A Violently Violaceous Rash of Infancy

An 8-month-old girl presented to the pediatric emergency department for a rapidly progressive, violaceous rash that started 12 hours prior. The rash began as small, round lesions on the arm that coalesced and spread without obvious pain or pruritus. One week prior, the patient had recovered from an apparent upper respiratory tract infection. The patient was afebrile and appeared nontoxic and playful on physical examination. Skin was remarkable for a violaceous, nonblanching rash involving the arms, legs, cheeks, and ears, which were edematous (Figures 1 and 2). Workup revealed a partial thromboplastin time of 43 seconds (normal 26.5-34.7 seconds) and a platelet count of 564 K/μL (normal 150-450 K/μL). Dermatology follow-up 4 days later revealed an improving rash.Figure 2Purpuric lesions of the face and edema of the ear.View Large Image Figure ViewerDownload Hi-res image Download (PPT) Acute hemorrhagic edema of infancy. Acute hemorrhagic edema of infancy is a rare disease characterized by mild fever, edema, and precipitous onset of a purpuric rash of the face, ears, and extremities in a nontoxic-appearing infant.1Karremann M. Jordan A.J. Bell N. et al.Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature.Clin Pediatr. 2009; 48: 323-326Crossref PubMed Scopus (34) Google Scholar,2Savino F. Lupica M.M. Tarasco V. et al.Acute hemorrhagic edema of infancy: a troubling cutaneous presentation with a self-limiting course.Pediatr Dermatol. 2013; 30: e149-e152Crossref Scopus (29) Google Scholar It is an acute cutaneous leukocytoclastic vasculitis.1Karremann M. Jordan A.J. Bell N. et al.Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature.Clin Pediatr. 2009; 48: 323-326Crossref PubMed Scopus (34) Google Scholar,3Saraclar Y. Tinaztepe K. Adaliolu 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: 473-483Abstract Full Text PDF PubMed Scopus (139) Google Scholar Though similar to Henoch-Schönlein purpura, immunohistologic differences suggest a separate entity.4da Silva Manzoni A.P.D. Viecili J.B. De Andrade C.B. et al.Acute hemorrhagic edema of infancy: a case report.Int J Dermatol. 2004; 43: 48-51Crossref PubMed Scopus (46) Google Scholar Unlike Henoch-Schönlein purpura, which normally presents after age 2, acute hemorrhagic edema of infancy affects children between 4 and 24 months of age.2Savino F. Lupica M.M. Tarasco V. et al.Acute hemorrhagic edema of infancy: a troubling cutaneous presentation with a self-limiting course.Pediatr Dermatol. 2013; 30: e149-e152Crossref Scopus (29) Google Scholar The etiology is unknown, but is often precipitated by infections, drugs, or vaccination.4da Silva Manzoni A.P.D. Viecili J.B. De Andrade C.B. et al.Acute hemorrhagic edema of infancy: a case report.Int J Dermatol. 2004; 43: 48-51Crossref PubMed Scopus (46) Google Scholar Acute hemorrhagic edema of infancy follows a benign clinical course with spontaneous, complete recovery and usually spares visceral organs.4da Silva Manzoni A.P.D. Viecili J.B. De Andrade C.B. et al.Acute hemorrhagic edema of infancy: a case report.Int J Dermatol. 2004; 43: 48-51Crossref PubMed Scopus (46) Google Scholar Differential diagnosis includes Henoch-Schönlein purpura, meningococcal infection, purpura fulminans, Kawasaki disease, and nonaccidental trauma.1Karremann M. Jordan A.J. Bell N. et al.Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature.Clin Pediatr. 2009; 48: 323-326Crossref PubMed Scopus (34) Google Scholar Workup may include a complete blood count, coagulation tests, and urinalysis to rule out severe disorders.1Karremann M. Jordan A.J. Bell N. et al.Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature.Clin Pediatr. 2009; 48: 323-326Crossref PubMed Scopus (34) Google Scholar Antihistamines, nonsteroidal anti-inflammatory drugs, or steroids are likely unnecessary.1Karremann M. Jordan A.J. Bell N. et al.Acute hemorrhagic edema of infancy: report of 4 cases and review of the current literature.Clin Pediatr. 2009; 48: 323-326Crossref PubMed Scopus (34)