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A 4-year-old girl presents with reddish, purple lesions on bilateral lower legs
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A 4-year-old girl without significant past medical history presented to the pediatric dermatology clinic for evaluation of a rash that had been ongoing for the past week. She recently had a few days of cough, rhinorrhea, sore throat and fever that had resolved before the presentation of the rash.
Figure 1. Red-to-purple papules symmetrically distributed over the posterior, dependent surfaces of the lower legs, thighs, and buttocks.
Source: Streicher JL
The patient’s mother first noticed skin lesions on the daughter’s ankles that appeared abruptly and spread quickly to involve her bilateral legs and arms. The patient reported that the skin lesions were itchy and painful. Of note, the patient also had complained of severe ankle pain a few days before presentation. Her mother had taken her to an outside ED where she had X-rays completed, which were normal. The patient had diarrhea, but no abdominal pain, nausea or vomiting. She was finishing a course of amoxicillin that had been started for her sore throat and cough.
On exam, the patient was afebrile and well-appearing. She had red-to-purple papules with central hemorrhagic crusting scattered over her bilateral lower legs extending up to her lower buttocks. She also had similar lesions involving her bilateral extensor arms. There was no joint swelling or hepatosplenomegaly. Her abdomen was nontender to palpation, and no lymphadenopathy was appreciated.
Case Discussion
Henoch-Schönlein purpura (D) is a small-vessel, Ig-A–mediated leukocytoclastic vasculitis that is most commonly seen in children aged 4 to 11 years. The exact etiology is unknown; however, it is hypothesized to be a hypersensitivity reaction that induces vascular damage, most often seen in the setting of a recent viral or bacterial infection. Henoch-Schönlein purpura (HSP) classically presents with palpable purpura distributed over dependent areas, including the bilateral lower legs, buttocks, and extensor arms with systemic involvement including genitourinary, musculoskeletal, gastrointestinal and/or central nervous systems. HSP is the most common form of vasculitis seen in children. Skin involvement is often the first manifestation, but it can be preceded by joint pain or abdominal pain.
Jenna L. Streicher
Marissa J. Perman
The differential diagnosis includes urticarial vasculitis, acute hemorrhagic edema of infancy (AHEI) and Rocky Mountain spotted fever. In contradistinction to HSP, urticarial vasculitis appears as hives (urticaria). However, the hives do not resolve within 24 to 48 hours as typically seen with urticaria. The skin lesions are usually reported to be painful and do not have a predisposition to any particular area on the body nor a symmetrical distribution. Urticarial vasculitis can be seen in patients with autoimmune disorders or paraneoplastic syndromes.
AHEI has many overlapping features with HSP; however, it presents in much younger children from 2 months to 2 years of age. The skin lesions are characterized by larger pink to purple annular or polycyclic plaques that have a target-like appearance and can resemble hives. It usually presents acutely with fever and edema of the face and extremities. Children with AHEI usually do not have systemic involvement.
Rocky Mountain spotted fever presents with petechial macules that are nonpalpable. These lesions usually start on the palms, soles, wrists and ankles and spread inward. Patients often have concomitant headache, fever and malaise. The disease has a rapid progression with high mortality rate so a high level of suspicion is imperative.
This patient was diagnosed with HSP based upon her characteristic skin findings and history of arthralgias. Her urinalysis and blood pressure were normal. The family was counseled about her risk for developing renal involvement and/or gastrointestinal symptoms including symptoms of intussusception.
The patient subsequently developed intermittent abdominal pain and hema-tochezia that self-resolved over 6 to 8 weeks. Urinalysis and blood pressure were repeated weekly while her rash was present and then monthly for 3 to 4 additional months. Fortunately, she did not develop nephritis, which can be seen in 10% to 60% of children with HSP. Her skin lesions resolved over 2 months.
HSP can present with characteristic purpuric, palpable papules distributed over the dependent upper and lower extremities. Practitioners should be aware of the cutaneous findings in order to appropriately counsel families and monitor for other systemic involvement including gastrointestinal and genitourinary symptoms. Patients should be followed closely and referred to nephrology if there is concern for renal involvement.
- References:
- Eichenfield LF and Frieden IJ. Neonatal and Infant Dermatology. 3rd ed. Elsevier; 2015.
- Paller AS and Mancini AJ. Hurwitz Clinical Pediatric Dermatology: A Textbook of Skin Disorders of Childhood and Adolescence. 5th ed. Elsevier; 2016.
- For more information:
- Jenna L. Streicher, MD, is a pediatric dermatology fellow at The Children’s Hospital of Philadelphia. She can be reached at streicherj@email.chop.edu.
- Marissa J. Perman, MD, is an attending physician at The Children’s Hospital of Philadelphia.
Disclosures: Streicher and Perman report no relevant financial disclosures.