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Open Access 11.08.2023 | case report/

Diagnostic difficulties in pediatric annular dermatoses

verfasst von: Doris Weiss, MD, Philipp Weber, MD, Amélie Hampel, MD, Julia Tittes, MD, Wolfgang Weninger, MD, Prof. Tamar Kinaciyan, MD

Erschienen in: Wiener Medizinische Wochenschrift



The polymorphic presentation of annular dermatoses in the pediatric population renders them a diagnostic challenge to the clinician. They include various distinct disease entities that can be vaguely categorized according to the age of onset. Herein, we report on a young girl with clinical characteristics of Wells’ syndrome, while histological findings favored the diagnosis of annular erythema of infancy (AEI). Although morphological and histological similarities do exist, AEI and eosinophilic annular erythema (EAE) of childhood are considered as distinct entities in the literature. Wells’ syndrome (WS) is an eosinophilic dermatosis and histologically characterized by eosinophilic dermal infiltration with the hallmark feature of “flame figures.” Based on this case, we discuss and review the differential diagnoses of annular dermatoses in children.

Prior presentation

Case report (poster at ESPD Annual Meeting 2021)

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Case report

A one-and-a-half-year-old girl presented to our pediatric dermatology clinic with erythematous papules and annular succulent plaques with a greyish-green center, confined to the right buttock (Fig. 1a, b). The parents had been applying a topical steroid ointment prescribed by a private practitioner for a few days, without efficacy.
The child’s medical history included trigonocephaly for which she had had surgical reconstruction, but was otherwise uneventful.
Our initial clinical differential diagnoses included Wells’ syndrome and eosinophilic annular erythema of childhood.
Histopathology, obtained from the border of the red to the greyish area of an annular lesion, revealed a superficial and deep perivascular and interstitial lymphocytic infiltrate with abundant CD68-positive macrophages (Fig. 1c, d).
White blood cell count and eosinophils were within the normal range. Eosinophilic cationic protein (ECP) and total serum IgE were both elevated, at 85.5 µg/L (normal value 13.3 µg/L) and 22.0 klU/L (age-adapted reference value < 10 kU/L), respectively. Family history of atopy and specific IgE antibodies to common inhalant and nutritive allergens were negative.
The lesions were treated with topical clobetasol propionate and occlusive dressing for 10 days, followed by once daily application that led to complete remission within 3 consecutive weeks. Since then, the patient has remained asymptomatic for the past 9 months without any further therapy.


