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Saudi Journal of Kidney Diseases and Transplantation
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Table of Contents   
CASE REPORT  
Year : 2020  |  Volume : 31  |  Issue : 4  |  Page : 874-876
Pellagra Disease in a Hemodialysis Patient


1 Department of Nephrology, Dialysis and Transplantation, La Rabta Hospital, Tunis, Tunisia
2 Department of Research Laboratory of Kidney Diseases (LR00SP01), Charles Nicolle Hospital; Department of Faculty of Medicine, Tunis El Manar University, Tunis, Tunisia

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Date of Submission06-Jun-2019
Date of Acceptance22-Jul-2019
Date of Web Publication15-Aug-2020
 

   Abstract 


Pellagra usually results from niacin deficiency and presents with the classic triad of dermatitis, diarrhea, and dementia. It is most commonly associated with malnutrition and poverty. We report a case of pellagra in a hemodialysis (HD) patient with breast neoplasia, aged 68-years, female, on HD unit for seven years. Her original nephropathy was indeterminate. The patient was followed up for homozygous beta-thalassemia and breast neoplasia with hepatic metastases on chemotherapy. The body mass index of the patient was 18.5 kg/m2. Physical examination showed a thickening of the epidermis with a scaly surface, pigmented, and atrophied areas. We noted neuropsychiatric signs (apathy, irritability, anorexia, and depression) and digestive symptomatology (diarrhea). The laboratory tests revealed hypoproteinemia at 55 g/L, hypoalbuminemia at 21 g/L, and hypocholesterolemia at 0.8 g/L. The diagnosis of pellagra disease was made. Vitamin and protein supplementation was initiated, but the patient committed suicide by puncture of her arteriovenous fistula, causing hemorrhagic shock. Pellagra is usually reported to be associated with malnutrition, chronic alcoholism, and some chemotherapeutic agents. In our patient, pellagra was caused by malnutrition and co-morbidities. Pellagra disease requires multidisciplinary care and can be frequently seen in HD patients due to the associated malnutrition.

How to cite this article:
Barrah S, Jebali H, Kheder R, Krid M, Smaoui W, Beji S, Hmida FB, Fatma LB, Rais L, Zouaghi MK. Pellagra Disease in a Hemodialysis Patient. Saudi J Kidney Dis Transpl 2020;31:874-6

How to cite this URL:
Barrah S, Jebali H, Kheder R, Krid M, Smaoui W, Beji S, Hmida FB, Fatma LB, Rais L, Zouaghi MK. Pellagra Disease in a Hemodialysis Patient. Saudi J Kidney Dis Transpl [serial online] 2020 [cited 2020 Oct 25];31:874-6. Available from: https://www.sjkdt.org/text.asp?2020/31/4/874/292325



   Introduction Top


Pellagra is a nutritional disorder caused by niacin (Vitamin B3) deficiency resulting from inadequate dietary nicotinic acid and/or its precursors, the essential amino-acid tryptophan.[1]

It is a systemic disease characterized by three criteria: diarrhea, skin lesions, and neurological impairment (usually dementia).[2] The pellagra syndrome has historically been characterized by the 4 D’s: Dermatitis, Diarrhea, and Dementia leading to Death.[3]

Chronic malnutrition is a common problem in patients with end-stage renal disease on hemodialysis (HD) leading to this syndrome especially in developing countries, but it is underdiagnosed.[1]


   Case Report Top


Informed consent was obtained from the patient’s relatives before publishing the case.

We report the case of a 68-year-old female patient on dialysis in our HD unit for seven years. Her original nephropathy was indeterminate. The patient was followed-up for homozygous beta-thalassemia and breast neoplasia with hepatic metastases on chemotherapy. In 1994, the patient received chemotherapy anthracyclines with a good response. In 1998, the patient had a recurrence of her neoplasia with bone metastases. She received chemotherapy with fluorouracil, epirubicin, and cyclophosphamide (FEC-75). In 2002, she received tamoxifen and hormonal treatment (letrozole). Clinical examination revealed normal vital signs, cachexia, and dehydration, accompanied by extensive pigmentation and scaling eruptions on a red base along the dorsal of his hands and the sun-exposed surface of his arms. The body mass index of the patient was 18.5 kg/m2. Furthermore, she had hypoproteinemia at 55 g/L, hypoalbumi- nemia at 21 g/L, hypocholesterolemia at 0.8 g/L, urea rate before dialysis at 0.7 g/L and a normal PRU at 77.2%. All these clinical and biological signs indicated the diagnosis of malnutrition. Upper gastrointestinal endoscopy showed multinodular antral gastropathy. We made a diagnosis of pellagra disease because of the combination of skin lesions, digestive symptomatology (diarrhea in our case), and neuropsychiatric signs (apathy, irritability, anorexia, and depression). Skin lesions included hyperpigmentation of the exposed areas along with photosensitivity. The derma- tological examination found a thickening of the epidermis with a scaly surface, pigmented, and atrophied areas [Figure 1], [Figure 2], [Figure 3]. Vitamin and protein supplementation was initiated, but the patient committed suicide by puncture of her arteriovenous fistula, causing hemorrhagic shock.
Figure 1:

