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CASE REPORT
Year : 2017  |  Volume : 18  |  Issue : 2  |  Page : 110-112

Disseminated granuloma annulare-like presentation of juvenile Wells' syndrome


1 Department of Dermatology and Pediatric Dermatology, National Reference Centre for Rare Skin Diseases, Hopital Pellegrin, CHU Bordeaux, France; Department of Dermatology, CHU-Treichville, Abidjan, Cote d'ivoire
2 Department of Dermatology, CHU-Treichville, Abidjan, Cote d'ivoire
3 Department of Dermatology and Pediatric Dermatology, National Reference Centre for Rare Skin Diseases, Hôpital Pellegrin, CHU Bordeaux, France

Date of Web Publication27-Mar-2017

Correspondence Address:
Celestin Kouadio Ahogo
Department of Dermatology, CHU-Treichville, Abidjan, Cote d’ivoire

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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/2319-7250.203002

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  Abstract 

A 5-year-old boy was referred because of urticarial flares with angioedema. Antihistamines and systemic steroids were not useful to prevent flares. At examination, at distance of a flare, chronic red-brownish plaques were found on the legs, trunk, and back, resembling granuloma annulare. The skin biopsy showed epithelioid and giant-cell granulomas, elastophagia, and “flames figures” surrounded with many eosinophils. A diagnosis of disseminated granuloma annulare-like variant of Wells' syndrome as described earlier by Caputo was made. A treatment with dapsone was initiated and enabled to stop flares. Wells' syndrome is a rare clinicopathologic entity with a chronic benign course and often misleading clinical presentation. Our case illustrates the clinical polymorphism and benefit of dapsone therapy.

Keywords: Flame figures, granuloma annulare, hypereosinophilia, Wells' syndrome


How to cite this article:
Ahogo CK, Gbery I P, Vagamon B, Fauconneau A. Disseminated granuloma annulare-like presentation of juvenile Wells' syndrome. Indian J Paediatr Dermatol 2017;18:110-2

How to cite this URL:
Ahogo CK, Gbery I P, Vagamon B, Fauconneau A. Disseminated granuloma annulare-like presentation of juvenile Wells' syndrome. Indian J Paediatr Dermatol [serial online] 2017 [cited 2020 Aug 12];18:110-2. Available from: http://www.ijpd.in/text.asp?2017/18/2/110/203002


  Introduction Top


Wells' syndrome is a rare inflammatory condition whose origin is still debated. An inappropriate hypersensitivity reaction is often postulated.[1] It usually runs a chronic recurrent clinical course with a good prognosis. Clinical features are varied, making diagnosis difficult. Pathological findings characterized by a dermal inflammatory infiltrate of eosinophils with flames figures are quite distinctive in this clinical context.[2],[3] Few pediatric cases have been reported, often with paucilesional presentation.[4],[5] We report an unusual case of Wells' syndrome with a disseminated granuloma annulare-like presentation.


  Case Report Top


A 5-year-old boy was referred to our unit in June 2011, for episodes of urticaria on the limbs and trunk, with edema of the hands. His skin condition had started in the neonatal period with a remitting diffuse pustulosis. Edematous lesions began when he was 6-month-old. Flares repeated 2–3 times each year and tend to recur on the same sites [Figure 1]a. There was neither itching nor fever during flares. The boy was otherwise in good health and growth, and developmental milestones were normal. After 3 years, some lesions evolved to red-brownish plaques with a slightly elevated border. The first skin biopsy, performed on a late remitting lesion, showed a dermal elastolysis without major inflammation. Antihistamines and steroids were prescribed during flares and were only partially efficient according to the family.
Figure 1: (a) Pseudo-urticarial lesions during flares. (b) Red-brownish plaques resembling granuloma annulare on the legs. (c) Posttreatment lesions

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At the time, he was referred to our unit, at distance of a flare, he had a few plaques on the back of his thighs, on the left leg, the upper back, and the arm, which were resembling granuloma annulare [Figure 1]b. This aspect of granuloma annulare had started from approximately 5 months. Clinical examination was otherwise normal.

A new skin biopsy was obtained on the raised border of a plaque. It revealed epithelioid and giant-cell granulomas in the upper dermis. Features of elastophagia were also observed with “flame figures” [Figure 2], surrounded by numerous eosinophils. Full blood count, renal and liver function tests, and C-reactive protein were normal. The presence of eosinophilic flame figures and the numerous eosinophils raised the hypothesis of Wells' syndrome. The diagnosis of Wells' syndrome in a disseminated granuloma annulare-like variant was eventually made upon clinicopathologic confrontation.
Figure 2: Epithelioid and giant-cell granulomas with elastophagia and flame figures, HES stain

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A treatment with dapsone 1 mg/kg daily was initiated and enabled to stop the flares. Treatment was well tolerated with only a moderate anemia. More than 6 months later, existing lesions had partially disappeared [Figure 1]c, and no new lesion was noted.


  Discussion Top


Wells initially reported eight cases of eosinophilic cellulitis characterized by inflammatory edematous plaques mimicking infectious cellulitis with eosinophilic dermal infiltrate on histological examination.[6],[7] The term “Wells' syndrome” was later coined, and further reports highlighted its clinical aspect. It usually affects adults, both men and women equally.[2] Wells' syndrome in children is rare and characterized by its polymorphism. In literature, the percentage of pediatrics cases is not well known indeed around thirty cases have been reported to date.[4] The “classical” description consists of an acute pruritic dermatitis mimicking infectious cellulitis. Plaques appear in a few days and disappear within weeks to months normally without scarring. Skin atrophy or residual brownish pigmentation can happen.[2],[4],[5] Bullae are not uncommon.[4],[8] Although the initial description was that of a cellulitis, a wide range of clinical manifestations has later been described. In the largest series to date,[5] Caputo et al. proposed diagnostic criteria and classified patients in seven types: Plaque-type, granuloma-annulare-like, urticaria-like, papulovesicular, bullous, papulonodular, and fixed drug eruption-like. Our case is unusual because of its disseminated granuloma annulare presentation, which has not been previously reported in a child. Systemic involvement is usually absent although some reports describe fever, joint swelling, and adenopathy.[4] Although a benign condition, Wells' syndrome often runs a chronic course with multiple recurrences. Differential diagnoses such as urticaria, granuloma annulare, bacterial cellulitis, or hypereosinophilic syndrome should be considered.

