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CASE REPORT
Year : 2020  |  Volume : 21  |  Issue : 3  |  Page : 215-217

A rare vasculitis with a dramatic presentation: Acute hemorrhagic edema of infancy


1 Department of Dermatology, Venereology and Leprosy, Maulana Azad Medical College, New Delhi, India
2 Department of Pathology, Maulana Azad Medical College, New Delhi, India

Date of Submission24-Jul-2018
Date of Decision16-Sep-2018
Date of Acceptance18-Mar-2020
Date of Web Publication30-Jun-2020

Correspondence Address:
Dr. Jaspriya Sandhu
Department of Dermatology, Venereology & Leprosy Dayanand Medical College Ludhiana, Punjab 141 001
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijpd.IJPD_84_18

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  Abstract 


Acute hemorrhagic edema of infancy is a disease marked by its dramatic presentation of purpuric plaques in a cockade or targetoid pattern. A 1-year-old boy was brought by his mother to our outpatient department with large purpuric plaques in annular and cockade pattern over the cheeks and bilateral lower limbs. Both the pinna showed purpura in a peculiar reticulate pattern, with vesiculation seen at the margin of the lesions along with mild edema. The skin biopsy showed cutaneous small-vessel vasculitis; direct immunofluorescence showed fibrinogen deposits around the small cutaneous vessels. The unusual features of our case were the reticulate pattern of purpura seen over the ears.

Keywords: Acute hemorrhagic edema of infancy, rare cutaneous vasculitis, reticulate purpura


How to cite this article:
Relhan V, Sandhu J, Garg VK, Khurana N. A rare vasculitis with a dramatic presentation: Acute hemorrhagic edema of infancy. Indian J Paediatr Dermatol 2020;21:215-7

How to cite this URL:
Relhan V, Sandhu J, Garg VK, Khurana N. A rare vasculitis with a dramatic presentation: Acute hemorrhagic edema of infancy. Indian J Paediatr Dermatol [serial online] 2020 [cited 2020 Aug 9];21:215-7. Available from: http://www.ijpd.in/text.asp?2020/21/3/215/288505




  Introduction Top


Vasculitides is a fascinating and esoteric field in medicine with a myriad of clinical features, though sometimes obvious clinically, at times may evade diagnosis. Among children, small vasculitis triggered by drugs, infections, or vaccination is fairly common. One such rare cutaneous-only vasculitis is acute hemorrhagic edema of infancy (AHOI). A disease marked by its dramatic presentation of purpuric plaques in a cockade or targetoid pattern. However, there is a striking dichotomy between the alarming clinical picture and an otherwise healthy child.

Here, we report a case of a 1-year-old boy with AHOI, with an unusual reticulate pattern of purpura over the ears, along with urticarial wheals and purpuric plaques.


  Case Report Top


A 1-year-old boy was brought by his mother to our outpatient department with large purpuric plaques in annular and cockade pattern over the cheeks and bilateral lower limbs [Figure 1]a, [Figure 1]b and [Figure 2]b. Both the pinna showed purpura in a peculiar reticulate pattern, with vesiculation seen at the margin of the lesions along with mild edema [Figure 1]c. Also, seen on the buttocks were multiple erythematous oval plaques, resembling urticarial wheals, ranging in size from 0.5 cm × 0.5 cm to 2 cm × 2 cm [Figure 2]a. The lesions appeared over the span of 48 h and were preceded by an episode of coryza. The child was alert and playful but did not allow examination of the lesions over the ear, possibly due to tenderness because of edema.
Figure 1: (a) Hemorrhagic plaques over pinna with vesiculation seen at the margin of the lesions. (b) Large purpuric plaques in annular and cockade pattern over the cheeks. (c) Pinna showed purpura in a peculiar reticulate pattern

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Figure 2: (a) Multiple erythematous oval plaques, resembling urticarial wheals over buttocks. (b) Purpuric plaques seen over lower limbs

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The routine hematological investigations (complete blood count, kidney function test, liver function test (KFT, LFT), urine microscopy) were within the normal limits, except for mild leukocytosis. The urine examination was within the normal limits.

A skin biopsy with direct immunofluorescence (DIF) was done. The skin biopsy showed cutaneous small-vessel vasculitis [Figure 3]. DIF showed fibrinogen deposits around the small cutaneous vessels.
Figure 3: (a) Skin biopsy showing leukocytic infiltration around vascular bundles in the dermis (H and E, ×200). (b) Higher magnification showing neutrophilic infiltration in the vessel wall (H and E, ×400)

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We made a diagnosis on the basis of the classical clinical picture and histo-pathological grounds.

