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Unusual case of cervical syphilis with Piringer-Kuchinka-like lymphadenitis
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British Journal of Oral and Maxillofacial Surgery 52 (2014) e141–e143
Short communication
Unusual case of cervical syphilis with Piringer-Kuchinka-like lymphadenitis Manuel Moreno-Sánchez ∗ , Raúl González-García, Carlos Moreno García, Florencio Monje Department of Oral and Maxillofacial – Head and Neck Surgery, University Hospital Infanta Cristina, Avenida de Elvas s/n, 06080, Badajoz, Spain Accepted 6 September 2014 Available online 29 September 2014
Abstract Syphilis rarely presents with cervical lymphadenopathy. We describe a patient with a cervical mass associated with weight loss, asthenia, and night sweats. The mass was excised and histological examination suggested Piringer-Kuchinka lymphadenitis, which is usually present in cervical toxoplasmosis. To the best of our knowledge, Piringer-Kuchinka lymphadenitis has not previously been reported in cervical syphilis. © 2014 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Keywords: Cervical syphilis; Piringer-Kuchinka lymphadenitis; Cervical mass
Introduction
Case report
Syphilis is a sexually-transmitted disease caused by infection with the spirochete Treponema pallidum.1 The incidence decreased dramatically after the introduction of penicillin for treatment, but the disease began to reappear in the 1990s.2 Its pathogenesis is classified into three stages: primary (a chancre, followed by bloodborne spread of the causative organism); secondary (a subsequent skin rash); and tertiary (the most advanced stage with degeneration of the central nervous and cardiovascular systems).1–4 Most primary lesions are genital (85%) and anal (10%), and it appears only rarely as cervical lymphadenopathy (incidence less than 1%).5,6 Here we report the case of a 35-year-old man with unexplained cervical lymphadenopathy.
The patient was referred to the Department of Oral and Maxillofacial Surgery with a 3-week history of a persistent, isolated mass in the left neck associated with weight loss, asthenia, fever, and night sweats. Physical examination showed a palpable, firm, non-tender mass at level II/III with no adhesions or alteration in skin colour. We found no other lesions in the head and neck. His medical history and review of systems gave no clue to the diagnosis. Computed tomography (CT) of the neck showed a necrotic, left cervical lymph node that was adherent to the internal jugular vein (Fig. 1). These findings suggested a metastatic tumour or abscess. Serological tests showed no sign of Epstein-Barr virus, cytomegalovirus, or toxoplasmosis. Fine needle aspiration was consistent with a reactive lymphadenopathy. The patient then had serological tests for syphilis and HIV. The mass was excised through a lateral cervicotomy approach for diagnostic and therapeutic purposes, though no primary lesion was found (Fig. 2). Histological examination showed epithelioid microgranulomas that were characterised by clusters of epithelioid histiocytes and a few accompanying cells compatible with Piringer-Kuchinka lymphadenitis (Fig. 3).
∗
Corresponding author. Tel.: +34 600063326. E-mail address: [email protected] (M. MorenoSánchez).
http://dx.doi.org/10.1016/j.bjoms.2014.09.004 0266-4356/© 2014 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
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M. Moreno-Sánchez et al. / British Journal of Oral and Maxillofacial Surgery 52 (2014) e141–e143
Fig. 1. Computed tomogram of the neck showing a necrotic left cervical lymph node adherent to the internal jugular vein.
Fig. 2. Operative photograph showing the well-encapsulated cervical mass under the sternocleidomastoid muscle.
No Mycobacteria were detected. The results of the serological tests showed the presence of syphilis but showed no evidence of HIV. Amplified polymerase chain reaction targeting the Tr. pallidum DNA polymerase A confirmed the diagnosis. He was treated with penicillin G intravenously for 14 days. Three months later he reported the successful resolution of all his symptoms.
Discussion Isolated cervical lymphadenopathy in young adults has many underlying causes, including autoimmune diseases, lymphoproliferative disorders, cancers of the head and neck, and infectious diseases,7 and many diseases that present with cervical lymphadenopathy also show similar signs such as fever, asthenia, and weight loss. Persistent cervical lymphadenopathy is often investigated by fine needle aspiration or open biopsy of the lymph nodes. Infectious agents that lead to cervical lymphadenopathy are
Fig. 3. Microgranuloma consisting of small aggregates of epithelioid histiocytes together with scarce accompanying lymphoid cells that are characteristic of Piringer-Kuchinka lymphadenitis (haematoxylin and eosin, original magnification ×40).
