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Inguinal adenopathy with rash in a 6-year-old febrile female
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A 6-year-old female is admitted from the ED for evaluation and treatment of a febrile illness with a rash. The history of her chief complaint began 8 days earlier when she noted a mildly painful lump in her right inguinal area. Less than 48 hours later, she noted the onset of a rash that first appeared in the same area as the right inguinal lump, but rapidly spread in every direction.
James H. Brien
She was evaluated by her primary provider the next day, who did a complete blood count, finding her white blood cell count to be 18,000, and gave her a prescription for Keflex (cephalexin, Shionogi) and a dermatology consult, which was for 5 days later. During this interval, she continued to have low-grade fevers, nasal congestion and persistence of the rash and inguinal mass, although it did not change in size or significance. Upon being seen in the dermatology clinic, she was directed to the ED for evaluation and admission.
A rash that had a scaly appearance (Figure 1) was also noted which consisted of widely scattered discrete lesions that are hyperpigmented, papular and macular lesions. Some of these also had some scale on them (Figures 2-4).
Source: Brien JH
Except for some mild nasal congestion at the onset, she had no other complaints, such as nausea, vomiting, diarrhea, headache, cough, muscle or joint pains, and her past medical history is unremarkable. She has had no recent travel or insect exposure, there have been no known sick contacts and her immunizations are up to date.
Her review of systems is positive only for the chief complaint. She has not taken any prescription medications recently or on a regular basis. However, her parent did give her some baby aspirin and a decongestant for the fever and congestion before the onset of the rash.
Her lips were also inflamed with some bleeding (Figure 5), and her palms and soles had numerous discrete macular lesions that were initially described to be petechiae (Figure 6).
Examination revealed normal vital signs, with the only positive findings being a 4 x 4 cm, firm mass in the right inguinal area that was nonfluctuant, and had minimal pain on firm palpation. A rash that had a scaly appearance (Figure 1) was also noted which consisted of widely scattered discrete lesions that are hyperpigmented, papular and macular lesions. Some of these also had some scale on them (Figures 2-4). Her lips were also inflamed with some bleeding (Figure 5), and her palms and soles had numerous discrete macular lesions that were initially described to be petechiae (Figures 6 and 7).
The patient's palms and soles had numerous discrete macular lesions that were initially described to be petechiae (Figure 7).
Lab tests performed on admission included a complete blood count, erythrocyte sedimentation rate, complete metabolic profile, coagulation studies and urine analysis, all of which were normal. Pending tests include cultures of blood and urine, Rickettsial titers and a skin lesion biopsy.
Case Discussion
The lesions on the palms and soles caused some initial confusion until it was made clear that they were not petechiae, but rather macular lesions — “fixed” annular targetlike lesions with some central necrosis — that were consistent with erythema multiforme (A).
The fixed lesions of erythema multiforme will persist for a few days to more than a week. The diagnosis in this case was confirmed by the skin biopsy, revealing “central necrosis surrounded by a rim of perivenular inflammation or vacuolar interface dermatitis,” with the interpretation being consistent with erythema multiforme. While it is very uncommon, aspirin can trigger erythema multiforme (which was the dermatologist’s best guess), but so could the ingredients in the decongestant, or more likely, the virus causing the febrile illness, or undetermined cause of the swelling of the right inguinal node. It could be that the node was an unrelated finding in that it never enlarged further, and resolved without further complications.
While the actual cause in this case will remain a mystery, erythema multiforme is known to be triggered by a wide range of infectious agents, including mycoplasma and, most commonly, herpes simplex virus (HSV) infections. Because of the appearance of her lips, a couple of suspicious lesions in the right lower lip area were tested by PCR for HSV, but came back negative.
We should not miss the opportunity to point out that no child should be given aspirin, or any other salicylate, for any illness unless specifically indicated, such as in Kawasaki disease. The risk is low, but there is an association with the use of aspirin — especially with influenza and varicella — and the development of Reye syndrome, a severe hepatic encephalopathy syndrome with a high mortality due to brain swelling. This condition has all but disappeared over the last few decades.
In years past, this condition might be called erythema multiforme minor (with only one mucous membrane area being inflamed), as opposed to erythema multiforme major (those with at least two mucous membranes involved), which many experts considered synonymous with Stevens-Johnson syndrome. However, this classification is no longer used by most academic dermatologists, in favor of going back to the older terms of E. multiforme for erythema multiforme minor, and Stevens-Johnson syndrome in place of erythema multiforme major.
Children with Stevens-Johnson syndrome almost always have a mixed rash of macules, papules and vesicles (Figure 8), with inflammation of the eyes and/or lips (Figure 9).
