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Print version ISSN 0365-0596
An. Bras. Dermatol. vol.86 no.6 Rio de Janeiro Nov./Dec. 2011
Cutaneous manifestations of kwashiorkor: a case report of an adult man after abdominal surgery*
Danielle MannI; Carolina PresottoII; Stella Maria Facó QueenIII; Elisa Fontenelle de OliveiraIV; Alexandre Carlos GrippV
IPhysician, resident in dermatology at the Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Hospital Universitário Pedro Ernesto - Universidade do Estado do Rio de Janeiro - HUPE / UERJ) - Rio de Janeiro (RJ), Brazil
IIPhysician graduated in medicine from the Catholic University of Rio Grande do Sul ( Pontifícia Universidade Católica do Rio Grande do Sul - PUC-RS) - Graduate student in dermatology at the Teaching Hospital Pedro Ernesto (Hospital Universitário Pedro Ernesto - Universidade do Estado do Rio de Janeiro - HUPE / UERJ) - Rio de Janeiro (RJ), Brazil
IIIPediatrician at the Public Workers' Hospital of the State of Rio de Janeiro (Hospital dos Servidores do Estado do Rio de Janeiro - HSE-RJ) - Graduate student in dermatology at the Teaching Hospital Pedro Ernesto (Hospital Universitário Pedro Ernesto - Universidade do Estado do Rio de Janeiro - HUPE / UERJ) - Rio de Janeiro (RJ), Brazil
IVSubstitute Professor at the Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Hospital Universitário Pedro Ernesto - Universidade do Estado do Rio de Janeiro - HUPE / UERJ); dermatologist at the City Hospital Jesus (Hospital Municipal Jesus); head of the outpatient clinic of Pediatric Dermatology of the Dermatology Institute Prof. Azulay - Charity Hospital of Rio de Janeiro (Instituto de Dermatologia Prof. Azulay - Santa Casa de Misericórdia do Rio de Janeiro) - Rio de Janeiro (RJ), Brazil
VMaster degree in dermatology - Assistant Professor of Dermatology and in charge of the Dermatology Clinic at the Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Hospital Universitário Pedro Ernesto - Universidade do Estado do Rio de Janeiro - HUPE / UERJ) - Rio de Janeiro (RJ), Brazil
Kwashiorkor is a type of protein-energy malnutrition where diet protein deficit is found, in spite of appropriate caloric intake. Cutaneous manifestations include xerosis, with abnormally dry skin that has a flaking enamel paint aspect, a typical red to gray-white hair color, the "flag sign" and more evident edema in lower limbs and face, giving it a full moon appearance. This article reports a case of a male adult patient who had undergone Whipple surgery for treatment of chronic pancreatitis associated with pseudotumor of the pancreatic head that progressed to cutaneous manifestations of kwashiorkor after pulmonary tuberculosis.
Keywords: Pancreatitis, chronic; Protein-energy malnutrition; Tuberculosis, pulmonary
Kwashiorkor is a form of protein-energy malnutrition where there is diet protein deficit, although caloric intake is adequate. Fat reserves and muscle mass are not affected at first, giving an illusory aspect of adequate nutrition. There is a connection with acute diseases such as traumatism and sepsis, as well as chronic diseases involving inflammatory responses that increase protein-energy requirements when consumption is limited.1
A duodenopancreatectomy or Whipple procedure is a therapeutic option for chronic pancreatitis, when the cephalic portion of the pancreas is compromised. Resection involves head and body of the pancreas, duodenum, part of the jejunum, the distal choledochus and the gall bladder.2
This article reports the case of an adult male patient previously subjected to the Whipple procedure, who presented pulmonary tuberculosis and progressed to clinical kwashiorkor, also verified by laboratory tests.
A 43-year old man, black, lawyer, had been subjected to the Whipple procedure three years before, due to chronic pancreatitis associated with pseudotumor on the pancreas head. It progressed to about seven daily bowel movements, weight loss of 13 kg for some time, without loss of appetite. He had been using iron sulfate and folic acid replacement. Two years after the surgery the patient was diagnosed with pulmonary tuberculosis, which was treated for six months. He began to notice skin depigmentation and body hair rarefaction.
The physical examination showed moon facies, dry reddish hair that was easily detached, depapillated tongue, angular cheilitis, onychodystrophy of fingernails and toenails, periungual desquamation and fissures, plantar keratosis and fissures, edema in hands and lower limbs up to the knees, diffuse hair rarefaction, ichthyosiform skin, hypochromia and desquamation of superciliary and central face regions, hypopigmentation under desquamative body areas, hyperemia and hair rarefaction in the genital area (Figures 1 to 3).
Laboratory tests demonstrated normochromic normocytic anemia (Hct 24% Hb 7.4 g/dl), hypoalbuminemia (1.9 g/dl), positive fecal fat test, normal vitamin B12, folic acid, ferritin, amylase and lipase levels. Negative serologies for HIV and hepatitis C. Hepatitis B with a pattern of cured prior disease. Non reactive PPD. High digestive endoscopy revealed subtotal gastrectomy with Billroth II reconstruction .
The histopathological examination revealed confluent hyperkeratosis and parakeratosis with focuses of cell vacuolation in the granulous layer of straightened epidermis. The papillary dermis showed edema and moderate perivascular infiltrate with lymphocytes (Figure 4).