usually viruses, bacteria, or Mycobacteria (tuberculosis). Nevertheless, syphilis should also be considered as a possible cause of isolated cervical lymphadenopathy, as we have shown. Other authors have reported similar cases.5,6,8 Lesions have been reported in the head and neck on the lips, tongue, tonsil, and mucosal patches in the aerodigestive tract, nose, and ear (otosyphilis).1 As with our patient, there may be no evidence of a primary chancre. A swelling in the neck may be the only presenting symptom. The firm texture and the non-tenderness of our patient’s lymph node was thought to be in keeping with malignancy and resulted in lymphadenectomy. However, this is also typical for syphilitic lymphadenitis, characterised by a painless, firm, enlargement of the lymph nodes.5 Histologically, the triad of epithelioid microgranulomas, follicular hyperplasia, and monocytoid B cell hyperplasia is known as Piringer-Kuchinka lymphadenitis,9 and usually indicates toxoplasma infection. However, these findings can be associated with other microorganisms such as syphilis, albeit rarely. To the best of our knowledge, Piringer-Kuchinka lymphadenitis has not previously been reported in a cervical syphilis. Clinicians should be wary of syphilis in patients with unexplained lymphadenopathy, including Piringer-Kuchinka lymphadenitis, that usually indicates cervical toxoplasma infection.
Conflict of interest We have no conflict of interest.
Ethics statement/confirmation of patient’s permission Not required.
M. Moreno-Sánchez et al. / British Journal of Oral and Maxillofacial Surgery 52 (2014) e141–e143
References 1. Hook III EW, Marra CM. Acquired syphilis in adults. N Engl J Med 1992;326:1060–9. 2. Little JW. Syphilis: an update. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100:3–9. 3. Read PJ, Donovan B. Clinical aspects of adult syphilis. Intern Med J 2012;42:614–20. 4. Scott CM, Flint SR. Oral syphilis—re-emergence of an old disease with oral manifestations. Int J Oral Maxillofac Surg 2005;34:58–63. 5. Van Crevel R, Grefte JM, van Doorninck D, et al. Syphilis presenting as isolated cervical lymphadenopathy: two related cases. J Infect 2009;58:76–8.
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6. Sato J, Tsubota H, Himi T. Syphilitic cervical lymphadenopathy. Eur Arch Otorhinolaryngol 2003;260:283–5. 7. Anthony PP, Knowles SA. Lymphadenopathy as a primary presenting sign: a clinicopathological study of 228 cases. Br J Surg 1983;70: 412–4. 8. Ikenberg K, Springer E, Bräuninger W, et al. Oropharyngeal lesions and cervical lymphadenopathy: syphilis is a differential diagnosis that is still relevant. J Clin Pathol 2010;63:731–6. 9. Piringer-Kuchinka A, Martin I, Thalhammer O. Superior cerviconuchal lymphadenitis with small groups of epithelioid cell proliferation. Virchows Arch (Pathol Anat) 1958;331:522–35 (in German).
British Journal of Oral and Maxillofacial Surgery 52 (2014) e141–e143
Short communication
Unusual case of cervical syphilis with Piringer-Kuchinka-like lymphadenitis Manuel Moreno-Sánchez ∗ , Raúl González-García, Carlos Moreno García, Florencio Monje Department of Oral and Maxillofacial – Head and Neck Surgery, University Hospital Infanta Cristina, Avenida de Elvas s/n, 06080, Badajoz, Spain Accepted 6 September 2014 Available online 29 September 2014
Abstract Syphilis rarely presents with cervical lymphadenopathy. We describe a patient with a cervical mass associated with weight loss, asthenia, and night sweats. The mass was excised and histological examination suggested Piringer-Kuchinka lymphadenitis, which is usually present in cervical toxoplasmosis. To the best of our knowledge, Piringer-Kuchinka lymphadenitis has not previously been reported in cervical syphilis. © 2014 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
Keywords: Cervical syphilis; Piringer-Kuchinka lymphadenitis; Cervical mass
Introduction
Case report
Syphilis is a sexually-transmitted disease caused by infection with the spirochete Treponema pallidum.1 The incidence decreased dramatically after the introduction of penicillin for treatment, but the disease began to reappear in the 1990s.2 Its pathogenesis is classified into three stages: primary (a chancre, followed by bloodborne spread of the causative organism); secondary (a subsequent skin rash); and tertiary (the most advanced stage with degeneration of the central nervous and cardiovascular systems).1–4 Most primary lesions are genital (85%) and anal (10%), and it appears only rarely as cervical lymphadenopathy (incidence less than 1%).5,6 Here we report the case of a 35-year-old man with unexplained cervical lymphadenopathy.
The patient was referred to the Department of Oral and Maxillofacial Surgery with a 3-week history of a persistent, isolated mass in the left neck associated with weight loss, asthenia, fever, and night sweats. Physical examination showed a palpable, firm, non-tender mass at level II/III with no adhesions or alteration in skin colour. We found no other lesions in the head and neck. His medical history and review of systems gave no clue to the diagnosis. Computed tomography (CT) of the neck showed a necrotic, left cervical lymph node that was adherent to the internal jugular vein (Fig. 1). These findings suggested a metastatic tumour or abscess. Serological tests showed no sign of Epstein-Barr virus, cytomegalovirus, or toxoplasmosis. Fine needle aspiration was consistent with a reactive lymphadenopathy. The patient then had serological tests for syphilis and HIV. The mass was excised through a lateral cervicotomy approach for diagnostic and therapeutic purposes, though no primary lesion was found (Fig. 2). Histological examination showed epithelioid microgranulomas that were characterised by clusters of epithelioid histiocytes and a few accompanying cells compatible with Piringer-Kuchinka lymphadenitis (Fig. 3).
