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A patient, aged 50 years, presented with complaints of a full body desquamating skin rash that had lasted several weeks. He had been an inpatient for over a month with treatment-resistant diarrhea and failure to thrive.
The patient had been diagnosed with HIV/AIDS prior to presentation, and after a prolonged hospital course was found to have cytomegalovirus (CMV) colitis, confirmed by CMV polymerase (PCR) of the plasma.
Physical exam revealed bilateral pitting edema, diffuse patchy erythema and desquamation on the trunk and all extremities. The desquamated areas resembled flaky paint and left behind patches of hypopigmentation.
The patient had abdominal ascites as well. A chart review revealed that the patient’s total protein and albumin had been low and decreasing since admission. A punch biopsy of the skin was performed.
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Kwashiorkor, also known as protein-energy malnutrition, is a condition that results from inadequate protein intake or absorption. It is common in children in developing countries who are unable to reach their protein intake goals, as well as patients with chronic diseases causing malabsorption in the developed countries. 1
The condition typically presents as marked muscle wasting, anasarca, and a desquamating rash. Previous reports have shown its similar features to other nutritional deficiencies. 1
The differential for a diffuse desquamating rash includes Stevens-Johnson syndrome or toxic epidermal necrolysis, bullous pemphigoid, and pemphigus vulgaris. 4 The diagnosis of kwashiorkor can be made with the clinical history of failure to thrive, chronic malabsorption, and appropriate lab work-up.
Blood tests show markedly decreased albumin and total protein, leading to the anasarca and indicating poor protein intake. Skin biopsy shows confluent parakeratosis, pallor of the keratinocytes with vacuolation in the superficial layers, which can be used to identify this condition as a nutritional deficiency. 1
Treatment of kwashiorkor involves identifying the underlying cause and reversing the process. Underlying causes include infectious diseases (as with this patient), genetic diseases such as cystic fibrosis, chronic gastrointestinal conditions such as Crohn disease, anatomic abnormalities, or insufficient protein intake. 1, 5, 6
This case demonstrates kwashiorkor should be considered in patients with characteristic desquamating rash with failure to thrive and chronic illness.
Jason Preissig, MD, is a graduate of Baylor College of Medicine.
Keyan Matinpour, MD, is a dermatology resident at Baylor College of Medicine.
Paul M. Rodriguez-Waitkus MD, PhD, is a dermatopathology fellow at Baylor College of Medicine.
Sylvia Hsu, MD, is a professor of dermatology at Baylor College of Medicine and Chief of the Dermatology Service at Ben Taub Hospital.
References
- Liu T, Howard RM, Mancini AJ, Weston WL, Paller AS, Drolet BA, Esterly NB, Levy ML, Schachner L, Frieden IJ. Arch Dermatol. 2001; 137(5):630-6.
- Brewster DR, Manary MJ, Menzies IS, O’Loughlin EV, Henry RL. Arch Dis Child. 1997;76(3):236-41.
- Alam M, Grossman ME, Longley BJ, Schneiderman PI. Cutis. 2001;67(4):321-4, 327.
- KP, Andea A, Hughey LC. Pediatr Dermatol. 2012; doi: 10.1111/j.1525-1470.2012.01747.x
- Al-Mubarak L, Al-Khenaizan S, Al Goufi T. Eur J Pediatr. 2010; doi: 10.1007/s00431-009-0981-5
- Krikler D, Schrire V. Lancet. 1958;1(7019):510-1.