Targetoid Skin Lesions in a Child: Acute Hemorrhagic Oedema of Infancy and Its Differential Diagnosis

Abstract

Acute hemorrhagic oedema of infancy (AHEI) is a cutaneous leukocytoclastic small-vessel vasculitis presenting with localized purpuric large skin plaques that are frequently associated with fever and oedema. It must be promptly differentiated from a number of diseases with similar dermatologic manifestations with potentially severe clinical courses that require adequate monitoring and prompt therapy to avoid the risk of a negative evolution. A 15-month-old girl with a negative personal medical clinical history was admitted for the sudden appearance of petechiae on the soft palate. The patient was moderately febrile during the following two days, with a maximum ear temperature of 38.3 °C. The fever disappeared on the third day, whereas the hemorrhagic rash progressively increased and extended to the limbs, face and auricles associated with a strong oedematous component. Moreover, on the second day of hospitalization, bilateral oedema of the metacarpophalangeal joints with joint pain appeared. The blood and serological tests showed an increase in C-reactive protein concentration (3.58 mg/dL) in the absence of leukocytosis and with a normal platelet count (180,000/mm3). The examination of the peripheral smear showed the presence of some large mononuclear elements with hyperbasophile cytoplasm. No alterations in platelet morphology were evidenced. The skin manifestations progressively diminished and disappeared spontaneously within 3 weeks, leaving no sequelae. Conclusion: This case shows the classic skin lesions of AHEI that require differentiation from those of more severe diseases that need prompt recognition and therapy. In this case, the age of the patient, the lack of systemic involvement and the favorable clinical course without therapy were typical. However, as these patients may present to the emergency department with an impressive clinical picture, the condition must be promptly diagnosed to avoid unnecessary diagnostic procedures and to reassure parents.

Acute Hemorrhagic Infancy of Edema: A Purpuric Rash in 6-Month-Old Infant

“… 2 These symptoms often present in the setting of a mild upper respiratory illness or other prodromal illness that causes mild fever. 1 , 3 Symptoms of AHEI are usually limited to the skin. In addition to the symptoms listed above, general edema including scrotal edema and auricular edema are common.…”

“… 2 Coagulation studies, liver function tests, and kidney function tests remain normal. 1 Skin biopsy studies demonstrate leukocytoclastic vasculitis of the small blood vessels in the dermis layer of the skin. Immunoglobulin A (IgA) deposition is found in <25% of biopsy samples of children with AHEI.…”

“… 5 , 6 HSP presents at an older age, has systemic findings, and has predominance of purpura on the buttock and lower extremities compared with AHEI. 1 , 6 …”

“…Acute hemorrhagic edema of infancy (AHEI) is a benign leukocytoclastic vasculitis that has previously had many names including Finkelstein’s disease, Seidlmayer’s disease, and postinfectious cockade purpura. 1 , 2 It is a benign process that presents with localized purpura, which is frequently associated with fever and edema. It usually resolves within a few weeks without intervention.…”

Whole Exome Sequencing Revealed a Candidate Gene for Finkelstein-Seidlmayer Disease

“…Some patients reported a temporal association with vaccines injections. Up now, no genes are associated with the FSD [1][2][3][4][5][6].…”