloma/patologia , Humanos , Transtornos Necrobióticos/tratamento farmacológico , Transtornos Necrobióticos/patologia , Paraproteinemias/tratamento farmacológico , Paraproteinemias/patologia , Xantomatose/tratamento farmacológico , Xantomatose/patologiaRESUMO
We conducted the current study to define within the spectrum of the neutrophilic dermatoses a group of patients with an urticarial rash clinically and a neutrophilic dermatosis histopathologically. We reviewed the literature on neutrophilic urticaria and we report here a series of patients with this unique presentation. We reviewed all cutaneous biopsies submitted to our department between 2000 and 2006 in which histopathologic evaluation was compatible with this entity. We then retrieved the patient medical records and obtained information about follow-up and associated diseases. This allowed us to identify 9 patients with an urticarial eruption that was characterized histopathologically by a perivascular and interstitial neutrophilic infiltrate with intense leukocytoclasia but without vasculitis and without dermal edema. Four patients also had small foci of necrobiotic collagen bundles. The eruption consisted of pale, flat or only slightly raised, nonpruritic macules, papules, or plaques. Elementary lesions resolved within 24 hours. Purpura, angioedema, and facial swelling were not seen, but dermographism was present in 1 patient. Six patients had fever, 7 had polyarthritis, and 6 had leukocytosis. Seven patients had associated systemic diseases: adult-onset Still disease (3 patients), systemic lupus erythematosus (3 patients), and Schnitzler syndrome (1 patient).A similar rash has been reported previously in the literature, mostly in patients with systemic inflammatory diseases, but the majority of patients reported under the undefined designation of "neutrophilic urticaria" did have a different clinicopathologic presentation. Thus, we suggest naming this eruption "neutrophilic urticarial dermatosis," to emphasize that this entity expands the broad group of cutaneous manifestations of neutrophilic aseptic disease. This entity bears important medical significance as it is strongly indicative of an associated systemic disease, mainly Schnitzler syndrome, adult-onset Still disease, lupus erythematosus, and the hereditary autoinflammatory fever syndromes.
Assuntos
Síndrome de Sweet/diagnóstico , Adolescente , Adulto , Idoso , Biópsia , Colágeno/ultraestrutura , Comorbidade , Diagnóstico Diferencial , Feminino , Humanos , Lúpus Eritematoso Sistêmico/diagnóstico , Lúpus Eritematoso Sistêmico/patologia , Masculino , Pessoa de Meia-Idade , Transtornos Necrobióticos/diagnóstico , Transtornos Necrobióticos/patologia , Neutrófilos/patologia , Síndrome de Schnitzler/diagnóstico , Síndrome de Schnitzler/patologia , Pele/patologia , Doença de Still de Início Tardio/diagnóstico , Doença de Still de Início Tardio/patologia , Síndrome de Sweet/patologia , Adulto JovemAssuntos
Granuloma/patologia , Transtornos Necrobióticos/diagnóstico , Xantomatose/patologia , Biópsia por Agulha , Células da Medula Óssea/patologia , Quimioterapia Combinada , Feminino , Granuloma/diagnóstico , Granuloma/tratamento farmacológico , Humanos , Imuno-Histoquímica , Masculino , Transtornos Necrobióticos/tratamento farmacológico , Índice de Gravidade de Doença , Xantomatose/diagnóstico , Xantomatose/tratamento farmacológicoRESUMO
Granulomatous slack skin disease (GSS) is a very rare form of T-cell lymphoma, with only 52 cases reported in the literature. In the recent World Health Organization-European Organization for Research and Treatment of Cancer consensus classification GSS is considered to be a variant of mycosis fungoides. We describe a patient with GSS and histologic evidence of necrobiosis, which has not been previously reported.
Assuntos
Linfoma Cutâneo de Células T/complicações , Linfoma Cutâneo de Células T/diagnóstico , Transtornos Necrobióticos/complicações , Transtornos Necrobióticos/diagnóstico , Neoplasias Cutâneas/complicações , Neoplasias Cutâneas/diagnóstico , Biópsia , Linfócitos T CD4-Positivos/metabolismo , Linfócitos T CD4-Positivos/patologia , Humanos , Antígenos Comuns de Leucócito/metabolismo , Linfoma Cutâneo de Células T/patologia , Masculino , Pessoa de Meia-Idade , Transtornos Necrobióticos/patologia , Neoplasias Cutâneas/patologia , Organização Mundial da SaúdeRESUMO
We present a case report of necrobiotic xanthogranuloma (NXG) in a 76-year-old Caucasian lady occurring as a nodule in a blepharoplasty scar. NXG is a rare histiocytic disease with progressive orbital and systemic features. Management options of excision biopsy or chemotherapy are discussed.
