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Mayo Clin Proc, March 2001, Vol 76
Pellagra in 2 Homeless Men
Pellagra in 2 Homeless Men
STEFAN G. KERTESZ, MD
Pellagra is a nutritional disease with cutaneous, gas-
diet. Appropriate suspicion for a diagnosis of pellagra
trointestinal, and neuropsychiatric manifestations. Be-
requires attention to a combination of socioeconomic and
cause of the diversity of pellagra’s signs and symptoms,
behavioral risk factors for nutritional deficiency. The
diagnosis is difficult without an appropriate index of suspi-
combination of homelessness, alcohol abuse, and failure to
cion. Untreated, pellagra is fatal. Two cases of pellagra in
eat regularly—particularly, failure to make use of shelter-
contemporary homeless people are described. Complete
based meal programs—may identify people at special risk
evaluation supported a clinical diagnosis of pellagra after
in contemporary settings.
exclusion of other possibilities. Signs and symptoms re-
solved after institution of niacin therapy and change in
Mayo Clin Proc. 2001;76:315-318
Pellagra, a disease primarily due to niacin deficiency, tobacco. He had lived on the street and avoided shelters for was epidemic in the United States before 1950. Al- 4 months. Examination revealed bronze-colored skin with though contemporary medical literature focuses on the his- rare scale formation, poor dentition, and low-grade fever.
tory of its eradication,1 some reports suggest that pellagra Initial laboratory studies showed the following: hematocrit, continues to occur2 but remains underappreciated, resulting 33.8%; mean corpuscular volume, 104 fL; normal leuko- in delayed diagnosis. Two series from Japan describe 28 cyte and platelet counts; normal renal function test results; patients who died of undiagnosed pellagra and underscore serum alanine aminotransferase, 76 U/L; serum aspartate the importance of identifying populations at special risk.3,4 aminotransferase, 126 U/L; albumin, 3.5 g/dL; total pro- To my knowledge, no report to date has described pella- tein, 5.6 g/dL; and erythrocyte sedimentation rate, 23 mm/1 gra in contemporary homeless people, despite the fact that h. Chest radiograph, electrocardiogram, and serial creatine the homeless are subject to relevant vulnerabilities of alco- kinase determinations were normal. Additional work-up holism, social dislocation, and irregular access to food.
included the following tests, the results of which were This article describes 2 cases of pellagra in this population.
normal: serum thyrotropin, cortisol, vitamin B , folate, iron studies, and rapid plasma reagin test; stool cultures and examinations for ova and parasites; sequential blood cul- tures for bacteria, mycobacteria, and fungi; serum histo- A 58-year-old homeless man was admitted to a Boston plasma antigen assay; serum gastrin and vasoactive intesti- hospital in October 1998 with chest pain and fatigue. Re- nal peptide levels; 24-hour urinary 5-hydroxyindoleacetic view disclosed multiple complaints, including fever, night acid, catecholamines, vanillylmandelic acid, and meta- sweats, 4.5-kg weight loss, a burning sensation in the nephrines; and computed tomography of the torso and jaw.
mouth, nausea, vomiting, diarrhea involving watery, large- Two teeth were extracted. The symptoms improved volume movements several times daily, cutaneous burning slightly without specific intervention, and the patient was sensations, increased skin pigmentation, cough, and pro- discharged; a multivitamin, thiamine, and folate were pre- nounced emotional lability over the preceding months.
scribed. At outpatient follow-up 1 week later, the patient The patient’s history was unremarkable, including nega- reported minimal improvement. A diagnosis of nutritional tive purified protein derivative and negative serum human deficiency was entertained. A dietary history revealed that immunodeficiency virus test results. The patient reported the patient had subsisted on 2 to 3 alcoholic beverages and drinking 2 or 3 alcoholic beverages per day as well as using a daily snack, usually corn chips, for 4 months. He hadavoided homeless shelters and shelter-based meal pro-grams. Information obtained later suggested that the patient From the Boston Health Care for the Homeless Program and the had a history of bipolar illness, although neither mania nor Section of General Internal Medicine, Boston Medical Center, Bos- depression was evident at the time of admission. A pre- sumed diagnosis of pellagra led to the institution of supple- Address reprint requests and correspondence to Stefan G. Kertesz, mental niacin, 100 mg orally twice daily; the patient also MD, Section of General Internal Medicine Research Unit, 91 E Con-cord St, Suite 200, Boston, MA 02118 (e-mail: skertesz@bu.edu).
