慢性酒精中毒合并烟酸缺乏病1例

烟酸缺乏病是由于人体烟酸缺乏或不足而产生，临床症状主要表现在皮肤、胃肠道及神经系统。笔者于1995年6月14日在我院皮肤科门诊治疗1例长期大量饮酒而导致烟酸缺乏的患者，现将治疗情况报告如下：     

烟酸缺乏症的研究进展

烟酸缺乏症又名糙皮病,是一种营养缺乏性疾病,同时也是累及多个系统的复杂性皮肤病,本文从流行病学、病因和发病机制、临床表现、实验室检查、诊断和鉴别诊断、治疗等方面对烟酸缺乏症的研究进展做一综述。     

1例烟酸缺乏症患者的护理体会

烟酸缺乏症是主要由于烟酸缺乏而引起的以皮肤黏膜、消化系统和神经系统症状为主的疾病。我科于2007年6月18日收治1例烟酸缺乏症患者,经过积极治疗及精心护理,患者于20天后康复出院,现将临床护理经验介绍如下。     

烟酸缺乏症的研究进展

烟酸缺乏症又名陪拉格,是一种营养缺乏性疾病,同时又是光敏性疾病。本文从流行病学、病因及发病机理、临床特征、实验室检查、诊断及鉴别诊断和治疗等方面对烟酸缺乏症的研究进展作一综述。     

烟酸缺乏症3例

报告3例烟酸缺乏症。3例患者口服烟酰胺治疗后皮损改善明显。其中1例出现幻视,幻触等戒酒综合征症状,病情发展迅速,提示与烟酸缺乏症精神异常鉴别的重要性。     

烟酸肌醇酯致固定性药疹1例

患者男性，60岁。因糖尿病等入院。入院后给予胰岛素及口服降糖药治疗，入院第3天开始加服烟酸肌醇酯400mgt．i．d。当服至总量为1．2g时，患者发现龟头部肿胀、瘦痒。检查：龟头部有一水肿性红斑，约3cmX3cm。遂停眼烟酸肌醉酯（口服降糖药未停），口服息斯敏10mgq．d，局部水肿消退、搔痒减轻。患者又服烟酸肌醉酯400mg，约3小时后龟头部又懂痒。立即停烟酸肌醉酯并继续口服息斯敏10mgq．d.，一周后皮疹消退。烟酸肌醇酯致固定性药疹1例@姜新红$山东德州地区中医院     

烟酸缺乏症1例

患者女，24岁。精神遭受打击后开始厌食3个月，双臂、面颈出现暗红斑2个月，口腔溃疡、腹泻半个月。实验室检查结果显示贫血、低蛋白血症。诊断为烟酸缺乏症。给予复合维生素B、烟酰胺、富马酸亚铁治疗2周，症状明显缓解。     

208例SLE临床和免疫学特征分析

为探讨皮肤科门诊定期就诊的SLE患者临床特征，自身抗体分布及长期皮质类固醇治疗引起的副作用，统计分析了208例SLE的首发症状及病程中临床表现和自身抗体分布情况，并分年龄、性别组进行分析，比较。结果：所有患者一般特征，临床表现和免疫学特征和国内外报道基本一致，首发症状为蝶形红斑者占60.58%，少年组及年轻男性组内脏受累重，皮类固醇副作用以浅表真菌感染最常见。     

血管炎的诊断

血管炎是一种病理学概念，是指组织病理学上血管壁及其周围组织的炎症性改变，包括血管内皮肿胀、红细胞外溢、血管壁及周围有纤维蛋白样物质沉积及炎细胞浸润，严重者有血栓形成、甚至整个血管的破坏。皮肤血管炎可表现出谱系改变，临床表现复杂多变，诊断和鉴别诊断富有挑战性。1临床表现一般而言，皮肤血管炎常伴有不同程度的系统症状，如发热、不适、体重减轻和关节痛等。皮损表现为红斑、丘疹、紫癜、皮下结节、水疱、血疱、坏死、溃疡等，多见于下肢，而上肢、躯干及头面部皮损少见，如果存在多提示有严重疾病或系统性血管炎。不同的临床表现，可以提示可能累及的血管大小（见表1）。     

局限型大疱性类天疱疮1例

患者女,75岁,手、足起红斑、大疱伴瘙痒8月。皮损局限于手足,结合组织病理、免疫病理确诊为局限型大疱性类天疱疮。予口服氨苯砜、四环素、烟酸片及外用强效皮质类固醇激素等治疗后症状明显缓解。     

Pellagra: a sporadic pediatric case with a full triad of symptoms

Pellagra is clinically manifested by a photosensitive dermatitis, diarrhea, and dementia. The full triad of symptoms is usually not well developed in infants and children. We report a case of a 14-year-old boy with classic symptoms of pellagra. All his symptoms responded to treatment with nicotinic acid.     

