A Pseudo-Tumoral Facial Mass revealing a Tertiary Syphilis
p. 21-24
Résumés
A 69-year-old man was referred for asymptomatic erythematous pseudo-tumoral nodule on the left malar area evolving for ten years. Systemic examination was unremarkable. The skin biopsy revealing a perivascular and perifollicular inflammatory infiltrate with numerous plasma cells and lymphocytes, a syphilis serological testing was ordered. Serum venereal disease research laboratory (VDRL) test was negative, but Treponema pallidum test using enzyme linked immunosorbent assay (ELISA) method was positive with a ratio of 10.3. A diagnosis of pseudo-tumoral tertiary syphilis was suggested. Three injections of benzathine benzylpenicillin 2.4 million units intramuscularly at weekly interval led to complete healing of the nodular lesion and confirmed the suspected diagnosis. Although syphilis is well known for having a panel of clinical and histological features and “simulates every other disease”1, we describe an unusual pseudo-tumoral tertiary syphilis with complete resolution under appropriate treatment by benzathine benzylpenicillin. In recent years, the incidence of syphilis has increased in Europe, representing a major health problem. Atypical presentations of syphilis should be known by dermatologists and considered as a differential diagnosis for nodular pseudo-tumoral skin lesions.
Un homme de 69 ans était adressé pour la prise en charge d’un nodule pseudo-tumoral érythémateux asymptomatique de la joue gauche évoluant depuis dix ans. Le reste de l’examen clinique était sans particularités notables. La biopsie cutanée révélant un infiltrat inflammatoire périvasculaire et périfolliculaire riche en plasmocytes et en lymphocytes, une sérologie syphilis était prescrite. Le test VDRL (venereal disease research laboratory) était négatif, mais le test spécifique de Treponema pallidum en ELISA (enzyme linked immunosorbent assay) était positif avec un ratio de 10,3. Un diagnostic de syphilis tertiaire pseudo-tumorale était suggéré. Trois injections intra-musculaires de benzathine benzylpénicilline 2,4 millions d’unités à une semaine d’intervalle permettaient une disparition complète de la lésion nodulaire et confirmaient définitivement le diagnostic. Bien que la syphilis soit connue pour avoir un panel de présentations cliniques et histologiques et être « la grande simulatrice1 », nous décrivons une présentation exceptionnelle de syphilis tertiaire pseudo-tumorale de résolution complète sous traitement approprié par benzathine benzylpénicilline. Ces dernières années, l’incidence de la syphilis a augmenté en Europe, représentant un problème majeur de santé publique. Les présentations cliniques atypiques de syphilis devraient être connues des dermatologues afin de l’évoquer en diagnostic différentiel devant une lésion nodulaire pseudo‑tumorale.
Texte intégral
Case Report
1A 69-year-old man was referred for asymptomatic erythematous nodular lesion on the left malar area evolving for ten years (figure 1A). His past medical history was notable for coronary stents placed in the setting of atherosclerosis-related myocardial infarction. Physical examination revealed a painless and not pruritic pseudo-tumoral nodule on the left cheek that slowly enlarged with years despite iterative use of topical corticosteroids and antibiotics. There were neither lesions on mucosal surfaces, palms and soles, nor lymphadenopathy. Systemic examination was unremarkable.
Fig. 1

(A)‑Asymptomatic nodular pseudo-tumoral lesion of the left cheek evolving for ten year
(B) Complete healing of the nodular lesion after three injections of benzathine benzylpenicillin 2.4 million units
2All laboratory tests were within normal limits, including blood count, liver enzymes and creatinine. HIV, HBV, and HCV serological assays were negative. Antinuclear, anti-dsDNA, anti-soluble nuclear antigens and anti-neutrophil cytoplasmic antibodies were all negative. A skin biopsy revealed a perivascular and perifollicular inflammatory infiltrate with numerous plasma cells and lymphocytes (figure 2). The significant presence of plasma cells in skin biopsy prompted us to order a syphilis serological testing. Serum venereal disease research laboratory (VDRL) test was negative, but Treponema pallidum test using enzyme linked immunosorbent assay (ELISA) method was positive with a ratio of 10.3.
Fig. 2

(A) Skin biopsy revealing a perivascular and perifollicular inflammatory infiltrate with numerous plasma cells and lymphocytes (B).
