Mondor’s disease of penis: a forgotten disease
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《性传输感染》
1 Department of Dermatology, Venereology and Leprology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
2 Department of Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
ABSTRACT
Background: Mondor’s disease of penis is an uncommon genital condition involving the penile sulcus in a circumferential manner. Although this disorder is almost always self limited, it is associated with considerable psychological stress and sexual disharmony.
Methods: All patients attending the sexually transmitted disease clinic during 1991–2003 were examined for evidence of Mondor’s disease of penis. Detailed history and clinical findings were recorded in a specially designed proforma. Histopathological examination and staining with CD31 and CD34 monoclonal antibodies was also done in 11 patients.
Results: 18 out of 1296 patients attending the sexually transmitted diseases (STD) clinic during the study period were found to have penile Mondor’s disease, giving an incidence of 1.39%. 17 patients had history of one or more episodes of STDs. Histopathological specimens showed prominent vessels with plump endothelial cells and thickened blood vessel walls. The occasional vessel showed complete occlusion of its lumen.
Conclusions: In our study we did not find any evidence of lymphatic involvement. Non-lymphatic vessels, mainly veins, were predominantly involved. In our opinion Mondor’s phlebitis of penis or Mondor’s disease of penis are better terms to describe the condition rather than non-venereal sclerosing lymphangitis.
Abbreviations: STD, sexually transmitted diseases
Keywords: Mondor’s disease; penis; non-venereal sclerosing lymphangitis
Mondor’s disease of penis is an uncommon genital condition presenting as a painless or tender cord-like lesion involving the coronal sulcus in a circumferential manner, or rarely the prepuce and the distal part of the penile shaft. The disorder was originally described by Hoffman1 in 1923, who thought it to be "simulation of primary syphilis by gonorrhoeal lymphangitis." Subsequently various names have been used to describe it such as circular indurated lymphangitis, non-venereal sclerosing lymphangitis of the penis,2 benign transient lymphangiectasis, and localised lymphoedema.3 Braun-Falco4 described for the first time the phlebitis of the dorsal vein of the penis and later it was termed Mondor’s disease of the penis.5 The aetiopathogenesis of this disorder is still controversial as, on histopathology, lymphatic channels as well as non-lymphatic vessels have been equally incriminated.
Most of the studies in the past have reported only a few patients and information from India is scanty. So, this study was undertaken to evaluate Mondor’s disease of penis in our sexually transmitted disease (STD) clinic attendees during past 13 years.
METHODS
All patients attending the STD clinic during 1991–2003 were examined for evidence of Mondor’s disease of penis. Detailed history and clinical findings were recorded in a specially designed proforma which included demographic and socioeconomic data, as well as information on disease related factors like trauma, masturbation, sexual practices, contraceptive measures, and STDs. They were investigated for the presence of any STD by relevant investigations. Biopsy for histopathological examination was done in 11 patients. In addition to the routine haematoxylin and eosin staining the sections were also treated with CD31 and CD34 monoclonal antibodies. No treatments were recommended, although all the patients were told about the transient nature of the disease and reassured.
RESULTS
Eighteen of 1296 patients attending the STD clinic during the study period were found to have Mondor’s disease of penis (incidence 1.39%). The age of the patients ranged from 18–45 years (mean 29.4 years). Three of these 18 patients were married. All the patients were uncircumcised. Duration of lesions at presentation varied from 1–4 weeks (mean 2.3 weeks).
Fifteen patients had one or more episodes of STDs in the past (table 1). The time between the episode of STD and their visit to the clinic varied from 4–12 weeks (mean 6.8 weeks). Two patients, one each with genital warts and chancroid, developed lesions while undergoing treatment.
The majority of the patients were asymptomatic; only six (33.3%) patients had mild to moderate pain during erection. Most of the patients became aware of the lesions during local cleansing, foreplay, or subsequent sexual activity. None of the patients experienced local oedema during onset or in the course of the disease. All the patients denied homosexual activity. The details of the sexual behaviour/attributes of the patients are given in table 2.
