Rasa MEDOVIC, Marija MEDOVIC, Zoran IGRUTINOVIC, Gordana KOSTIC, Biljana VULETIC, Slavica MARKOVIC, Aleksandar VLAHOVIC, Gordana RISTIC

Acute hemorrhagic edema of infancy – is it really a mild, benign disease?

Background. Acute hemorrhagic edema of infancy (AHEI) is a rare vasculitis, which presents dramatically as palpable purpuric skin lesions on the limb, face and auricles, with swelling of these parts and low-grade fever, in children up to 2 years of age. To date, approximately 400 cases have been described in the literature. The etiology remains mostly unknown. With or without treatment, AHEI goes to spontaneous recovery within 1-3 weeks, usually without any complications. To our knowledge, compartment syndrome as complication of AHEI has only been reported in one case. We present an unusual case of AHEI with serious complications due to compartment syndrome of the right-hand fingers. Case. A 16–month–old male child presented with fever and sudden appearance and rapid spread of palpable, painless, non–itching ecchymotic hematomas on the thigh, cheeks, earlobes, forearms, dorsum of hands and feet, with mild edema of these regions. Complete systemic examination and all vital parameters were normal for age. There was no history of bleeding disorders in the family. Except low hemoglobin on complete blood count and increased D-dimer values, all other laboratory investigations were in the normal range. Changes on the right forearm and hand expanded on almost the entire dorsal side and all surfaces of the fingers, with pronounced swelling and formation of bullous lesions, which were spreading and cracking. Skin biopsy confirmed nonspecific small–vessel vasculitis. That required the use of Methylprednisolone, low-molecular weight heparin, antibiotics and debridement of necrotic eschar, with necrectomy of the affected fingers. Conclusions. Early recognition of AHEI is important to avoid unnecessary investigation and therapy. On the other hand, our reported case warns that unexpected complications may occur.

PDF

___

1. Arnold KA, Gao J, Stein SL. A review of cutaneous hypersensitivity reactions in infants: from common to concerning. Pediatr Dermatol 2019; 36: 274-282.

2. Lava SAG, Milani GP, Fossali EF, Simonetti GD, Agostoni C, Bianchetti MG. Cutaneous manifestations of small-vessel leukocytoclastic vasculitides in childhood. Clin Rev Allergy Immunol 2017; 53: 439-451.

3. Miconi F, Cassiani L, Savarese E, et al. Targetoid skin lesions in a child: acute hemorrhagic oedema of infancy and its differential diagnosis. Int J Environ Res Public Health 2019; 16: 823.

4. Halicioglu O, Akman SA, Sen S, Sutcuoglu S, Bayol U, Karci H. Acute hemorrhagic edema of infancy: a case report. Pediatr Dermatol 2010; 27: 214-2155.

5. Ferrarini A, Benetti C, Camozzi P, et al. Acute hemorrhagic edema of young children: a prospective case series. Eur J Pediatr 2016; 175: 557-561.

6. Serra E Moura Garcia C, Sokolova A, Torre ML, Amaro C. Acute hemorrhagic edema of infancy. Eur Ann Allergy Clin Immunol 2016; 48: 22-26.

7. Parker L, Shahar-Nissan K, Ashkenazi-Hoffnung L, et al. Acute hemorrhagic edema of infancy: the experience of a large tertiary pediatric center in Israel. World J Pediatr 2017 13: 341-345.

8. Fiore E, Rizzi M, Ragazzi M, et al. Acute hemorrhagic edema of young children (cockade purpura and edema): a case series and systematic review. J Am Acad Dermatol 2008 ; 59: 684-695.

9. Risikesan J, Koppelhus U, Steiniche T, Deleuran M, Herlin T. Methylprednisolone therapy in acute hemorrhagic edema of infancy. Case Rep Dermatol Med 2014; 2014: 853038.

10. Moradinejad MH, Entezari P, Mahjoub F, Ziaee V. Acute hemorrhagic edema of infancy; a report of five Iranian infants and review of the literature. Iran J Pediatr 2011; 21: 107-112.

11. Mreish S, Al-Tatari H. Hemorrhagic lacrimation and epistaxis in acute hemorrhagic edema of infancy. Case Rep Pediatr 2016; 2016: 9762185.

12. Watanabe T, Sato Y. Renal involvement and hypocomplementemia in a patient with acute hemorrhagic edema of infancy. Pediatr Nephrol 2007; 22: 1979-1981.

13. Obeid M, Haley J, Crews J, Parhizgar R, Johnson L, Camp T. Acute hemorrhagic edema of infancy with abdominal pain and elevated transaminases. Pediatr Dermatol 2008; 25: 640-641.

14. Yu JE, Mancini AJ, Miller ML. Intussusception in an infant with acute hemorrhagic edema of infancy. Pediatr Dermatol 2007; 24: 61-64.

15. Al Sufyani MA. Acute hemorrhagic edema of infancy: unusual scarring and review of the English language literature. Int J Dermatol 2009; 48: 617-622.

16. Dotan M, Nahum E, Weigl D, Bilavsky E. Compartment syndrome because of acute hemorrhagic edema of infancy: a case report and literature review. J Pediatr Orthop B 2014; 23: 419- 421.

17. Snow IM. Purpura, urticaria and angioneurotic edema of the hands and feet in a nursing baby. JAMA 1913; 61: 18-19.

18. Lai-Cheong JE, Banerjee P, Hill V, Kenny P, Ross J. Bullous acute haemorrhagic oedema of skin in infancy. Clin Exp Dermatol 2007; 32: 467-468.

19. Chesser H, Chambliss JM, Zwemer E. Acute hemorrhagic edema of infancy after coronavirus infection with recurrent rash. Case Rep Pediatr 2017; 2017: 5637503.

20. Canpolat C, Bakir M. A case of purpura fulminans secondary to transient protein C deficiency as a complication of chickenpox infection. Turk J Pediatr 2002; 44: 148-151.

21. Yilmaz D, Kavakli K, Ozkayin N. The elevated markers of hypercoagulability in children with Henoch-Schönlein purpura. Pediatr Hematol Oncol 2005; 22: 41-48.

22. Ma TT, Huang YM, Wang C, Zhao MH, Chen M. Coagulation and fibrinolysis index profile in patients with ANCA-associated vasculitis. PLoS One 2014; 9: e97843.

23. Ho CH. Can very high level of D-dimer exclusively predict the presence of thromboembolic diseases? J Chin Med Assoc 2011; 74: 151-154