obtained regular meals from a shelter. All symptoms, in- 2001 Mayo Foundation for Medical Education and Research Mayo Clin Proc, March 2001, Vol 76
Pellagra in 2 Homeless Men
Figure 1. Patient 2. Left, at time of presentation. Two weeks into the course of the illness, cutaneousweeping, edema, erythema, fissuring, and scale were noted over the ears, face, and collar area. Right, 1year later.
cluding cutaneous burning sensations, rash, diarrhea, and diagnostic considerations included photodermatitis, actinic mood alteration, resolved within 2 weeks.
dermatitis, and pellagra. The patient was prescribed a mul-tivitamin, niacin (100 mg orally daily), and triamcinolone cream; the patient reported that his rash resolved over the A 55-year-old homeless man presented to a shelter next few weeks. A photograph obtained 1 year later docu- clinic in July 1998 with several days of a weeping eczema- mented complete resolution (Figure 1, right).
tous rash of the face, ears, and neck (Figure 1, left). Physi- After resolution of his symptoms, the patient reported cal examination demonstrated extensive cutaneous weep- that, for several months preceding his illness, he had ing, edema, erythema, fissuring, and scale over the ears, missed meals for up to 4 days at a time, subsisting on face, collar area, and forearms. He had no other known coffee, donuts, and alcohol, while generally avoiding medical conditions. He received topical corticosteroids and homeless shelters. He recalled a burning sensation in his antibiotics for a diagnosis of allergic contact dermatitis mouth, increased psychological irritability, and continuous abdominal upset without diarrhea, all of which subsided The rash did not abate after 1 week, and the patient was with vitamin therapy and regular meals. The distribution of referred to a dermatology service. Evaluation included nor- the patient’s rash, the nonspecific dermatological work-up, mal blood cell counts, liver function test results, urinalysis, and the resolution of all symptoms with niacin therapy and urine porphyrins. A serum antinuclear antibody assay were judged to represent a diagnosis of pellagra.
was positive with a speckled pattern on Hep2 cells at a titerof 1:160, but there were no joint, renal, or hematologic abnormalities. Biopsy of the left preauricular skin showed This report describes pellagra in 2 homeless men, with compact orthokeratosis, mild epidermal hyperplasia, focal diagnosis based on clinical presentation, negative or non- spongiosis, mild to moderate superficial perivascular specific findings after extensive evaluation, and resolution lymphohistiocytic infiltrates with occasional eosinophils of cutaneous, gastrointestinal, and psychiatric symptoms and neutrophils, and mild papillary dermal fibrosis; these after change in food intake and therapy with niacin. A findings were nonspecific. The dermatology service’s final standard textbook recommends diagnosis based on clinical Mayo Clin Proc, March 2001, Vol 76
Pellagra in 2 Homeless Men
assessment and response to therapy because most postu- vitamins, surveys have had difficulty identifying an in- lated laboratory indicators, including urinary nicotinamide creased prevalence of niacin deficiency among alcoholics and serum niacin levels, have not proved reliable5-7; one since the fortification of flour began after World War II.18,19 assay involving high-performance liquid chromatography The pre-1950 case literature identifying an association be- is not commonly available.8 In the absence of definitive tween alcoholism and pellagra20 did not describe patients’ laboratory results, this report relied on clinical diagnosis.
socioeconomic status. Nevertheless, indigence and alco- A functional deficiency of niacin plays a central role in holism together may pose special nutritional risk. In 1923, the pathophysiology of pellagra; however, contemporary Shattuck21 suggested that dietary inadequacy due to poverty research has shown that an interplay of niacin and other could account for the development of pellagra among alco- metabolic factors actually determines whether the disease holics. Of 15 alcoholics with pellagra in the 1977 series by will develop in a given person.9 Coexistent deficiencies of Spivak and Jackson,2 14 were classified as indigent.
