Kwashiorkor_1113 Derm Clinic
For the past two months, a man, aged 49 years, experienced persistent nausea, vomiting, and diarrhea. Dermatology was consulted for treatment of a rash that had been present for one month. Medical history was significant for HIV and end-stage renal disease.
Physical examination revealed superficial desquamation with underlying erosions on the trunk and extremities with a “flaky paint” appearance. Erythematous plaques were noted in the intertriginous areas, most prominently in the groin. Lab results were significant for an albumin level of 0.8 g/dL.
Submit your diagnosis to see full explanation.
A nutritional disease is caused by either insufficiency or excess of one or more dietary essentials.1 Kwashiorkor is a nutritional disease in which there is protein deficiency despite normal caloric intake. The disease is defined as a total body weight of 60% to 80% of ideal body weight with either edema or hypoalbuminemia, or both.2
Nutritional diseases, including kwashiorkor, are endemic in the developing world. In developed countries, protein deficiency occurs predominantly in the chronically ill and in hospitalized patients.3
Protein deficiency can be attributable to either exogenous (primary) causes or endogenous (secondary) causes. Examples of exogenous causes include poverty, old age, alcoholism, psychiatric disorders (e.g., anorexia nervosa, bulimia), diets, and child neglect. Causes of endogenous protein deficiency include intestinal malabsorption (e.g., bowel bypass, cystic fibrosis), gastroenteritis or other GI diseases, metabolic disease, chronic systemic disease (e.g., malignancy), and HIV.3
Kwashiorkor is characterized by skin and hair changes and edema. In children with kwashiorkor, impaired growth and the characteristic potbelly are often seen.
Peeling of the skin is a cutaneous finding specific to kwashiorkor. In mild cases, only a superficial desquamation is seen; it is referred to as “enamel paint spots.” In severe cases, large erosions can be seen underlying the desquamation; these have been described as “flaky paint.”3
Dyschromia is a common finding in kwashiorkor for a multitude of reasons. Pallor caused by skin distension from edema and loss of pigment may be seen in some cases. Hypopigmentation may be seen following abrasions, wounds, and ulcerations. Large “flaky paint” ulcerations often heal with hypopigmentation coupled with a characteristic hyperpigmented border.2 Hyperpigmentation can be seen in areas subject to trauma or friction, including the flexures, groin, buttocks, and elbows.2 Other cutaneous findings of kwashiorkor include erythema, thinning, petechiae, ecchymoses, and purpura.3
The hair in patients with kwashiorkor is often sparse, dry, and brittle. A reddish tinge may be noted as well. Alternating bands of pale and dark coloration along a single strand of hair is a specific hair finding in patients with protein deficiency. The dark hair corresponds to periods of good nutrition, while the pale hair occurs during periods of poor protein intake. This finding is referred to as “the flag sign.”2,3 Curly-haired individuals with kwashiorkor may develop straight hair.2 The nails in patients with kwashiorkor are often soft and thin.2,3 The mucosa may often be involved and can present with cheilitis, xerophthalmia, and vulvovaginitis.
Systemic features of kwashiorkor include edema, moon facies, and potbelly. Children are often anorexic, irritable, and apathetic. If there is a prolonged period of deficient protein intake, children may develop failure to thrive with delayed growth and mental development.3 Bilateral parotitis is another possible systemic feature associated with kwashiorkor. As seen in many other nutritional deficiencies, patients with kwashiorkor may develop diarrhea.
By definition, kwashiorkor patients are noted to have hypoalbuminemia with albumin levels <2.5 g/dL. They may have some level of immune deficiency and are often noted to have low alpha and beta globulins. Over time, these individuals may also develop a gamma-globulin deficiency. Impaired cellular immunity may also develop.3 This deficiency in humoral and cellular immunity may predispose patients to superimposed bacterial and fungal infections.
The diagnosis of kwashiorkor is made based on clinical and laboratory findings. Skin biopsies are useful in confirming an underlying nutritional deficiency; however, a skin biopsy cannot determine which specific nutrient is deficient. The most notable histologic findings for all nutritional deficiencies are pallor of the upper epidermis, a superficial perivascular lymphocytic infiltrate, and confluent parakeratosis.1 If a skin biopsy of the “enamel pain spots” is obtained, a thickened, pigmented stratum corneum with an underlying stratum lucidum may be seen.3 Some patients may develop hepatomegaly. If a liver biopsy or imaging study is performed, fatty-liver change may be noted.3 Given the sparse hair seen clinically in kwashiorkor patients, it is not surprising that histologic examination of scalp biopsies reveal a decreased number of follicles in the growth phase (anagen follicles) and an increased number of follicles in the resting phase (telogen follicles). Scalp biopsies may also reveal severe atrophy, shaft constriction, and depletion of pigment in anagen follicles, which explains the pale, brittle, lusterless hair seen in patients with protein deficiency.
The differential diagnosis of kwashiorkor includes such other nutritional deficiencies as zinc deficiencies and pellagra. Zinc deficiency may be inherited or acquired; the inherited form is known as acrodermatitis enteropathica. Like patients with kwashiorkor, patients with zinc deficiency can present with stomatitis, alopecia, diarrhea, irritabiliy, and failure to thrive. Unlike patients with kwashiorkor, patients with zinc deficiency often present with glossitis and photophobia. A low zinc level will be diagnostic. A quick screening test for zinc deficiency is a low serum alkaline phosphatase level, because alkaline phosphatase is a zinc-dependent enzyme.4 Pellagra is caused by vitamin B3 deficiency and is characterized by the triad of dermatitis, diarrhea, and dementia. The dermatitis seen in pellagra is distinct from that of kwashiorkor because it is photodistributed. When this photosensitive eruption forms a broad band around the neck, it is referred to as “Casal necklace.”3 Diagnosis is often confirmed by the clinical response to niacin supplementation, as niacin levels are typically only obtained for research purposes.
Kwashiorkor is treated through the implementation of a balanced diet with adequate protein and caloric intake.3 Cutaneous findings are all reversible with therapy.3 While awaiting resolution of cutaneous lesions with protein supplementation, symptomatic relief can be achieved with topical moisturizers and ointments. Fatal complications of kwashiorkor include hypoglycemia with hypothermia, coma, and bacterial or parasitic disease, so careful monitoring of these patients is required.3 High mortality rates are seen in patients with HIV.
The protein deficiency of the man in this case was felt to be multifactorial, as he was diagnosed with dumping syndrome in addition to chronic active ileitis and cytomegalovirus colitis in the setting of HIV. After several weeks of trophic tube feeds with total parenteral nutrition, improvement in his skin was noted.
Audrey Chan, MD, is a third-year dermatology resident at Baylor College of Medicine in Houston.
- James WD, Berger TG, Elston DM. Andrews’ Diseases of the Skin: Clinical Dermatology. 11th ed. Philadelphia, Pa.: Saunders Elsevier; 2011:469.
- Schachner LA, Hansen RC, eds. Pediatric Dermatology. 4th ed. Philadelphia, Pa.: Mosby Elsevier; 2011:1263-1264.
- Bolognia JL, Jorizzo JL, Rapini RP, eds. Dermatology. 2nd ed. St. Louis, Mo.: Elsevier-Mosby; 2008:661-663.
- Cho YE, Lomeda RA, Ryu SH, et al. Zinc deficiency negatively affects alkaline phosphatase and the concentration of Ca, Mg and P in rats. Nutr Res Pract. 2007;1:113-119. Available at www.ncbi.nlm.nih.gov/pmc/articles/PMC2882585/.
All electronic documents accessed October 15, 2013.