Necrobiose lipoídica é uma condição rara de pele onde o colágeno, uma proteína importante da pele, se danifica. Caracteriza-se por manchas que aparecem nas pernas, geralmente abaixo dos joelhos. É mais comum em mulheres, e geralmente surgem várias manchas. São manchas um pouco elevadas, brilhantes, de cor vermelho-amarronzada. O centro dessas manchas costuma ser amarelado e pode desenvolver feridas abertas que demoram a cicatrizar. Infecções podem ocorrer, mas são raras. Alguns pacientes sentem coceira, dor ou sensações estranhas. Geralmente ocorre com mais frequência em pessoas com diabetes, em pessoas que têm histórico familiar de diabetes ou uma tendência a desenvolver a doença, mas também pode aparecer em quem não tem diabetes. Cerca de 11% a 65% dos pacientes com necrobiose lipoídica também têm diabetes, mas a causa exata ainda não é conhecida. O tratamento é difícil. A doença é geralmente crônica, com uma evolução que varia e que pode deixar cicatrizes.
Introdução
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Necrobiose lipoídica é uma condição rara de pele onde o colágeno, uma proteína importante da pele, se danifica. Caracteriza-se por manchas que aparecem nas pernas, geralmente abaixo dos joelhos. É mais comum em mulheres, e geralmente surgem várias manchas. São manchas um pouco elevadas, brilhantes, de cor vermelho-amarronzada. O centro dessas manchas costuma ser amarelado e pode desenvolver feridas abertas que demoram a cicatrizar. Infecções podem ocorrer, mas são raras. Alguns pacientes sentem coceira, dor ou sensações estranhas. Geralmente ocorre com mais frequência em pessoas com diabetes, em pessoas que têm histórico familiar de diabetes ou uma tendência a desenvolver a doença, mas também pode aparecer em quem não tem diabetes. Cerca de 11% a 65% dos pacientes com necrobiose lipoídica também têm diabetes, mas a causa exata ainda não é conhecida. O tratamento é difícil. A doença é geralmente crônica, com uma evolução que varia e que pode deixar cicatrizes.
Escala de raridade
<1/50kMuito rara
1/20kRara
1/10kPouco freq.
1/5kIncomum
1/2k
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Sinais e sintomas
O que aparece no corpo e com que frequência cada sintoma acontece
Partes do corpo afetadas
+ 8 sintomas em outras categorias
Características mais comuns
Os sintomas variam de pessoa para pessoa. Abaixo estão as 21 características clínicas mais associadas, ordenadas por frequência.
Linha do tempo da pesquisa
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Genética e causas
O que está alterado no DNA e como passa nas famílias
Nenhum gene associado encontrado
Os dados genéticos desta condição ainda estão sendo catalogados.
Diagnóstico
Os sinais que médicos procuram e os exames que confirmam
Tratamento e manejo
Remédios, cuidados de apoio e o que precisa acompanhar
Onde tratar no SUS
Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)
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Activating mutations in the ABCC8 gene are extremely rare and cause ABCC8-MODY. The phenotype is variable with onset of diabetes in childhood/early adulthood. Retinopathy is the most common reported complication. We describe a 31-year-old primigravida woman referred to and seen at our antenatal ambulatory diabetes clinic at 6 weeks plus 5 days gestation. She had a strong family history of diabetes and was diagnosed at the age of 11 years. Genetic testing revealed an activating pathogenic c.4139G>A variant in the ABCC8 gene. She was managed with glibenclamide, sitagliptin, and dapagliflozin. Her complications included mild bilateral non-proliferative retinopathy and necrobiosis lipoidica. Her BMI was 19 kg/m2, and HbA1c was 68 mmol/mol. Oral agents were discontinued, and insulin therapy was commenced. At 22 weeks gestation, routine retinal screening identified progression to bilateral active proliferative diabetic retinopathy (time in range for pregnancy (TIRp) 68%, HbA1c 39 mmol/mol). She received four sessions of panretinal photocoagulation (PRP) bilaterally between 22 and 33 weeks gestation. There was no associated loss of vision or nephropathy. TIRp was ≥70% for the remainder of the pregnancy. She delivered a 3.9 kg unaffected female infant at 38 weeks via elective caesarean section without maternal or neonatal complications. Bilateral active proliferative retinopathy persisted up to 61 weeks postnatally and required additional PRP. Forty-six weeks post-partum, after ceasing breastfeeding, insulin was switched to glibenclamide and dapagliflozin. This is the first case report of rapid progression of clinically significant diabetic retinopathy during pregnancy in a woman with ABCC8-MODY. This is an unusual finding as there is a relatively low risk of significant progression of diabetic retinopathy in women with type 2 diabetes during pregnancy. Rapid progression of diabetic retinopathy during pregnancy is an uncommon complication in women with type 2 diabetes. This woman with ABCC8-MODY developed rapidly progressive clinically significant diabetic retinopathy without nephropathy during pregnancy. There is a paucity of literature regarding pregnancy-related complications of ABCC8-MODY. This case highlights the risks of end organ disease in a rare form of diabetes.
S1-Guideline for diagnosis and therapy of necrobiosis lipoidica.