Pellagra a review exploring causes and mechanisms,including isoniazid-induced pellagra

Pellagra is a clinical syndrome caused by a deficiency of niacin (nicotinic acid) and/or its precursor tryptophan. The cardinal manifestations are 4 D’s: dermatitis, diarrhoea, dementia and in worst case death. Increased use of isoniazid prophylaxis along with antiretroviral therapy in countries where latent tuberculosis is common has been associated with increased presentations with pellagra.     

Letter: Pellagra as the initial presentation of Crohn disease

Pellagra is a nutritional disease caused by the deficiency of niacin. We describe a case of pellagra as the initial presentation of Crohn disease, which has been rarely described in the literature.     

Pellagra: dermatitis, dementia, and diarrhea

Pellagra defines systemic disease as resulting from a marked cellular deficiency of niacin. It is characterized by 4 "D's": diarrhea, dermatitis, dementia, and death. Diagnosis of pellagra is difficult in the absence of the skin lesions, and is often facilitated by the presence of characteristic ones. The dermatitis begins as an erythema. Acute pellagra resembles sunburn in its first stages, but tanning occurs more slowly than typically in sunburn. Exacerbation follows re-exposure to sunlight. In this work we review the findings of this once mysterious disorder, one that still challenges clinicians world-wide.     

Pellagra: a clinical, histopathological, and epidemiological study of 7 cases

Background and objectivesIn the developed world, pellagra is a rare condition that is restricted to a small number of at-risk groups. It mainly affects alcoholic patients and those with dietary deficiencies, with intestinal malabsorption, or in treatment with certain drugs. The aim of this study was to analyze the clinical, histopathological, and epidemiological characteristics of patients diagnosed with pellagra in our hospital and to compare the results with the findings traditionally described for this disease.Patients and methodsWe undertook a retrospective study of patients with clinical or pathological evidence of pellagra who were seen in our hospital between 1998 and 2009.ResultsSeven patients met the inclusion criteria. All were men and the most common predisposing factors were alcoholism and dietary deficiency. All exhibited photosensitivity mainly affecting the forearms and the upper surface of the feet, where the lesions were more severe. The most consistent histopathological findings were the presence of dilated blood vessels with extravasation and little or no inflammatory infiltrate. Various changes were observed in the epidermis, including those suggestive of mild pellagra, such as epidermal pallor and some degree of ballooning of the keratinocytes. Other abnormalities such as epidermal necrosis and hyperkeratosis were also observed. In most patients, pellagra was not initially suspected. Additional noncutaneous findings were observed in almost all cases.ConclusionsPellagra should be ruled out in patients with lesions on sun-exposed areas. Predisposing factors for pellagra should be assessed along with the social situation of patients and the presence of digestive or neurological abnormalities.     

Tryptophanstoffwechsel bei kranken mit pellagra

Zusammenfassung Der Tryptophanstoffwechsel wurde bei einer Patientin mit Hartnup-Syndrom und 4 Patientinnen mit Pellagra untersucht, indem die 24 Std-Harnausscheidungen von N¹-Methylnicotinamid, 3-Hydroxyanthranilsäure, Kynurenin, Tryptamin, Gesamt-Indol-3-Essigsäure und 5-Hydroxyindolessigsäure vor und nach oraler Gabe von 5 g DL-Tryptophan bestimmt wurden.Alle Patientinnen zeigten eine verminderte Ausscheidung von N¹-Methylnicotinamid, ansonsten waren die Ausscheidungsmuster uneinheitlich. Aus den Resultaten wird geschlossen, daß Malabsorption von Trytophan, Mangelernährung oder Enzymblockaden im Bereiche des Kynureninstoffwechselweges die wichtigsten Stoffwechselfaktoren für die erniedrigte Nicotinsäuresynthese bei den untersuchten Patientinnen darstellen.Zusätzlich wurden Beziehungen zwischen biochemischen Parametern und klinischen Symptomen beobachtet. Reduktion der Harnausscheidungen von N¹-Methylnicotinamid war assoziiert mit den typischen Hautveränderungen der Pellagra. Abnorm hohe Ausscheidungen von Tryptamin fanden sich in Zusammenhang mit den klinischen Zeichen einer paranoid-halluzinatorischen Psychose, während bei einer weiteren Patientin mit subdepressiver Symptomatik verminderte Harnausscheidungen von 5-Hydroxyindolessigsäure bestimmt wurden.Die Untersuchungsergebnisse sowie die Rolle des Indolaminstoffwechsels bei psychischen Störungen werden diskutiert.