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   Discussion Top


Pellagra disease is related to malnutrition that currently affects developing countries and results from a lack of niacin (Vitamin B3).[4] Dermatitis caused by pellagra is a bilaterally symmetrical erythema at the sites of solar exposure. Dermatitis begins in the form of erythema with acute or intermittent onset gradually changing to an exudative eruption on the dorsal part of the hand, face, neck, and chest with pruritus and burning. In addition to skin changes, gastrointestinal involvement may lead to intractable diarrhea, stomatitis, and glossitis, while the neurocognitive impairment - pellagrous encephalopathy - may present as apathy, memory loss, disorientation, depression or delirium.[5] Stupor and death result if pellagra is left untreated.[2],[3],[4],[5] [Figure 1], [Figure 2], [Figure 3]. The dermatological examination found a thickening of the epidermis with a scaly surface, pigmented and atrophied areas.

The treatment of pellagra consisted of exo genous administration of niacin.[6] Topical management of skin lesions with emollients may reduce discomfort. The therapy should also include other B vitamins, zinc, and magnesium as well as a diet rich in calories. The prevention is based on nutritional education and the discontinuation of alcohol.[7],[8] In this case, we emphasize the importance of nutrition in dialysis patients, which is an important factor in the prognosis of morbidity and mortality. Malnutrition is frequent in dialysis patients despite effective dialysis, as in our case, probably due to co-morbidities (neoplasia in our case leading to hyper catabolism).[1] Cancer often causes malnutrition and specific vitamin and protein deficiencies. Chemotherapy also causes deficiencies by promoting anorexia, stomatitis, and alimentary tract disturbances. Anti-metabolite drugs in particular, inhibit synthesis of essential vitamins, purines, and pyrimidines.[9],[10] Nutrition and vitamin supplementation is necessary in patients on dialysis, orally or parenterally, administered during dialysis.[1] Pellagra disease has a poor prognosis due to delayed diagnosis because it is an unrecognized pathology.

Niacin deficiency should especially be suspected in the following conditions: (i) malnutrition (homelessness, anorexia nervosa or severe co-morbid conditions such as end-stage malignancy or HIV); (ii) malabsorption (e.g. Crohn’s or Hartnup disease); (iii) chronic alcoholism; (iv) hemodialysis or peritoneal dialysis; v) drugs such as isoniazid, ethio- namide, 6-mercaptopurine, and estrogens; (vi) carcinoid syndrome (due to excess turnover of tryptophan, precursor of niacin, to serotonin).[5],[6],[7],[8] In our case, our patient had breast neoplasia with hepatic metastases, on chemotherapy, and she was treated by maintenance HD.


   Conclusion Top


Pellagra is a historically old but certainly not completely eradicated disease. Clinicians should maintain a high index of suspicion, in certain patient groups, to diagnose early, and cure this potentially fatal but treatable disease. This disease requires multi-disciplinary care (dermatologist, gastroenterologist, neurologist and, psychiatrist.

Conflict of interest: None declared.



 
   References Top

1.
Waterlot Y, Sabot JP, Marchal M, Vanherweghem JL. Pellagra: Unusual cause of paranoid delirium in dialysis. Nephrol Dial Transplant 1986;1:204-5.  Back to cited text no. 1
    
2.
Kesty K, De Luca JF, Taylor SL, Sangueza OP. Scaly erythematous acral eruption. Am J Dermatopathol 2016;38:50-1, 77-8.  Back to cited text no. 2
    
3.
Terada N, Kinoshita K, Taguchi S, Tokuda Y. Wernicke encephalopathy and pellagra in an alcoholic and malnourished patient. BMJ Case Rep 2015;2015:bcr2015209412.  Back to cited text no. 3
    
4.
Leonhard N, Aeberhard C, Birrenbach T, Stanga Z. An uncommon diagnosis. Praxis (Bern 1994) 2015;104:1151-6.  Back to cited text no. 4
    
5.
Hegyi J, Schwartz RA, Hegyi V. Pellagra: Dermatitis, dementia, and diarrhea. Int J Dermatol 2004;43:1-5.  Back to cited text no. 5
    
6.
Bernardo Cofiño J, Menéndez Calderón MJ, Quevedo de Quevedo A. Pellagra. Med Clin (Barc) 2015;145:e7.  Back to cited text no. 6
    
7.
Ikenouchi-Sugita A, Sugita K. Niacin deficiency and cutaneous immunity. Nihon Rinsho Meneki Gakkai Kaishi 2015;38:37-44.  Back to cited text no. 7
    
8.
Savvidou S. Pellagra: A non-eradicated old disease. Clin Pract 2014;4:637.  Back to cited text no. 8
    
9.
Thornton AM, Drummond CJ. An unexpected case of pellagra. Med J Aust 2014;200:546-8.  Back to cited text no. 9
    
10.
Hendricks WM. Pellagra and pellagra like dermatoses: Etiology, differential diagnosis, dermatopathology, and treatment. Semin Dermatol 1991;10:282-92.  Back to cited text no. 10
    

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Correspondence Address:
Sana Barrah
Department of Nephrology, Dialysis and Transplantation, La Rabta Hospital, Tunis
Tunisia
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DOI: 10.4103/1319-2442.292325

PMID: 32801252

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