Necrobiosis lipoidica (NL) is a rare granulomatous skin disease of unknown etiology that occurs frequently in association with diabetes mellitus and other comorbidities. The predilection site is the lower leg, particularly the pretibial areas. The exact pathogenesis remains unclear. Vascular disorders with microangiopathic changes and an autoimmune genesis are discussed. Necrobiosis lipoidica occurs three to six times more frequently in women. Men tend to show a more severe course and develop ulcerations more frequently. The diagnosis can often be established based on typical clinical and dermatoscopic findings. A biopsy should be performed in clinically unclear cases, in the presence of ulceration, or if there are signs of malignant transformation. Overall, the scientific data available for NL are still insufficient and there is a need for further research. However, as patients often experience a severely impaired quality of life, it is important to be aware of the limited scientific evidence and to translate it into practical therapeutic recommendations. This short version of the S1 guideline of the German Dermatological Society (DDG) summarizes the current evidence and, incorporating expert assessments, provides specific recommendations for everyday clinical practice.
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Obesity is a major global health concern characterized by excessive fat accumulation, which significantly increases the risk of numerous comorbidities. While lifestyle modifications and pharmacotherapy are commonly employed, bariatric surgery is recognized as a highly effective treatment option. These procedures alter gastrointestinal anatomy, restricting food intake and modifying nutrient absorption, thereby reducing hunger and increasing satiety. Beyond weight reduction, bariatric surgery can improve or resolve obesity-related conditions, including type 2 diabetes, hypertension, sleep apnea, and dyslipidemia. In addition to obesity, patients undergoing bariatric surgery frequently present with diverse skin disorders, such as hidradenitis suppurativa, psoriasis, necrobiosis lipoidica, skin tags, acanthosis nigricans, striae, keratosis pilaris, hyperhidrosis, plantar hyperkeratosis, intertrigo, pseudoacanthosis nigricans, lymphedema, bacterial infections, and confluent and reticulated papillomatosis. Bariatric surgery has been reported to improve or resolve conditions such as acanthosis nigricans, confluent and reticulated papillomatosis, necrobiosis lipoidica, hidradenitis suppurativa, psoriasis, hirsutism, skin tags, intertrigo, keratosis pilaris, and pebble fingers. Conversely, it may precipitate or exacerbate other conditions, including xeroderma, sporotrichosis, prurigo pigmentosa, bowel-associated dermatitis-arthritis syndrome, pellagra, disseminated intravascular coagulation, purpura, vasculitis, panniculitis, and alopecia. The relationship between obesity, weight loss, and skin health in patients undergoing bariatric surgery is complex, involving mechanisms such as inflammation, hormonal alterations, and mechanical stress on the skin. This study aims to investigate the effects of bariatric surgery on the progression and development of skin disorders, evaluating both potential improvements and the emergence of new conditions postoperatively. In summary, bariatric surgery exerts multifaceted and sometimes conflicting effects on skin health.
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JAAD case reportsRemission of necrobiosis lipoidica diabeticorum with a JAK1/2 inhibitor: A case report.
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International wound journal[Rare skin condition in a child with diabetes mellitus].
Nederlands tijdschrift voor geneeskundeSarcoidosis With Cutaneous Manifestations of Necrobiosis Lipoidica.
The American Journal of dermatopathologyCutaneous Manifestations of Diabetes Mellitus and Prediabetes.
Acta medica portuguesaNecrobiosis lipoidica in a patient with monoclonal gammopathy of underdetermined significance.
The Australasian journal of dermatologyGranulomatous Phlebitis in Necrobiosis Lipoidica.
The American Journal of dermatopathologyNon-infectious granulomatous dermatoses.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGYouth-Onset Type 2 Diabetes Manifestations in other Specialties: Its Many Disguises.
Annals of nutrition & metabolismConcomitant necrobiosis lipoidica and splenic abscess.
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JAAD case reportsNecrobiosis lipoidica with bone involvement successfully treated with infliximab.
Rheumatology (Oxford, England)Fatal squamous cell carcinoma from necrobiosis lipoidica diabeticorum in a diabetic patient.
Endocrinology, diabetes & metabolism case reportsClinical Features and Comorbidities of Patients With Necrobiosis Lipoidica With or Without Diabetes.
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CutisPapular spreading necrobiosis lipoidica: a rare clinical presentation of this pathology.
Clinical and experimental dermatologyThe 2017 Banting Memorial Lecture The diabetic lower limb - a forty year journey: from clinical observation to clinical science.
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The Journal of dermatological treatmentAtypical presentation of necrobiosis lipoidica in a pediatric patient.
Pediatric dermatologyConcomitant granuloma annulare and necrobiosis lipoidica: do they have a related pathogenesis?
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Dermatologic clinicsPotassium Iodide for Cutaneous Inflammatory Disorders: A Monocentric, Retrospective Study.
Dermatology (Basel, Switzerland)Penile necrobiosis lipoidica: case report and literature review.
Clinical and experimental dermatologyAn atypical case of papular necrobiosis lipoidica masquerading as sarcoidosis.
JAAD case reportsNecrobiosis lipoidica associated with sarcoidosis.