Stevens-Johnson syndrome was first described in 1922 by Albert M. Stevens (1884–1945) and Frank C. Johnson (1894–1934), two pediatricians from the Children’s Medical Division at Columbia University and Bellevue Hospital in New York, who reported two males, aged 7 and 8 years, with excellent black and white pictures of each, in the American Journal of Diseases of Children 24(6): 526-33. Many experts now believe it to be a milder form of toxic epidermal necrolysis (TEN), a severe rash that results in the complete separation of the epidermis from the dermis due to a complex immune-mediated reaction.
Children with Stevens-Johnson syndrome almost always have a mixed rash of macules, papules and vesicles (Figure 8), with inflammation of the eyes and/or lips (Figures 9 and 10, showing the progression from admission and about 72 hours later). The mouth sores are very painful, impairing the patient’s ability to eat or drink, which is often the cause for admission. The inflammation of the eyes can result in permanent damage, including blindness, and should always be co-managed by an ophthalmologist. If Stevens-Johnson syndrome is in the differential of a rash presentation, beware of rapid progression to TEN, which can be fatal without proper intensive care. Normally, TEN can be diagnosed at presentation, because the progression is fairly rapid; at least, that’s the experience with all the cases I have seen.
Children with Stevens-Johnson syndrome almost always have inflammation of the eyes and/or lips (Figure 10).
Erythema multiforme is often confused with annular or giant urticaria; a benign cutaneous hypersensitivity reaction, often referred to as urticaria multiforme, but usually easy to distinguish, as urticaria has transient lesions, that change from one day to the next, and without central necrosis, but rather pale or dusky centers (Figure 11). I, personally, find the term “urticaria multiforme” less desirable, as the nomenclature can be confused with erythema multiforme, and these conditions are confusing enough already; but then I’m fairly simple-minded.
There was initial concern for possible Rocky Mountain spotted fever (RMSF); so much so that the patient was empirically treated with doxycycline for 7 days. However, in looking back, with the advantage of retrospective 20/20 vision, RMSF would be very unusual to present as this child did.
Patients with RMSF typically have fever, which this child did not have around the time of admission. Also, RMSF patients almost always complain of headache, along with other systemic complaints, including generalized aches and pains, nausea, vomiting, diarrhea and abdominal pain. The rash is typically maculopapular with petechiae that starts on the distal extremities around the wrists and ankles, then rapidly spreads to the rest of the body (Figure 12, courtesy of the James W. Bass, MD, collection). RMSF patients often have thrombocytopenia, anemia, and leukopenia and they may have abnormal coagulation studies.
Erythema multiforme is often confused with annular or giant urticaria; a benign cutaneous hypersensitivity reaction, often referred to as urticaria multiforme, but usually easy to distinguish, as urticaria has transient lesions, that change from one day to the next, and without central necrosis, but rather pale or dusky centers (Figure 11). The rash is typically maculopapular with petechiae that starts on the distal extremities around the wrists and ankles, then rapidly spreads to the rest of the body (Figure 12, courtesy of the James W. Bass, MD, collection).
With the exception of the rash on the palms and soles, none of these other findings was present in this patient. Additionally, RMSF is very unusual in central Texas, but not unheard of, especially with the high mobility of our population. I have 26 years of practice in various parts of Texas and have not seen a documented case here. The two cases I have seen consisted of one case when I was in the Army stationed in Washington, D.C., and a second case in Hawaii, in a military-dependent child who became infected just prior to moving with his family from North Carolina. He had been apparently incubating the illness as RMSF is not otherwise found in Hawaii.
It is not unusual for erythema multiforme and Stevens-Johnson syndrome patients to have palmar and plantar involvement of the rash, making it appropriate to include RMSF in the differential. However, the serology for Rickettsial antibodies was negative, and the skin biopsy confirmed erythema multiforme. Having said all this, one should not wait to “confirm” the diagnosis of RMSF to initiate therapy, as a delay in treatment can result in worsening disease, with increased morbidity and mortality.
Lastly, the treatment of erythema multiforme is generally symptomatic with perhaps antihistamines and topical therapy with wet compresses. If evidence of an infectious agent is found, then, of course, treatment can be directed against it. These patients often end up being treated with steroids; however, there are no evidence-based studies of which I am aware that show benefit. Please let me know if you know of a good, scientific study to the contrary.
For more information:
James H. Brien, DO, is with the department of infectious diseases at McLane Children’s Hospital, Baylor Scott & White Health, Texas A&M College of Medicine in Temple, Texas. He also is a member of the Infectious Diseases in Children Editorial Board. Brien can be reached at: jhbrien@aol.com.
Disclosure: Brien reports no relevant financial disclosures.