Acute Hemorrhagic Edema of Infancy

Onychomadesis is a reversible, painless, noninflammatory condition in which there is proximal shedding of the nail plate from the nail matrix. It can occur in fingernails, toenails or both. It may be secondary to systemic disorders, high fevers, bullous dermatoses, Kawasaki disease, infections (streptococcal infections and measles), zinc deficiency, local trauma, acute paronychia, and drug reactions. In addition to these causes, many cases are idiopathic. HFMD is a common pediatric viral illness that is characterized by vesicular eruptions that involve the palms, the soles, and the oral cavity. The median latency period between HFMD and onychomadesis is 40 days. The mechanism of nail matrix arrest after infection remains unclear. Transverse leukonychia and Beau lines reflects milder interruptions in ungula growth and may occur simultaneously in the same patient or a result of the same disease process.


Introduction
Acute hemorrhagic edema of infancy (AHEI) is a benign cutaneous small vessel leucocytoclastic vasculitis.The disease characterized by low-grade fever, acral inflammatory edema and large rosetta-, annular-, or targetoid-shaped ecchymotic purpura primarily over the face and extremities.The outbreak is frequently preceded by an immunization, drug intake or various infections.In spite of violent and alarming clinical presentation in a nontoxic infant, spontaneous recovery without any sequelaes occurs within a few weeks.Although some have suggested that AHEI is a purely cutaneous variant of Henoch-Schönlein purpura, most authors prefer to regard it as a separate clinical entity among the cutaneous small vessel vasculitic diseases of infancy.

Background
Although, AHEI was first described by Snow as a purely cutaneous variant of Henoch-Schönlein purpura in American literature in 1913 (Snow, 1913), it was well-recognised by Seidlmayer (Seidlmayer, 1936) and Finkelstein (Finkelstein, 1938) in European literature in 1930ies.Since then in Europe, many cases have been reported under different clinical terms, including Finkelstein disease and Seidmayer's 'cockade' purpura or syndrome.The name AHEI first appeared in Spanish written and used by Del Carril (Del Carril et al., 1936).Other names used for the disease include purpura en cocarde avec oedema, urticarial vasculitis of infancy, acute benign cutaneous leukocytoclastic vasculitis (Saraclar et al., 1990) of infancy and infantile post-infectious iris-like purpura and oedema.Already, more than 100 cases of AHEI have been published in medical literature worldwide.However, the current number of reported cases are being still unknown.This is probably because, the most papers on the subject being written different languages in the world and AHEI is called Henoch-Schönlein purpura in the United States.Although most of the prints were in case reports (Babic et al., 2008;Can et al., 2006;Di Lernia et al., 2004;Garty et al., 2002;Michael, 2006;Millard et al., 1999;Macea et al., 2003;Obeid et al., 2008;Serna et al., 1994, Silveira & Braganca, 2006), case series were also published (Alp et al., 2009;Caksen et al., 2002;Fiore et al., 2008;Gelmetti et al., 1985;Gonggryp & Todd, 1998;Ince et al., 1995;Karremann et al., 2009;Krause et al., 1996;Legrain et al., 1991;Poyrazoglu et al., 2003;Saraclar et al., 1990;Sites et al., 2008).Today, AHEI is considered as a separate entity, but not a variant of Henoch-Schönlein purpura, with clinical findings and prognosis.

Epidemiology
The exact incidence of AHEI is not well known because of rarity of the disease.So, multicenter epidemiologic studies are needed.Some authors believe that AHEI is often mistaken for other diseases and, therefore, its incidence may be more frequent.It is also probable that, using the synonyms for AHEI in the same population for different cases and to consider the disease as Henoch-Schönlein purpura may change the incidence.

Effect of seasons
AHEI, usually presents during the winter months.This may be associated with its potential etiology.However, some have presented in the spring, autumn (Fiore et al., 2008;Legrain et al., 1991;Poyrazoglu et al., 2003) and summer (Saraclar et al., 1990).

Conclusion
Acute hemorrhagic edema of infancy is an uncommon cutaneous leukocytoclastic vasculitis.Various infections, drug use and vaccination are discussed as triggering factors more than www.intechopen.com Acute Hemorrhagic Edema of Infancy 389 90 years after the first description of the disease.Also, the benign and self-limited course and excellent prognosis with mild to absent systemic involvement is well established.Today, it has been considered as a distinct entity either than a variant of Henoch-Schönlein purpura.Physicians might develop the skills necessary to consider the diagnosis of acute hemorrhagic edema of infancy when presented with a non-toxic infant having large targetoid purpuric lesions and edema in nonpitting character.

Acknowledgment
I give my thanks and gratitude to Prof. Ismail Reisli from Department of Pediatric Immunology for his suggestions about writing the text.I also thank to Assoc.Prof. Hatice Toy from Department of Pathology for the pictures of histopathology and immunofluorescence studies.
Fig. 5. Ecchymotic and purpuric lesions of upper extremities in a 10-month-old girl.
a. Between 4 months and 2 years of age.b.Purpuric or ecchymotic target-like skin lesions with edema especially on the face, auricles and extremities with or without mucosal involvement.c.Lack of systemic disease or visceral involvement.d.Spontaneous recovery within 4-24 days.Common laboratory investigations are follows.a. Leukocytosis with lymphocytosis or eosinophilia.b.Elevated erythrocytes sedimentation rate or C-reactive protein.c. Leucocytoclastic vasculitis with or without fibrinoid necrosis involving small blood vessels in the dermis on histological examination.d.Negative immunofluorescence studies are usual or rarely positivity of C 3 , C 1q , al.,