Assuntos
Cicatriz Hipertrófica/patologia , Doenças Palpebrais/patologia , Granuloma/patologia , Transtornos Necrobióticos/patologia , Doenças Orbitárias/patologia , Xantomatose/patologia , Idoso , Biópsia por Agulha , Cicatriz Hipertrófica/complicações , Quimioterapia Combinada , Doenças Palpebrais/diagnóstico , Doenças Palpebrais/tratamento farmacológico , Feminino , Seguimentos , Granuloma/diagnóstico , Granuloma/cirurgia , Humanos , Imuno-Histoquímica , Melfalan/administração & dosagem , Transtornos Necrobióticos/diagnóstico , Transtornos Necrobióticos/cirurgia , Doenças Orbitárias/diagnóstico , Doenças Orbitárias/cirurgia , Prednisolona/administração & dosagem , Recidiva , Medição de Risco , Resultado do Tratamento , Xantomatose/diagnóstico , Xantomatose/cirurgiaRESUMO
Necrobiotic xanthogranuloma (NXG) is an uncommon granulomatous disease involving the skin and extracutaneous tissues. In its typical clinical presentation, it consists of multiple indurate yellow-brownish plaques or nodules, usually located on the periorbital area. The lesions often are bilateral and symmetric and frequently show tendency to atrophy and ulceration. Most cases are asymptomatic, and oral mucosal erosions are sometimes present. Ophthalmologic complications are observed in many patients, and NXG may involve other extracutaneous sites. Paraproteinemia is closely associated with NXG, demonstrating a monoclonal gammapathy, and other hematologic or lymphoproliferative disorders also may appear in association with NXG. Large areas of degenerated collagen alternating with granulomatous inflammation, multinucleate giant cells, cholesterol clefts, and lymphoid follicles are the most outstanding histopathologic characteristics of NXG. The prognosis for NXG is good, but no curative treatment is available. Chemotherapy with alkylating agents is the most frequently administered treatment, but oral and intralesional corticosteroids, interferon alpha, plasmapheresis, surgical excision, and radiation therapy also have been used for treatment of this disorder, with variable results.
Assuntos
Granuloma/diagnóstico , Transtornos Necrobióticos/diagnóstico , Xantomatose/diagnóstico , Diagnóstico Diferencial , Granuloma/patologia , Granuloma/terapia , Humanos , Transtornos Necrobióticos/patologia , Transtornos Necrobióticos/terapia , Prognóstico , Xantomatose/patologia , Xantomatose/terapiaAssuntos
Edema/etiologia , Transtornos Necrobióticos/diagnóstico , Doenças Orbitárias/etiologia , Edema/cirurgia , Feminino , Humanos , Cirrose Hepática/complicações , Linfócitos/patologia , Macrófagos/patologia , Pessoa de Meia-Idade , Transtornos Necrobióticos/complicações , Transtornos Necrobióticos/cirurgia , Doenças Orbitárias/cirurgia , Plasmócitos/patologia , Esplenomegalia/complicações , Trombocitopenia/complicaçõesRESUMO
Histiocytoses are a heterogeneous group of disorders that are characterized by the proliferation and accumulation of reactive or neoplastic histiocytes. Three classes of histiocytoses have been defined: class I, Langerhans cell disease; class II, non-Langerhans cell histiocytic disease without features of malignancy; and class III, malignant histiocytic disorders. Although the disorders in classes I and II usually have a benign appearance on histology and are commonly non-aggressive and self-healing, some can cause debilitating or even fatal outcomes. Such cases beg the question: what stimulates aggressive behavior of a classically benign disease? New molecular information may now provide insight into the driving force behind many of the aggressive histiocytoses. In this article, we review Langerhans cell disease and seven aggressive histiocytoses that can involve skin, discuss histologic features that may forecast a poor prognosis, and discuss the molecular findings that help to explain the pathophysiology of these aggressive histiocytic disorders.
Assuntos
Dermatopatias , Histiocitose/classificação , Histiocitose/patologia , Histiocitose/fisiopatologia , Histiocitose de Células de Langerhans/diagnóstico , Histiocitose de Células de Langerhans/patologia , Histiocitose de Células de Langerhans/fisiopatologia , Histiocitose de Células não Langerhans/diagnóstico , Histiocitose de Células não Langerhans/patologia , Histiocitose de Células não Langerhans/fisiopatologia , Histiocitose de Células não Langerhans/terapia , Humanos , Linfo-Histiocitose Hemofagocítica/patologia , Linfo-Histiocitose Hemofagocítica/fisiopatologia , Transtornos Necrobióticos/diagnóstico , Transtornos Necrobióticos/patologia , Transtornos Necrobióticos/fisiopatologia , Transtornos Necrobióticos/terapia , Prognóstico , Síndrome do Histiócito Azul-Marinho/patologia , Síndrome do Histiócito Azul-Marinho/fisiopatologia , Síndrome do Histiócito Azul-Marinho/terapia , Dermatopatias/diagnóstico , Dermatopatias/patologia , Dermatopatias/fisiopatologia , Dermatopatias/terapia , Xantogranuloma Juvenil/patologia , Xantogranuloma Juvenil/fisiopatologia , Xantogranuloma Juvenil/terapiaRESUMO
Granuloma multiforme is a rare granulomatous skin disease, usually reported in sub-Saharan African countries. The exact aetiology of granuloma multiforme is still unknown. We report the case of a patient who presented clinical and histopathological features of granuloma multiforme that can be considered the first described case in Tunisia.