3A diagnosis of pseudo-tumoral tertiary syphilis was suggested.The patient was heterosexual, not married, and didn’t remember any risky sexual exposure. Cerebrospinal fluid (CSF) analysis with CSF-VDRL test were negative. A full-body computed tomography scan didn’t show any systemic involvement. Three injections of benzathine benzylpenicillin 2.4 million units intramuscularly at weekly interval led to complete healing of the nodular lesion (figure 1B), without any Jarisch-Herxheimer reaction, and confirmed the suspected diagnosis. There wasn’t any clinical recurrence on follow-up examination three years later.
Discussion
4Cutaneous lesions revealing tertiary syphilis are very rare. A prospective study following 1978 patients with primary or secondary syphilis from 1891 to 1951 showed that 28% of patients with untreated syphilis would develop late signs such as late cutaneous syphilis (16% of tertiary syphilis), cardiovascular syphilis, or neurosyphilis2. Most of late cutaneous syphilis appear in the superficial, nodulo-ulcerative type or the deeper, more destructive, gummatous type. Histologically, granulomas are confined to the dermis within a lymphocytic and plasma cell infiltrate. The significant presence of plasma cells is characteristic and should bring to mind the diagnosis of syphilis. We present a rare case of pseudo-tumoral facial mass revealing a tertiary cutaneous syphilis. Differential diagnosis could be lupus tumidus, cutaneous lymphoma, sarcoidosis, rhinoscleroma, lepra, or even sporotrichosis3. By reviewing electronic litterature, we could only find three cases of nodular cutaneous tertiary syphilis. These publications didn’t share the clinical features of our case, making it even more unusual and interesting. The first case was a 52-year-old black man with a 10-month history of well-circumscribed pruritic violaceous papules and nodules on the left suprascapular4. The second one was a 57-year-old black woman presenting with enlarging plaques on the face for 30 years, previously diagnosed as discoid lupus erythematosus but unresponsive to hydroxychloroquine and triamcinolone cream5. The third case was a 28-year-old Caucasian man presenting with a two-year history of persistent, multiple raised cutaneous plaques with central clearing, revealing a tertiary cutaneous syphilis with HIV co-infection6. However, by searching in classical textbooks, we have found a case of superficial cutaneous tertiary syphilis of the left cheek, looking similar to our case7.
Conclusion
5Although syphilis is well known for having a panel of clinical and histological features and “simulates every other disease”8 we describe an unusual pseudo-tumoral tertiary syphilis with complete resolution under appropriate treatment by benzathine benzylpenicillin. In recent years, the incidence of syphilis has increased in Europe, representing a major health problem. Atypical presentations of syphilis should be known by dermatologists and considered as a differential diagnosis for nodular pseudo-tumoral skin lesions.
Notes de bas de page
1 W. Osler, Aequanimitas, Philadelphia, Blakiston, 1932, p. 134.
2 E.-G. Clark, N. Danbolt, « The Oslo Study of the natural course of untreated syphilis. An epidemiologic investigation based on a re-study of the Boeck-Bruusgaard material », Medical Clinics of North America, n° 48 (3), 1964, p. 613-623.
3 J. Cappelli, Le infezioni sessuali, Milano, Vallardi, 1955, p. 151.
4 S.-S. Matsuda-John et al., « Nodular late syphilis », Journal of American Academy of Dermatology, n° 9 (2), 1983, p. 269-272.
5 G. Chung et al., « Tertiary syphilis of the face », Journal of American Academy of Dermatology, n° 24 (5), 1991, p. 832-835.
6 TN Revathi et al., « Benign nodular tertiary syphilis: A rare presenting manifestation of HIV infection », Dermatology Online Journal, n° 17 (2), 2011, p. 5.
7 AH. Rudolph, « Syphilis (Figure 21) », in Demis DJ, Clinical Dermatology, Harper and Row, n° 3, 1985, p. 16-22.
8 W. Osler, op. cit. p. 134.
Auteurs
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Michael Benzaquen
Service de dermatologie, CHU Nord, APHM, Aix-Marseille Université, Marseille, France
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Caroline Horreau
Service de dermatologie, CHU Nord, APHM, Aix-Marseille Université, Marseille, France
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Marie-Christine Koeppel
Service de dermatologie, CHU Nord, APHM, Aix-Marseille Université, Marseille, France
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Philippe Berbis
Service de dermatologie, CHU Nord, APHM, Aix-Marseille Université, Marseille, France
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