www.mbcb-journal.orgMed Buccale Chir Buccale 2015;21:177-181 Les auteurs, 2015DOI: 10.1051/mbcb/2015033

www.mbcb-journal.org

Up-to date review and case report

Sweets syndrome revealed by oral pustulosis

Jean-Christophe Fricain1,4,*, Vincent Sibaud2, Sebastien Lepreux3, Alain Taieb4,Franck Boralevi4, Jean-Marie dElbe1

1 Department of Dentistry and Oral Health, University Hospital of Bordeaux, France2 Claudius Regaud institute, Toulouse, France3 Department of Pathology, University Hospital of Bordeaux, France4 Department of Dermatology, University Hospital of Bordeaux, France

(Received 5 July 2015, accepted 8 July 2015)

Abstract Introduction: Neutrophilic dermatoses are conditions characterized by a dermal neutrophilicinfiltration associated with variable leukocytoclasia. Oral involvement occurs but is rare and is of less importancethan the cutaneous involvement. Observation: The case of a 71-year-old man with neutrophilic dermatoses inwhom the major manifestations of the disease were recurrent oral ulcers associated with tongue pustulosis isreported. Oral and skin biopsies revealed a dense neutrophilic infiltrate in the lamina propria. Clinical symptomswere quickly resolved with colchicine treatment. The clinical course allowed us to diagnose Sweet's syndromeassociated with oral pustulosis. Discussion: The main differential diagnosis of Sweet's syndrome is from Behetsdisease. Overlap exists between the manifestations of the two diseases, so they must be differentiated. Therecurrence of oral ulcers with little inflammatory halo and the quick resolution of ulcers associated with pustules,as well as the histological patterns, were not compatible with Behet's disease.

Rsum Introduction : Les dermatoses neutrophiles sont caractrises par une infiltration neutrophiliquecutane associe une leucocytoclasie. Les manifestations orales sont rares et moins bruyantes quau niveau de lapeau. Observation : Le cas d'un homme de 71 ans avec une dermatose neutrophile dont les principalesmanifestations de la maladie taient des ulcres buccaux rcurrents associs une pustulose linguale est rapport.Les biopsies buccales et cutanes ont rvl un infiltrat neutrophilique dense dans la lamina propria. Lessymptmes cliniques ont t rapidement rsolus avec de la colchicine. L'volution clinique a permis dediagnostiquer un syndrome de Sweet associ une pustulose orale. Discussion : Le principal diagnostic diffrentieldu syndrome de Sweet est la maladie de Behet. Des chevauchements existent entre les manifestations cliniquesdes 2 entits. Mais celles-ci nont pas le mme pronostic et doivent tre diffrencies. La rcurrence des ulcresbuccaux avec un discret halo inflammatoire, la rsolution rapide des ulcres associs aux pustules ainsi que lesaspects histologiques du cas dcrit, ntaient pas compatibles avec la maladie de Behet.

Introduction

Neutrophilic dermatoses (ND) are a collection of diseasescharacterized by the accumulation of neutrophils in the skinwithout an identifiable infectious agent [1]. Neutrophilic der-matoses, including Sweets syndrome (SS), pyoderma gangreno-sum (PG), subcorneal pustular dermatosis (Sneddon-Wilkinsondisease (SW)) and rheumatoid neutrophilic dermatitis, are

inflammatory conditions of the skin often associated withunderlying systemic disease [2]. Some deserve great attentiondue to major symptoms and the concept of reactive disease orfor being a marker for other pathologies, especially neoplastic dis-eases. Common to all, there is a disorder of stimuli and prolifer-ation of neutrophils, expressed by cellular skin infiltration [3]. Afew cases of oral involvement have been reported in patientswith ND and were mainly associated with SS [4-9].

Key words:neutrophilicdermatosis / Sweet'ssyndrome / Behet'sdisease / ulceration

Mots cls :dermatoseneutrophilique /syndrome de Sweet /maladie de Behcet /ulcration

177

* Correspondence: jean-christophe.fricain@u-bordeaux2.fr

This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), whichpermits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited

Article publi par EDP Sciences

http://creativecommons.org/licenses/by/4.0http://dx.doi.org/10.1051/mbcb/2015033http://publications.edpsciences.org

Med Buccale Chir Buccale 2015;21:177-181 J.C. Fricain et al.

The case of a patient presenting for evaluation of chronicand painful oral lesions is reported. The associated clinicalsymptoms allowed us to diagnose SS.

Observation

A 71-year-old male patient was hospitalized for painful oralulcers associated with pruritic pustular lesions of the lowerlimbs. His past medical history revealed pneumonia three yearsbefore, oral ulcerations for which he had been hospitalizedmany years ago and Launois-Bensaude lipomatosis. Morerecently, he consulted a dermatologist for skin pustulosis, oralulcers and diffuse scrotal erosions.