Annular dermatoses in pediatric patients include a broad spectrum of differential diagnoses that can only be definitely diagnosed based on subtle clinical variations in conjunction with histopathologic features. In some cases, laboratory investigations are mandatory to rule out serious complications (Table 1).
Table 1
Differential diagnoses of annular erythema in children
Clinical characteristics
Neonatal period
Neonatal lupus erythematosus
6–12 weeks
Maternal anti-SS-A Ab (95%)
anti-SS-B Ab
Annular/polycyclic patches and plaques
“Owl eyes“/“racoon eyes“
Lesions resolve without scarring within 4–6 months (Ab degradation)
Cardiac manifestations (AV block, cardiomyopathy)
Clinical picture & serologic testing in infant & mother
Histology: Interface dermatitis, KC damage, perivascular, periadnexal lymphocytic infiltrate
DIF: granular IgG deposition at dermo-epidermal junction
Familial annular erythema
Usually soon after delivery
Autosomal dominant inheritance
Annular erythema with centrifugal spreading
Very rare
Positive family history is the key
Histology: superficial & deep perivascular infiltrate of marked neutrophils, few eosinophils & histiocytes
Annular erythema of infancy (AEI)
Typically, within 3–11 months
Recurrent annular erythematous plaques
Slow spreading
Resolving without sequelae within weeks
Histology: perivascular lymphocytic infiltrate with eosinophils
Neutrophilic figurate erythema of infancy (NFEI)
Within days postpartum–13 months
Similar to AEI
No associated diseases/significant laboratory abnormalities
Histology: perivascular & interstitial neutrophils, nuclear dust with some lymphocytes ± eosinophils
Absence of vasculitis
Eosinophilic annular erythema (EAE)
Typically after the age of 1 year
Annular erythematous lesions on trunk and extremities
Recurrent and recalcitrant course over years
Histology: superficial and deep perivascular lymphohistiocytic infiltrate with abundant eosinophils
Wells’ syndrome
Typically 1–9 years
Potential triggers: arthropod bites, vaccinations
Painfully infiltrated plaques with greenish hue
Bullous form
Systemic symptoms (fever, arthralgia, malaise)
Peripheral eosinophilia in > 50%, elevated ECP
Histology: superficial & deep perivascular & interstitial eosinophilic infiltrate with “flame figures”
Erythema annulare centrifugum (EAC)
Primarily adults affected, rare in children
EAC is a clinical reaction pattern, not a clinicopathological entitiy
Erythematous papules that enlarge centrifugally, ± scaling at the inner margin
Individual lesions resolve within weeks
Chronic course
Histology: perivascular lymphohistiocytic infiltrate, acanthosis, parakeratosis and spongiosis
Erythema chronicum migrans (ECM)
Children after the age of 2
Borrelia burgdorferi, B. afzelii, B. garinii
“Bull’s eye”—expanding annular erythema with central clearing
Manifests particularly in the head and neck region in children or with multilocular lesions
Clinical picture determines diagnosis
Serology: unreliable in early manifestations or for monitoring purposes after successful therapy
Histology: superficial & deep dermal lymphocytic infiltrate ± plasma cells and eosinophils
Erythema marginatum (rheumaticum)
School children, teenagers, and adolescents
Acute rheumatic fever post-group A streptococcal infection
Recurrent centrifugally expanding erythematous annular patches & plaques
Major Jones criterion for rheumatic fever (others include carditis, polyarthritis, chorea, and subcutaneous nodules)
Histology: interstitial and perivascular neutrophilic infiltrate without vasculitis.
Erythrocyte extravasation in later stages
Annular lichenoid dermatitis of youth (ALDY)
≥ 10 years of age
Asymptomatic annular patches with an erythematous brownish border and central hypopigmentation
Mostly on groin and flanks
Histology: lichenoid dermatitis limited to the tips of rete ridges with an intraepidermal lymphocytic infiltrate
Ab antibody, DIF direct immunofluorescence, ECP eosinophil cationic protein, KC Keratinocyte
Annular erythema of infancy (AEI) and eosinophilic annular erythema (EAE) of childhood are described as distinct, rare inflammatory conditions of infancy and childhood [15]. There are only a few cases previously published in the literature; therefore, the true incidence of these dermatoses is unknown and is likely underreported [17].
In 1981, Peterson and Jarratt originally coined the term AEI, for recurrent rapidly evolving arcuate and annular benign skin lesions, predominantly in infants from 3 to 11 months of age with spontaneous resolution within the first year of life. Histology consists of perivascular lymphocytes with scattered eosinophils [6]. Histiocytic infiltration has been described in HE staining but neither in that abundancy nor immunohistochemically (Fig. 1c, d; [7]). In contrast, EAE is thought to represent the morphological expression of a spectrum of eosinophilic dermatoses in older children that often follow a more recalcitrant clinical course, requiring immunomodulatory drugs. Histology shows a superficial and deep, predominantly perivascular, lymphocytic infiltrate with abundant eosinophils without “flame figures,” which are characteristic, but not pathognomonic, for Wells’ syndrome [1, 3, 8]. The only differences between AEI and EAE are the age of disease onset and the eosinophil count in histology (some in AEI and abundant in EAE), which results in a more transient rash in AEI or recalcitrant in EAE [3, 8].
Wells’ syndrome is a rare pruritic inflammatory skin disease of unknown etiology with several morphologic variants that have been described in recent years (papulonodular, papulovesicular, plaque-type, urticarial, or bullous). It classically presents as localized erythematous swelling (hence the term eosinophilic cellulitis), and subsequently progresses to indurated plaques with a characteristic green-greyish hue in the central part of the plaque.
In the present case, clinical findings were primarily compatible with Wells’ syndrome. The lack of eosinophils in histology, however, suggests otherwise, leaving room for debate. The natural tissue lifespan of eosinophils ranges from 2 to 5 days.7 Treatment with topical steroids downregulates interleukin (IL)-5, which is crucial for the recruitment and survival of eosinophils in tissue [9]. In hindsight it can only be speculated whether prior topical steroid treatment could obscure histopathologic findings (i.e., eradicate all tissue eosinophils).
Elevated levels of ECP in the absence of peripheral eosinophilia as well as the clinical presentation of annular succulent plaques with a greyish-green center (Fig. 1a, b) indicate eosinophil activation.
The age of onset after the first year of life would suggest EAE as the favorable diagnosis, while Wells’ syndrome does not have an age-specific predilection and is even considered to be rare in children [3, 8]. From our own experience, we know that Wells’ syndrome is probably underdiagnosed in many patients due to its polymorphic picture, as we have seen and successfully treated three children alone in the past 2 years. As the etiology is elusive in many cases, we propose that eosinophilic annular dermatoses in children might pose an academically unsatisfactory diagnostic dilemma. It remains to speculate whether they are different presentations of an identical pathophysiological process that lie within a spectrum and should not be exclusive of one another.