All the patients presented with subcutaneous, skin coloured, cord-like thickening on the penis. In 15 patients the lesions were parallel to the coronal sulcus (giving the appearance of a double coronal sulcus) (fig 1) and three patients had lesions in the midline over dorsum of penis. The lesions varied in size from 1–3 cm. Tenderness could be elicited in only two patients.
HIV serology was negative in all patients while VDRL was still reactive in two patients with a previous history of syphilis (1:2 and 1:4 dilutions). None of our patients had any other local or systemic diseases including diabetes mellitus or hypertension.
Histopathological study was carried out in 11 cases and all the specimens showed prominent vessels with plump endothelial cells, proliferation of connective tissue of the vessel wall, and partially granulating thrombus (fig 2). Veins were found to be sclerosed in six patients while one showed sclerosis of both arteries and veins. No pathology in lymphatics could be visualised in any of the specimens. Sparse perivascular cellular infiltrate composed of lymphocytes, histiocytes, and plasma cells was noticed in the dermis. The intimal layer and tunica media of blood vessels could be easily differentiated in seven specimens with elastic tissue stain (elastic Van Gieson). CD31 and CD34 monoclonal antibodies revealed endothelin staining in all these patients.
Spontaneous clearing of lesions occurred in all patients over a period of 4–8 weeks (mean 5.6 weeks). None had any recurrence of similar lesion over the 4–6 month follow up period.
DISCUSSION
Penile Mondor’s disease is a benign pathology of the superficial dorsal penile vein affecting sexually active men.2 In this study most of the subjects were young and under 45 years of age. Although lesions are classically asymptomatic, some patients have pain or discomfort, especially on erection.5,6 The majority of our patients were asymptomatic and only one third presented with mild to moderate pain and tenderness
The cause of penile Mondor’s disease remains unclear. Non-venereal causes such as enteroviral infection, irritation from menstrual blood, tuberculosis and circumferential scarring from circumcision have been suggested but there has been no clinical or investigational support.2,7 Mechanical trauma has been most frequently postulated theory.8,9 Patients have a history of increased sexual activity immediately before the appearance of the lesion. Usually the lesions occur shortly (24–48 hours) after sexual intercourse. The majority of the reports indicate that temporary abstinence results in prompt involution of lesions and return to sexual activity can lead to recurrence. Most of our patients had history of abstinence of few weeks because of the past STDs. However, absence of recurrence after resumption of the sexual activity indicates that trauma may not be the only factor or the different aetiological factors might result in a common response.
Various STDs have been associated with this condition.3,6 Although one fourth of the 105 previously reported cases have demonstrated a close temporal relation to STDs, it is difficult to determine whether these patients have a greater incidence of STDs than an otherwise analogous at-risk population or that frequent sexual activity exposes them to both the acquisition of an STD and development of Mondor’s phlebitis.
Controversy regarding the lymphatic versus venous origin of this condition has been discussed in the literature.5,10,11 Lymphatics were not involved in any of our patients; the veins were predominantly involved which is in consonance in some of the previous studies.5,10–12 The striking feature noticed in all patients was plump endothelial cells (lymphatic vessels–flat endothelial cells). We could easily differentiate the intimal layer from connective tissue in the blood vessel lumen with elastic tissue stain (loss of differentiation between the intimal layer and connective tissue). The positive staining of vascular endothelial cells on staining with CD31 and CD34 monoclonal antibodies also hints towards a venous pathology. But anatomical and histological differentiation between the veins and lymphatic vessels of the penis can sometimes be difficult and this issue can only be settled by penile sonography with colour Doppler imaging5 and endothelin staining. In our opinion "Mondor’s disease of penis" or "Mondor’s phlebitis of penis" are better terms for description of the condition rather than non-venereal sclerosing lymphangitis.
Mondor’s disease of penis is a rare condition often associated with significant morbidity and the attending physicians should be aware of this benign, self resolving condition to prevent misdiagnosis and overaggressive treatment with its attendant physical and emotional trauma. We believe proper explanation, strong reassurance, and temporary sexual abstinence should remain the mainstay of therapy, unless the disease is very symptomatic or the lesions persist for a longer than average healing time.
CONTRIBUTORS
BK, clinical evaluation; SG, clinical evaluation; TN, clinical evaluation and data analysis; BDR, histopathology and special staining.
