Dermatitis, Diarrhea and Alopecia: What Is Your Diagnosis?

History and Clinical Presentation




A 36-year-old black woman presents to the
emergency room with the chief complaint of a desquamating, painful skin
rash around her mouth, abdomen, groin, inner thighs, and buttocks, which
has been present for the past 8-10 days. She also noticed increased
peeling of the skin on her hands and feet. The patient has not been able
to eat normally for a few days because of increasing tongue pain.


History


Eight years ago she had gastric bypass
surgery and has lost more than 230 lb. Since her surgery, she has
suffered from chronic abdominal pain and diarrhea as a result of short
gut syndrome, and she reports worsening of diarrhea for the past 4
months. The patient also complains of chronic, progressive hair loss.


Physical Examination


Physical exam reveals large, red-brown
plaques with desquamating sheets of scale and underlying superficial
erosions and denuded tissue on the patient's buttocks (Figures 1 and 2)
and inner thighs (Figure 3). On oral exam, similar perioral, red-brown
desquamation and smooth, edematous glossitis are seen (Figure 4).
Desquamation is also evident on the palms of her hands (Figure 5) and
soles of her feet. Diffuse, patchy, nonscarring alopecia is present on
the scalp.




Figure 1. Red-brown plaques and desquamation of patient's buttocks.




Figure 2. Red-brown plaques and desquamation of patient's buttocks.




Figure 3. Plaques and desquamation of patient's upper thigh.




Figure 4. Perioral plaques and glossitis.




Figure 5. Palms of hands, showing skin desquamation.



Diagnostic Evaluation



Histopathology


On hematoxylin and eosin (H&E) stain,
atrophic epidermis with palor high and confluent parakeratosis and
alternating orthokeratosis are visualized. The keratinocytes within the
epidermis are relatively normal (Figures 6 and 7).




Figure 6. H&E stain, low magnification (10x).




Figure 7. H&E stain, high magnification (40x).
What is your diagnosis?

• Lichen planus
  
• Mastocytosis
  
• Necrolytic acral erythema
  
• Glucagonoma syndrome
  
• Acquired zinc deficiency
  
• Diagnosis
  
  
  
  Laboratory Studies
  
  
  The patient's glucagon level was within
  normal limits and hepatitis C antibody levels were negative. Measurement
  of serum zinc revealed a level that was below normal limits at 50 μg/dL
  (normal range, 70-150 μg/dL).
  
  
  Discussion: The Missing Zinc
  
  
  Given the clinical, histologic, and
  laboratory findings, the patient was diagnosed with acquired zinc
  deficiency and associated exfoliative dermatitis.
  Zinc is one of the most important elements in
  the human body, mediating the production of lipids, proteins, and
  nucleic acids. It also plays an essential role in normal wound healing,
  reproduction, cardiovascular health, olfaction, and immunity. The
  recommended daily allowance of zinc is 15 mg but can range from 3 to 25
  mg depending on age.^[1-3]
  A wide variety of foods contain zinc. Amounts of zinc contained in food
  are determined by the protein content of the food source. Oysters
  contain higher levels of zinc per serving than any other nutritional
  source. Other excellent sources include beans, whole grains, nuts, green
  leafy vegetables, and dairy products.^[1,2]
  
  Zinc deficiency is divided into acquired and
  genetic forms. Acrodermatitis enteropathica (the genetic form) is
  inherited in an autosomal recessive fashion.^[4] Mutations in the transmembrane zinc transporter protein SLC39A4 on chromosome 8 affect normal intestinal absorption of zinc.^[5,6]
  Children affected by this condition usually present with periorificial
  and acral erythematous, scaly plaques, diarrhea, and alopecia within the
  first few months of life as breast-feeding is discontinued.^[4,7] Secondary infection with Staphylococcus aureus or Candida albicans is also common.^[2]
  
  Acquired zinc deficiency is seen in
  association with a number of conditions affecting either zinc intake or
  absorption (such as high-fiber diets, poor nutritional intake,
  malabsorption syndromes, and gastrointestinal disease) or excessive zinc
  losses, including alcoholism, malignancy, pregnancy, drugs
  (penicillamine), and extensive burns.^[2,3]
  Acquired forms of zinc deficiency can look fairly similar to genetic
  forms upon presentation, with the classic triad of exfoliative
  dermatitis, diarrhea, and alopecia. The condition can be further
  complicated by impaired immunity, poor wound healing, anorexia, or
  delayed puberty.^[1,2,8]
  
  Zinc deficiency is diagnosed by the classic
  triad of clinical features (dermatitis, diarrhea, and alopecia),
  histologic findings (epidermal atrophy and necrosis) and low plasma zinc
  levels. Most patients will have plasma zinc levels below 60 μg/dL.^[1]The differential diagnosis includes glucagonoma and necrolytic acral erythema secondary to hepatitis C infection.^[2]
  After the diagnosis of zinc deficiency is confirmed, zinc should be
  supplemented at a dose of 1-3 mg/kg/day for life. Response to treatment
  is fairly rapid; improvement in cutaneous findings can be seen as early
  as 1-2 weeks.^[1-3]
  
  
  
  
  
  
  Follow-up
  
  
  The lower small bowel absorptive area in this
  patient following her bariatric procedure led to her acquired zinc
  deficiency. She was admitted to the hospital for nutritional support,
  intravenous fluids, and zinc supplementation. Her exfoliative dermatitis
  dramatically improved after only 15 days of zinc supplementation
  (Figure 8).
  
  
  
  
  Figure 8. Improvement in cutaneous findings between presentation (day 1) and almost 3 weeks of treatment (day 20).