Physical examination revealed a slight fever of 37.8 C.Pustular lesions were present on the four limbs, abdomen andupper back (Fig. 1). They were associated with painful oralulcers of the tongue, lips, cheeks and pharynx, and pustuleson the tongue (Figs. 2, 3 and 4). Axillary and inguinal lymphnodes were present.

The pathergy test was negative. Complete blood examina-tion revealed leukocytosis (14,1x109/L) and an inflammatorysyndrome with the C-reactive protein (CRP) concentration at154 mg/L. The autoantibody profile was characterized by a pos-itive anti-nuclear antibody at 1/100. Human leukocyte antigen(HLA) typing was negative for the B51 locus. EBV, CMV, HIV,HBV and HCV serologies were negative. A slight monoclonalpeak was found on serum protein electrophoresis. The beta 2microglobulin serum level and myelogram were normal. Themonoclonal IgG/kappa gammopathy found in immunoelectro-phoresis was classified as monoclonal gammopathy of unde-termined significance (MGUS). A thoraco-abdominopelvic CTscan was done and ruled out underlying malignancy.

Histological study of oral biopsies showed a nonspecificinfiltrate of neutrophils in the lamina propria with exocytosisand without leukocytoclastic vasculitis (Figs. 5, 6). The skinbiopsy showed a subcorneal pustule (Fig. 7) and a marked neu-trophilic infiltrate in the absence of vasculitis consistent withneutrophilic dermatosis. The oral and cutaneous immunofluo-rescence studies were negative.

Bacteriological samples from skin pustules were negative.Treatment with colchicine (Colchimax) led to regression

of oral and skin lesions in one week. Within several days theformation of new skin lesions ceased, followed by a markedimprovement of eruptions. After 1 month, only sequelar cuta-neous pigmented lesions remained. There were neither mucosallesions nor lymphadenopathy. Colchicine treatment was con-tinued for 4 months. Five months after having the colchicinestopped, the patient was hospitalized for recurrence of erosiveoral lesions, which were again treated with colchicine.

Because of the slight fever, the neutrophilic infiltrate withinflammatory syndrome, and the rapid resolution of both oraland skin lesions with colchicine, the final diagnosis wasSweet's syndrome with oral involvement.

178

Discussion

Neutrophilic dermatoses are disorders of stimuli and pro-liferation of neutrophils expressed by cellular skin infiltrationwithout an identifiable infectious agent. SS is the most com-mon type of ND and is characterized by a constellation of find-ings which include pyrexia, elevated neutrophil count, tendererythematous skin lesions (papules, nodules, pustules andplaques), and on the skin biopsy a diffuse infiltrate of matureneutrophils typically located in the upper dermis. It can alsopresent as a pustular dermatosis [10]. The symptoms and clinical

Fig. 1. Appearance of the skin lesions: diffuse pustulosis affectingthe four limbs.Fig. 1. Pustulose affectant les 4 membres.

Med Buccale Chir Buccale 2015;21:177-181 J.C. Fricain et al.

manifestations typically respond promptly after initiation ofsystemic corticosteroid therapy [10].

Our patient met the criteria for SS developed by Su and Liuand modified by von den Driesch [11-12]. In addition to thetwo major criteria (abrupt onset of tender or painful ery-thematous or violaceous plaques or nodules and neutrophilic

Fig. 3. Ulcerative lesion on the oral mucosae: a white-yellowfibrinous center without peripheral erythema.Fig. 3. Ulcration de la joue avec un fond fibrineux et une absence dehalo priphrique.

Fig. 2. Large and painful ulceration on the lower lip, with a whitefibrinous base without a peripheral red halo.Fig. 2. Large ulcration de la lvre infrieure sans halo rythmateuxpriphrique.

infiltration of the dermis without leukocytoclastic vasculitis),he had three minor criteria (elevation of the CRP level, leuko-cytosis and preceding slight fever) [2]. Like our case report,cases of SS with recurrent oral ulcerations were reported [1,4-7].They had tender erythematous nodular or vesicle-like papularskin eruptions on both forearms [5], over the face, neck,palms and legs [4], multicentric erythema and pustules pro-gressing to ulcers on the legs, face, trunk and extremities [7],

Fig. 4. Pustule on the tongue.Fig. 4. Pustule de la langue.

Fig. 5. Histological details of the mucosal biopsy of the pustulespecimen revealing suppurative inflammatory infiltrate in the lami-na propria (hematoxylin and eosin stain; magnification 10).Fig. 5. Coupe histologique de la biopsie dune pustule rvlantlinfiltrat inflammatoire (coloration : hmatoxyline et osine,grandissement 10).

179

Med Buccale Chir Buccale 2015;21:177-181 J.C. Fricain et al.

and erythematous plaques on the legs [6]. Painful oral l