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Case Reports
. 2025 Feb 17;17(2):e79179.
doi: 10.7759/cureus.79179. eCollection 2025 Feb.

An Atypical Presentation of Henoch-Schönlein Purpura With Features of Acute Hemorrhagic Edema of Infancy and Koebnerization: A Case Report

Amira Basyouny  1 , Ahmad Imam  2   3 , Kamal A Abouzaid  2
Affiliations
Case Reports

An Atypical Presentation of Henoch-Schönlein Purpura With Features of Acute Hemorrhagic Edema of Infancy and Koebnerization: A Case Report

Amira Basyouny et al. Cureus. .

Abstract

Immunoglobulin A vasculitis, also known as Henoch-Schönlein purpura (HSP), is a type of vasculitis that is most commonly found in children. Palpable purpura is a key feature and is often associated with joint, abdominal, or renal involvement. This case report presents an atypical presentation of HSP in a four-year-old male, characterized by overlapping features of Acute Hemorrhagic Edema of Infancy (AHEI) and Koebnerization (isomorphic response), which complicates the diagnosis. The patient initially presented with redness around his mouth and nose, followed by hematuria and the development of palpable purpura on the lower extremities and buttocks. Vascular swellings were noted on the palms, feet, and face, and cockade purpura appeared on the upper extremities. The patient also exhibited episodic joint pain, genital edema, and Koebnerization on the lower back and buttocks. The diagnosis of this case was challenged by the presence of AHEI-like features and Koebnerization. The patient responded well to treatment with prednisone and colchicine. This atypical case presentation of HSP underscores the clinical overlap between HSP and AHEI, as well as the rare occurrence of Koebnerization in HSP. It emphasizes the importance of recognizing atypical presentations to ensure timely and accurate diagnosis. Further research is required to explore the frequency, pathophysiology, and clinical implications of such cases to enhance diagnostic accuracy and management in pediatric vasculitis.

Keywords: acute hemorrhagic edema of infancy; atypical presentation; dermatology; henoch-schönlein purpura (iga vasculitis); koebnerization; pediatrics.

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Conflict of interest statement

Human subjects: Consent for treatment and open access publication was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work.

Figures

Figure 1
Figure 1. Erythema around the nose and mouth.
Figure 2
Figure 2. Palpable purpura of both lower limbs with signs of erosion and necrosis (black arrow).
Figure 3
Figure 3. Swelling of the forehead, palms, and dorsum of the foot, accompanied by cockade purpura (large, round, and purplish skin lesion with an annular targetoid pattern) (black arrow) in the forearm of both upper limbs and dorsum of the foot.
Figure 4
Figure 4. Redness and edema of the external genitalia.
Figure 5
Figure 5. Koebnerization of the buttocks and lower back region caused by the compression of the elastic band of the clothes on the skin.
See this image and copyright information in PMC

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