Conflict of interest

D. Weiss, P. Weber, A. Hampel, J. Tittes, W. Weninger, and T. Kinaciyan declare that they have no competing interests.

Ethical standards

For this case report no studies with human subjects or animals were performed. All investigations performed were in accordance with the ethical standards. As no images or other information within the manuscript identifies the patient, consent was not deemed necessary.
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Zurück zum Zitat Rongioletti F, Fausti V, Kempf W, Rebora A, Parodi A. Eosinophilic annular erythema: an expression of the clinical and pathological polymorphism of Wells syndrome. J Am Acad Dermatol. 2011;65(4):e135–e7.CrossRefPubMed Rongioletti F, Fausti V, Kempf W, Rebora A, Parodi A. Eosinophilic annular erythema: an expression of the clinical and pathological polymorphism of Wells syndrome. J Am Acad Dermatol. 2011;65(4):e135–e7.CrossRefPubMed
Zurück zum Zitat Kunz M, Hamm K, Brocker EB, Hamm H. Annular erythema in childhood—a new eosinophilic dermatosis. Hautarzt. 1998;49(2):131–4.CrossRefPubMed Kunz M, Hamm K, Brocker EB, Hamm H. Annular erythema in childhood—a new eosinophilic dermatosis. Hautarzt. 1998;49(2):131–4.CrossRefPubMed
Zurück zum Zitat Abarzua A, Giesen L, Silva S, Gonzalez S. Eosinophilic annular erythema in childhood—Case report. An Bras Dermatol. 2016;91(4):503–5.CrossRefPubMedPubMedCentral Abarzua A, Giesen L, Silva S, Gonzalez S. Eosinophilic annular erythema in childhood—Case report. An Bras Dermatol. 2016;91(4):503–5.CrossRefPubMedPubMedCentral
Zurück zum Zitat Paulitschke V, Tittes J, Tanew A, Radakovic S. Eosinophilic annular erythema in a 20-month-old girl. Hautarzt. 2021;72(4):332–6.CrossRefPubMed Paulitschke V, Tittes J, Tanew A, Radakovic S. Eosinophilic annular erythema in a 20-month-old girl. Hautarzt. 2021;72(4):332–6.CrossRefPubMed
Zurück zum Zitat Patrizi A, Savoia F, Varotti E, Gaspari V, Passarini B, Neri I. Neutrophilic figurate erythema of infancy. Pediatr Dermatol. 2008;25(2):255–60.CrossRefPubMed Patrizi A, Savoia F, Varotti E, Gaspari V, Passarini B, Neri I. Neutrophilic figurate erythema of infancy. Pediatr Dermatol. 2008;25(2):255–60.CrossRefPubMed
Zurück zum Zitat Peterson AO Jr., Jarratt M. Annular erythema of infancy. Arch Dermatol. 1981;117(3):145–8.CrossRefPubMed Peterson AO Jr., Jarratt M. Annular erythema of infancy. Arch Dermatol. 1981;117(3):145–8.CrossRefPubMed
Zurück zum Zitat Hebert AA, Esterly NB. Annular erythema of infancy. J Am Acad Dermatol. 1986;14(2 Pt 2):339–43.CrossRefPubMed Hebert AA, Esterly NB. Annular erythema of infancy. J Am Acad Dermatol. 1986;14(2 Pt 2):339–43.CrossRefPubMed
Zurück zum Zitat Hamidi S, Prose NS, Selim MA. Neutrophilic figurate erythema of infancy: A diagnostic challenge. J Cutan Pathol. 2019;46(3):216–20.CrossRefPubMed Hamidi S, Prose NS, Selim MA. Neutrophilic figurate erythema of infancy: A diagnostic challenge. J Cutan Pathol. 2019;46(3):216–20.CrossRefPubMed
Diagnostic difficulties in pediatric annular dermatoses
verfasst von
Doris Weiss, MD
Philipp Weber, MD
Amélie Hampel, MD
Julia Tittes, MD
Wolfgang Weninger, MD
Prof. Tamar Kinaciyan, MD
Springer Vienna
Erschienen in
Wiener Medizinische Wochenschrift
Print ISSN: 0043-5341
Elektronische ISSN: 1563-258X