Case Report: Acute hemorrhagic edema of infancy (Seidlmayer purpura) – a dramatic presentation for a benign disease

Abstract

We present a case of an 11-month-old girl who was referred to our unit for an erythematous rash that appeared on the face and extremities. Personal and family history was not relevant. Laboratory tests were normal. During recovery, diameter and colour intensity of the cutaneous lesions increased, but after some weeks, lesions had a self-limited resolution without any treatment. Based on clinical and laboratory findings, a diagnosis of acute hemorrhagic edema of infancy (AHEI) was made. AHEI is a rare cutaneous leukocytoclastic vasculitis that usually affects children aged between 4 and 24 months. Etiology is unknown but almost of 75% of cases are preceded by infectious episodes, vaccinations or use of medications. In contrast to the dramatic cutaneous eruption, clinical conditions are usually optimal. Classically, AHEI is characterized by a triad of symptoms: fever, edema and purpura. Skin lesions are erythematous, annular, medallion-like, purpuric plaques that have a rapid onset and appear on the face and extremities, sparing trunk and mucosal membranes. Initially interpreted as a variant of Henoch-Schönlein purpura, now it is considered a distinct disease. In the majority of cases the disease is benign and self-limited without a visceral involvement, so a conservative approach is most often chosen.PubMed Abstract | Publisher Full Text 3.Homme JL, Block JM: Acute hemorrhagic edema of infancy and common mimics. Am J Emerg Med. 2016; 34(5): 936.e3-6. PubMed Abstract | Publisher Full Text 4. Cunha DF, Darcie AL, Benevides GN, et al.: Acute Hemorrhagic Edema of Infancy: an unusual diagnosis for the general pediatrician. Autopsy Case Rep. 2015; 5(3): 37-41. PubMed Abstract | Publisher Full Text | Free Full Text 5. Fotis L, Nikorelou S, Lariou MS, et al.: Acute hemorrhagic edema of infancy: a frightening but benign disease. Clin Pediatr (Phila). 2012; 51(4): 391-3. PubMed Abstract | Publisher Full Text 6. Miorin E, Meneghini A, Don B, et al.: Edema emorragico acuto del lattante, descrizione di un caso clinico e revisione della letteratura. Medico e Bambino pagine elettroniche. 2002; 5(3). Reference Source 7. Alhammadi AH, Adel A, Hendaus MA: Acute hemorrhagic edema of infancy: a worrisome presentation, but benign course. Clin Cosmet Investig Dermatol. 2013; 6: 197-9. PubMed Abstract | Publisher Full Text | Free Full Text 8. Krause I, Lazarov A, Rachmel A, et al.: Acute haemorrhagic oedema of infancy, a benign variant of leucocytoclastic vasculitis. Acta Paediatr. 1996; 85(1): 114-7. PubMed Abstract | Publisher Full Text 9. Savino F, Lupica MM, Tarasco V, et al.: Acute hemorrhagic edema of infancy: a troubling cutaneous presentation with a self-limiting course. Pediatr Dermatol. 2013; 30(6): e149-52. PubMed Abstract | Publisher Full Text 10. Tagliabue A, Bettinelli A, Cogliati F: Edema acuto emorragico della prima infanzia (porpora di seidlmayer). Medico e Bambino pagine elettroniche. 2009; 12(6). Reference Source

Köbner and Pastia Signs in Acute Hemorrhagic Edema of Young Children: Systematic Literature Review

“…The Köbner sign occurred in a total of 32 cases, comprised of 21 males and 11 females 11 [ 8 , 9 , 10 , 11 , 12 , 13 , 14 , 15 , 16 , 17 ] months of age. A total of 42 Köbner signs ( Table 2 ) were documented in the mentioned 32 cases [ 14 , 21 , 23 , 24 , 27 , 31 , 37 , 40 , 43 , 45 , 47 , 49 , 50 , 54 , 59 , 60 , 61 , 63 , 65 , 66 , 67 , 69 , 72 , 78 , 79 , 80 , 81 , 83 , 84 , 85 , 86 , 89 ]. Lower legs, thighs, waistline, and groins were the most affected regions.…”

Acute hemorrhagic edema of infancy (AHEI): Alarming cutaneous presentation of a benign and self‐limited disease

“…19 Despite the rapid progression of lesions, AHEI is a self-limited disease with spontaneous and complete resolution, often occurring within 1-3 weeks. 20 Affected patients rarely have recurrent attacks and complications, like scarring lesions as well as renal, gastrointestinal, or joint involvement. AHEI has no specific treatment, and conservative management is the most common approach.…”

Acute haemorrhagic oedema‐like eruption in an adult

“…Genetic factors have been investigated due to family-based investigations, but genetic studies revealed no role for HLA-B27, HLA-Cw6 or HLA-DR, PSTPIP2, LPIN2 and NOD2. 1,2 Variants rs10889677, rs2201841, rs7517847 of IL-23R and rs2243248 of IL-4 showed strong associations with SAPHO syndrome. 3 Our results for rs7517847 of IL-23R and rs2243248 of IL-4 were similar to Guo's results.…”

“…AHE, also known as Seidlmayer purpura, is a rare and benign leukocytoclastic vasculitis, typically described in young children and usually triggered by infections, drugs or vaccination. 1 The main clinical characteristics are symmetrically purpuric medallion-or cockade-shaped patches, associated with painful oedema and predominantly located on cephalic extremity or limbs. Although cutaneous presentation may be dramatic, in children, disease remains non-toxic, visceral involvement is rare and the disease is self-remitting within a few days.…”