Banda, P

Kumwenda, JJ

Mulambia, C

Segula, D

English

PubMed

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Malawi Medical Journal; 24(1) 19-20 March 2012  19Case Report - A forgotten Dermatological DiseaseA 42 year old woman was admitted to hospital with a one month history of  progressive forgetfulness, irritability and confusion. There was no history of  tremor or confabulation. Reportedly, she also had fever two weeks prior to admission. There was no history of  headache, neck-ache or neck stiffness. Further inquiry revealed she had developed rash around her neck and in the distal parts of  all four limbs a month prior to the onset of  the altered mental status. There was no history of  rash involving the mucosae or of  having taken any drugs in the period preceding the rash. The guardians also reported that the patient had had diarrhea which was watery and had lasted a week prior to admission. There was no history of  vomiting.She had no history of  tuberculosis, diabetes or hypertension. She had been staying in Lilongwe until the illness. She is married with seven children. One child died of  an unknown cause. There was no other family member who had been reported with a similar illness. Her husband was employed and worked as a gardener. To generate extra income for her family, she has been distilling kachasu (local spirits) for commercial purposes since 1999. She had a history of  alcohol consumption (4 units per day) since 2005.Reportedly, she was intoxicated most days of  the week. She had a 1 pack-year history of  smoking.Her meals mainly consisted of  nsima and vegetables. However, she would often neglect herself  and fail to eat when intoxicated from alcohol.On examination, she was irritable and confused. Her Glasgow Coma Score was 14. She had no neck stiffness or tenderness. There was no flapping tremor or stigmata of  chronic liver disease.Her blood pressure was 120/60 mmHg, pulse rate 96/minute, respiratory rate 20/minute and temperature 37.90C. There was a hyperpigmented well-marginated rash around her neck involving most of  C3 and C4 dermatomes (figure 1 and 2). She had similar rashes in other sun exposed areas of  the body.These were more pronounced in the dorsal aspects of  the hands and feet (figure 3 and 4).There was a vesicular rash on her upper lip which had begun crusting (figure 1).Her cardiovascular, respiratory, abdominal examinations were normal. Her initial investigation results on admission revealed that she was HIV negative. Her full blood count was as follows: WBC 3.6 x 109/L, Hb12.2g/dL MCV 96 fL, MCH 32.3 pg, Platelets 223 x 109/L. The cerebrospinal fluid (CSF) analysis was normal. There was no growth from the CSF and blood after 7 days. She had tested VDRL negative. The renal function results showed creatinine of  0.59mg/dl, BUN of  6mg/dl, sodium of  141 mmol/L and calcium of  2.3mmol/L (corrected for albumin which was normal)QuestionWhat is the diagnosis?What is the name of the hyperpigmentation around the neck?DiscussionPellagra was first described by a Spanish physician Don Gaspar Casal in 1735. He called the disease “mal de la rosa,” because all the affected patients had the typical reddish and glossy rash on the dorsum of  the hands and feet. The rash around the neck carries his name: Casal’s collar or necklace 1, 2. Casal noted the disease among the poor of  Spain. The disease is characterized by the four D’s: Dermatitis, Diarrhea, and Dementia and in some cases Death. The dermatitis of  pellagra is characteristically present in sun exposed area. Dementia is evidenced by the progressive forgetfulness.3  Pellagra results from a systemic cellular deficiency of  Niacin (Vitamin B3). Niacin can be directly obtained from diet or synthesized from an essential amino acid tryptophan. It is estimated that 60mg of  tryptophan will produce 1mg of  niacin in the presence of  vitamin B2 and B6. 3 The daily allowance recommended for niacin is 15- 20mg. Primary pellagra results when the diet is deficient in niacin or tryptophan. The niacin is converted into nicotinamide in the body which is a component of  co-enzyme 1 and coenzyme2. These compounds are involved in glycolysis, protein and amino acid metabolism, pyruvate metabolism, pentose biosynthesis, generation of  ATP bonds, glycerol metabolism and free fatty acid metabolism. Secondary pellagra results when there is enough dietary niacin but disease processes interfere with its intake, absorption or metabolism. The secondary causes include (but are not limited to) chronic alcoholism and a variety of  chronic bowel disorders. Sporadic cases among alcoholics have been reported.4, 5 Our patient had a considerable history of  alcohol consumption which makes it very likely to have been the cause of  the pellagra. Primary pellagra was unlikely in view of  the fact that it was absent in the other members of  the family who had been taking the same diet. Other