riboflavin and pyridoxine, although unmeasured in our Homelessness does confer an increased risk of dietary inadequacy. Contemporary homeless people frequently Although both patients fulfilled diagnostic criteria miss meals and have substantial rates of dietary insuffi- based on clinical manifestations in 3 organ systems, this ciency when evaluated by nutritional surveys of vitamin diagnostic approach would overlook other cases. Only 22% intake, although few studies have assessed niacin in par- of patients in one series presented with symptoms in 3 ticular.22-25 Anthropometric studies provide additional evi- systems.2 For this reason, identification of epidemiological dence of malnutrition in homeless people, but the absolute rate of reported nutritional deficiency disease in this popu- A search of the MEDLINE database identified no previ- lation remains low.13,26 At least one study found shelter- ous reports of pellagra in homeless people. Given the sub- based meal programs to be reasonably well balanced with stantial literature describing delayed diagnosis leading to respect to protein and carbohydrate,27 and access to free patient deaths,2-4 the occurrence of this disease in the con- food sources is likely to prevent malnutrition among the temporary homeless may be underappreciated. The home- less population has grown during the past decade, with Because both patients in this report had avoided home- estimates of the homeless ranging from 440,000 to 842,000 less shelter-based meal programs for several months before people during any given week in 1996.11 Although avail- the development of symptoms, they are likely to have been able studies identify the broad range of medical conditions especially susceptible to nutritional deficiency.
affecting homeless people,12,13 the published literature hasonly rarely described the specific nutritional deficiency diseases to which homeless persons are subject.14 For this The present cases, diagnosed after delay, underscore a reason, pellagra may continue to go unrecognized unless contemporary dilemma. A fatal but easily treated disease is physicians develop an index of suspicion for its occurrence.
readily overlooked because of its rarity. Neither a history of Pellagra’s manifestations include a rash over sun-ex- alcohol use nor homelessness alone has a strong positive posed areas that develops in spring or summer. The simul- predictive value for a diagnosis of pellagra. Homelessness taneous occurrence of a severe rash of the face, forearms, coupled with alcoholism in patients who do not obtain and collar region, as was seen in patient 2, suggests classic meals from shelter-based meal programs, however, appears pellagra. But the literature contains reports of more subtle to identify a group at special risk.
findings, including diffuse, mild hyperpigmentation,2 aswas seen in patient 1. The literature also documents the I gratefully acknowledge the editorial advice of Drs Mark occurrence of pellagra without rash (“pellagra sine pella- Moskowitz, John Noble, and James O’Connell in the preparationof this article and the assistance of Dr James O’Connell in obtain- gra”), affecting 15% of patients in a compilation of 4121 cases.15 Patients without rash may die of pellagra’s neuro-logic sequelae.4 Gastrointestinal manifestations include not only diarrhea but also glossitis, nausea, vomiting, and post- Rajakumar K. Pellagra in the United States: a historical perspec- prandial discomfort.16 Neuropsychiatric manifestations tive. South Med J. 2000;93:272-277.
vary and can include cognitive dysfunction, memory im- Spivak JL, Jackson DL. Pellagra: an analysis of 18 patients and areview of the literature. Johns Hopkins Med J. 1977;140:295-309.
pairment, insomnia, anxiety, depression, acute psychosis, Ishii N, Nishihara Y. Pellagra encephalopathy among tuberculous seizures, ataxia, and spastic paraparesis.3,17 patients: its relation to isoniazid therapy. J Neurol Neurosurg Psy- Whether the nutritional vulnerability of these 2 patients Ishii N, Nishihara Y. Pellagra among chronic alcoholics: clinical might best be attributed to alcohol use or to homelessness and pathological study of 20 necropsy cases. J Neurol Neurosurg itself can be debated. Despite alcohol’s effect on many Mayo Clin Proc, March 2001, Vol 76
Pellagra in 2 Homeless Men
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