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Flaky Paint Dermatosis

Flaky Paint Dermatosis Case An African American woman in her 60s was admitted to the intensive care unit (ICU) for altered mental status, respiratory failure, and skin sloughing. Her medical history was notable for a recent myocardial infarction but otherwise negative for human immunodeficiency virus (HIV), substance abuse, gastrointestinal tract surgery, or any other chronic disease. Over the months preceding admission, she had become progressively withdrawn from her family, increased her daily alcohol consumption, and noted an unintentional 13.6-kg weight loss, progressive extremity swelling, and decreased nutritional intake. Her dermatologic examination was significant for generalized shiny, enamel-like, hyperpigmented scale in an irregular pattern; when removed, it demonstrated underlying hypopigmentation (Figure, A and B). The physical examination was notable for marked pitting edema and ascites; the mucous membranes, hair, and nails were normal. Figure. View LargeDownload A, Thigh; B, foot; C, a skin biopsy specimen from the leg (hematoxylin-eosin, original magnification ×40). Laboratory testing demonstrated numerous electrolyte abnormalities, acute renal failure, low serum iron level (7 μg/dL), low prealbumin level (5 g/dL), hypoalbuminemia (1.7 g/dL), hypogammaglobulinema, zinc deficiency (47 μg/dL), and undetectable vitamin C and pyridoxine levels. (To convert serum iron to micromoles per liter, multiply by 0.179; to convert albumin to grams per liter, multiply by 10; to convert serum zinc to micromoles per liter, multiply by 0.153.) Ultrasonography of the abdomen revealed steatohepatitis. A skin biopsy specimen was obtained from the leg (Figure, C). What is your diagnosis? Read the Discussion. Discussion Diagnosis Kwashiorkor Microscopic Findings and Clinical Course Results from a biopsy (Figure, C) revealed prominent hyperkeratosis and parakeratosis overlying an effaced epidermis with papillary dermal pallor. Direct immunofluorescence testing was negative. These findings are not pathognomonic for kwashiorkor but are commonly seen in nutritional deficiencies. The patient was experiencing diuresis, and findings from nerve conduction studies and electromyography demonstrated a sensory and motor polyneuropathy. Over the 2-week ICU course, the patient progressively experienced decompensation; despite a timely diagnosis and meticulous nutritional replacement, she ultimately died from multiple organ failure, and care was withdrawn. The cutaneous manifestations of kwashiorkor progress from xerosis and atrophy to widespread hyperkeratosis and hyperpigmentation. Ultimately, the fragile skin peels away in an irregular pattern to reveal an underlying hypopigmentation that gives the appearance of flaking paint (Figure, A). The shiny, varnished-looking quality of the hyperpigmentation is the most common dermatologic finding in kwashiorkor (Figure, B). Other common findings include dry, brittle hair with alternating bands of normal and reddish yellow coloration; these document periods of adequate and poor nutrition. Nail plates are generally soft and thin with subtle fissures and ridges or more prominent koilonychia.1 Historically, the pathophysiologic mechanisms of kwashiorkor and marasmus have been thought to be simple protein-energy malnutrition (PEM). Marasmus was thought to progress into kwashiorkor, which was diagnosed clinically with additional findings of edema, hypoproteinemia, and the typical skin findings noted herein.2 However, recent research has yielded alternate theories of dysadaptation, including micronutrient deficiencies, slow protein catabolism, oxidative stress, and antidiuretic hormone (ADH) excess. The latter theory postulates that the release of ferritin from hepatocellular death stimulates the kidneys to produce excessive amounts of ADH with consequent water retention leading to anasarca.2 Although the incidence of PEM is markedly higher in underfed populations, developed nations where obesity is rampant are not immune. PEM in the pediatric literature is robust owing to parental ignorance and infant fad diets, but few cases have been reported in adults. As with pediatric cases and in the case of our patient, hypoproteinemia is rarely an isolated laboratory finding: other electrolyte, vitamin, and mineral deficiencies are common. The adult literature casuistically associates PEM with specific conditions such as HIV, substance abuse disorders, history of gastric bypass surgery, and anorexia nervosa.3-8 Our patient’s increased daily alcohol consumption and decreased nutritional intake are likely the causative factors leading to her presentation, diagnosis, and ultimate death. This case highlights the classic clinical findings associated with kwashiorkor and unique risk factors associated with adult populations in developed countries. It underscores that dermatologic manifestations of kwashiorkor can be underrecognized in developed countries. It also emphasizes the importance of a thorough nutritional evaluation of critically ill patients. Back to top Article Information Corresponding Author: J. Austin Cox, MD, Department of Dermatology, Naval Medical Center San Diego, 34800 Bob Wilson Dr, San Diego, CA 92134 (james.austin.cox@us.army.mil). Funding/Support: None reported. Published Online: November 27, 2013. doi:10.1001/jamadermatol.2013.5520. Additional Contributions: We are indebted to Gopal Patel, MD. Section Editor: Mary S. Stone, MD; Assistant Section Editors: Soon Bahrami, MD; Carrie Ann R. Cusack, MD; Molly A. Hinshaw, MD; Arni K. Kristjansson, MD; Lori D. Prok, MD. References 1. Grover Z, Ee LC. Protein energy malnutrition. Pediatr Clin North Am. 2009;56(5):1055-1068.PubMedGoogle ScholarCrossref 2. Ahmed T, Rahman S, Cravioto A. Oedematous malnutrition. Indian J Med Res. 2009;130(5):651-654.PubMedGoogle Scholar 3. Jaffe AT, Heymann WR. Kwashiorkor/zinc deficiency overlap following partial gastrectomy. Int J Dermatol. 1998;37(2):134-137.PubMedGoogle ScholarCrossref 4. Albers SE, Brozena SJ, Fenske NA. A case of kwashiorkor. Cutis. 1993;51(6):445-446.PubMedGoogle Scholar 5. Mann D, Presotto C, Queen SM, Oliveira EF, Gripp AC. Cutaneous manifestations of kwashiorkor: a case report of an adult man after abdominal surgery. An Bras Dermatol. 2011;86(6):1174-1177.PubMedGoogle ScholarCrossref 6. Lewandowski H, Breen TL, Huang EY. Kwashiorkor and an acrodermatitis enteropathica-like eruption after a distal gastric bypass surgical procedure. Endocr Pract. 2007;13(3):277-282.PubMedGoogle ScholarCrossref 7. Alam M, Grossman ME, Longley BJ, Schneiderman PI. Kwashiorkor in patients with AIDS. Cutis. 2001;67(4):321-324, 327.PubMedGoogle Scholar 8. Kuhl J, Davis MD, Kalaaji AN, Kamath PS, Hand JL, Peine CJ. Skin signs as the presenting manifestation of severe nutritional deficiency: report of 2 cases. Arch Dermatol. 2004;140(5):521-524.PubMedGoogle ScholarCrossref http://www.deepdyve.com/assets/images/DeepDyve-Logo-lg.png JAMA Dermatology American Medical Association

