Summer 2024 - Vol. 19, No. 2


PHOTO QUIZ FROM URGENT CARE
 
Acute Abdominal Pain Followed by a Rash
 
geissinger

Jared S. Geissinger, PA-C
Penn Medicine Lancaster General Health Urgent Care
 
CASE HISTORY
A 17-year-old male initially presents to Urgent Care for evaluation of a lesion behind his knee with no known trigger or trauma. A single plaque measures approximately 3 mm, but he is otherwise asymptomatic.

Several days later, the patient develops severe abdominal pain with CT-confirmed duodenitis. He then develops a similar but more prominent rash on the lower extremities (see Fig. 1), particularly around the ankles (see Fig. 2).

henoch purpura HSP     henoch purpura HSP
Fig. 1 (left). Several days after a single plaque presents behind the patient’s knee, a more prominent rash develops on the lower extremities.
Fig. 2 (right). Larger areas of rash also apprear around the ankles after several days.

A few areas present with a target appearance; joint pain continues, mainly in the bilateral knees.

These symptoms and the progression of disease lead to a diagnosis of Henoch-Schönlein purpura (HSP).

QUESTIONS
  1. What is the typical age distribution of HSP?
  2. What are the signs and symptoms, as well as the diagnostic criteria, of HSP?
  3. What is the significance of HSP, and what organ is most important to monitor?
  4. What is the treatment for HSP?
ANSWERS
  1. HSP generally occurs in children and teens 3-15 years of age and is seen less commonly in adults.
  2. Signs and symptoms include rash presenting as palpable purpura, which may or may not be itchy; arthralgia; and acute abdominal pain. Diagnostic criteria include purpura (usually palpable and in clusters) or petechiae, with lower limb predominance with or without thrombocytopenia or coagulopathy.
  3. HSP can cause kidney involvement and may lead to kidney deterioration.
  4. Supportive care includes having patients stay well hydrated, and they can use NSAIDs for joint pain and prednisone for more concerning symptoms. Frequent monitoring of kidney function is important via blood pressure measurements, urinalysis, and sometimes bloodwork. A kidney biopsy is warranted in severe cases.
DISCUSSION
HSP is a relatively rare, often self-limiting condition that is almost always associated with skin changes and can have significant implications for kidney health, potentially related to the age of onset.1 Although it most commonly occurs in children aged 3-7 years old and teens with most cases occurring before age 17, HSP can present in adults as well.2-4 A later age of onset is associated with adverse outcomes related to kidney function.

Because of its implications for kidney function, a prompt and accurate diagnosis is important for this condition.5 Supportive care should accompany close monitoring of kidney health.5,6 In this case, the rash was first diagnosed as erythema multiforme, as a few lesions were target-like. Serial trending of kidney function demonstrated deterioration.

This patient was referred to nephrology, and ultimately a kidney biopsy was performed. The skin changes around the ankles worsened, and skin grafts needed to be performed.

Subsequently, the patient fully recovered and is now in good health.

REFERENCES
1. Ozen S, Pistorio A, Iusan SM, et al. EULAR/PRINTO/PRES criteria for Henoch-Schönlein purpura, childhood polyarteritis nodosa, childhood Wegener granulomatosis and childhood Takayasu arteritis: Ankara 2008. Part II: final classification criteria. Ann Rheum Dis. 2010;69(5):798-806.
2. Oni L, Sampath S. Childhood IgA vasculitis (Henoch Schönlein purpura) — advances and knowledge gaps. Front Pediatr. 2019;7:257.
3. Piram M, Mahr A. Epidemiology of immunoglobulin A vasculitis (Henoch-Schönlein): current state of knowledge. Curr Opin Rheumatol. 2013;25(2):171-178.
4. Blanco R, Martínez-Taboada VM, Rodríguez-Valverde V, García-Fuentes M, González-Gay MA. Henoch-Schönlein purpura in adulthood and childhood: two different expressions of the same syndrome. Arthritis Rheum. 1997;40(5):859-864.
5. Ghrahani R, Ledika MA, Sapartini G, Setiabudiawan B. Age of onset as a risk factor of renal involvement in Henoch-Schönlein purpura. Asia Pac Allergy. 2014;4(1):42-47.
6. Saulsbury FT. Henoch-Schönlein purpura in children. Report of 100 patients and review of the literature. Medicine (Baltimore). 1999;78(6):395-409.