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LETTER TO EDITOR
Year : 2017  |  Volume : 18  |  Issue : 1  |  Page : 67-69

Acute hemorrhagic edema of infancy: Panicked parents and playful child


1 Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry, India
2 Department of Dermatology, Global Hospital, Chennai, Tamil Nadu, India
3 Department of Pathology, Venereology and Leprology, JIPMER, Puducherry, India

Date of Web Publication12-Dec-2016

Correspondence Address:
Devinder Mohan Thappa
Department of Dermatology, Venereology and Leprology, JIPMER, Puducherry
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.184327

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How to cite this article:
Behera B, Singh N, Thappa DM, Rajesh NG. Acute hemorrhagic edema of infancy: Panicked parents and playful child. Indian J Paediatr Dermatol 2017;18:67-9

How to cite this URL:
Behera B, Singh N, Thappa DM, Rajesh NG. Acute hemorrhagic edema of infancy: Panicked parents and playful child. Indian J Paediatr Dermatol [serial online] 2017 [cited 2019 Aug 21];18:67-9. Available from: http://www.ijpd.in/text.asp?2017/18/1/67/184327



Sir,

A ten-month-old male child was brought to the emergency medical services with complaints of multiple asymptomatic annular erythematous rashes over extremities and trunk for one day duration. The parents gave history of fever, vomiting, and bloody diarrhea five days before the onset of the skin rashes. There was no history of abdominal pain, joint pain or swelling, oliguria or hematuria. The parents were looking very anxious and worried but in contrast, the child was active, playful and feeding well. Cutaneous examination revealed multiple annular purpuric targetoid or medallion-like plaques over the face [Figure 1], bilateral arms, legs, and buttocks [Figure 2]. Bilateral lower extremities showed moderate nonpitting edema. General and systemic examination were within normal limits. Provisional diagnoses of acute hemorrhagic edema of infancy (AHEI) and urticarial vasculitis were considered. The skin biopsy taken from the purpuric rash revealed leukocytoclastic vasculitis [Figure 3], and direct immunofluorescence (DIF) was negative. Thus, we diagnosed the case as AHEI and child was kept under observation since the parents were very apprehensive. In one week, all the skin lesions subsided, and the child was discharged without any complication.
Figure 1: Annular purpuric plaques over face and arm

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Figure 2: Annular purpuric targetoid plaques over buttocks

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Figure 3: Histology of skin biopsy demonstrating leukocytoclastic vasculitis (H and E, ×400)

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AHEI is an uncommon benign, small-vessel leukocytoclastic vasculitis, which mainly affects male children between six months and two years of age.[1],[2]

The exact etiology of AHEI is uncertain. Various triggering factors include viral and bacterial infections especially upper respiratory tract infections, immunization and drugs.[3] In our case, history of fever, vomiting, and bloody diarrhea suggest an episode of prior gastroenteritis.

Clinically, AHEI is characterized by sudden onset of nonpruritic annular purpuric plaques with well-defined borders and central vascular patterns, called as the medallion-like lesions. These ecchymotic or cockade or targetoid lesions mainly affect the extremities, face, and ears and are preceded by tender, asymmetrical edema of the extremities, face and scrotum.[2] The trunk is usually spared and mucosal involvement is rare.[3] It can be associated with constitutional symptoms such as fever and rarely, joint pains, and gastrointestinal symptoms. The general condition of the child is often good despite the explosive nature of the disease.[2],[3]

The clinical mimics of AHEI include hypersensitive angitis, Henoch-Schönlein purpura (HSP), urticaria, urticarial vasculitis, Kawasaki disease, purpura fulminans, Sweet's syndrome, erythema multiforme, meningococcal septicemia, and viral hemorrhagic rashes.[3] The importance lies in differentiating AHEI from HSP as the latter can have organ and life-threatening complications. The scenario is complicated by the fact that both HSP and AHEI can have some similarities like both follow infections, have a seasonal occurrence, are more common in males, and show leukocytoclastic vasculitis on histopathology.[3] The differentiating features between AHEI and HSP include age of the child (3–24 months in AHEI vs. 3–6 years in HSP), skin lesions (medallion-like lesions in AHEI vs. smaller purpuric lesions in HSP), lack of internal organ involvement in AHEI, elevated serum immunoglobulins (IgA) level in HSP, frequent fibrinoid necrosis of vessel wall in HSP, positive DIF staining for IgA and C1q in the vessel wall in HSP, frequent relapses of the disease in HSP and more rapid resolution of the lesions in AHEI.[4] Occasionally IgA deposition in the vessel wall can be positive in AHEI.[5]

The clinical course of the disease is benign which resolves within 2-3 weeks period without any complications.[6] We are reporting this rare case to highlight the importance of recognizing this benign disorder early to avoid unnecessary investigations and medications and to properly counsel the parents to alleviate their anxiety and apprehension.

Declaration of Patient Consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the parents have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The parents understand that their child's names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial Support and Sponsorship

Nil.

Conflicts of Interest

There are no conflicts of interest.

 
  References Top

1.
Caksen H, Odabas D, Kösem M, Arslan S, Oner AF, Atas B, et al. Report of eight infants with acute infantile hemorrhagic edema and review of the literature. J Dermatol 2002;29:290-5.  Back to cited text no. 1
    
2.
Chowdhoury SR, Ganguly S, Mukherjee S, Samanta M, Datta K. Acute hemorrhagic edema of infancy. Indian J Pediatr 2014;81:811-3.  Back to cited text no. 2
    
3.
Dongre A, Adhe V, Kothari D, Kardekar S, Khopkar U. Acute hemorrhagic edema of infancy: A report of two cases. Indian J Dermatol Venereol Leprol 2012;78:121.  Back to cited text no. 3
[PUBMED]  Medknow Journal  
4.
Jindal SR, Kura MM. Acute hemorrhagic edema of infancy-a rare entity. Indian Dermatol Online J 2013;4:106-8.  Back to cited text no. 4
[PUBMED]  Medknow Journal  
5.
Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A. Acute hemorrhagic edema of infancy (AHEI) – A variant of Henoch-Schönlein purpura or a distinct clinical entity? J Allergy Clin Immunol 1990;86(4 Pt 1):473-83.  Back to cited text no. 5
    
6.
Avhad G, Ghuge P, Jerajani H. Acute hemorrhagic edema of infancy. Indian Dermatol Online J 2014;5:356-7.  Back to cited text no. 6
[PUBMED]  Medknow Journal  


    Figures

  [Figure 1], [Figure 2], [Figure 3]



 

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