Dermatology Online Journal

Acute hemorrhagic edema of infancy
Daniel J Michael MD
Dermatology Online Journal 12 (5): 10

University of California Davis Department of Dermatology. dan.michael@ucdmc.ucdavis.edu

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Abstract

An 11-month-old boy initially presented to an outside hospital with fever, rhinorrhea, swelling, and papular lesions involving the left foot. He was diagnosed with necrotizing fasciitis and he subsequently underwent debridement of the lower left leg. Tissue cultures were submitted and were negative. Histopathological examination revealed a subcutaneous leukocytoclastic vasculitis. The patient was then transferred to the University of California Davis Medical Center at which time he was noted to have erythematous nontender annular and targetoid patches and plaques with purpuric centers; the lesions were scattered over the legs, right foot, flanks, and pinnae. The clinical and histopathological findings supported a diagnosis of acute hemorrhagic edema of infancy. Supportive care was maintained and the lesions and associated edema resolved. Acute hemorrhagic edema of infancy is a form of leukocytoclastic vasculitis that, despite a rapid and dramatic onset, has a benign prognosis.

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Clinical synopsis

An 11-month-old boy presented to an outside hospital with a several day history of mild fever and rhinorrhea. One day prior to presentation the patient's mother noted apparent insect bites on the patient's left leg. She became concerned when she noticed his left foot had become swollen and brought him to a local emergency department.

On admission, the patient was alert, distractible, and in no acute distress. Several erythematous round flat-topped papules smaller than 0.5 cm were noted on his left leg and torso. Moderate non-pitting edema was noted over his left foot. Over the course of the next several hours the involved foot developed a pale dusky discoloration and the edema spread proximally to involve most of the left lower leg.

The patient was subsequently taken to the operating room for debridement, because of the possible risk of necrotizing fasciitis and progressive spread of the eruption proximally. Debridement of the skin and subcutaneous tissue (excluding muscular fascia) was performed and samples sent for gram stain, culture and sensitivities, and histopathologic examination. The patient was started on penicillin and clindamycin. No microorganisms were identified and the cultures remained sterile. Histopathology revealed a subcutaneous leukocytoclastic vasculitis. Direct immunofluorescence studies showed perivascular C3, fibrinogen, and IgM (Fig. 1).

The patient was subsequently transferred to the University of California Davis Medical Center in stable condition for further management. The next day, additional erythematous annular lesions were noted over the patient's right leg, trunk, upper arms and ears. In addition, the right foot was noted to be edematous with a slightly dusky appearance.

Figure 1	Figure 2
Figures 1 and 2. Annular erythematous papules and plaques on the right lower leg and ankle.

Physical examination revealed a well-appearing, interactive baby boy. Erythematous non-tender 0.4 cm to 4 cm annular and targetoid patches and plaques with purpuric centers were scattered over the upper left leg, upper and lower right leg and foot, flanks, and pinnae (Fig. 2).

Figure 3	Figure 4
Figure 3. Erythematous macules and patches on the right plantar foot Figure 4. Targetoid erythematous plaque on the arm.

Figure 5	Figure 6
Figures 5 and 6. Leukocytoclastic vasculitis with fibrinoid necrosis. Hematoxylin and eosin stain.

The clinical and histopathological findings suggested a diagnosis of acute hemorrhagic edema of infancy (AHEI). The patient remained stable with conservative management and routine wound care. Subsequent exploration of the wound revealed no evidence of necrotizing fasciitis. Over the course of the next several weeks, the dermatologic lesions resolved and the patient was transferred to Shriners Hospital Burn Service for wound management with planned skin grafts.

Comment

Acute hemorrhagic edema of infancy (AHEI) is a relatively uncommon form of leukocytoclastic vasculitis that typically presents in children from age 4 months to 2 years [1, 2]. This condition was first described by Snow in 1913 [2, 3]. Onset is often dramatic with petechiae, ecchymoses, and annular, nummular or targetoid purpuric lesions usually involving the extremities, face, or ears. The edema is often asymmetric and begins distally and can progress proximally [2].

Although the etiology is unknown, pathophysiologically it is an immune complex mediated vasculitis often preceded by a prodromal period [4, 5]. Association with prior viral infection (upper respiratory tract infection, otitis media, viral conjunctivitis), bacterial infection (streptococcal or staphylococcal pharyngitis, pulmonary tuberculosis, pneumonia, urinary tract infection), vaccination, or medication (penicillin, cephalosporin, trimethoprim-sulfamethoxazole, or paracetamole) have been documented [5, 6]. AHEI is believed by most to be a mild variant of Henoch-Schönlein purpura (HSP) [7], but is considered a distinct entity by others [6, 8]. It has been noted that while typical AHEI has features that distinguish it from HSP (lack of IgA deposition, but presence of C1q deposition) [4, 6], some cases without such histopathologic differences may represent a pediatric variant of HSP [4].

Treatment remains controversial. While it has been reported that systemic corticosteroids and antihistamines do not alter the course [4, 9], some authors support the use of corticosteroids [6] and antihistamines [7] to hasten resolution. The acute onset and possible confusion with other more severe conditions emphasizes the importance of early diagnosis. The differential diagnosis includes HSP, meningococcemia, erythema multiforme, urticarial vasculitis, Kawasaki disease, and necrotizing fasciitis [2].

References

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2. Bolognia JL, Jorrizo JL, Rapini RP. Dermatology. C.V. Mosby - 2003-06-01. 387-388. ISBN: 0323024092

3. Snow IM. Purpura, urticaria and angioneurotic edema of the hands and feet in a nursing baby. JAMA. 1913;61:18-9

4. Legrain V, Lejean S, Taieb A, Guillard JM, Battin J, Maleville J. Infantile acute hemorrhagic edema of the skin: study of ten cases. J Am Acad Dermatol. 1991 Jan;24(1):17-22. PubMed

5. Roh MR, Chung HJ, Lee JH. A case of acute hemorrhagic edema of infancy. Yonsei Med J. 2004 Jun 30;45(3):523-6. PubMed

6. da Silva Manzoni AP, Viecili JB, de Andrade CB, Kruse RL, Bakos L, Cestari TF. Acute hemorrhagic edema of infancy: a case report. Int J Dermatol. 2004 Jan;43(1):48-51. PubMed

7. Saraclar Y, Tinaztepe K, Adalioglu G, Tuncer A. Acute hemorrhagic edema of infancy (AHEI)--a variant of Henoch-Schonlein purpura or a distinct clinical entity? J Allergy Clin Immunol. 1990 Oct;86(4 Pt 1):473-83. PubMed

8. Krause I, Lazarov A, Rachmel A, Grunwald MM, Metzker A, Garty BZ, Halevy S, Nitzan M. Acute haemorrhagic oedema infancy, a benign variant of leucocytoclastic vasculitis. Acta Paediatr 1996 Jan;85(1): 114-7. PubMed

9. Crowe MA, Jonas PP. Acute hemorrhagic edema of infancy. Cutis. 1998 Aug;62(2):65-6. PubMed

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