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A 1-year-old female with worsening diaper rash
A 1-year-old female presented to her primary provider with a common diaper rash. It was treated in the usual fashion with various creams, including an antifungal cream, and eventually topical steroids. During a period of about 2 months, the rash would wax and wane but never cleared. In fact, it seemed to be somewhat progressive when the primary provider noted that it had become raised with some areas of bleeding. At this point, it was thought to be secondarily infected, and she was admitted to the hospital for further evaluation and treatment.
James H. Brien
Her medical history was positive for several unrelated problems, such as being born prematurely at 27 weeks’ gestation; residual pulmonary disease that was stable, requiring no oxygen; Hirschsprungs disease with surgical repair; and esotropia. Her history was also positive for having atopic dermatitis with frequent use of steroid creams, and she had Down syndrome.
There’s been no trauma to the area and no travel or animal exposure. Her immunizations are up-to-date and she lives with both parents, who are healthy, in an environment with no exposure to toxins.
Figure 1. Examination in the hospital revealed normal vital signs and numerous, raised, dark maroon lesions of various size, in a somewhat linear pattern, covering the entire perineum.
Source: Brien JH
Examination in the hospital revealed normal vital signs and numerous, raised, dark maroon lesions of various size, in a somewhat linear pattern, covering the entire perineum, as shown in Figure 1. Additionally, there was some residue of steroid cream mixed in, and on closer examination, the lesions appeared to have numerous white, pinpoint lesions within each. There was no discharge noted from any of the lesions, but some evidence of bleeding was apparent in the diaper. The surrounding skin and rest of her exam appeared normal.
What’s Your Diagnosis?
A. Hemangioma
B. Langerhans cell histiocytosis
C. Granuloma gluteale infantum
D. Severe Candida diaper dermatitis
Case Discussion
The answer turned out to be C, granuloma gluteale infantum (GGI). A rare condition of unclear etiology, GGI was first reported with a clear description in the early 1970s; however, similar cases may date back to the late 19th century.
In reviewing the literature, one thing is clear: There is no clear underlying cause. GGI was originally known as “vegetating potassium bromide toxic dermatitis” because bromide-containing ointments were used for diaper rashes in the “old days.” The predominance of opinion appears to favor the cause nowadays being the overuse of potent topical steroids, whereas others feel the cause may be related to an unusual reaction to Candida infections. There’s at least one report of lesions containing starch granules, which may explain the tiny white spots seen within the lesions in this case. However, this also has not been a consistent feature. Certainly, corn starch remains a popular home remedy for diaper dermatitis, which may explain its presence here, if that’s what those spots were.
This is a visual diagnosis, in that nothing else looks quite like it. In some respects, it’s like measles; once you have seen a case, you will likely have no trouble recognizing it the next time. In those subjected to biopsy, the lesions consist of a dense, transdermal granulomatous infiltrate of a variety of cells, and tend to have a linear orientation along skin creases. However, biopsy is usually not needed.
Figure 2. Hemangiomas are common in infants and young children. However, they would be unusual in this location, but not unheard of.
Whatever the cause, the course is typically self-limiting and requires no specific treatment. In fact, topical steroids are often used, but some experts say that may actually exacerbate the problem. Our dermatologists recommended using a barrier ointment to protect the skin and an oral antihistamine for itching. If there is evidence of secondary infection with Candida, perhaps oral fluconazole might be tried rather than anything topical.
Frequent diaper changes with good hygiene should go without saying. Considering that this condition does not occur in those not wearing diapers, it is usually self-limiting, as most of us are eventually out of our diapers. But for those who continue to be diaper-dependent, following the above advice should be adequate.
Hemangiomas are common in infants and young children. However, they would be unusual in this location, but not unheard of, as shown in Figure 2. This photo is of a young infant with an unusual lesion that turned out to be a hemangioma in the cleft of the buttock. The fact that there was a lesion there at birth should make one think of a congenial lesion like a hemangioma rather than an acquired problem.
Figures 3 and 4. LCH, a potentially life-threatening infiltrative condition, previously known as histiocytosis X, with several subtypes, may present in the newborn (Figures 3 and 4).
Langerhans cell histiocytosis (LCH), a potentially life-threatening infiltrative condition, previously known as histiocytosis X, with several subtypes, may present in the newborn (Figures 3 and 4) or in the older infant (Figure 5) with an unusual skin rash resembling common diaper dermatitis or eczema at first, but soon becomes apparent that it must be something else because of a lack of response to therapy. This disorder is uncommon, but being increasingly recognized. The diagnosis must be confirmed with a biopsy because the skin manifestations may closely resemble other more common conditions noted above. The more severe variety generally requires chemotherapy similar to cancer patients and is usually followed by oncologists. Fatal cases are usually caused by respiratory disease.
Figures 5 and 6. LCH may present also in the older infant (Figure 5).If not properly managed, especially in the presence of damaged skin, such as atopic dermatitis, for example, it can be fairly severe, as shown in Figure 6.
Candida diaper dermatitis is a common complication of diaper rashes that usually begin as simple cutaneous reactions to irritants in stool and urine. As the skin breaks down, it’s easy for the ubiquitous Candida spores to invade in this warm, moist, nutrient-rich environment. If not properly managed, especially in the presence of damaged skin, such as atopic dermatitis, for example, it can be fairly severe, as shown in Figure 6. However, the beefy-red, moist erythema does not resemble GGI, making differentiating from even severe Candida dermatitis easy.
Columnist Comments
I hope you have a wonderful Thanksgiving, with family all around. Please remember our military personnel who are away from home during the holidays. It’s a special kind of melancholy that they feel at times like these. Please keep in touch and let me know if you have questions or suggestions for this column, or anything else that comes up. I’m always happy to hear from you.
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- James H. Brien, DO, a member of the Infectious Diseases in Children Editorial Board, as well as Vice Chair for Education at The Children’s Hospital at Scott and White, and is the Associate Professor of Pediatrics at Texas A&M University, College of Medicine, Temple, Texas, can be reached at jhbrien@aol.com.
Disclosure: Brien reports no relevant financial disclosures.