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CASE REPORT
Year : 2015  |  Volume : 16  |  Issue : 2  |  Page : 90-92

Well's syndrome in a child: A rare case report


Department of Dermatology, Sree Balaji Medical College, Chennai, Tamil Nadu, India

Date of Web Publication9-Apr-2015

Correspondence Address:
C Zohra Begum
42 F6, Kalpana Flats, Haridoss 2nd Street, Kolathur, Chennai 600 099, Tamil Nadu
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.152131

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  Abstract 

Well's syndrome is an uncommon condition of unknown etiology, clinically characterized by pruritic or tender cellulitis like eruption and histologically by flame figures in the dermis. We report a case of Well's syndrome in an 18-month-old boy who presented with urticaria like eruptions.

Keywords: Eosinophilic cellulitis, flame figures, Well′s syndrome


How to cite this article:
Thomas J, Begum C Z, Tamilarasi S. Well's syndrome in a child: A rare case report. Indian J Paediatr Dermatol 2015;16:90-2

How to cite this URL:
Thomas J, Begum C Z, Tamilarasi S. Well's syndrome in a child: A rare case report. Indian J Paediatr Dermatol [serial online] 2015 [cited 2020 Jul 16];16:90-2. Available from: http://www.ijpd.in/text.asp?2015/16/2/90/152131


  Introduction Top


Well's syndrome [1] or eosinophilic cellulitis is a rare inflammatory disorder of unknown etiology, clinically characterized by two phases. The first phase begins as erythematous, centrifugally spreading plaques, usually following a prodrome of burning sensation and pain. They later evolve into the second phase in 1-3 weeks, characterized by induration, which resolves with morphea like skin atrophy or hyperpigmentation, by 4-8 weeks.


  Case report Top


An 18-month-old boy was brought by his parents with erythematous, edematous plaques associated with mild scaling all over the body [Figure 1] and [Figure 2]. The skin lesions started soon after birth, were pruritic but not tender. The differential diagnoses considered were urticaria and atopic dermatitis. The child was started on antibiotics and antihistamines with no improvement. Complete blood count was done which revealed leukocytosis with eosinophilia. Urine examination and other routine investigations were normal.
Figure 1: Erythematous, edematous plaques on the forearm of an 18-month-old boy

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Figure 2: Urticaria-like plaques associated with mild scaling seen over the scalp

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Histology showed an acanthotic epidermis and dense eosinophilic infiltrate with "flame figures" in the upper dermis [Figure 3] and [Figure 4]. On the basis of lesional morphology, peripheral eosinophilia and cutaneous histopathological features a diagnosis of Well's syndrome was made, and the patient was started on a short course of systemic steroids to which he responded well.
Figure 3: Histology showing an acanthotic epidermis and dense eosinophilic infiltrate with "flame figures" in the upper dermis (low power view)

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Figure 4: "Flame figures" are seen in the dermis, they represent intense eosinophilic degranulation of major basic protein coating collagen bundles in the dermis (high power view, magnification ×40)

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  Discussion Top


Well's syndrome is usually considered as a differential diagnosis for any atypical cellulitis or bacterial cellulitis not responding to a course of antibiotics. This condition is rare in children and classically presents with erythematous, edematous plaques. Other clinical variants that have been documented include urticaria like, granuloma annulare like, papulovesicular, bullous, nodular and fixed drug eruption like lesions. [2]

Systemic involvement is rare, but fever, arthralgia, [3] lymphadenopathy and anterior uveitis [4] has been reported. The exact etiology is not known, but evidence suggests that it is a hypersensitivity response to various triggering factors like insect bites, drugs, infection, inflammatory disorders, thiomersal containing vaccines, malignancy or idiopathic. Congenital [5],[6] and familial cases [3] have also been reported in literature.

Histopathological findings involve an acute stage, subacute stage, and resolving stage. The acute stage is characterized by dermal edema and an eosinophilic infiltrate. "Flame figures" are seen in the subacute stage, they represent intense eosinophilic degranulation of major basic protein coating collagen bundles in the dermis. In the resolving stage, phagocytic histiocytes palisade around the flame figures, and a granuloma may form in an attempt to eliminate the flame figures. These flame figures are not specific to eosinophilic cellulitis and occur in other conditions, such as bullous pemphigoid, tinea pedis, or insect bite reactions. The peripheral eosinophilia correlates with increased serum interleukin-5 (IL-5) and eosinophilic cationic proteins seen in the majority of patients. IL-5 is responsible for mobilizing eosinophils from the bone marrow, degranulation and ultimately tissue destruction. [7]

Patients respond well to a course of oral steroids, Prednisolone at a dosage of 2 mg/kg/day, gradually tapered over 2 to 3 weeks. [7] In recalcitrant cases, a combination of prednisolone, dapsone and antihistamines, [8] minocycline, [9] PUVA therapy [10] and cyclosporine has been found to be effective.

This case is reported for its rare and challenging clinical presentation.

 
  References Top

1.
Wells GC. Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp Dermatol Soc 1971;57:46-56.  Back to cited text no. 1
[PUBMED]    
2.
Carlesimo M, Fidanza L, Mari E, Feliziani G, Narcisi A, De Marco G, et al. Wells syndrome with multiorgan involvement mimicking hypereosinophilic syndrome. Case Rep Dermatol 2009;1:44-48.  Back to cited text no. 2
    
3.
Kamani N, Lipsitz PJ. Eosinophilic cellulitis in a family. Pediatr Dermatol 1987;4:220-4.  Back to cited text no. 3
    
4.
Miller BA, Geist CE. Iritis and Wells' syndrome. Int J Dermatol 1992;31:672.  Back to cited text no. 4
[PUBMED]    
5.
Garty BZ, Feinmesser M, David M, Gayer S, Danon YL. Congenital Wells syndrome. Pediatr Dermatol 1997;14:312-5.  Back to cited text no. 5
    
6.
Kuwahara RT, Randall MB, Eisner MG. Eosinophilic cellulitis in a newborn. Pediatr Dermatol 2001;18:89-90.  Back to cited text no. 6
[PUBMED]    
7.
Gilliam AE, Bruckner AL, Howard RM, Lee BP, Wu S, Frieden IJ. Bullous "cellulitis" with eosinophilia: Case report and review of Wells' syndrome in childhood. Pediatrics 2005;116:e149-55.  Back to cited text no. 7
    
8.
Lee MW, Nixon RL. Eosinophilic cellulitis case report: Treatment options. Australas J Dermatol 1994;35:95-7.  Back to cited text no. 8
    
9.
Stam-Westerveld EB, Daenen S, Van der Meer JB, Jonkman MF. Eosinophilic cellulitis (Wells' syndrome): Treatment with minocycline. Acta Derm Venereol 1998;78:157.  Back to cited text no. 9
[PUBMED]    
10.
Diridl E, Hönigsmann H, Tanew A. Wells' syndrome responsive to PUVA therapy. Br J Dermatol 1997;137:479-81.  Back to cited text no. 10
    


    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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