Assuntos
Transtornos Necrobióticos/patologia , África Subsaariana/epidemiologia , Diagnóstico Diferencial , Granuloma Anular/diagnóstico , Humanos , Índia/epidemiologia , Masculino , Pessoa de Meia-Idade , Transtornos Necrobióticos/diagnóstico , Transtornos Necrobióticos/epidemiologia , Tunísia/epidemiologiaRESUMO
We report a 65-year-old Japanese woman who presented with a nodule on the nose near the left eye. Histological examination of the totally resected lesion revealed the typical features of necrobiotic xanthogranuloma (NXG). However, her laboratory investigation revealed no paraproteinemia. We believe that the present case represents a cutaneous variant of NXG without accompaniment of paraproteinemia or any systemic involvement.
Assuntos
Granuloma/diagnóstico , Transtornos Necrobióticos/diagnóstico , Dermatopatias/diagnóstico , Xantomatose/diagnóstico , Idoso , Diagnóstico Diferencial , Feminino , Granuloma/patologia , Humanos , Transtornos Necrobióticos/patologia , Nariz/patologia , Paraproteinemias , Dermatopatias/patologia , Xantomatose/patologiaRESUMO
BACKGROUND: Necrobiotic xanthogranuloma is an extremely rare form of histiocytosis that presents clinically as yellowish infiltrated plaques or nodules. Ocular involvement is seen in over 80% of cases. Histopathology reveals numerous xanthomous histiocytes and collagen necrobiosis. Benign monoclonal gammopathy associated with myeloma is found in 80% of patients, but the course is normally long, with 100% survival at 10 years. CASE REPORT: A 76-year-old man presented skin lesions that subsequently became ulcerated. The clinical appearance and histopathological examination resulted in diagnosis of necrobiotic xanthogranuloma. No monoclonal gammopathy or myeloma was seen. The disease was marked by sensitivity to corticosteroids with failure of other therapies (cyclophosphamide, alpha interferon), onset ofcorticosteroid dependency, iatrogenic Cushing's syndrome and diabetes, which were in part responsible for the infectious complications and subsequent death of the patient. DISCUSSION: Necrobiotic xanthogranuloma is difficult to treat, even in the absence of myeloma or monoclonal gammopathy. Corticosteroids are probably the most efficacious treatment, but can give rise to multiple complications, resulting in this particular case in death of the patient.
Assuntos
Granuloma/diagnóstico , Transtornos Necrobióticos/diagnóstico , Xantomatose/diagnóstico , Idoso , Evolução Fatal , Humanos , Masculino , Choque Séptico/etiologia , Úlcera/etiologiaRESUMO
Necrobiotic xanthogranuloma is a rare cutaneous condition that can be mistaken for atypical necrobiosis lipoidica. It has a strong association with a paraproteinaemia, which may progress to frank haematological malignancy. We describe four patients with variable cutaneous features, and their treatment response.
Assuntos
Granuloma/diagnóstico , Transtornos Necrobióticos/diagnóstico , Xantomatose/diagnóstico , Idoso , Idoso de 80 Anos ou mais , Antineoplásicos Alquilantes/uso terapêutico , Clorambucila/uso terapêutico , Ciclofosfamida/uso terapêutico , Feminino , Granuloma/tratamento farmacológico , Humanos , Masculino , Pessoa de Meia-Idade , Transtornos Necrobióticos/tratamento farmacológico , Esteroides/uso terapêutico , Resultado do Tratamento , Xantomatose/tratamento farmacológicoRESUMO
The clinical features of necrobiotic xanthogranuloma (NX) have been presented in a series of case reports, but there are hardly any reports on the clinical long-term course of this rare and usually chronic entity. Therapeutic recommendations are mostly based on individual observations, lacking general therapeutic guidelines. To illustrate a typical chronic and progressive course of NX, we report the case of a 64-year-old woman with periorbital NX, ocular involvement and IgG paraproteinemia. Diagnosed with NX for the first time in 1993, the patient was already presented in this journal in 1995. Since then a series of therapeutic options including medication with dapsone, chlorambucil, interferon-alpha, clofazimine, melphalan, fumaric acid esters, surgical treatment and other physical therapies such as radiation, plasmapheresis, and photodynamic therapy have been applied. None of these therapeutic approaches, however, showed a satisfying long-term effect. At present the patient is undergoing cyclophosphamide-dexamethasone pulse therapy.