Neutrophilic figurate erythema of infancy L98.2

Annessi et al. 1977

Neutrophilic figural erythema (NFE) is a rare benign entity, is considered a variant of adult urticarial vasculitis, and is one of the anular erythemas of infancy. Although it is an inflammatory dermatosis of the pediatric age group, adult cases have also been reported.

NFE is typically a disease of the young child. Adult-onset cases have been described (Kuo YW et al. 2021).

Mostly unknown

Upper and lower extremities, face and chest region.

The term "neutrophilic figured erythema of childhood", NFE, is histologically defined and characterized by a neutrophil-rich dermal infiltrate with nuclear dust. Differentiation from Sweet syndrome is difficult as both diseases can have analogous histopathological substrates, although NFE is more likely to be associated with leukocytoclastic vasculitis.

Clinically, neutrophilic figured erythema in children is characterized by asymptomatic or mildly pruritic, annular or circinate, red plaques with raised, firm, red borders and (coarse) lamellar scaling. Although the disease is self-limiting and no residual atrophy or scarring remains, cases with a chronic persistent course have been reported.

A variant of NFE is chronic recurrent annular neutrophilic dermatosis (CRAND). It was first described in 1989. CRAND is a rarely described form of neutrophilic dermatosis characterized by a ring-like and chronic course and histologic involvement similar to Sweet syndrome, but without common signs, biologic abnormalities, or underlying systemic pathology. It appears to affect adult women after the age of 40.

Deep variant of erythema anulare centrifugum. Histology is diagnostic. No leukocytoclasia. Age is rather unusual in childhood.

Tuberculoid leprosy: histology is diagnostic

Subacute cutaneous lupus erythematosus, which can often present with anular foci. Histo and immunohisto and ANA are diagnostic

Sweet syndrome: no evidence of leukocytoclastic vasculitis, unusual age.

Acute tinea due to Malassezia species: lack of fungal evidence. Missing source of infection.

A two-year-old girl presented with a gradually spreading, circinate, elevated red plaque on the right leg that had been present for 7-10 days. The mother reported a primary red plaque with centrifugal growth. Ultimately, the ring-shaped structures had formed. There were no systemic complaints. The child was otherwise healthy. No medication was taken. The family history was unremarkable. Anamnestically, analogous skin symptoms were reported, which had healed without consequences after a few weeks under blanching local therapy. The current lesions appeared at new sites.

Clinic: partly annular, partly circulatory erythematous, raised, firm, non-sensitive plaques with a fine scaling of the surface. Systemic and general examinations showed normal results except for a slight pallor.

Histo: unremarkable epidermis, dense neutrophilic infiltrate throughout the dermis and around the vessels. Abundant nuclear dust. Histopathologic findings were consistent with leukocytoclastic vasculitis.

Laboratory: decreased hemoglobin (9.2 gm %), hypochromic and microcytic erythrocytes, elevated ESR. The other biochemical parameters were in the normal range. Antinuclear antibodies (Hep-2 cell line) as well as anti-Ro and anti-La were negative.

Therapy: oral prednisolone (1 mg/kg) for 5 days. Partial regression of the lesions. Continuation of oral prednisolone therapy for a further two weeks. Including complete regression of all lesions with slight hyperpigmentation. Reduction of prednisolone therapy over a further 2 weeks.

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2. Ghosh SK et al. (2012) Neutrophilic figurate erythema recurring on the same site in a middle aged healthy woman. Indian J Dermatol Vnerol Leprol 78:505-508.

3. Hamidi S et al. (2019) Neutrophilic figurate erythema of infancy: A diagnostic challenge. J Cutan Pathol 46:216-220.

4. Kuo YW et al (2021) Pregnancy-associated neutrophilic figurate erythema. Indian J Dermatol Venereol Leprol 88:99-101.
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8. Saha A (2022) Neutrophilic Figurate Erythema- A Masquerading Presentation. Indian J Dermatol 67: 94.
9. Trébol I et al (2007) Paraneoplastic neutrophilic figurate erythema. Br J Dermatol 156:396-398.

10. Wallach D et al. (2018) Pyoderma gangrenosum and Sweet syndrome: The prototypic neutrophilic dermatoses. Br J Dermatol 178, 595-602.