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References (9)

Publisher
American Medical Association
Copyright
Copyright © 2014 American Medical Association. All Rights Reserved.
ISSN
2168-6068
eISSN
2168-6084
DOI
10.1001/jamadermatol.2013.5520
pmid
24285001
Publisher site
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Abstract

Case An African American woman in her 60s was admitted to the intensive care unit (ICU) for altered mental status, respiratory failure, and skin sloughing. Her medical history was notable for a recent myocardial infarction but otherwise negative for human immunodeficiency virus (HIV), substance abuse, gastrointestinal tract surgery, or any other chronic disease. Over the months preceding admission, she had become progressively withdrawn from her family, increased her daily alcohol consumption, and noted an unintentional 13.6-kg weight loss, progressive extremity swelling, and decreased nutritional intake. Her dermatologic examination was significant for generalized shiny, enamel-like, hyperpigmented scale in an irregular pattern; when removed, it demonstrated underlying hypopigmentation (Figure, A and B). The physical examination was notable for marked pitting edema and ascites; the mucous membranes, hair, and nails were normal. Figure. View LargeDownload A, Thigh; B, foot; C, a skin biopsy specimen from the leg (hematoxylin-eosin, original magnification ×40). Laboratory testing demonstrated numerous electrolyte abnormalities, acute renal failure, low serum iron level (7 μg/dL), low prealbumin level (5 g/dL), hypoalbuminemia (1.7 g/dL), hypogammaglobulinema, zinc deficiency (47 μg/dL), and undetectable vitamin C and pyridoxine levels. (To convert serum iron to micromoles per liter, multiply by 0.179; to convert albumin to grams per liter, multiply by 10; to convert serum zinc to micromoles per liter, multiply by 0.153.) Ultrasonography of the abdomen revealed steatohepatitis. A skin biopsy specimen was obtained from the leg (Figure, C). What is your diagnosis? Read the Discussion. Discussion Diagnosis Kwashiorkor Microscopic Findings and Clinical Course Results from a biopsy (Figure, C) revealed prominent hyperkeratosis and parakeratosis overlying an effaced epidermis with papillary dermal pallor. Direct immunofluorescence testing was negative. These findings are not pathognomonic for kwashiorkor but are commonly seen in nutritional deficiencies. The patient was experiencing diuresis, and findings from nerve conduction studies and electromyography demonstrated a sensory and motor polyneuropathy. Over the 2-week ICU course, the patient progressively experienced decompensation; despite a timely diagnosis and meticulous nutritional replacement, she ultimately died from multiple organ failure, and care was withdrawn. The cutaneous manifestations of kwashiorkor progress from xerosis and atrophy to widespread hyperkeratosis and hyperpigmentation. Ultimately, the fragile skin peels away in an irregular pattern to reveal an underlying hypopigmentation that gives the appearance of flaking paint (Figure, A). The shiny, varnished-looking quality of the hyperpigmentation is the most common dermatologic finding in kwashiorkor (Figure, B). Other common findings include dry, brittle hair with alternating bands of normal and reddish yellow coloration; these document periods of adequate and poor nutrition. Nail plates are generally soft and thin with subtle fissures and ridges or more prominent koilonychia.1 Historically, the pathophysiologic mechanisms of kwashiorkor and marasmus have been thought to be simple protein-energy malnutrition (PEM). Marasmus was thought to progress into