Clinics and Practice 2014; volume 4:637
Pellagra: a non-eradicated old The term pellagra - from pelle agra, Italian for rough skin - was first used by Frappoli in Correspondence: Savvidou Savvoula, Ring Road disease 1771 due to its dermatologic manifestations.3 N. Efkarpia-Thessaloniki, Zip Code 56429, However, since Goldberger implicated vitamin Thessaloniki, Greece. Savvoula Savvidou B deficiency as the cause of pellagra in 1926,3 Tel. +30.2313323180 - Mobile: +30.6977774301 - Fax: +30.2313323165. 1st Department of Internal Medicine, the pellagra syndrome has historically been E-mail: [email protected] Papageorgiou University Hospital of characterized by the 4 D’s: Dermatitis, 1,3 Thessaloniki, Thessaloniki, Greece Diarrhea, and Dementia leading to Death. Key words: pellagra, niacin, vitamin B3 deficien- Pellagra was a major widespread cause of cy, pellagrous encephalopathy. death until the early 20th century, but fortifica- tion of flour with niacin has led practically to Acknowledgements: the author would like to its eradication in developed countries. thank Dr. Sotiriadis D, Professor of Dermatology Abstract However, several recent reports suggest that and head of 2nd Dermatology Department, pellagra may even be underdiagnosed.3-6 Papageorgiou University Hospital of We report a case of a 50-year old homeless Thessaloniki, for his significant contribution. male who presented with pellagra and pella- Received for publication: 18 March 2014. grous encephalopathy. The characteristic - if Revision: Not required. not pathognomonic - skin lesions of pellagra Case Report Accepted for publication: 11 April 2014. support its diagnosis on solely clinical grounds. Clinicians should keep a high index A 50-year old man was brought to the emer- This work is licensed under a Creative Commons of suspicion, in certain patient groups, in gency department due to disturbed mental sta- Attribution NonCommercial 3.0 License (CC BY- NC 3.0). order to early diagnose and cure this potential- tus. The patient was reported to be homeless, ly fatal but treatable disease. but no other information about his medical ©Copyright S. Savvidou, 2014 status or living conditions was available. The Licensee PAGEPress, Italy patient was unreliable in providing history of Clinics and Practice 2014; 4:637 alcohol abuse or describing symptoms like doi:10.4081/cp.2014.637only Introduction fever in his recent past. Clinical examination revealed normal vital Pellagra is a nutritional disorder caused by signs, cachexia and dehydration, accompanied was observed. Laboratory investigation showed niacin (vitamin B3) deficiency, which leads to by extensive pigmentation and scaling erup- usemild elevation of total white blood cell count, systemic disease with clinical manifestations tions on a red basis along the dorsa of his from the skin, gastrointestinal tract and the hands and the sun-exposed surface of his arms slightly elevated creatinine and low serum nervous system.1-3 Niacin forms into nicotinic (Figure 1). The patient was confused and dis- albumin. The remaining of the routine labora- acid and nicotinamide, which serve as precur- orientated but responded sufficiently to simple tory tests, including glucose, electrolytes and sors of two important coenzymes of cellular commands. Neurological examination revea - aminotransferases, were found within normal metabolism, namely nicotinamide adenine led no abnormalities concerning his cranial range. dinucleotide (NAD) and NAD-phosphate.1 nerves and tendon reflexes. No flapping tremor The patient was admitted for further inves-
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Figure 1. Skin lesions of pellagra on the extensor surface of the patient’s forearm (A) and dorsa of his hand (B).
[page 22] [Clinics and Practice 2014; 4:637] Case Report tigation. Clinical follow-up did not reveal fever typical photosensitive distribution with well- high index of suspicion, in certain patient or other symptoms or signs indicative of an defined borders, and is mostly observed on the groups, in order to early diagnose and cure this underlying infection. Intravenous fluids and face, the neck - forming the so-called Casal’s potentially fatal but treatable disease. high-caloric nutrition was administered. necklace -, the dorsa of the hands and the During hospitalization the patient was subject- extensor surface of the forearms.1-3 In addition ed to computed tomography imaging which to skin changes, gastrointestinal involvement excluded any brain or other pathology. may lead to intractable diarrhea, stomatitis References Following neurological assessment and the and glossitis, while the neurocognitive impair- negative imaging studies, the disturbed men- ment - pellagrous encephalopathy - may pres- 1. Russel RM. Vitamin and trace mineral tal status was attributed to metabolic factors. ent as apathy, memory loss, disorientation, deficiency and excess. In: Kasper DL, Furthermore, as the characteristic skin lesions depression or delirium. Stupor and death Braunwald E, Fauci AS, et al., eds. in an obviously malnourished homeless man result if pellagra is left untreated.3,4 Harrison’s principles of internal medicine. were compatible with pellagra, pellagrous Niacin deficiency should especially be sus- 16th ed. New York: McGraw-Hill; 2005. pp encephalopathy was the preferable diagnosis. pected in the following conditions:1,3,4 i) malnu- 404-405. Although serologic and urinary assays con- trition (homelessness, anorexia nervosa or 2. MacDonald A, Forsyth A. Nutritional defi- firming niacin deficiency were unavailable in severe comorbid conditions like end-stage ciencies and the skin. Clin Exp Dermatol our hospital, treatment with nicotinamide 300 malignancy or HIV); ii) malabsorption (e.g. 2005;30:388-90. mg orally in a daily basis was started. The skin Crohn’s or Hartnup disease); iii) chronic alco- 3. Oldham MA, Ivkovic A. Pellagrous lesions improved significantly but did not holism; iv) hemodialysis or peritoneal dialysis; encephalopathy presenting as alcohol resolve completely. Eighteen days later the v) drugs like isoniazid, ethionamide, 6-mer- withdrawal delirium: a case series and lit- patient died due to hospital-acquired pneumo- captopurine and estrogens; vi) carcinoid syn- erature review. Addict Sci Clin Pract nia, without showing any remarkable improve- drome (due to excess turnover of tryptophan, 2012;7:12. ment in his mental status. precursor of niacin, to serotonin). 4. Pipili C, Cholongitas E, Ioannidou D. The diagnostic importance of photosensitivity dermatoses in chronic alcoholism: report onlyof two cases. Dermatol Online J 2008; Discussion Conclusions 14:15. 5. Brown TM. Pellagra: an old enemy of time- Patients with pellagra have a brown discol- In conclusion, pellagra is a historically old less importance. Psychosomatics 2010;51: oration of the skin, especially in sun-exposed but certainly not completely eradicateduse dis- 93-7. areas. In advanced stages, increased pigmen- ease.5 The characteristic - if not pathognomon- 6. Okan G, Yaylaci S, Alzafer S. Pellagra: will tation usually leads to thin varnish-like erup- ic - skin lesions support its diagnosis on solely we see it more frequently? J Eur Acad tive scales. The characteristic skin rash has a clinical grounds.1,3 Clinicians should keep a Dermatol Venereol 2009;23:365-6.
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