Henoch-Schönlein purpura with epididymo-orchitis: A rare extra-cutaneous manifestation

Abstract

Henoch-Schönlein Purpura (HSP), or IgA vasculitis, is a systemic inflammatory disorder primarily affecting children. While common symptoms include purpura, abdominal pain, and arthritis, testicular involvement is rare. We report a case of a young boy presenting with severe scrotal pain and swelling, later identified as epididymo-orchitis secondary to HSP. Diagnosis was confirmed via Doppler ultrasound, and treatment with corticosteroids and antibiotics resulted in rapid symptom resolution. This case highlights the importance of recognizing rare extra-cutaneous manifestations of HSP and underscores the need for prompt diagnosis and tailored management to achieve favorable outcomes.

Highlights

•

Epididymo-orchitis as a rare manifestation of Henoch-Schönlein Purpura (IgA vasculitis).
•

Doppler ultrasound aids in distinguishing orchitis from testicular torsion in vasculitis.
•

Corticosteroid therapy resolves severe scrotal symptoms and reduces inflammation.
•

Case underscores vigilance for atypical presentations in pediatric systemic vasculitis.
•

Early diagnosis and tailored treatment ensure favorable outcomes in rare IgA vasculitis cases.

1 Introduction

Henoch-Schönlein purpura (HSP), also known as IgA vasculitis (IgAV), is a systemic inflammatory disorder caused by the deposition of immune complexes in the walls of small blood vessels. This leads to inflammation and subsequent leakage of blood. It is the most common cause of vasculitis in the pediatric population, affecting 10 to 20 per 100,000 children per year. The peak incidence of IgAV occurs in children between the ages of 4 and 7, although it can affect individuals of any age, with a slight male predominance.

Numerous risk factors have been linked to the development of IgAV, but the exact mechanisms by which these factors induce the disorder remain unknown. Genetic and environmental factors, such as infections, immunizations, insect bites, foods, and drugs, are believed to elicit this abnormal immune response.

The clinical manifestations of IgAV can vary, but the most common features include skin rash, gastrointestinal tract involvement, arthritis, and IgA nephropathy. Involvement of other organs, such as the central nervous system (CNS), lungs, and testicles, is rare. Testicular involvement in IgAV is relatively uncommon, with an estimated incidence of 1.3 % in male patients.

While the clinical manifestations of IgAV are well-documented, rare complications such as epididymo-orchitis are less frequently reported, potentially leading to delays in diagnosis and management. Documenting cases like this is crucial to expanding the understanding of IgAV’s full clinical spectrum and guiding timely, evidence-based interventions for atypical presentations.

Diagnosis of IgAV is based on the characteristic rash and one or more additional features, such as abdominal pain, arthritis, renal involvement (evidenced by urine analysis), histological evidence of IgA deposition in the glomeruli (as proliferative glomerulonephritis) or blood vessels (as leukocytoclastic vasculitis). Scrotal tenderness, and swelling are often observed on examination, and Doppler ultrasound may reveal decreased blood flow. IgA levels are elevated in up to 40 % of cases. Additionally, patients with scrotal involvement in IgAV tend to have lower serum IgA levels compared to those without this manifestation. Treatment in these cases may be conservative, medical (using steroids), or surgical.

This case underscores the rarity and diagnostic complexity of epididymo-orchitis as a manifestation of HSP. Scrotal involvement occurs in male IgAV patients, and isolated epididymo-orchitis are exceedingly uncommon. This unique presentation adds to the limited pool of cases and highlights the necessity for heightened clinical suspicion when scrotal pain occurs alongside systemic vasculitis symptoms.

2 Case presentation

A previously healthy boy in his early childhood presented with diffuse, colicky abdominal pain, which was followed by a palpable purpuric rash on both lower extremities. The rash began on the dorsum of his feet and gradually spread to his knees and thighs. His family sought medical advice at an outpatient clinic, where he was diagnosed with HSP and managed as an outpatient ( Table 1 ).

Table 1

The child’s initial laboratory results.

	Test	Result	Normal Range
Urine analysis (UA)	Color	Yellow Straw
	Turbidity	Turbid
	RBCs	1–2	0- 5 HPF
	WBCs	1–2	0-5 HPF
	Bacteria	Few	0-10 HPF
	protein	Negative
Hematology	WBC	11.7 ∗10 ³	(5.5–15.5)∗10 ³cell/L
	RBC	4.65	3.9–5.3 cell/L
	НЬ%	11.6	10.5–14 g/dL
	MCV	78.1	70-74 fl
Special Tests	Creatinine	0.43	0.7–1.2 mg/dl
	BUN	12	5–18 mg/dl
	AST	20	0–50 U/L
	ALT	22	0–41 U/L

Two days later, he presented to our emergency department with severe scrotal pain and swelling. This was accompanied by tactile fever, joint pain, swelling, and limping. The patient throat was clear, and there was no history of nausea, vomiting, bloody stool, hematuria, scrotal trauma, or urinary tract infections.

On arrival, the child appeared to be in pain, but his vital signs were within normal ranges. Physical examination revealed a petechial and purpuric rash diffusely distributed over both feet, extending to the knees and thighs. The rash was palpable, maculopapular, polymorphic, non-blanchable, and red to purple in color. His abdomen was soft and non-tender, as demonstrated in Fig. 1 . His chest was clear on auscultation, with good bilateral air entry and no additional sounds.

Genital examination showed an erythematous and edematous scrotal wall that was tender to palpation. The pain was not alleviated by scrotal elevation, as shown in Fig. 2 . Doppler ultrasound of the scrotum revealed an enlarged left epididymis and spermatic cord, both of which were edematous and heterogeneous with increased vascularity on color Doppler. Additionally, there was slightly increased vascularity in the left testicle with a surrounding mild to moderate reactionary hydrocele. The scrotal wall was markedly edematous. The right testicle was located in the inguinal canal and appeared normal in size, shape, and echotexture. These findings were consistent with left-sided epididymitis-funiculitis with possible early orchitis.