December 2006

A 3-year-old girl with type 1B glycogen storage disease and multiple associated problems was in the hospital to have a gastric button placed for better nutrition. On the day of admission, she had a fever spike with a white blood cell count of 3,000 and an absolute neutrophil count of 1,500. Her urine analysis was normal, she had some blood and urine cultures obtained, and she started empiric treatment with IV cefepime (a 4th-generation cephalosporin with an enhanced spectrum of activity). With no additional fevers, she underwent the G-button placement two days later. Her postoperative course was complicated with respiratory distress, which kept her in the pediatric intensive care unit for a couple of days. Upon arrival back to the general pediatric ward, a rash was found on the right side of her neck, which rapidly spread and worsened in severity overnight (figure 1).

Examination revealed normal vital signs and a small, 3-year-old girl in no acute distress, with a Port-a-cath that was accessed for fluids and the antibiotic, a massively enlarged liver and the rash on her neck. It was beefy-red with some skin breakdown in the central part of the rash (figure 2), and a maculopapular component extending down the right side of her back as shown in figures 3-4.

What’s Your Treatment?

1. Fluconazole PO (Per Gastric Tube)
2. Clindamycin IV
3. Silver sulfadiazine cream
4. Mupirocin cream

Answer

My answer and recommendation was systemic fluconazole (A), to treat this cutaneous candidiasis.

In fact, this patient had a history of several Candida infections, mostly Candida albicans, involving various mucocutaneous surfaces. That should come as no surprise since it has been known since the early 1980s that Glycogen Storage Disease type 1B is associated with neutropenia and impaired migration of neutrophils (Beaudent AL, et al. Neutropenia and impaired neutrophil migration in type 1B glycogen storage disease. J Pediatr. 1980;97:906-910). This impairment places these patients at increased risk of bacterial and fungal infections. They ultimately get caught in a Catch-22 situation: The frequent use of broad-spectrum antibiotics, like cefepime, sets the stage for opportunistic fungal overgrowth and invasion. All it takes to get started is damage to the skin surface or an area with an inadequate barrier, such as a G-button site. In this case, she had some skin breakdown in the area of the right side of her neck due to moisture. For the same reason, the rash can spread as shown in figures 3-4. This is much like the severe “diaper dermatitis” seen in babies with severe seborrhea, which spreads well beyond the confines of the diaper, as shown in figures 5-6.

Fluconazole is a fairly nontoxic choice that is very effective for mucocutaneous Candida infections. However, one should remain aware that patients who frequently receive antibiotics and antifungal agents might be at risk of being colonized and therefore infected with resistant organisms. If this patient had a history of resistant Candida in her past, it may be more prudent to use IV Amphotericin B, pending culture and sensitivity results. Topical therapy is probably not needed, but it’s hard to resist using some nystatin cream or powder along with the systemic therapy.

Clindamycin is not a bad choice if you think the patient has cellulitis, as it is generally effective against Staphylococcus aureus (even most methicillin-resistant strains) and group A streptococcus. But the visual appearance of the skin in this patient, with its raw, weepy surface, is not characteristic of cellulitis, which is more likely to have a diffuse erythema with varying degrees of swelling under normal-appearing skin, as shown in figure 7, a child with right sided cervical cellulitis.

Silver sulfadiazine cream (Silvadene, Monarch Pharmaceuticals), is a broad-spectrum topical cream that is generally used on burn patients (figure 8, a child with an iron burn to the right side of the neck) to prevent secondary infections, particularly those due to Pseudomonas aeruginosa and group A strep. More about this remarkable product in a future column.

Mupirocin (Bactroban) is also an excellent topical antibiotic with broad-spectrum activity, that has many uses, but a Candida infection is not one of them.

By the way, the patient did well with the Candida clearing up, even though her other problems remain.

Commentary

As I have mentioned in past years, the holiday season is a difficult time for military personnel who are separated from their families, especially if they are deployed overseas. If you are looking for something altruistic to do to help someone less fortunate, consider participating in one of the many care package programs, and send something to someone you’ll probably never see. It could be the best thing that soldier will remember from a very difficult time.

The Brien family wishes you all a wonderful holiday season and a safe and healthy new year. Please keep in touch at jhbrien@aol.com.