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Tryptophan metabolism in patients with pellagra

Summary Tryptophan metabolism was investigated in 1 patient with Hartnupsyndrome and 4 patients with pellagra by determining 24 h-urinary excretions of N¹-methylnicotinamide, 3-hydroxyanthanilic acid, kynurenine, tryptamine, total indole-3-acetic acid and 5-hydroxyindoleacetic acid before and after oral administration of 5 g DL-tryptophan.All patients showed decreased excretions of N¹-methylnicotinamide, with varying excretion patterns. From the results it is concluded that malabsorption of tryptophan, malnutrition or enzymatic blockades within the kynurenine pathway represent the main metabolic factors for the decreased synthesis of nicotinic acid in the investigated patients.In addition, associations between biochemical parameters and clinical symptoms were observed. Decreased urinary excretions of N¹-methylnicotinamide were found to be associated with the typical skin lesions of pellagra. Abnormally high excretions of tryptamine were observed in connection with the clinical signs of a paranoid-halluzinatory psychosis while in another patient with a subdepressive state urinary excretions of 5-hydroxyindoleacetic acid were reduced.The results of the investigation as well as the rôle of indolamine metabolism in psychic disturbances are discussed.

     

Identification of an error in the tryptophan → niacin pathway in carriers of some dermatoses conditioned or aggravated by sunlight

Summary The study of the tryptophanniacin pathway showed a defect of the kynureninase activity at the level of the 3-hydroxykynurenine3-hydroxyanthranilic acid step in a series of present acquired pellagra carriers (23) and in 5 of a series of 12 chronic erythematodes carriers. The same defect, associated with a deficit of the kynureninase activity also at the level of the kynurenineanthranilic acid step, was observed in 52% of cases of a series of porphyria cutanea tarda carriers (23), in one case of Rothmund-Thomson syndrome and in a group of rats in which a hepatic porphyria was provoked.The above-mentioned error in metabolism is brought on by the sunlight and by artificial light from 400 to 750 m-in fact it may also be provoked or aggravated by both. It causes a reduction of the niacin synthesis, and this probably has a particular pathogenetic importance in both pellagra and porphyria cutanea tarda. This error is quickly corrected by intramuscular administration of normal human gastric juice. This treatment also provokes a recovery of the clinical signs of pellagra.

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Zusammenfassung Die Untersuchung des Tryptophan-Niacin-Stoffwechsels ergab einen Kynureninasemangel bei der Umwandlung von 3-Hydroxykynurenin in 3-Hydroxyanthranilsäure bei 23 Patienten mit Pellagra und bei 5 von insgesamt 12 Patienten mit chronischem Erythematodes. Ein Kynureninasemangel (bei Umwandlung von Kynurenin in Anthranilsäure) wurde gleichfalls bei 52% von Patienten mit Porphyria cutanea tarda (23), einem Fall mit Rothmund-Thomson-Syndrom sowie bei Ratten mit experimentell ausgelöster hepatischer Porphyrie beobachtet.Diese Stoffwechselstörung wird durch Sonnenlicht oder künstliches Licht mit einer Wellenlänge von 400–750 m ausgelöst oder verschlimmert. Die daraus resultierende Verminderung der Niacin-Synthese ist wahrscheinlich von besonderer pathogenetischer Bedeutung für die Pellagra und die Porphyria cutanea tarda. Intramuskuläre Injektionen normalen menschlichen Magensaftes führen zu einer raschen Beseitigung der Stoffwechselstörung und veranlassen ein Verschwinden der klinischen Symptomatik bei Pellagra.

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Dedicated to Professor Isidoro Bosco on the occasion of his 65th birthday.     

Isoniazid induced pellagra despite pyridoxine supplementation

Although pellagra is a recognized complication of isoniazid therapy, the diagnosis may be overlooked or delayed--sometimes with life-threatening consequences. We report a case of isoniazid-induced pellagra which occurred despite pyridoxine supplementation. Drug withdrawal and supplementation with niacin led to a rapid and sustained clinical improvement. The possible mechanisms of isoniazid induced pellagra are discussed.     

Pellagra. A challenging differential diagnosis in burn injuries

With this case report we want to emphasize the importance of performing a thorough physical examination of the burn and detailed review of the patient's history.This is a challenging case because it deals with an uncommon disease nowadays, pellagra, which presents lesions with an appearance very similar to burns; on the other hand the management of pellagra is different to the management of the common burns we are used to handling day-to-day.With this case report we will be able to revise the broad list of different injuries that can bring up an issue related to a correct diagnosis, caused by a large diversity of different etiologies with cutaneous expression.We will look over the diagnostic process of pellagra, management, treatment and results in this patient.