Dermatologic Therapy, Vol. 26, 2013, 486–488 Printed in the United States · All rights reserved
© 2013 Wiley Periodicals, Inc.
DERMATOLOGIC THERAPY ISSN 1396-0296
THERAPEUTIC HOTLINE A case of tertiary neurosyphilis presenting with moth-eaten bone lesions Xue-Gang Xu*†, Yuan-Hong Li†, Xing-Hua Gao†, Hong-Duo Chen† & Ya-Qin Zhang* *Department of Dermatology, The Second Hospital of Jilin University, Changchun, and †Department of Dermatology, No.1 Hospital of China Medical University, Shenyang, China
ABSTRACT: Syphilis, the “great imitator,” with regard to skin diseases, is a chronic systemic infectious disease with a clinical course that waxes and wanes. The incidence of tertiary syphilis had decreased drastically these decades. We report a case of tertiary neurosyphilis presenting with moth-eaten bone lesions of the lower extremities. To the best of our knowledge, we have not seen such reports. KEYWORDS: bone lesions, tertiary syphilis, Treponema pallidum
Introduction
Case report
Tertiary syphilis is a rare systemic disease that may present with mucocutaneous, cardiac, ophthalmologic, neurologic, or osseous abnormalities. Though the incidence of primary and secondary syphilis is still increasing these years, the incidence of tertiary syphilis had remarkably decreased due to the widespread use of penicillin. Physicians may therefore fail to recognize its clinical features. To highlight this problem, we present the clinical features of a patient with a recent diagnosis of tertiary syphilis.
A 40-year-old female presented for evaluation of a 2 years history of numerous nonitchy erythematous plaques and nodules on the face, chest, and back along with intense pain in the upper and lower extremities. Scattered erythematous plaques and papules presented on her face 4 years ago. Two years earlier, numerous scattered erythemas gradually arose on the trunk accompanied with intense pain in the extremities, which was more severe by degrees and did not improve with oral antirheumatic medicines. The movement of the extremities gradually limited associating with vision weakness during the last year. Physical examination revealed an afebrile patient with poor general condition and both eyes’ vision was 0.2. Dermatological examination revealed gummatou lesions: numerous, nonpruritic, variably sized, solid, dull-red plaques, and papulonodular lesions without significant scaling disseminated on the face and trunk. Large nodular lesions were present on the face especially on the peri-nose areas about
Address correspondence and reprint requests to: Ya-Qin Zhang, MD, PhD, Associate Professor, Department of Dermatology, The Second Hospital of Jilin University, 218 Zi-qiang Street, Changchun, 130041, China, or email: [email protected]. Co-correspondence: Hong-Duo Chen, MD, Department of Dermatology, No.1 Hospital of China Medical University, 155 North Nanjing Street, Shenyang, 110001, China, or email: [email protected]. Disclosure statement: The authors disclose no potential financial conflict of interest with this study.
486
Tertiary neurosyphilis
FIG. 1. Skin eruptions of the face.
1 cm in diameter (FIG. 1). The upper and lower extremities severely swelled with significant tenderness. On enquiry, the patient admitted having extramarital sexual contact with a new partner 4 years ago after her divorce 10 years ago. Laboratory data disclosed the following values: Treponema pallidum hemagglutination assay (TPHA) test was positive and rapid plasma reagin (RPR) test was 1 : 128; cerebrospinal fluid tests: Pandy’s test (+), TPHA (+), increasing leukocytes of 8 ¥ 106/L, protein 815 mg/L (80–430 mg/L), glucose 2.56 mmol/L (2.5–4.4 mmol/L), Cl- 106 mmol/L (118–129 mmol/L). Results of angiography of both optical fundus showed thinner and stenosis of the arteries. X-ray imaging showed moth-eaten damages of bone cortex (FIG. 2) and periosteal proliferation of the long bones of the upper extremities (FIG. 3). Results of Doppler echocardiography were normal. Diagnosis of tertiary neurosyphilis with bone lesions and ocular complications was determined. Then she was given penicillin G 1, 800 U/day for 14 days and then intramuscular benzathine penicillin 2.4 M units weekly for 3 weeks. There was a marked clinical response posttreatment and a gradual resolution of papulonodular eruptions, vision weakness, and bone tenderness. Three months later, RPR titer was negative and TPHA was positive, and the patient was still in follow-up.