REFERENCES
Hoffmann E. Vortauschung primarer syphilis durch gonosshvische lymphangitis (gonorrhoischer psuedoprimaraffekt). Munch Med Wschr 1923;70:1167–8.
Nickel WR, Plumb RT. Nonvenereal sclerosing lymphangitis of the penis. Arch Dermatol 1962;86:761–3.
McMillan A. Lymphocoele and localized lymphoedema of the penis. Br J Vener Dis 1976;52:409–11.
Braun-Falco O. Sur Klinik, Histologie und Pathogenese der strangformigen, oberflachlichen phlebitiden. Dermotol Wochenschr 1955;132:705–15.
Sasso F, Gulino G, Alcini TP. Penile Mondor’s disease: an under estimated pathology. Br J Urol 1996;77:729–32.
Von Berde K. Further contribution to the knowledge of the nonvenereal diseases: lymphangitis of the coronary sulcus. Dermatol Wochenschr 1937;105:1532–40.
Broaddus SB, Leadbetter GW. Surgical management of persistent, symptomatic nonvenereal sclerosing lymphangitis of the penis. J Urol 1982;127:987–8.
Sieunarine K. Non-venereal sclerosing lymphangitis of the penis associated with masturbation. Br J Urol 1987;59:194–5.
Lassus A, Niemi KM, Valle SL, et al. Sclerosing lymphangitis of the penis. Br J Vener Dis 1972;48:545–8.
Aragona P, Piazza R, Artibani W, et al. The so-called Hoffman’s lymphangitis of the penis: is it a lymphangitis or phlebitis? Int Urol Nephrol 1988;20:139–45.
Findlay GH, Whiting DA. Mondor’s phlebitis of the penis. A condition miscalled ‘non-venereal sclerosing lymphangitis’. Clin Exp Dermatol 1977;2:65–7.
Tanii T, Hamada T, Asai, et al. Mondor’s phlebitis of the penis: to study with factor VIII related antigen. Acta Derm Venereol 1984;64:337–40.(B Kumar, T Narang, B D Ra)
2 Department of Pathology, Postgraduate Institute of Medical Education and Research, Chandigarh, India
ABSTRACT
Background: Mondor’s disease of penis is an uncommon genital condition involving the penile sulcus in a circumferential manner. Although this disorder is almost always self limited, it is associated with considerable psychological stress and sexual disharmony.
Methods: All patients attending the sexually transmitted disease clinic during 1991–2003 were examined for evidence of Mondor’s disease of penis. Detailed history and clinical findings were recorded in a specially designed proforma. Histopathological examination and staining with CD31 and CD34 monoclonal antibodies was also done in 11 patients.
Results: 18 out of 1296 patients attending the sexually transmitted diseases (STD) clinic during the study period were found to have penile Mondor’s disease, giving an incidence of 1.39%. 17 patients had history of one or more episodes of STDs. Histopathological specimens showed prominent vessels with plump endothelial cells and thickened blood vessel walls. The occasional vessel showed complete occlusion of its lumen.
Conclusions: In our study we did not find any evidence of lymphatic involvement. Non-lymphatic vessels, mainly veins, were predominantly involved. In our opinion Mondor’s phlebitis of penis or Mondor’s disease of penis are better terms to describe the condition rather than non-venereal sclerosing lymphangitis.
Abbreviations: STD, sexually transmitted diseases
Keywords: Mondor’s disease; penis; non-venereal sclerosing lymphangitis
Mondor’s disease of penis is an uncommon genital condition presenting as a painless or tender cord-like lesion involving the coronal sulcus in a circumferential manner, or rarely the prepuce and the distal part of the penile shaft. The disorder was originally described by Hoffman1 in 1923, who thought it to be "simulation of primary syphilis by gonorrhoeal lymphangitis." Subsequently various names have been used to describe it such as circular indurated lymphangitis, non-venereal sclerosing lymphangitis of the penis,2 benign transient lymphangiectasis, and localised lymphoedema.3 Braun-Falco4 described for the first time the phlebitis of the dorsal vein of the penis and later it was termed Mondor’s disease of the penis.5 The aetiopathogenesis of this disorder is still controversial as, on histopathology, lymphatic channels as well as non-lymphatic vessels have been equally incriminated.