∗
Corresponding author. Tel.: +34 600063326. E-mail address: [email protected] (M. MorenoSánchez).
http://dx.doi.org/10.1016/j.bjoms.2014.09.004 0266-4356/© 2014 The British Association of Oral and Maxillofacial Surgeons. Published by Elsevier Ltd. All rights reserved.
e142
M. Moreno-Sánchez et al. / British Journal of Oral and Maxillofacial Surgery 52 (2014) e141–e143
Fig. 1. Computed tomogram of the neck showing a necrotic left cervical lymph node adherent to the internal jugular vein.
Fig. 2. Operative photograph showing the well-encapsulated cervical mass under the sternocleidomastoid muscle.
No Mycobacteria were detected. The results of the serological tests showed the presence of syphilis but showed no evidence of HIV. Amplified polymerase chain reaction targeting the Tr. pallidum DNA polymerase A confirmed the diagnosis. He was treated with penicillin G intravenously for 14 days. Three months later he reported the successful resolution of all his symptoms.
Discussion Isolated cervical lymphadenopathy in young adults has many underlying causes, including autoimmune diseases, lymphoproliferative disorders, cancers of the head and neck, and infectious diseases,7 and many diseases that present with cervical lymphadenopathy also show similar signs such as fever, asthenia, and weight loss. Persistent cervical lymphadenopathy is often investigated by fine needle aspiration or open biopsy of the lymph nodes. Infectious agents that lead to cervical lymphadenopathy are
Fig. 3. Microgranuloma consisting of small aggregates of epithelioid histiocytes together with scarce accompanying lymphoid cells that are characteristic of Piringer-Kuchinka lymphadenitis (haematoxylin and eosin, original magnification ×40).
usually viruses, bacteria, or Mycobacteria (tuberculosis). Nevertheless, syphilis should also be considered as a possible cause of isolated cervical lymphadenopathy, as we have shown. Other authors have reported similar cases.5,6,8 Lesions have been reported in the head and neck on the lips, tongue, tonsil, and mucosal patches in the aerodigestive tract, nose, and ear (otosyphilis).1 As with our patient, there may be no evidence of a primary chancre. A swelling in the neck may be the only presenting symptom. The firm texture and the non-tenderness of our patient’s lymph node was thought to be in keeping with malignancy and resulted in lymphadenectomy. However, this is also typical for syphilitic lymphadenitis, characterised by a painless, firm, enlargement of the lymph nodes.5 Histologically, the triad of epithelioid microgranulomas, follicular hyperplasia, and monocytoid B cell hyperplasia is known as Piringer-Kuchinka lymphadenitis,9 and usually indicates toxoplasma infection. However, these findings can be associated with other microorganisms such as syphilis, albeit rarely. To the best of our knowledge, Piringer-Kuchinka lymphadenitis has not previously been reported in a cervical syphilis. Clinicians should be wary of syphilis in patients with unexplained lymphadenopathy, including Piringer-Kuchinka lymphadenitis, that usually indicates cervical toxoplasma infection.
Conflict of interest We have no conflict of interest.
Ethics statement/confirmation of patient’s permission Not required.
M. Moreno-Sánchez et al. / British Journal of Oral and Maxillofacial Surgery 52 (2014) e141–e143
References 1. Hook III EW, Marra CM. Acquired syphilis in adults. N Engl J Med 1992;326:1060–9. 2. Little JW. Syphilis: an update. Oral Surg Oral Med Oral Pathol Oral Radiol Endod 2005;100:3–9. 3. Read PJ, Donovan B. Clinical aspects of adult syphilis. Intern Med J 2012;42:614–20. 4. Scott CM, Flint SR. Oral syphilis—re-emergence of an old disease with oral manifestations. Int J Oral Maxillofac Surg 2005;34:58–63. 5. Van Crevel R, Grefte JM, van Doorninck D, et al. Syphilis presenting as isolated cervical lymphadenopathy: two related cases. J Infect 2009;58:76–8.
e143
6. Sato J, Tsubota H, Himi T. Syphilitic cervical lymphadenopathy. Eur Arch Otorhinolaryngol 2003;260:283–5. 7. Anthony PP, Knowles SA. Lymphadenopathy as a primary presenting sign: a clinicopathological study of 228 cases. Br J Surg 1983;70: 412–4. 8. Ikenberg K, Springer E, Bräuninger W, et al. Oropharyngeal lesions and cervical lymphadenopathy: syphilis is a differential diagnosis that is still relevant. J Clin Pathol 2010;63:731–6. 9. Piringer-Kuchinka A, Martin I, Thalhammer O. Superior cerviconuchal lymphadenitis with small groups of epithelioid cell proliferation. Virchows Arch (Pathol Anat) 1958;331:522–35 (in German).