Discussion There has been a dramatic emergence of syphilis in China during these years. The incidence of syphilis was increasing from 0.07/100 000 in 1991 to 19.49/ 100 000 in 2008 in China. The presentation of syphilis, often described as the great mimic, can be
FIG. 2. X-ray manifestations of the lower extremities (white arrow: moth-eaten lesions).
FIG. 3. X-ray manifestations of the upper extremities (arrow: periosteal proliferation).
varied and atypical. Early recognition of the clinical manifestations of syphilis is important for the treatment, recovery of patients and the prevention of this disease. The tertiary stage may begin as early
487
Xu et al.
as 1 year after infection or at any time during an infected person’s lifetime. The incidence of tertiary syphilis has decreased drastically with the use of penicillin for the treatment of primary and secondary stages (1,2). Over the past 20 years, however, cases have been reported sporadically (3–6). Tertiary syphilis may present with mucocutaneous, cardiac, ophthalmologic, neurologic, or osseous abnormalities. Skin lesions may appear as nodular, noduloulcerative, or gummatous. The nodules appear in groups, asymmetrical in distribution, and may heal with atrophic, noncontractile scars, with peripheral hyperpigmentation. Bone lesions are marked by osteitis with periosteal changes involving the hard palate, nasal septum (formation of “saddle nose”), tibia, and clavicle, with nocturnal pain and local swelling. Cardiovascular syphilis occurs approximately in 10% of cases of untreated syphilis, including aortic aneurysms, aortic insufficiency, coronary stenosis, and myocarditis. Neurosyphilis includes five major categories as: asymptomatic, meningeal, meningovascular, parenchymatous, and gummatous. In our case, the patient presented as erythematous plaques and papulonodules on the trunk and face with ophthalmologic syphilis, neurologic syphilis, and rarely seen moth-eaten osseous abnormalities. And these damages gradually resolved after treating with penicillin, which also confirmed the diagnosis. Bone lesions in tertiary syphilis are similar to these in secondary syphilis (1,7), and are more severe, which may cause pathologic fracture or joint malformation. Bone involvement often occurs in the long bones like shins, accompanied by swelling and tenderness. Skulls
488
are also commonly involved with severe headache, which may be mistaken for meningism and so on. The gummatous lesions can also affect the bones. Plain radiographs, computed tomography scans, and isotope bone scans are useful in clarifying the lesions. The incidence of syphilis has dramatically increased in the past decade (8,9). It is imperative that we refamiliarize ourselves with the features of this disease. We highlight this case to illustrate that bone pain without improvement with common treatments should prompt a search for syphilis.
References 1. Dourmishev LA, Dourmishev AL. Syphilis: uncommon presentations in adults. Clin Dermatol 2005: 23: 555–564. 2. Hercogova J, Vanousova D. Syphilis and borreliosis during pregnancy. Dermatol Ther 2008: 21: 205–209. 3. Pereira TM, Fernandes JC, Vieira AP, et al. Tertiary syphilis. Int J Dermatol 2007: 46: 1192–1195. 4. Dahmani O, Target N, Guy JP, et al. Progressive painless lower limbs weakness in a dialyzed patient: undiagnosed tertiary syphilis: a case report. Cases Journal 2010: 3: 23. 5. Jones S, Nguyen EQ, Nielsen TA, et al. Nodular tertiary syphilis mimicking granuloma annulare. J Am Acad Dermatol 2000: 42: 378–380. 6. Schramm M, Blume U, Kruger K, et al. Two recent cases of tertiary syphilis. Eur J Dermatol 1999: 9: 300–302. 7. Coyne K, Browne R, Anagnostopoulos C, Nwokolo N. Syphilitic periostitis in a newly diagnosed HIV-positive man. Int J STD AIDS 2006: 17: 421–423. 8. Righarts AA, Simms I, Wallace L, et al. Syphilis surveillance and epidemiology in the United Kingdom. Eurosurveillance Monthly Arch 2004: 9: 15–16. 9. Mackenzie H, Mahmalji W, Raza A. The gumma and the gonad: syphilitic orchitis, a rare presentation of testicular swelling. Int J STD AIDS 2011: 22: 531–533.