A peripheral eosinophilia is present in up to 50% of the cases. As a rule, other routine laboratory tests are normal. Histopathological findings depend on the stage of the disease. In the acute stage, dermal edema with eosinophilic infiltrate is a prominent feature. Granuloma with histiocytes and eosinophils later develops along with characteristic flame figures.[2],[3] Flames figures are amorphous or granular eosinophil material coating collagen bundles and elastic fibers. They result from the degranulation of eosinophils.[9] They are not pathognomonic and can be found in various dermatoses such as bullous pemphigoid and insect bites, tinea pedis, and other inflammatory conditions with numerous eosinophils.[2],[9]

The pathogenesis of Wells' syndrome is poorly understood. Wells' syndrome is thought to be a reactive disorder, though most of the time, no disease is associated. Some authors postulate a hypersensitivity reaction to various stimuli followed by, as in hypereosinophilic syndromes, a clonal proliferation of type 2 T-helper lymphocytes which would result in an increased IL-5 production and eosinophils recruitment.[10],[11] Various triggering factors have been reported drugs,[10] viral infections,[12] or digestive parasitoses [13] for instance. For some authors, Wells' syndrome might be part of the wider spectrum of “eosinophilic dermatoses” by analogy with neutrophilic dermatoses. Those authors advocate a thorough research of an associated condition, once Wells' syndrome is diagnosed.[11] In our case, no associated condition was found.

Treatment remains elusive and nonconsensual. Steroids are usually efficient at moderate doses (0.3–0.5 mg/kg/j) but do not prevent relapses. Antihistamines and dapsone are sometimes used as a first-line therapy. Numerous therapeutic agents have also been reported to be efficient: Phototherapy, colchicine, topical steroids, and azathioprine.[4] Since prognosis is good, more aggressive treatments are rarely considered. In our case, dapsone was chosen given the inefficiency of steroids and antihistamines.


  Conclusion Top


Wells' syndrome is a rare clinicopathologic entity with a chronic course. It is a benign condition with various and often misleading clinical presentations. Our case illustrates the clinical polymorphism of Wells' syndrome and the therapeutic interest of dapsone.

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Conflicts of Interest

There are no conflicts of interest.

 
  References Top

1.
Cruz MJ, Mota A, Baudrier T, Guimaraes S, Azevedo F. Recurrent Wells' syndrome associated with allergic asthma exacerbation. Cutan Ocul Toxicol 2012;31:154-6. Available from: http://www.ncbi.nlm.nih.gov/pubmed/22035426. [Last cited on 2012 Mar 17].  Back to cited text no. 1
    
2.
Moossavi M, Mehregan DR. Wells' syndrome: A clinical and histopathologic review of seven cases. Int J Dermatol 2003;42:62-7.  Back to cited text no. 2
    
3.
Consigny S, Courville P, Young P, Richard C, Gauthier V, Maillard V, et al. Histological and clinical forms of the eosinophilic cellulitis. Ann Dermatol Venereol 2001;128(3 Pt 1):213-6.  Back to cited text no. 3
    
4.
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. 4
    
5.
Caputo R, Marzano AV, Vezzoli P, Lunardon L. Wells syndrome in adults and children: A report of 19 cases. Arch Dermatol 2006;142:1157-61.  Back to cited text no. 5
    
6.
Wells GC. Recurrent granulomatous dermatitis with eosinophilia. Trans St Johns Hosp Dermatol Soc 1971;57:46-56.  Back to cited text no. 6
    
7.
Wells GC, Smith NP. Eosinophilic cellulitis. Br J Dermatol 1979;100:101-9.  Back to cited text no. 7
    
8.
Shams M, Hudgens J, Lesher JL Jr., Florentino F. Wells' syndrome presenting as a noninfectious bullous cellulitis in a child. Pediatr Dermatol 2012;29:224-6.  Back to cited text no. 8
    
9.
Leiferman KM, Peters MS. Reflections on eosinophils and flame figures: Where there's smoke there's not necessarily Wells syndrome. Arch Dermatol 2006;142:1215-8.  Back to cited text no. 9
    
10.
Seçkin D, Demirhan B. Drugs and Wells' syndrome: A possible causal relationship? Int J Dermatol 2001;40:138-40.  Back to cited text no. 10
    
11.
Delaporte E. From Wells syndrome to eosinophilic disease. Ann Dermatol Venereol 2001;128(3 Pt 1):207-11.  Back to cited text no. 11
    
12.
Cherng E, McClung AA, Rosenthal HM, Hicks J, Levy ML. Wells' syndrome associated with parvovirus in a 5-year old boy. Pediatr Dermatol 2012;29:762-4. Available from: http://www.ncbi.nlm.nih.gov/pubmed/22150362. [Last cited on 2012 Mar 17].  Back to cited text no. 12
    
13.
Canonne D, Dubost-Brama A, Segard M, Piette F, Delaporte E. Wells' syndrome associated with recurrent giardiasis. Br J Dermatol 2000;143:425-7.  Back to cited text no. 13
    


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  [Figure 1], [Figure 2]



 

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