kwashiorkor, which was diagnosed clinically with additional findings of edema, hypoproteinemia, and the typical skin findings noted herein.2 However, recent research has yielded alternate theories of dysadaptation, including micronutrient deficiencies, slow protein catabolism, oxidative stress, and antidiuretic hormone (ADH) excess. The latter theory postulates that the release of ferritin from hepatocellular death stimulates the kidneys to produce excessive amounts of ADH with consequent water retention leading to anasarca.2 Although the incidence of PEM is markedly higher in underfed populations, developed nations where obesity is rampant are not immune. PEM in the pediatric literature is robust owing to parental ignorance and infant fad diets, but few cases have been reported in adults. As with pediatric cases and in the case of our patient, hypoproteinemia is rarely an isolated laboratory finding: other electrolyte, vitamin, and mineral deficiencies are common. The adult literature casuistically associates PEM with specific conditions such as HIV, substance abuse disorders, history of gastric bypass surgery, and anorexia nervosa.3-8 Our patient’s increased daily alcohol consumption and decreased nutritional intake are likely the causative factors leading to her presentation, diagnosis, and ultimate death. This case highlights the classic clinical findings associated with kwashiorkor and unique risk factors associated with adult populations in developed countries. It underscores that dermatologic manifestations of kwashiorkor can be underrecognized in developed countries. It also emphasizes the importance of a thorough nutritional evaluation of critically ill patients. Back to top Article Information Corresponding Author: J. Austin Cox, MD, Department of Dermatology, Naval Medical Center San Diego, 34800 Bob Wilson Dr, San Diego, CA 92134 (james.austin.cox@us.army.mil). Funding/Support: None reported. Published Online: November 27, 2013. doi:10.1001/jamadermatol.2013.5520. Additional Contributions: We are indebted to Gopal Patel, MD. Section Editor: Mary S. Stone, MD; Assistant Section Editors: Soon Bahrami, MD; Carrie Ann R. Cusack, MD; Molly A. Hinshaw, MD; Arni K. Kristjansson, MD; Lori D. Prok, MD. References 1. Grover Z, Ee LC. Protein energy malnutrition. Pediatr Clin North Am. 2009;56(5):1055-1068.PubMedGoogle ScholarCrossref 2. Ahmed T, Rahman S, Cravioto A. Oedematous malnutrition. Indian J Med Res. 2009;130(5):651-654.PubMedGoogle Scholar 3. Jaffe AT, Heymann WR. Kwashiorkor/zinc deficiency overlap following partial gastrectomy. Int J Dermatol. 1998;37(2):134-137.PubMedGoogle ScholarCrossref 4. Albers SE, Brozena SJ, Fenske NA. A case of kwashiorkor. Cutis. 1993;51(6):445-446.PubMedGoogle Scholar 5. Mann D, Presotto C, Queen SM, Oliveira EF, Gripp AC. Cutaneous manifestations of kwashiorkor: a case report of an adult man after abdominal surgery. An Bras Dermatol. 2011;86(6):1174-1177.PubMedGoogle ScholarCrossref 6. Lewandowski H, Breen TL, Huang EY. Kwashiorkor and an acrodermatitis enteropathica-like eruption after a distal gastric bypass surgical procedure. Endocr Pract. 2007;13(3):277-282.PubMedGoogle ScholarCrossref 7. Alam M, Grossman ME, Longley BJ, Schneiderman PI. Kwashiorkor in patients with AIDS. Cutis. 2001;67(4):321-324, 327.PubMedGoogle Scholar 8. Kuhl J, Davis MD, Kalaaji AN, Kamath PS, Hand JL, Peine CJ. Skin signs as the presenting manifestation of severe nutritional deficiency: report of 2 cases. Arch Dermatol. 2004;140(5):521-524.PubMedGoogle ScholarCrossref

Journal

JAMA DermatologyAmerican Medical Association

Published: Jan 1, 2014

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