© 2013 Wiley Periodicals, Inc.
DERMATOLOGIC THERAPY ISSN 1396-0296
THERAPEUTIC HOTLINE A case of tertiary neurosyphilis presenting with moth-eaten bone lesions Xue-Gang Xu*†, Yuan-Hong Li†, Xing-Hua Gao†, Hong-Duo Chen† & Ya-Qin Zhang* *Department of Dermatology, The Second Hospital of Jilin University, Changchun, and †Department of Dermatology, No.1 Hospital of China Medical University, Shenyang, China
ABSTRACT: Syphilis, the “great imitator,” with regard to skin diseases, is a chronic systemic infectious disease with a clinical course that waxes and wanes. The incidence of tertiary syphilis had decreased drastically these decades. We report a case of tertiary neurosyphilis presenting with moth-eaten bone lesions of the lower extremities. To the best of our knowledge, we have not seen such reports. KEYWORDS: bone lesions, tertiary syphilis, Treponema pallidum
Introduction
Case report
Tertiary syphilis is a rare systemic disease that may present with mucocutaneous, cardiac, ophthalmologic, neurologic, or osseous abnormalities. Though the incidence of primary and secondary syphilis is still increasing these years, the incidence of tertiary syphilis had remarkably decreased due to the widespread use of penicillin. Physicians may therefore fail to recognize its clinical features. To highlight this problem, we present the clinical features of a patient with a recent diagnosis of tertiary syphilis.
A 40-year-old female presented for evaluation of a 2 years history of numerous nonitchy erythematous plaques and nodules on the face, chest, and back along with intense pain in the upper and lower extremities. Scattered erythematous plaques and papules presented on her face 4 years ago. Two years earlier, numerous scattered erythemas gradually arose on the trunk accompanied with intense pain in the extremities, which was more severe by degrees and did not improve with oral antirheumatic medicines. The movement of the extremities gradually limited associating with vision weakness during the last year. Physical examination revealed an afebrile patient with poor general condition and both eyes’ vision was 0.2. Dermatological examination revealed gummatou lesions: numerous, nonpruritic, variably sized, solid, dull-red plaques, and papulonodular lesions without significant scaling disseminated on the face and trunk. Large nodular lesions were present on the face especially on the peri-nose areas about
Address correspondence and reprint requests to: Ya-Qin Zhang, MD, PhD, Associate Professor, Department of Dermatology, The Second Hospital of Jilin University, 218 Zi-qiang Street, Changchun, 130041, China, or email: [email protected]. Co-correspondence: Hong-Duo Chen, MD, Department of Dermatology, No.1 Hospital of China Medical University, 155 North Nanjing Street, Shenyang, 110001, China, or email: [email protected]. Disclosure statement: The authors disclose no potential financial conflict of interest with this study.
486
Tertiary neurosyphilis
FIG. 1. Skin eruptions of the face.
1 cm in diameter (FIG. 1). The upper and lower extremities severely swelled with significant tenderness. On enquiry, the patient admitted having extramarital sexual contact with a new partner 4 years ago after her divorce 10 years ago. Laboratory data disclosed the following values: Treponema pallidum hemagglutination assay (TPHA) test was positive and rapid plasma reagin (RPR) test was 1 : 128; cerebrospinal fluid tests: Pandy’s test (+), TPHA (+), increasing leukocytes of 8 ¥ 106/L, protein 815 mg/L (80–430 mg/L), glucose 2.56 mmol/L (2.5–4.4 mmol/L), Cl- 106 mmol/L (118–129 mmol/L). Results of angiography of both optical fundus showed thinner and stenosis of the arteries. X-ray imaging showed moth-eaten damages of bone cortex (FIG. 2) and periosteal proliferation of the long bones of the upper extremities (FIG. 3). Results of Doppler echocardiography were normal. Diagnosis of tertiary neurosyphilis with bone lesions and ocular complications was determined. Then she was given penicillin G 1, 800 U/day for 14 days and then intramuscular benzathine penicillin 2.4 M units weekly for 3 weeks. There was a marked clinical response posttreatment and a gradual resolution of papulonodular eruptions, vision weakness, and bone tenderness. Three months later, RPR titer was negative and TPHA was positive, and the patient was still in follow-up.