The child had pain due to the edema and would not allow the ear to be touched, though otherwise well. Although steroids do not alter the course of the disease, the child had severe tenderness due to the edema of the pinna; hence, we gave a short course of steroids (prednisolone 0.5 mg/kg for 7 days) to prevent any damage to the pinna and the underlying cartilage.

There was dramatic improvement a week later, most lesions had disappeared, and only a few purpuric plaques over the left cheek and resolving crusted lesions on the ear remained [Figure 4].
Figure 4: (a) Follow-up at 1-week, purpuric lesions have subsided. (b) Follow-up at 1-week, vesicular lesions over the pinna have resolved with crusting

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  Discussion Top


AHOI was first described in 1913 by Snow, and later in 1938 by Finkelstein. Snow in his first description called it “purpura, urticarial, and angioneurotic edema of the hands and feet in a nursing baby.”[1] It is a rare, rapid onset, usually benign small-vessel cutaneous vasculitis usually seen in children aged<2 years with a slight male preponderance.[2]

The classical description of AHOI includes fever and purpuric plaques, which may be seen in a cockade or targetoid pattern. “Cockade” refers to a round or oval knot of ribbons worn as a badge or as a part of livery, it is derived from the French word coquard, the most famous being the cockade of the French revolution whose colors still remain in the modern-day flag of France (red, blue, and white). Although an uncommon vasculitis, there have been case reports from India [Table 1].
Table 1: Review of Literature: India

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AHOI is often considered to be a cutaneous-only variant of Henoch–Schonlein purpura (HSP), and it is argued whether it is even a distinct clinical entity.[7] IgA deposition on immunofluorescence is the hallmark of HSP, whereas fibrinogen and C3 are more commonly seen in AHOI. In our case, only fibrinogen deposits were seen. In keeping with its clinical course, a more appropriate name of “acute benign LCV of infancy” has been proposed.[7]

Although self-resolving in nature, there are isolated reports of healing with unusual scarring.[8] Due to the severe involvement of ears with tenderness and vesiculation, we gave a short course of steroids to tide over the acute stage of the disease. The unusual features of our case include the reticulate pattern of purpura seen over the ears. Furthermore, along with the classical purpuric lesions were round to oval erythematous plaques, resembling urticarial wheals present over the buttocks and thighs. The trigger for the disease is often an intercurrent infection, vaccine, or drugs seen in almost three-fourth of the cases.[9] In our case, the child had a mild upper respiratory tract infection (URTI), which may have served as a trigger for the disease.


  Conclusion Top


AHOI is a rare cutaneous-only vasculitis seen in children. Its benign course and self-resolving nature makes it even more important to be correctly identified lest it may be overtreated and overinvestigated, adding to the duress of already-worried caregivers.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their 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.
Snow IM. Purpura, urticaria and angioneurotic edema of the hands and feet in a nursing baby. JAMA 1913;61:18-9.  Back to cited text no. 1
    
2.
Fiore E, Rizzi M, Ragazzi M, Vanoni F, Bernasconi M, Bianchetti MG, et al. Acute hemorrhagic edema of young children (cockade purpura and edema): A case series and systematic review. J Am Acad Dermatol 2008;59:684-95.  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]  [Full text]  
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]  [Full text]  
5.
Avhad G, Ghuge P, Jerajani H. Acute hemorrhagic edema of infancy. Indian Dermatol Online J 2014;5:356-7.  Back to cited text no. 5
[PUBMED]  [Full text]  
6.
Roy KP, Madke B, Kar S, Yadav N. Acute Hemorrhagic Edema of Infancy. Indian J Dermatol 2015;60:624-5.  Back to cited text no. 6
[PUBMED]  [Full text]  
7.
Saraclar Y, Tinaztepe K, Adalioǧlu 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;86:473-83.  Back to cited text no. 7
    
8.
AlSufyani MA. Acute hemorrhagic edema of infancy: Unusual scarring and review of the English language literature. Int J Dermatol 2009;48:617-22.  Back to cited text no. 8
    
9.
Karremann M, Jordan AJ, Bell N, Witsch M, Dürken M. Acute hemorrhagic edema of infancy: Report of 4 cases and review of the current literature. Clin Pediatr (Phila) 2009;48:323-6.  Back to cited text no. 9
    


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