July 2004

The guest columnist and contributor for the second of this two-part series is Louis Giangiulio, MD, who is a board-certified pediatrician in the U.S. Army and who recently returned from a tour in Afghanistan. He is now back at Keller Army Community Hospital at West Point, where he is assigned as a general pediatrician.

In Afghanistan, he was attached to the 10th Mountain Division out of Fort Drum, N.Y. Dr. Giangiulio graduated from Jefferson Medical College in Philadelphia in 1999. His residency training was at the National Capital Consortium (Walter Reed Army Medical Center in Washington and the National Naval Medical Center in Bethesda, Md.). In response to my previous requests for input, Dr. Giangiulio documented in pictures several cases of various pediatric infectious diseases. This issue will feature two more brief cases. The hint for both is that they are fairly common in the Middle East.

CASE 2

Case 2 is an 8-year-old girl with a severe, disfiguring mucocutaneous condition that is shown in Figure 2. There is an additional lesion on her upper back as shown in Figure 3. It is unclear how long this had been going on, but apparently it had been quite a while. As can be seen in the figures, examination revealed that she had multiple lentigines with numerous soft tissue masses of various sizes on the face and the large lesion on her back. She was also noted to have severe involvement of her eyes with marked inflammation and associated blindness. A biopsy was obtained from the back lesion and sent to the regional Army medical center in Landstuhl, Germany, for tissue diagnosis. There, the tissue was noted to be teeming with spirochetes. What’s the most likely cause? Bejel Yaws Pinta Tertiary syphilis

Case 1

Case 1 was clinically diagnosed as cutaneous leishmaniasis, sometimes referred to as Delhi boil. So the answer is both C and D. Other synonyms include Baghdad boil, Oriental sore, tropical sore, Aleppo boil or button and others. This is a protozoan infection that is caused by Leishmania tropica and Leishmania major complex in the Middle East and Africa. In the Western Hemisphere, the disease is caused by Leishmania mexicana and Leishmania braziliensis complex. The organism is transmitted by the bite of the female sandfly.

The clinical forms of this disease are divided into cutaneous, mucocutaneous and visceral leishmaniasis. This form of the disease (kala-azar, which is Hindi for black poison or disease) is usually caused by Leishmania donovani complex, although Leishmania infantum has been identified as a cause in young children, especially in the Middle East and coastal regions of the Mediterranean.

The taxonomy of these parasites has become complex and beyond the scope of this column. This is another way of saying, “I’m not smart enough to figure it out,” sort of like trying to help my daughter with her high school math in the late 1980s. For anyone really interested in getting into the details of this, I would recommend any new tropical medicine or infectious disease text, or just “Google” it. There are a lot of very informative Internet sites, some containing pictures.

Dr. Giangiulio also pointed out one U.S. soldier he saw who presented with cutaneous leishmaniasis in Afghanistan. This was similar to the experience I had in 1990-1991 in Saudi Arabia and Iraq. Cutaneous leishmaniasis was a significant problem for those who were not compulsive with their repellent and insect netting during sleep. Three of the soldiers I saw are pictured in Figures 4, 5 and 6. Because of its location, the soldier with the facial lesion had to return to Walter Reed Army Medical Center, where treatment was available.

However, for those with uncomplicated cutaneous lesions, watching for spontaneous healing is reasonable, as was the case for the soldiers with the elbow and forearm lesions. This is usually preferred by these service men and women, as their sense of mission tends to override their concern for treatment. Getting the serviceman with the facial lesion to agree to return to Walter Reed for treatment had to be in the form of an order.

However, sometimes these lesions can continue to grow, resulting in large ulcers as shown in Figures 7 and 8, requiring therapy. Diagnosis of cutaneous lesions is usually made clinically, but it can be confirmed with a biopsy of the leading edge of the ulcer, where the organism can be found. Diagnosis of visceral leishmaniasis is usually made by finding the organism in the peripheral blood or bone marrow. Occasionally, one may need to perform liver or spleen aspirates to recover the organism.

Treatment of potentially disfiguring cutaneous leishmaniasis, mucocutaneous disease or visceral leishmaniasis is still the pentavalent antimonial compound, parenteral sodium stibogluconate. For those not in the military, this drug can be obtained from the CDC. It is usually given for three to four weeks. Liposomal amphotericin B can also be used for the treatment of visceral leishmaniasis, as long as the patient has a normal immune system. Other products, like pentamidine, have been successfully used, but it would be wise to consult an infectious disease specialist for assistance with these patients.

Prevention of leishmaniasis is primarily by avoiding the bite of the sandfly. However, as noted in the hint, there has been a long-standing practice in some areas of the leishmaniasis world where children are intentionally subjected to the bite of an infectious sand fly in an inconspicuous area, like the buttock, to develop a sore, and immunity in the process. This practice probably dates back several centuries. In modern times, the organism (promastigotes) can be recovered in the lab and used to inject under the skin for the same effect.