Discussion There has been a dramatic emergence of syphilis in China during these years. The incidence of syphilis was increasing from 0.07/100 000 in 1991 to 19.49/ 100 000 in 2008 in China. The presentation of syphilis, often described as the great mimic, can be
FIG. 2. X-ray manifestations of the lower extremities (white arrow: moth-eaten lesions).
FIG. 3. X-ray manifestations of the upper extremities (arrow: periosteal proliferation).
varied and atypical. Early recognition of the clinical manifestations of syphilis is important for the treatment, recovery of patients and the prevention of this disease. The tertiary stage may begin as early
487
Xu et al.
as 1 year after infection or at any time during an infected person’s lifetime. The incidence of tertiary syphilis has decreased drastically with the use of penicillin for the treatment of primary and secondary stages (1,2). Over the past 20 years, however, cases have been reported sporadically (3–6). Tertiary syphilis may present with mucocutaneous, cardiac, ophthalmologic, neurologic, or osseous abnormalities. Skin lesions may appear as nodular, noduloulcerative, or gummatous. The nodules appear in groups, asymmetrical in distribution, and may heal with atrophic, noncontractile scars, with peripheral hyperpigmentation. Bone lesions are marked by osteitis with periosteal changes involving the hard palate, nasal septum (formation of “saddle nose”), tibia, and clavicle, with nocturnal pain and local swelling. Cardiovascular syphilis occurs approximately in 10% of cases of untreated syphilis, including aortic aneurysms, aortic insufficiency, coronary stenosis, and myocarditis. Neurosyphilis includes five major categories as: asymptomatic, meningeal, meningovascular, parenchymatous, and gummatous. In our case, the patient presented as erythematous plaques and papulonodules on the trunk and face with ophthalmologic syphilis, neurologic syphilis, and rarely seen moth-eaten osseous abnormalities. And these damages gradually resolved after treating with penicillin, which also confirmed the diagnosis. Bone lesions in tertiary syphilis are similar to these in secondary syphilis (1,7), and are more severe, which may cause pathologic fracture or joint malformation. Bone involvement often occurs in the long bones like shins, accompanied by swelling and tenderness. Skulls
488
are also commonly involved with severe headache, which may be mistaken for meningism and so on. The gummatous lesions can also affect the bones. Plain radiographs, computed tomography scans, and isotope bone scans are useful in clarifying the lesions. The incidence of syphilis has dramatically increased in the past decade (8,9). It is imperative that we refamiliarize ourselves with the features of this disease. We highlight this case to illustrate that bone pain without improvement with common treatments should prompt a search for syphilis.
References 1. Dourmishev LA, Dourmishev AL. Syphilis: uncommon presentations in adults. Clin Dermatol 2005: 23: 555–564. 2. Hercogova J, Vanousova D. Syphilis and borreliosis during pregnancy. Dermatol Ther 2008: 21: 205–209. 3. Pereira TM, Fernandes JC, Vieira AP, et al. Tertiary syphilis. Int J Dermatol 2007: 46: 1192–1195. 4. Dahmani O, Target N, Guy JP, et al. Progressive painless lower limbs weakness in a dialyzed patient: undiagnosed tertiary syphilis: a case report. Cases Journal 2010: 3: 23. 5. Jones S, Nguyen EQ, Nielsen TA, et al. Nodular tertiary syphilis mimicking granuloma annulare. J Am Acad Dermatol 2000: 42: 378–380. 6. Schramm M, Blume U, Kruger K, et al. Two recent cases of tertiary syphilis. Eur J Dermatol 1999: 9: 300–302. 7. Coyne K, Browne R, Anagnostopoulos C, Nwokolo N. Syphilitic periostitis in a newly diagnosed HIV-positive man. Int J STD AIDS 2006: 17: 421–423. 8. Righarts AA, Simms I, Wallace L, et al. Syphilis surveillance and epidemiology in the United Kingdom. Eurosurveillance Monthly Arch 2004: 9: 15–16. 9. Mackenzie H, Mahmalji W, Raza A. The gumma and the gonad: syphilitic orchitis, a rare presentation of testicular swelling. Int J STD AIDS 2011: 22: 531–533.
