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A diagnosis at hand

Published:January 10, 2022DOI:https://doi.org/10.1016/j.ejim.2022.01.007

      1. Introduction

      A 45-year-old man was found unconscious on the sidewalk and brought to the Emergency Department. He could not provide anamnestic information, but the hospital electronic archive revealed he was homeless and had a history of excessive alcohol consumption (250–300 g daily intake). He suffered from depressive disorder and had attempted suicide 9 years before by caustic ingestion, which led to total gastrectomy and esophagojejunal anastomosis. Physical examination revealed a Glasgow Coma Scale score of 8 with no focal neurological signs. A tonic-clonic seizure occurred during examination. Vital signs and cardio-respiratory function were unremarkable. He appeared extremely dehydrated and emaciated. The skin on the dorsal surface of his hands was thickened, hyperpigmented and scaly (Fig. 1A). Laboratory findings showed severe anemia with anisocytosis and poikilocytosis, marked thrombocytosis and leucocytosis with evidence of hypersegmented neutrophils. A head CT-scan was performed, revealing bilateral intracerebral hemorrhage causing ventricle compression and midline shift. No neurosurgical intervention was recommended apart from observation. During the hospital stay, several episodes of watery diarrhea occurred. Coltural exams turned out negative.
      Fig 1
      Fig. 1Hyperpigmented, scaly lesions on the dorsal skin of the hand before (Panel A) and after therapy (Panel B).
      What is the diagnosis?

      2. Diagnosis

      Pellagra
      Intracerebral hemorrhage, possibly consequence of head trauma

      3. Discussion

      Pellagra is a systemic disease associated with chronic lack of niacin and tryptophan (its precursor). The nutritional deficiency leads to cutaneous, gastrointestinal and neurological manifestations. Although often considered a disease of the past, pellagra is not eradicated and continues to occur even in developed countries, but its underappreciation can lead to delayed diagnosis [
      • Savvidou S.
      Pellagra: a non-eradicated old disease.
      ]. Niacin deficiency should be suspected in any condition of malnutrition, malabsorption, chronic alcoholism, hemodialysis or peritoneal dialysis, carcinoid syndrome and administration of certain drugs [
      • Kyuraghi Lute S.
      • Sato R.
      Alcoholic pellagra as a cause of altered mental status in the emergency department.
      ]. The pellagra syndrome has historically been characterized by the four D's: Dermatitis, Diarrhea and Dementia, leading to Death. The symptoms rarely appear simultaneously and are often incomplete. The disease has a chronic course with periods of exacerbation and remission but, if untreated, eventually leads to multiorgan failure [
      • Kapoor R.
      • Saint S.
      • Kapoor J.R.
      • Johnson R.A.
      • Dhaliwal G.
      Clinical problem-solving. D is for delay.
      ].
      Our patient was at high risk of developing pellagra due to his personal history. He showed typical skin lesions and diarrhea; assessing the neurological features was made difficult by the concomitant intracerebral hemorrhage. Although serologic and urinary assays confirming niacin deficiency were unavailable in our hospital, a protein-rich diet and multivitamin supplementation were started. During hospitalization, the patient's level of consciousness gradually improved: he regained awareness and appeared partially oriented, slightly apathic and bradypsychic; the following CT-scans showed reabsorption of the hemorrhage. The skin lesions improved as well (Fig. 1B). Recurrent diarrhea decreased in frequency without reaching complete regression.

      References

        • Savvidou S.
        Pellagra: a non-eradicated old disease.
        Clin Pract. 2014; 4 (Mar 27): 637
        • Kyuraghi Lute S.
        • Sato R.
        Alcoholic pellagra as a cause of altered mental status in the emergency department.
        J Emerg Med. 2017; 53 (Oct): 554-557
        • Kapoor R.
        • Saint S.
        • Kapoor J.R.
        • Johnson R.A.
        • Dhaliwal G.
        Clinical problem-solving. D is for delay.
        N Engl J Med. 2014; 371 (Dec 4): 2218-2223