   |
|
E-IJD® - CORRESPONDENCE |
|
| Year : 2022 | Volume
: 67
| Issue : 1 | Page : 94 |
|
| Neutrophilic figurate erythema- A masquerading presentation |
|
|
Abhijit Saha
Department of Pediatric Dermatology, Institute of Child Heath, Kolkata, West Bengal, India
| Date of Web Publication | 19-Apr-2022 |
Correspondence Address:
Abhijit Saha
Department of Pediatric Dermatology, Institute of Child Heath, Kolkata, West Bengal
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/ijd.ijd_1033_20
|
|
How to cite this article:
Saha A. Neutrophilic figurate erythema- A masquerading presentation. Indian J Dermatol 2022;67:94 |
Sir,
Neutrophilic figurate erythema (NFE) is a rare, benign entity and is considered to be a variant of annular erythema of infancy. Though this is an inflammatory dermatosis of the pediatric age group, adult cases have also been reported.
A two-year-old female child was presented with gradually expanding elevated skin lesion over the right leg for the duration of seven to ten days. She did not have any systemic complaints and was otherwise healthy. There was no history of preceding drug intake or any insect bite. She had similar eruptions two months previous to this incident, which was resolved with some medicines without any sequela. The current lesions appeared at new sites. Cutaneous examination revealed a well-defined, erythematous, elevated, firm, and non-tender plaque [Figure 1]. There was relative clearing and fine scaling over the center with surface wrinkling at places and no sensory deficit could be elicited. She also had similar types of lesions over the right forearm, face, and chest [Figure 2] and [Figure 3]. The lesion over the forearm showed signs of resolution at one end. As per her case history, lesions started as a small papule and gradually increased in size to attain the present configuration. Systemic and general examinations showed results within normal limits except a mild pallor. On physical examination, the reticuloendothelial system showed no abnormality. The family history was unremarkable. | Figure 1: Well-defined, erythematous, elevated plaque with relative clearing and fine scaling over the center of the lesion over right leg
Click here to view |
We considered a deep variant of erythema annulare centrifugum (EAC), tuberculoid leprosy, and subacute cutaneous lupus erythematosus (SCLE) as differentials based on clinical presentation and planned further workup. Salient distinguishing features of differentials are tabulated in [Table 1]. The complete hemogram revealed low hemoglobin (9.2 gm %) with hypochromic and microcytic Red Blood Cell (RBC) and raised Erythrocyte Sedimentation Rate (ESR) (22/1st hour). A peripheral blood smear was otherwise normal. Other biochemical panels were within the normal limit. Antinuclear antibody (Hep 2 cell line) and Anti-Ro and anti-La were negative. Dermatophytosis was ruled out by direct examination of the skin scraping. Mantoux test and slit-skin smear were also found to be negative. We left no stone unturned and a thorough systemic evaluation revealed no abnormalities. As the clinical findings and laboratory investigations—including peripheral blood smear—were not suggestive of leukemia, we did not perform any invasive procedure, such as bone marrow examination. | Table 1: Salient distinguishing features of differential diagnoses (EAC, tuberculoid leprosy, SCLE, Sweet syndrome) and other annular erythemas
Click here to view |
We took a biopsy from the lesion of the leg and sent it for histopathological examination. Direct immunofluorescence could not be performed. Post-biopsy, we offered systemic (amoxicillin) and topical antibiotics. After three days, the child was again brought in by parents with new crops of the lesion at different sites with preferential involvement of buttocks [Figure 4]. The baby was offered oral prednisolone (1 mg/kg) for five days. There was a partial resolution of both the pre-existing as well as new crops of lesions. Oral prednisolone was continued for another two weeks in the same dosage resulting in complete resolution of all the lesions with mild residual dyspigmentation [Figure 5] and [Figure 6]. Prednisolone was gradually tapered through another two weeks and the patient was instructed to follow- up for another three months. No relapse was documented in the subsequent visits. The patient was lost to follow up there after. | Figure 5: Resolution of cutaneous lesions over right leg with mild residual dyspigmentation following treatment
