Girl presents with rapidly spreading rash, abdominal pain and fever
What’s your diagnosis?
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During the winter holidays, a 12-year-old girl was seen in her primary care clinic with fever, fatigue, cough and sore throat. At that time, she was thought to likely have strep throat, but her rapid strep test was negative.
Figure 1. Lesions on right ear. Image: James H. Brien, DO.However, since cases of group A streptococcal infections had been spiking in the community, she was prescribed amoxicillin anyway. Two days later, she presented with mild abdominal pain and a few scattered mixed urticarial-like lesions with a purpuric component localized on her left arm and wrist. She was advised to continue the amoxicillin and return if it worsened.
Figure 2. Lesions on left cheek. Image: James H. Brien, DO.Over the next 2 days, as her fever and abdominal pain continued, the palpable rash rapidly spread to her right arm and wrist, both legs, ankles and feet, with numerous scattered lesions about the trunk, face and ears.
Figure 3. Lesions on left lower leg. Image: James H. Brien, DO.She also developed some painful swelling of her wrists, knees, ankles and smaller joints of her feet and fingers. She denied any other pain or symptoms, including headache.
Figure 4. Lesions on swollen left knee. Image: James H. Brien, DO.Due to the progression of her illness and the diagnostic uncertainty, she was admitted to the local hospital for further evaluation. Her admitting vital signs revealed a temperature of 101.1°F/38.4°F, with other vital signs being normal. Her exam confirmed the findings noted earlier (Figures 1 to 7). Antimicrobials were held pending more information.
Figure 5. Swelling of left ankle and feet. Image: James H. Brien, DO.Her Initial laboratory workup included a normal complete metabolic profile and negative Epstein-Barr virus panel and antistreptolysin O titer. Abnormal results included moderately elevated inflammatory markers (erythrocyte sedimentation rate and C-reactive protein) and white blood cell count on the complete blood count test. Also, her urinalysis showed some microscopic hematuria and small amount of protein. Subsequently, her respiratory viral panel returned positive for respiratory syncytial virus. With no evidence implicating a bacterial infection, the antibiotics were held and attention was turned to other possibilities, such as allergic reaction, or a toxin or immune-mediated rash-producing illness.
Figure 6. Lesions and swelling of left foot. Image: James H. Brien, DO.Her family history is positive for a younger brother who had some mild respiratory symptoms 2 weeks earlier but is well now. Her other family members are well. Her only travel history is a recent skiing vacation in Colorado, returning 10 days prior to the illness. Her animal exposure includes a healthy family dog and cat.
Figure 7. Mild swelling of left wrist and joints of both hands. Image: James H. Brien, DO.What’s your diagnosis (what positive test would you predict)?
A. Coronary artery ectasia
B. Intracellular gram-negative coccobacilli
C. Intracellular gram-negative diplococci
D. Small vessel IgA vasculitis
Answer and discussion:
The best answer is D, small vessel IgA vasculitis, the underlying pathology of Henoch-Schönlein purpura (HSP). The clinical description in this case is not unusual, and the trigger is likely the RSV detected on the respiratory virus panel, which causes an immune response that produces IgA immune complexes that deposit on the small vessels of the skin, joints, kidneys, the gastrointestinal tract and rarely the central nervous system.
Over the last 24 years, I have shown two other cases of HSP (September 2000 and December 2018). As in this case, they presented in classic fashion, usually triggered by a virus or other infectious agent, including group A strep. However, HSP is uncommon, with approximately 10 cases per 100,000 per year, but there may be more cases because they are likely misdiagnosed. The majority are between ages of 3 to 16 years, with the characteristic features noted below:
- abdominal pain;
- arthralgia and/or arthritis;
- palpable purpura without thrombocytopenia or coagulopathy;
- renal disease; and
- rarely, CNS involvement.
The diagnosis is usually made clinically by recognition of the rash and other features. But in less obvious cases, a biopsy of a lesion may be needed, which will reveal the typical IgA small vessel vasculitis on immunofluorescence. Rarely, the course can be complicated by renal insufficiency or prolonged abdominal pain. Corticosteroids have been successfully used in those with significant abdominal pain. The intestinal involvement may result in intussusception, which should also be considered in those cases of severe, intractable pain. Steroids may also be helpful in renal involvement, but management of renal insufficiency should be done in consultation with a nephrologist.
Regarding the other choices:
Coronary artery ectasia (the pre-aneurysmal dilation of a coronary artery by cardiac ultrasound or catheterization) might be seen with Kawasaki disease, which would present with at least 5 days of fever and the characteristic mucocutaneous findings of a polymorphous rash (Figure 8) with mucous membrane inflammation, including the bulbar conjunctiva, lymph node swelling and edema of the hands and feet in a much younger child.
Figure 8. Polymorphous rash of Kawasaki disease. Image: James H. Brien, DO.Intracellular gram-negative coccobacilli, characteristic of Rickettsia rickettsii — the cause of Rocky Mountain spotted fever (RMSF) — would be seen on immunohistochemical staining on biopsy of a skin lesion. The rash of RMSF, however, tends to consist of small maculopapular and petechial lesions that initially appear about the wrists and ankles then spread (Figure 9). Additionally, severe headache is almost always one of the chief complaints, which was missing in this patient. Lastly, even though the family had been skiing in Colorado, the vector (dog tick and wood tick) is very uncommon in the Rocky Mountains, and even if they were there, they would not be very active in the winter.
Figure 9. Characteristic lesions of Rocky Mountain spotted fever. Image: James W. Bass, MD, MPH.Intracellular gram-negative diplococci should suggest sepsis with Neisseria meningitidis (meningococcemia). These children are typically very sick with sepsis and multiorgan insufficiency. The rash can initially resemble a measles rash that progresses to include petechiae and subsequent purpura (Figure 10).
A very nice summary-type reference is Pediatric Dermatology, A Quick Reference Guide, 4th Edition, by Anthony J. Mancini, MD, and Daniel P. Krowchuk, MD, published by the American Academy of Pediatrics in 2021. Also, you may find my older cases noted earlier to be useful for teaching.
Figure 10. Purpura associated with meningococcal sepsis. Image: James H. Brien, DO.Columnist comments:
This is my 400th column since beginning with Infectious Diseases in Childrenin April of 1989. I initially began writing these columns in Infectious Disease News, a “sister publication” in November 1988, but I do not count those. I have had a few breaks in the column; when I was deployed to the Gulf War in 1990-1991, then when I was assigned to the Office of the Army Surgeon General in 1995 and again when I retired from the Army and started working at Scott & White (now Baylor Scott & White Health) in June 1997. These breaks turned out to be brief, and after 35 years, I have only missed writing 20 columns, which has been uninterrupted since September 1997. I still have a lot of material and pictures, but how much longer?
I may perceive these little vignettes to be more useful than they really are, and I would hate to waste your time (and mine) if they are not measuring up. So, please let me know if you see opportunities for improvement. Also, cases submitted by readers are always welcome, and it adds some more variety to the routine. So please feel free to let me know if you have a case you would like to see in this column, and we will try our best to make it happen.
References:
Mancini AJ, Krowchuk DP, eds. Pediatric Dermatology, A Quick Reference Guide. 4th ed. American Academy of Pediatrics; 2021.
For more information:
Brien is a member of the Healio Pediatrics Peer Perspective Board and an adjunct professor of pediatric infectious diseases at McLane Children's Hospital, Baylor Scott & White Health, in Temple, Texas. He can be reached at jhbrien@aol.com.