Journal of cutaneous pathologyDermatoscopy of Granulomatous Disorders.
Dermatologic clinicsUlcerated Necrobiosis Lipoidica.
Acta medica portuguesaNecrobiosis lipoidica: a rare complication of diabetes.
The Pan African medical journalAtrophic and Annular Scarring Alopecia of the Scalp as a Finding in Underlying Systemic Sarcoidosis.
Acta dermatovenerologica Croatica : ADCSystemic therapies for leg ulcers.
Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDGImprovement in Ulcerative Necrobiosis Lipoidica After Janus Kinase-Inhibitor Therapy for Polycythemia Vera.
JAMA dermatologyUlcerated necrobiosis lipoidica: A cutaneous granulomatous reaction associated with systemic B-cell lymphoma.
Indian journal of dermatology, venereology and leprologyUnconventional use of fractional ablative CO2 laser in necrobiosis lipoidica.
Journal of cosmetic and laser therapy : official publication of the European Society for Laser DermatologyPhotodynamic therapy of necrobiosis lipoidica using methyl aminolevulinate: A retrospective follow-up study.
Photodiagnosis and photodynamic therapyKeratoacanthomatous Changes: Unifying the Histologic Spectrum of Actinic Granuloma.
The American Journal of dermatopathologyUstekinumab to target granulomatous dermatitis in recalcitrant ulcerative necrobiosis lipoidica: case report and proposed mechanism.
Dermatology online journalThe long-term effect of pulsed dye laser on Necrobiosis Lipoidica: A case study.
Journal of cosmetic and laser therapy : official publication of the European Society for Laser DermatologyGranulomatous skin disease with a histological pattern of palisading granuloma - an atypical facial necrobiosis lipoidica or more?
Postepy dermatologii i alergologiiNecrobiosis lipoidica with mucin deposition in a patient with autoimmune thyroiditis.
The Journal of dermatologyCutaneous Granulomatosis: a Comprehensive Review.
Clinical reviews in allergy & immunologyMiscellaneous skin disease and the metabolic syndrome.
Clinics in dermatologyGranulomatosis disciformis in a non-diabetic patient.
Anais brasileiros de dermatologiaDermatologic manifestations of endocrine disorders.
Translational pediatricsGranuloma annulare can occur on a scar, mimicking sarcoidosis.
Clinical and experimental dermatologyRefractory Ulcerated Necrobiosis Lipoidica: Closure of a Difficult Wound with Topical Tacrolimus.
Advances in skin & wound careXanthoma-like Skin Changes in an Elderly Woman with a Normal Lipid Profile.
Acta dermatovenerologica Croatica : ADCInterstitial Granulomatous Dermatitis (IGD).
Open access Macedonian journal of medical sciencesImage Gallery: Never miss the details: the importance of dermoscopy in the differential diagnosis of coexistent inflammatory disorders.
The British journal of dermatologyPainful rash on hospitalized patient with diabetic ketoacidosis (DKA).
European journal of internal medicineA case of annular elastolytic giant cell granuloma on unusual sites requiring the differential diagnosis of necrobiosis lipoidica.
European journal of dermatology : EJDNarrow-band UVB for pretibial (necrobiosis lipoidica-like) involvement of cutaneous sarcoidosis: a promising therapeutic option.
European journal of dermatology : EJDNecrobiosis lipoidica following Q-switched laser tattoo removal.
The Australasian journal of dermatologyA systematic review of the relationship between glycemic control and necrobiosis lipoidica diabeticorum in patients with diabetes mellitus.
International journal of dermatology[Evidence of compression therapy].
Der Hautarzt; Zeitschrift fur Dermatologie, Venerologie, und verwandte GebieteAssociações
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Referências e fontes
Bases de dados externas citadas neste artigo
Publicações científicas
Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.
- Subcutaneous Granuloma Annulare in a Middle-Aged Patient: A Case Report.
- Progression of diabetic retinopathy during pregnancy in a woman with ABCC8-MODY.
- S1-Guideline for diagnosis and therapy of necrobiosis lipoidica.Journal der Deutschen Dermatologischen Gesellschaft = Journal of the German Society of Dermatology : JDDG· 2026· PMID 41420334mais citado
- The Impact of Bariatric Surgery on the Development and Progression of Dermatologic Diseases: A Narrative Review.
- Ulcerated necrobiosis lipoidica: clinical and histopathological presentation of two cases.
- Association between palisaded and interstitial granulomatous dermatoses and haematological neoplasms: evidence beyond coincidence?
- Necrobiosis Lipoidica.
- Cutaneous Squamous Cell Carcinoma Developing in Necrobiosis Lipoidica - A Case Report with Literature Review.
- Necrobiosis Lipoidica in Pregnancy Complicated by Gestational Diabetes Mellitus: A Case Report.
Bases de dados e fontes oficiais
Identificadores e referências canônicas usadas para montar este verbete.
- ORPHA:542592(Orphanet)
- MONDO:0006583(MONDO)
- GARD:13040(GARD (NIH))
- Busca completa no PubMed(PubMed)
- Artigo Wikipedia(Wikipedia)
Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.
Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar
