HISTORY
Chief Complaint: Rash on Legs and Buttocks
A 5-year-old African American girl was seen through a telemedicine visit for concerns for Henoch-Schonlein purpura (HSP). The patient had a 4-day history of violaceous and erythematous pruritic papules on bilateral legs, buttocks, and elbows and a few scattered on her ears, chest, and abdomen. She also had a 2-day history of lower extremity arthralgias, abdominal pain, and a recent history of upper respiratory infection.
* Prior treatment: none
* Prior biopsy: none
* Skin history: no history of similar rashes; no history of skin disease
* Laboratory findings: none
IMAGE QUALITY ASSESSMENT
Fully satisfactory.
TELEDERMATOLOGY IMAGING READER REPORT1
A high-quality image was provided for this case. The image showed distinct and multiple palpable purpura on the dorsal surface of lower extremities and buttocks bilaterally (Figure 1).
INTERPRETATION OF IMAGES
The morphology and distribution of the lesions, as well as the history of present illness, are highly suggestive of HSP.
RECOMMENDATIONS
* Skin care and treatment recommendations: gentle skin care and moist wound care for any open or crusted papules.
* Education recommendations: Provide education about HSP and its clinical course including waxing and waning nature as well as continued observation for systemic findings.
* Medication recommendations: Acetaminophen and NSAIDs as needed for arthralgias.
RECOMMENDATION TO FOLLOW-UP
* Type of visit: primary care provider for ongoing monitoring of skin disease as well as for any increasing severity of gastroenterology symptoms and changes in urine color. Consider referral to nephrology if needed.
CLINICAL PEARL
HSP is a small-vessel vasculitis of early childhood that manifests as palpable purpura, arthralgias, gastrointestinal symptoms, and renal complications. The immunopathogenesis of HSP is related to the IgA1 subclass, and IgA and IgA-containing circulating immunocomplexes are elevated in the serum of patients with HSP (Habif, 2016; Heineke et al., 2017). HSP is diagnosed clinically in patients with palpable purpura or petechiae (high prevalence in the lower limbs) and one of the four following features: arthritis or arthralgias; abdominal pain; leukoclastic vasculitis with deposition of IgA immune complex on histology; and renal involvement including hematuria, proteinuria, and red blood cell casts (Linskey, Kroshinsky, Mihm, & Hoang, 2012; Trnka, 2013). Renal biopsy is not necessary to make the diagnosis. It is important to monitor kidney function in patients with HSP as the long-term morbidity with HSP is associated with nephritis (Trnka, 2013). Milder nephritis is commonly seen in 20%-50% of patients with HSP and resolves without any treatment (Habif, 2016). However, less than 2% of patients with HSP develop serious kidney complications (Hahn, Hodson, Willis, & Craig, 2015). Gastrointestinal complications may also occur, specifically intussusception, and abdominal ultrasound is indicated in cases of severe abdominal pain.
Management of HSP involves supportive care, symptomatic treatment, and, in rare cases, immune suppression. Immunosuppressive treatments are reserved for patients with severe abdominal pain or nephritis and limiting arthralgias. Prednisone is helpful in shortening the course of mild HSP nephritis, joint symptoms, and severe abdominal pain (Ronkainen et al., 2006). Currently, it is not recommended to treat patients with HSP prophylactically with steroids or antiplatelet agents to prevent the development of HSP nephritis (Habif, 2016). In addition, there is lack of evidence to recommend cyclophosphamide, cyclosporine, or mycophenolate mofetil in most patients with HSP (Hahn et al., 2015). There is a high likelihood (50%) of recurrence of HSP within the first 3 months. However, the overall prognosis is excellent for children and adults with HSP (Habif, 2016). Adult women with a history of childhood HSP are at an increased risk for preeclampsia during pregnancy. Children and young adults with HSP nephritis should get a continued long-term follow-up to monitor for kidney disease, especially during pregnancy (Habif, 2016).
REFERENCES