Click here to view |
 | Figure 6: Resolution of cutaneous lesions over right arm with mild residual dyspigmentation following treatment
Click here to view |
Histopathology revealed unremarkable epidermis with dense infiltration of neutrophils throughout the dermis and around the vasculatures. Nuclear dust particles were plenty. There was no evidence of vessel wall involvement [Figure 7]. Histopathological findings simulated Sweet syndrome but based on clinicopathological correlation, we finally stamped our diagnosis as NFE. | Figure 7: H & E (40×) showing neutrophils throughout the dermis and around vasculatures with plenty of nuclear dust
Click here to view |
The term NFE has evolved from the neutrophil-rich dermal infiltrate and nuclear dust in the histopathology section.[1],[2] Histopathologically, it is difficult to differentiate this entity from Sweet syndrome as both the conditions share similar findings [Refer to [Table 1]]. NFE is clinically characterized by asymptomatic or mildly pruritic, an arciform plaque with raised firm, erythematous border, and trailing scale. It can be subdivided into two types; with known etiology, such as malignancy,[3] and with signature pattern without any etiology. In our case, we failed to find out any definite etiology even after extensive workup. Though the disease is self-limiting without any residual atrophy or scarring, cases with chronic persistent course have been reported.[1] Our case had a history of previous episodes at the time of presentation, though post-treatment follow-up was uneventful. NFE is a disease of the pediatric population. Limited data are available where victims are adults.[3],[4],[5] One interesting finding of our case is the presence of surface wrinkling, which was not reported before. The appearance of new lesions following antibiotic therapy could be co-incidental or drug-induced precipitation of the entity. But there is no such prior observation or any established evidence in this regard, which is again due to the rarity of the entity and the resultant limited number of reports. However, our cognizance may bring about a new aspect for further research in this regard. Here in this context, it is to be mentioned that drugs has been found to be a causative agent in the etiopathogenesis of EAC, a close mimicker of NFE.
Several treatment options are mentioned in the literature, such as topical steroid, topical miconazole, oral steroid, antihistaminic, dapsone, colchicine, potassium iodide, etc. We offered oral steroids in our case and the outcome was satisfactory. The oral steroid was preferred as it is the fastest-acting drug among the documented treatment options and as the biopsy report was pending at that time, we considered the steroid as a safe and effective drug with regard to the differential factors we mentioned previously.
In a nutshell, NFE is a diagnostic challenge to the physician due to its rarity and masquerading presentation. Proper awareness and an inquisitive mind can pick up on the presence of this rare entity and avoid unnecessary investigations and interventions. It should be differentiated from its close serious differentials, which require altogether different approaches as far as prognosis and treatments are concerned.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial Support and Sponsorship
Nil.
Conflicts of Interest
There are no conflicts of interest.
 References | |  |
| 1. | Patrizi A, Savoia F, Varotti E, Gaspari V, Passarini B, Neri I. Neutrophilic figurate erythema of infancy. Pediatr Dermatol 2008;25:255-60.  |
| 2. | Annessi G, Signoretti S, Angelo C, Paradasi M, Puddu P. Neutrophilic figurate erythema of infancy. Am J Dermatopathol 1997;19:403-6. |
| 3. | Trébol I, González-Pérez R, García-Rio I, Arregui MA, Saracibar N, Carnero L, et al. Paraneoplastic neutrophilic figurate erythema. Br J Dermatol 2007;156:396-8. |
| 4. | Ozdemir M, Engin B, Toy H, Demirkesen C. Neutrophilic figurate erythema. Int J Dermatol 2008;47:262-4. |
| 5. | Ghosh SK, Bandyopadhyay D, Haldar S. Neutrophilic figurate erythema recurring on the same site in a middle aged healthy woman. Indian J Dermatol Vnerol Leprol 2012;78:505-8. |
[Figure 1], [Figure 2], [Figure 3], [Figure 4], [Figure 5], [Figure 6], [Figure 7]
[Table 1] |
|
|
|
|
|
|
|
|
|
|
|
|
| Article Access Statistics | | | Viewed | 1086 | | | Printed | 8 | | | Emailed | 0 | | | PDF Downloaded | 9 | | | Comments | [Add] | | |
|
|
