March 2004

A 5-week-old infant is admitted to the hospital for evaluation and treatment of a severe rash. The rash began about three weeks earlier (about 2 weeks of age) in the diaper area, subsequently spreading upward. He has received topical therapy with an over-the-counter diaper rash cream without improvement. He has no other problems noted. His activity and appetite are appropriate for his age. His past medical history, including pregnancy, labor and delivery are all unremarkable. His family history is also normal.

Examination revealed an active, alert baby with normal vital signs and a red rash and mouth lesions as shown in figures 1-4. The rash had a moist look in places, with wide-spread desquamation in other areas. As easily seen, the rash is much more intense in the diaper area. He was otherwise normal in appearance. Blood for a CBC showed a white blood cell count of 26,000 with cultures pending. A urinalysis was normal with urine culture pending. A fungal culture of one of the satellite lesions of the skin is also pending.

How Would You Treat?

1. Nafcillin
2. Fluconazole (Diflucan, Pfizer)
3. Prednisone
4. Ampicillin and cefotaxime (Claforan, Aventis)

Answer

This is a fairly severe example of mucocutaneous candidiasis in a baby with seborrhea. The baby was treated with fluconazole (Diflucan, Pfizer) with rapid improvement and went home in a couple of days after blood and urine cultures were negative, and remained well. His skin culture grew a heavy amount of Candida albicans. Many babies have some degree of candidiasis during the initial months of life. However, this baby’s infection was more severe probably due to a combination of the effect of seborrheic dermatitis and inadequate therapy. Simple Candida diaper dermatitis can usually be treated with topical nystatin, ketoconazole or clotrimazole, and oral thrush with nystatin suspension. In difficult cases, some of us “old-timers” were taught to use gentian violet in the mouth. In fact, one of our children had such a persistent Candida diaper rash and thrush, we wondered if her immune system was working right. This was 29 years ago, and the best thing our pediatrician could come up with was a nystatin cream/gentian violet paste for her diaper area and gentian violet along with the nystatin suspension for her thrush. As you can imagine, her appearance was somewhat unusual to friends and family. Invariably, gentian violet ends up going to other places as well, so there’s this child with purple dye all over her. (This pre-dated my addiction to photography; otherwise I would provide a picture). So, I understand the desperation that parents can feel with this stubborn problem that some infants have with Candida. Nowadays, for these stubborn cases, or those that are unusually severe like the one presented, I go right to oral fluconazole per directions in John D. Nelson’s Pocket Book of Pediatric Antimicrobial Therapy.

This is not to be confused with chronic mucocutaneous candidiasis, which is a rare, chronic immune deficiency associated with a T-lymphocyte disorder (figure 5). It is often seen in families and may be associated with Type-1 diabetes or other endocrinopathies. It is treatable with antifungal agents (figure 6), but has no cure, although bone marrow and thymus transplantation have been used successfully in some, but not others.

Nafcillin was not used because there was no evidence of cellulitis, only superficial inflammation. The elevated WBC count was probably secondary to the inflammation caused by the candidiasis. Prednisone was not used because the problem is a fungal infection, and does not need immune modulation, although a topical steroid might be reasonable for the first few days of systemic anti-fungal therapy. The ampicillin and cefotaxime option was not taken in this case, but I would not argue with anyone wanting to treat a young infant with a high WBC count pending culture. But remember, he was alert and afebrile, and we had a good reason for his leukocytosis.

To make things more confusing, the patient returned at 6½ months of age with a bump on his forehead (really, I’m not making this up). He was otherwise well except for mild seborrhea. The Candida had not reoccurred. Plain radiographs of his skull revealed the lytic lesion shown on the left frontal bone in figures 7 & 8.

Now, What’s your diagnosis?

1. Bone cancer
2. Cat scratch disease
3. Langerhan’s cell histiocytosis
4. Benign bone cyst

Answer

The most likely diagnosis under these circumstances would be Langerhan’s cell histiocytosis (LCH), previously known as histiocytosis X, one of the severe forms of proliferative disorders of mononuclear phagocytes. Up to 80% of patients with this disorder have bone lesions and about 50% have seborrhea. They often present with mucocutaneous candidiasis. The best way to diagnosis the condition is with a biopsy, which, in this case showed the patient presented here to have a benign bone cyst.

However, a 3-month-old female did present to our facility with LCH, and initially presented with a persistent rash consistent with candidiasis, but also had some bony abnormalities as shown in figures 9 & 10. The left mandible was essentially missing as well as having several skull lesions. This baby had biopsy-proven LCH and progressed to severe pulmonary disease. These cases are generally treated by oncologists as they frequently require significant immune suppression to get control of this process, usually starting out with prednisone.

Cat scratch disease and cancer can cause bone lesions, but usually not of the skull. However, they should at least be considered.

Postscript: Since I asked for names and contact information of pediatricians deployed to hostile areas with the three branches of the armed services a few months ago, I have received numerous responses. By the time this is published, I will hopefully have collected all the information and have it organized into a report. I would now like to entertain some ideas of what to do with the data. I think the obvious goal would be to learn something from those in these dangerous assignments, but also to try to bring a bit of happiness to their lives while they are there. Also, since these Middle East campaigns appear to be shaping up to be prolonged, with military personnel going on a rotation schedule, the list will need to be periodically updated. So, just e-mail me your thoughts and any additional information at jhbrien@aol.com, and we will incorporate your ideas into a plan. Please keep in touch.

For more information:

• James H. Brien, DO, Pediatric Infectious Disease, Scott and White’s Children’s Health Center and Associate Professor of Pediatrics, Texas A&M University, College of Medicine, Temple, Texas. E-mail: jhbrien@aol.com.