Most of the studies in the past have reported only a few patients and information from India is scanty. So, this study was undertaken to evaluate Mondor’s disease of penis in our sexually transmitted disease (STD) clinic attendees during past 13 years.
METHODS
All patients attending the STD clinic during 1991–2003 were examined for evidence of Mondor’s disease of penis. Detailed history and clinical findings were recorded in a specially designed proforma which included demographic and socioeconomic data, as well as information on disease related factors like trauma, masturbation, sexual practices, contraceptive measures, and STDs. They were investigated for the presence of any STD by relevant investigations. Biopsy for histopathological examination was done in 11 patients. In addition to the routine haematoxylin and eosin staining the sections were also treated with CD31 and CD34 monoclonal antibodies. No treatments were recommended, although all the patients were told about the transient nature of the disease and reassured.
RESULTS
Eighteen of 1296 patients attending the STD clinic during the study period were found to have Mondor’s disease of penis (incidence 1.39%). The age of the patients ranged from 18–45 years (mean 29.4 years). Three of these 18 patients were married. All the patients were uncircumcised. Duration of lesions at presentation varied from 1–4 weeks (mean 2.3 weeks).
Fifteen patients had one or more episodes of STDs in the past (table 1). The time between the episode of STD and their visit to the clinic varied from 4–12 weeks (mean 6.8 weeks). Two patients, one each with genital warts and chancroid, developed lesions while undergoing treatment.
The majority of the patients were asymptomatic; only six (33.3%) patients had mild to moderate pain during erection. Most of the patients became aware of the lesions during local cleansing, foreplay, or subsequent sexual activity. None of the patients experienced local oedema during onset or in the course of the disease. All the patients denied homosexual activity. The details of the sexual behaviour/attributes of the patients are given in table 2.
All the patients presented with subcutaneous, skin coloured, cord-like thickening on the penis. In 15 patients the lesions were parallel to the coronal sulcus (giving the appearance of a double coronal sulcus) (fig 1) and three patients had lesions in the midline over dorsum of penis. The lesions varied in size from 1–3 cm. Tenderness could be elicited in only two patients.
HIV serology was negative in all patients while VDRL was still reactive in two patients with a previous history of syphilis (1:2 and 1:4 dilutions). None of our patients had any other local or systemic diseases including diabetes mellitus or hypertension.
Histopathological study was carried out in 11 cases and all the specimens showed prominent vessels with plump endothelial cells, proliferation of connective tissue of the vessel wall, and partially granulating thrombus (fig 2). Veins were found to be sclerosed in six patients while one showed sclerosis of both arteries and veins. No pathology in lymphatics could be visualised in any of the specimens. Sparse perivascular cellular infiltrate composed of lymphocytes, histiocytes, and plasma cells was noticed in the dermis. The intimal layer and tunica media of blood vessels could be easily differentiated in seven specimens with elastic tissue stain (elastic Van Gieson). CD31 and CD34 monoclonal antibodies revealed endothelin staining in all these patients.
Spontaneous clearing of lesions occurred in all patients over a period of 4–8 weeks (mean 5.6 weeks). None had any recurrence of similar lesion over the 4–6 month follow up period.
DISCUSSION
Penile Mondor’s disease is a benign pathology of the superficial dorsal penile vein affecting sexually active men.2 In this study most of the subjects were young and under 45 years of age. Although lesions are classically asymptomatic, some patients have pain or discomfort, especially on erection.5,6 The majority of our patients were asymptomatic and only one third presented with mild to moderate pain and tenderness
The cause of penile Mondor’s disease remains unclear. Non-venereal causes such as enteroviral infection, irritation from menstrual blood, tuberculosis and circumferential scarring from circumcision have been suggested but there has been no clinical or investigational support.2,7 Mechanical trauma has been most frequently postulated theory.8,9 Patients have a history of increased sexual activity immediately before the appearance of the lesion. Usually the lesions occur shortly (24–48 hours) after sexual intercourse. The majority of the reports indicate that temporary abstinence results in prompt involution of lesions and return to sexual activity can lead to recurrence. Most of our patients had history of abstinence of few weeks because of the past STDs. However, absence of recurrence after resumption of the sexual activity indicates that trauma may not be the only factor or the different aetiological factors might result in a common response.