This is reminiscent of the practice of variolation, where crude attempts were made to prevent or modify the severity of smallpox by using infectious material from a patient with a mild case of the disease. In Asia, where the practice seemed to have been developed, material from dried lesions was blown up the nose of the one wishing to be “protected.” This was later refined in the early 18th century by inoculating some of the liquid from a lesion under the skin of one wishing to be protected. Although this practice could in itself occasionally result in severe or fatal disease, it was at a much lower rate. Through the efforts of Lady Montagu in Europe and Cotton Mather in the United States, this practice was spread widely, resulting in reduction in the morbidity and mortality of smallpox.

Obviously, more modern techniques are used today for both diseases, but not much different. By the way, to read more about the history of these and other vaccines, or just about any topic on vaccines, I would recommend the new (4th) edition of “Vaccines,” by Stanley A. Plotkin, MD, and Walter A. Orenstein, MD. The chapter on parasitic disease vaccines (chapter 48) was written by Peter J. Hotez, MD, PhD, a widely recognized expert in this area, and Jeffrey M. Bethony, PhD, an epidemiologist. Both are from The George Washington University in Washington.

Cutaneous myiasis is usually caused by the female human botfly (Dermatobia hominis), which places her larvae into the patient’s skin with the help of a biting insect, such as a mosquito, to the abdomen of which she has glued her eggs. As the larva matures, it creates a furuncle with a breathing pore (Figures 9 and 10). Most people want these removed as soon as possible, like a week ago. The best way is to sedate the child and remove the worm through a small incision (Figures 11 and 12). As can be seen, there’s little trouble distinguishing myiasis from leishmaniasis.

To have vaccinia, one needs to have received smallpox vaccine. So it’s ruled out by history.

Case 2

The answer to the second case is most consistent with bejel, also known as endemic or nonvenereal syphilis. The cause (Treponema pallidum endemicum) is a very similar spirochete to the one that causes syphilis (Treponema pallidum pallidum). The word bejel comes from the Arabic word bajlah, and the disease is endemic in the Middle East, as well as Africa, central Asia and Australia.

It is usually acquired orally, causing lesions in the mouth, although it can also directly contaminate an area of injured skin. Early lesions progress through stages, beginning with papules that ulcerate and go on to spread through the lymphatics and bloodstream to other sites, including skin and bone. Over time, if untreated, destructive gummatous lesions may develop, as shown in this patient (figures 2 and 3). The treatment is with penicillin G; however, advanced cases may require longer courses of treatment and months to heal. The ocular manifestations of bejel were detailed by Tabbara, al Kaff and Fadel in Ophthalmology (1989;96[7]:1087-1091) and can include uveitis, chorioretinitis and optic atrophy.

The other nonvenereal treponemal diseases, yaws and pinta, are caused by Treponema pallidum pertenue and Treponema carateum, respectively. Like bejel, they are diagnosed by their typical lesions and geographic location.

Yaws is found in warm, humid, tropical regions of Africa, Southeast Asia and equatorial America, whereas pinta is found in warm, arid, tropical climates of the northern aspect of South America and Mexico. Pinta comes from the Spanish word for “painted,” which describes the various colors of the relatively flat lesions at their various stages. Yaws probably dates back at least 1.5 million years, according to the findings of the Archaeological Institute of America. Yaws is known for its destructive lesions of the skin and bones and closely resembles tertiary bejel. Mucosal lesions, however, are rare with yaws.

The late manifestations of syphilis may have destructive gummas, making differentiation difficult. However, there will usually be a history of sexual activity and possibly a genital chancre. Of course, it has a worldwide distribution. Like bejel, all these diseases are best treated with penicillin G.

In closing, I would like to thank Dr. Giangiulio for his service to our country and, on behalf of these most helpless victims of war, thanks for his work as a military pediatrician. Although separated by about 12 years and a couple of borders, our experiences were very similar and mirror all the deployed military pediatricians and other physicians who have gone before and since. Geographic diseases may vary the specific experiences, but the desperation and malnutrition that accompany many of these children are a constant (Figures 13 and 14). Providing them with even momentary expertise might make the difference between living and dying. I’m no behavioral expert, and my predictions are often wrong, but I believe that the child who is rescued from these horrible problems will unlikely grow up to be a suicide bomber 10 or 20 years later and may even convince a few friends not to as well.

At this moment, the pediatric consultant to the Army Surgeon General and chief of pediatrics at Tripler Army Medical Center in Hawaii, Col. Chuck Callahan, is taking his turn with a tour in the Middle East. But we will probably not be aware of the quiet, routine lifesaving work he and all the military physicians will be doing to help the children, therefore chipping away at the foundation of terrorism.

Acknowledgements: Thanks again go to Maj. Phil Chadwick, the brigade surgeon, for his technical assistance with the pictures in Figures 1-3 and electronic transmission. Figure 11 is courtesy of my old dermatology friend and fellow Gulf War veteran Jim Keeling, COL, MC, USA (Retired). Figure 12 is courtesy of Paul Benson, also an Army dermatologist from Walter Reed.

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.
• Louis Giangiulio, MD, is a board-certified pediatrician in the U.S. Army, Keller Army Community Hospital, West Point, N.Y.