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A previously healthy, 4-year-old female with unusual skin lesions and mouth sores
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The rash began on her face and spread to the body and extremities in no particular pattern, with simultaneous onset of sores in her mouth. Two weeks earlier, she had a hoarse voice with some fever and was seen by her provider, who diagnosed her with an upper respiratory tract infection. She seemed to be better after about 4 days and remained well until just a few days prior to this visit.
The mother reported that the patient had some fever, but her temperature was not taken until she was seen in the ER, where she was afebrile. She had the clinical appearance of dehydration and was admitted for IV fluid therapy. Her past medical history was that of a previously healthy 4-year-old female with immunizations up to date — none given recently — and on no medications.
Source: James H. Brien, DO
Exam in the hospital revealed normal vital signs, and the patient remained afebrile during her brief hospital stay. She had about 50 discrete, round, erythematous lesions, surrounded by normal-appearing skin. The lesions were of various size, with some having dark-to-black-appearing centers. On close examination, some lesions appeared to have small vesicles in the center. Her mouth and lips were found to have several ulcerative lesions (Figures 1 and 2). Her other mucous membranes, including her eyes, were clear. She improved clinically with IV fluids, with no progression of mouth sores or the skin rash during this hospitalization of only a few days. A herpes simplex virus (HSV) PCR of one of the skin lesions was negative. Her respiratory virus panel was also negative, and her blood work revealed only mild elevation of her white blood cell count, her C-reactive protein and sedimentation rate. Her urinalysis revealed only an elevated specific gravity consistent with her dehydration.
Hint: 8 months later, the patient presented to her primary with a similar episode of stomatitis only, without rash.
What’s your diagnosis?
A. Stevens-Johnson syndrome
B. Disseminated mucocutaneous HSV
C. Erythema multiforme
D. Ecthyma gangrenosum
Case Discussion
The answer confirmed by dermatology was erythema multiforme, or EM (answer C). This condition is characterized by fixed, target-like lesions, often with necrotic centers. Many experts feel that the most common trigger for this condition is infection with HSV. With mouth and lip ulcerative lesions, HSV could very well have been the cause, although EM lesions can appear in this area as well. However, the HSV test was done on a skin lesion, which one would not expect to be positive, as these are reactions to the infection, not a local HSV infection. In cases triggered by HSV infections, recurrence is occasionally seen, which hints that the patient was having an additional episode of stomatitis 8 months later. It should be noted that if there is any question of ocular involvement, an ophthalmologist should be involved early on. Such was not the case with this patient. Treatment is supportive and, if due to HSV, acyclovir should be used.
Stevens-Johnson syndrome (Figure 3) is a type IV, or delayed hypersensitivity reaction, triggered by drugs such as sulfa or infections such as Mycoplasma. It is characterized by skin lesions that may appear as erythematous plaques as well as vesicles of various sizes, and at least two mucous membrane surfaces being involved. Typically, the lips are very inflamed, and the eyes may be as well. In these cases, ophthalmology consult is essential, as this can cause blindness. Treatment is supportive in cases induced by drugs, or if due to Mycoplasma or HSV, treatment should include appropriate antimicrobials.
Disseminated mucocutaneous HSV (Figure 4) is usually seen only in patients with underlying skin disorder such as eczema (eczema herpeticum) or in those with immune deficiencies. They may or may not have mouth involvement, and the diagnosis is usually easy to confirm by sampling any of the newer skin lesions for HSV by PCR. Treatment is with acyclovir, usually intravenously if it is severe enough for admission to the hospital, as well as meticulous attention to skin care of the underlying problem.
Ecthyma gangrenosum is almost always seen in the face of severe underlying immune deficiency, such as in patients on chemotherapy for cancer, and it is by far most commonly a result of Pseudomonas aeruginosa sepsis, with septic emboli causing necrotic vasculitis. These lesions typically began as small, erythematous papules or even vesicles with surrounding erythema but rapidly progress in size to dark lesions consistent with necrosis (Figure 5). This is usually a sign of a life-threatening infection and should be treated very aggressively.
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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.