Various STDs have been associated with this condition.3,6 Although one fourth of the 105 previously reported cases have demonstrated a close temporal relation to STDs, it is difficult to determine whether these patients have a greater incidence of STDs than an otherwise analogous at-risk population or that frequent sexual activity exposes them to both the acquisition of an STD and development of Mondor’s phlebitis.
Controversy regarding the lymphatic versus venous origin of this condition has been discussed in the literature.5,10,11 Lymphatics were not involved in any of our patients; the veins were predominantly involved which is in consonance in some of the previous studies.5,10–12 The striking feature noticed in all patients was plump endothelial cells (lymphatic vessels–flat endothelial cells). We could easily differentiate the intimal layer from connective tissue in the blood vessel lumen with elastic tissue stain (loss of differentiation between the intimal layer and connective tissue). The positive staining of vascular endothelial cells on staining with CD31 and CD34 monoclonal antibodies also hints towards a venous pathology. But anatomical and histological differentiation between the veins and lymphatic vessels of the penis can sometimes be difficult and this issue can only be settled by penile sonography with colour Doppler imaging5 and endothelin staining. In our opinion "Mondor’s disease of penis" or "Mondor’s phlebitis of penis" are better terms for description of the condition rather than non-venereal sclerosing lymphangitis.
Mondor’s disease of penis is a rare condition often associated with significant morbidity and the attending physicians should be aware of this benign, self resolving condition to prevent misdiagnosis and overaggressive treatment with its attendant physical and emotional trauma. We believe proper explanation, strong reassurance, and temporary sexual abstinence should remain the mainstay of therapy, unless the disease is very symptomatic or the lesions persist for a longer than average healing time.
CONTRIBUTORS
BK, clinical evaluation; SG, clinical evaluation; TN, clinical evaluation and data analysis; BDR, histopathology and special staining.
REFERENCES
Hoffmann E. Vortauschung primarer syphilis durch gonosshvische lymphangitis (gonorrhoischer psuedoprimaraffekt). Munch Med Wschr 1923;70:1167–8.
Nickel WR, Plumb RT. Nonvenereal sclerosing lymphangitis of the penis. Arch Dermatol 1962;86:761–3.
McMillan A. Lymphocoele and localized lymphoedema of the penis. Br J Vener Dis 1976;52:409–11.
Braun-Falco O. Sur Klinik, Histologie und Pathogenese der strangformigen, oberflachlichen phlebitiden. Dermotol Wochenschr 1955;132:705–15.
Sasso F, Gulino G, Alcini TP. Penile Mondor’s disease: an under estimated pathology. Br J Urol 1996;77:729–32.
Von Berde K. Further contribution to the knowledge of the nonvenereal diseases: lymphangitis of the coronary sulcus. Dermatol Wochenschr 1937;105:1532–40.
Broaddus SB, Leadbetter GW. Surgical management of persistent, symptomatic nonvenereal sclerosing lymphangitis of the penis. J Urol 1982;127:987–8.
Sieunarine K. Non-venereal sclerosing lymphangitis of the penis associated with masturbation. Br J Urol 1987;59:194–5.
Lassus A, Niemi KM, Valle SL, et al. Sclerosing lymphangitis of the penis. Br J Vener Dis 1972;48:545–8.
Aragona P, Piazza R, Artibani W, et al. The so-called Hoffman’s lymphangitis of the penis: is it a lymphangitis or phlebitis? Int Urol Nephrol 1988;20:139–45.
Findlay GH, Whiting DA. Mondor’s phlebitis of the penis. A condition miscalled ‘non-venereal sclerosing lymphangitis’. Clin Exp Dermatol 1977;2:65–7.
Tanii T, Hamada T, Asai, et al. Mondor’s phlebitis of the penis: to study with factor VIII related antigen. Acta Derm Venereol 1984;64:337–40.(B Kumar, T Narang, B D Ra)