causes of  secondary pellagra which were very unlikely in our patient but that need consideration in any patient with pellagra include prolonged diarrhoea, anorexia nervosa, tuberculosis of  the gastrointestinal system, chronic colitis, regional ileitis, severe ulcerative colitis, hepatic cirrhosis, carcinoid tumour, Hartnup’s syndrome and medication: 5-fluorouracil, pyrazinamide, 6-mercaptopurine, isoniazid, phenobarbital and chloramphenicol.6,7,8,9 Investigations in any patient who presents with confusion should include an HIV test and cerebrospinal fluid (CSF) analysis (unless there is a contra-indication). Our patient tested negative for HIV antibodies. Her CSF analysis was normal. Metabolic causes of  confusion need be considered as well. These include hyponatraemia, hypernatraemia or hypercalcaemia. In our patient, the electrolyte results were normal. In view of  the reported fever prior to admission, her blood was taken for culture and there was no growth. The reported fever was thought to be most likely due to herpes simplex reactivation as she had herpetic vesicular rash on her upper lips that had already started crusting when she presented.Segula D1,2, Banda P1, Mulambia C1, Kumwenda JJ1,1. Department of  Medicine, University of  Malawi College of  Medicine, Blantyre, Malawi2. Malawi-Liverpool-Wellcome (MLW) Trust Clinical Research Programme, Blantyre, MalawiMalawi Medical Journal; 24(1) 19-20 March 2012  120Clinical ProgressThe patient was treated with vitamin B tablets compound, strong. The adult dose for acute pellagra is nicotinamide 100mg orally every 6 hours for several days or until resolution of  major features.3   Each Vitamin B tablet compound (‘strong’) contains 20mg of  nicotinamde. It also contains pyridoxine and riboflavin which are needed in the synthesis of  niacin from dietary tryptophan. Therefore the concomitant administration of  these is desirable. There was gradual resolution of  the confusion. She had no diarrhoea during the admission. Nine days later, she was discharged.The patient together with her guardians was counselled on stopping alcohol, ensuring a diet rich in protein and niacin, and avoiding sun exposure. Food sources of  niacin and/or tryptophan include yeast, eggs, bran, peanuts, meat, poultry, fish, legumes and whole grain cereals. Maize contains considerable amount of  niacin but it is in bound form and therefore not readily bioavailable. She was advised to continue to take  vitamin B compound, until the resolution of  the skin lesions. The nicotinamide was stepped down to 50mg every 8 – 12 hours on discharge.1 Other supportive management included emollients for the skin lesions.The prognosis of  pellagra is excellent when treated appropriately.3 On the other hand, untreated pellagra will slowly progress, and lead to death within 4 -5 years. Our patient was scheduled for a follow up visit after one month, but she did not attend.  ConclusionThis is a classical case of  pellagra. It is likely that there are many patients with pellagra who are not being diagnosed. During the time in which we have been writing up this case, three other cases have been diagnosed in this hospital. This case report serves to remind us to think of  pellagra in patients with any of  the component symptoms - dermatitis, diarrhoea or dementia, and especially in patients with all three of  these. . It also illustrates the importance of  taking a thorough social and dietary history, as these may give the clues to the diagnosis.References1. Major RH: Don Gaspar Casal, Francois Thierry and pellagra. Bull Hist Med 1944; 16:351-3612. Major RH: Classic description of the disease. Springfield, III, Charles C Thomas, 3rd Ed 1945; 607-6153. Hegyi J, Schwartz RA, Hegyi V, Pellagra: Dematitis, Dementia and Diarrhea. International Journal of Dermatology 2004, 43, 1-5.4. Lorentzen HF, Fugleholm AM, Weismann K. Zinc deifiency and Pellagra in alcohol abuse. Ugerkr Laeger 2000, 162:6854 – 6856.5. Kertesz SG. Pellagra in 2 homeless men. Mayo Clin Proc 2001; 76: 315-318.6. Darvay A, Basarab T, Mc Gregor JM, et al. Isoniazid induced pellagra despite pyridoxine supplementation. Clin Exp Dermatol 1999; 24: 167-1697. Castiello RJ, Lynch PJ. Pellagra and the carcinoid syndrome. Arch Dermatol 1972; 105:574-5778. Rapaport MJ. Pellagra in a patient with anorexia nervosa. Arch Dermatol 1985; 121:255-2579. Stevens HP, Ostlere LS, Begent RHJ, et al. Pellagra secondary to 5-fluorouracil. Br J Dermatol 1993; 128: 578-58010. Hegyi V, Schwartz RA. Pellagra. In: James WD, Elston D, eds. Emedicine Dermatology. St Petersburg: eMedicine Corp, in press. /eMedicine Dermatology [Journal serialonline].2002: Available at: http://author.emedicine.com/derm/topic621.htm.

Case Report: A forgotten Dermatological Disease

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Case Report: A forgotten Dermatological Disease

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