Neutrophilic Figurate Erythema of Infancy

Abstract

We report here a new case of neutrophilic figurate erythema of infancy in a 1-year and 9-month-old white boy. Neutrophilic figurate erythema of infancy is a rare inflammatory dermatosis which is part of the figurate inflammatory dermatoses of infancy and is considered a variant of annular erythema of infancy. The disease is clinically characterized by annular erythematous lesions, sometimes with a polycyclic configuration, and histologically by a dermal neutrophilic infiltration with leukocytoclasia. Differential diagnosis mainly includes clear-cut severe diseases with a well-known etiology, such as neonatal lupus erythematosus and its variant erythema gyratum atrophicans transiens neonatale, erythema chronicum migrans and erythema marginatum rheumaticum, and diseases of unknown origin and with less clear limits, such as erythema annulare centrifugum and its variant familial annular erythema. Anamnesis, laboratory findings, clinical features, and histology allow the correct diagnosis and therefore, having excluded severe diseases, parents should be reassured as neutrophilic figurate erythema of infancy, as the annular erythema of infancy, is a benign disease, in most cases unassociated with other conditions and usually self-limiting over a few months, even though a chronic course may rarely occur. Our case was characterized by a chronic persistent course and by a complete resolution of the lesions only during febrile episodes.

Neutrophilic figurate erythema of infancy associated with juvenile myelomonocytic leukemia

“…3 It is characterized by papular erythematous eruptions with rapid centrifugal enlargement to annular or polycyclic asymptomatic plaques with indurated borders devoid of vesicles, crusts or desquamation. 4,5 Frequently, the eruptions begin on the face and then spread centrifugally to the limbs. The patches tend to disappear within 2---4 weeks, but the disease course is chronic.…”

“…1,3 Histologically, NFEI is characterized by a superficial and deep, perivascular and interstitial infiltrate of neutrophils associated with leukocytoclasis but without other signs of vasculitis. 1,2,4,5 Differential diagnosis of NFEI includes annular erythemas of infancy and dermatoses with a prominent neutrophilic infiltrate such as Sweet syndrome (SS) and pyoderma gangrenosum, urticarial lesions of dermatitis herpetiformis or linear IgA dermatosis, early lesions of bullous lupus erythematosus, Still disease, Sjögren's syndrome and early leukocytoclastic vasculitis. 2 Sweet syndrome (SS) was very important in our differential diagnosis, considering the association with JMML presented in this case.…”

Cold Induced Neutrophilic Figurate Erythema

“…Once a diagnosis of NFE of infancy is made, parents should be reassured, as this entity and its variants are unassociated with other conditions in most cases. 8,10 Up to now drug induced NFE has not been reported before. In this report, we wanted to emphasize that our case is the first case of NFE which might have been induced by a herbal drug; Ginkgo biloba that has neuroprotective effects.…”

“…7 Similar cold aggravation and presence of hot induced lesions were also remarkable in 1-year and 9-month-old white two boys with idiopathic NFE of infancy reported previously. 8 We believe that the diagnosis of cryoglobulinemia has to be excluded in the presence of this status. Complete resolution of the lesions were previously reported during febrile episodes in these cases and in a few cases with erythema annulare centrifigum (EAC).…”

“…Complete resolution of the lesions were previously reported during febrile episodes in these cases and in a few cases with erythema annulare centrifigum (EAC). 8,9 After disregulation of some cytokine production in the skin triggered by cold, the skin lesions resolve with an increase in interferon if the body temperature rises to a degree higher than normal. 9 Moreover, NFE has been reported to show some clinical differences in paediatric cases such as benign, selflimiting course when compared with adult cases.…”