The patient was referred to the outpatient clinic and a special diet was prescribed, according to orientation of nutritional support (hypercaloric oral diet, hyperproteic and with medium-chain triglycerides), vitamin supplements, oligoelements and oral replacement of pancreatic enzymes. He was discharged one month later for outpatient clinic follow-up with progressive elevation of serum albumin, decrease of edema, weight gain of around 5 Kg, decrease to two daily bowel movements and better general disposition. After three months of treatment, important improvement in skin and hair pigmentation could be verified (Figures 5 and 6).
The clinical diagnosis of Kwashiorkor is confirmed by signs that include: easily detached hair; edema; skin fissures; faulty wound healing; skin anergy. Symptoms that are considered sine qua non: albumin < 2.8 g/dl, transferrin < 150 mg/dl or total iron binding capacity < 200mcg/dl.1
Regarding cutaneous manifestations, generalized cutaneous dermatitis is compared to flaking enamel paint with a skin fissure pattern, suggesting a cracked or "crazy" pavement. Large erosion areas may be present in the most severe cases.3
Increased cutaneous pigmentation can be observed in extensors surfaces of arms and legs or in irritated areas and not necessarily in sun-exposed areas (which contrasts with pellagra). Depigmentation after desquamation may occur in these areas or it may be generalized. Hair alterations typically include development of a reddish coloration, which may evolve to a white-gray color. The flag sign occurs due to moments of nutritional improvement and worsening. Edema, a consequence of hypoproteinemia, is present in the lower limbs, but may also affect hands and face (moon facies). It is already present in internal organs before being detected in the limbs and face. In adults the symptom complex may be less pronounced, being manifest mainly as acquired xerosis or ichthyosis and may result from decreased secretion of sebaceous glands or associated deficit of micronutrients.3,4,5
Histopathology findings include superficial perivascular infiltrate with lymphocytes, pale keratinocytes in band-like form along the upper part of epidermis and confluent parakeratosis. The most specific findings are paleness, ballooning degeneration and keratinocyte necrosis in band-like form along the upper part of epidermis; they are considered almost pathognomonic of dermatitis, due to nutritional deficit. However, these findings are not pathognomonic of kwashiorkor and may be found in other conditions that have nutritional deficit as causal factor.6,7
The diagnosis of energy-protein malnutrition with predominant characteristics of kwashiorkor was confirmed in our patient, based on ectoscopic alterations found on physical examination corroborated by laboratory tests. It was related to a secondary form of energy-protein malnutrition due to anatomic alterations imposed on abdominal visceral organs by the surgical procedure performed, since structures that are fundamental for nutrient digestion and absorption, such as the stomach, duodenum, gall bladder and pancreas were partially or entirely removed. Therefore, not only protein and lipid intake was compromised, but also hydro- and liposoluble vitamins and oligoelements such as iron and calcium (both absorbed in the duodenum), indispensable for human body homeostasis. Thus, it is understood that, in an already weakened body, an intercurrent infection such as tuberculosis in the described case would unbalance and aggravate the picture, leading to the onset of energy-protein malnutrition of the Kwashiorkor type.
1. Heimburger DC. Desnutrição e avaliação nutricional. In: Fauci AS, Kasper DL, Longo DL, Braunwald E, Hauser SL, Jameson JL, Loscalzo J, editores. Harrison Medicina Interna. 17. ed. Rio de Janeiro: Mc Graw Hill; 2009. p.450-4. [ Links ]
2. Manso JEF, Renteria JM, Eulálio JMR. Pancreatite crônica. In: Vieira OM, Chaves CP, Manso JEF, Eulálio JMR, editores. Clínica Cirúrgica: fundamentos teóricos e práticos. Rio de Janeiro: Atheneu; 2002. p.339-52. [ Links ]
3. Jen M, Shah KN, Yan AC. Cutaneous changes in nutricional disease. In: Wolff K, Goldsmith LA, Katz SI, Gilchrest BA, Paller AS, Leffell DJ, editores. Fitzpatrick's Dermatology in General Medicine. 7th ed. New York: Mc Graw Hill; 2008. p.1201-18. [ Links ]
4. Ruiz-Maldonado R, Orozco-Covarrubias L. Nutritional diseases. In: Bolognia JL, Jorizzo JL, Rapini RP, editores. Dermatology. 2nd ed. Spain: Mosby Elsevier; 2008. p.661-73. [ Links ]
5. Lee BY, Hogan DJ, Ursine S, Yanamandra K, Bocchini JA. Personal observation of skin disorders in malnutrition. Clin Dermatol. 2006;24:222-7. [ Links ]
6. Liu T, Howard RM, Mancini AJ, Weston WL, Paller AS, Drolet BA, et al. Kwashiorkor in the United States: fad diets, perceived and true milk allergy, and nutritional ignorance. Arch Dermatol. 2001;137:630-6. [ Links ]
7. Abbott RA, Robson A, O'Donoghue N. Acquired loss of hair pigment associated with a flexural dermatosis. Clin Exp Dermatol. 2009;34:735-6. [ Links ]
Mailing address: Received on 09.03.2010. * Study carried out at the Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Hospital Universitário Pedro Ernesto - Universidade do Estado do Rio de Janeiro - HUPE / UERJ) - Rio de Janeiro (RJ), Brazil.
Travessa Leopoldina Vieira, 38/602, Alcântara
CEP: 24452-190 - São Gonçalo - RJ, Brazil
Approved by the Advisory Board and accepted for publication on 30.08.2010.
Conflict of interest: None
Financial funding: None
Received on 09.03.2010.
* Study carried out at the Teaching Hospital Pedro Ernesto - State University of Rio de Janeiro (Hospital Universitário Pedro Ernesto - Universidade do Estado do Rio de Janeiro - HUPE / UERJ) - Rio de Janeiro (RJ), Brazil.