Post-circumcision non-venereal sclerosing lymphangitis

A 22-year-old male presented with complaints of an asymptomatic cord-like swelling over the penis since six months. Although the lesion was painless, the appearance of the lesion worried him. The patient had a history of repeatedly retracting remnant foreskin (post circumcision) over the shaft of the penis. The patient was otherwise healthy and did not give any history of vigorous sexual activity, penile manipulation, penile or scrotal swellings, urethral discharge, genital ulcers, or prior lesions of similar nature. Examination revealed a single 1.5 mm wide non-tender skin-colored serpiginous cord-like induration on the lateral aspect of the penis just below the coronal sulcus [Figure 1a-c]. There were no signs of inflammation, and the overlying skin was normal and freely mobile. Routine hematological and urine investigations, as well as a screening test for sexually transmitted infections were negative. The close differential diagnosis for our patient is Mondor’s disease which is characterized by an initial presentation of severe pain and erythema; in contrast, our patient had a chronic course of illness and was completely asymptomatic. On cutaneous examination, thrombophlebitis of the penis is usually seen on the dorsum of the penis with a thrombosed vessel adhered to the penile skin covering it. In contrast, non-venereal sclerosing lymphangitis will have a serpiginous morphology with freely mobile overlying skin.^[1] The diagnosis of non-venereal sclerosing lymphangitis was considered in view of the distinct clinical appearance of painless serpiginous cord-like induration following circumcision.^[2] The patient was advised to abstain from the habit of repeated retraction of remnant foreskin and was counseled about the self-limiting benign nature of the disease.

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Figure 1:

(a-c) Presence of single 1.5 mm wide non-tender skin colored serpiginous cord-like induration on lateral aspect of penis just below the coronal sulcus.

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Non-venereal sclerosing lymphangitis also known as “gonorrheal pseudochancre,” “non-venereal plastic lymphangitis of the penis,” or “benign transient lymphangiectasias of the penis” is a benign condition of the penis caused by lymphatic channel blockage.^[1] It usually manifests as a painless skin-colored, firm, translucent, linear, and cord-like enlargement around the coronal sulcus, with the overlying skin being freely movable and not associated with erosions or ulcers. One of the most frequent risk factors is repeated trauma from intense sexual activity. Furthermore, sexually transmitted infections and circumcision might also be contributing factors. The main differential diagnosis is thrombophlebitis of superficial dorsal penile vein also known as penile Mondor’s disease. In terms of clinical appearance, Mondor’s disease presents as a painful rigid cord-like structure with adherent overlying skin over the dorsum of the penis while non-venereal sclerosing lymphangitis presents as an asymptomatic serpiginous cord with freely mobile skin around the coronal sulcus. In doubtful cases, a Doppler can be done which shows echogenic, non-compressible veins in Mondor’s disease while being normal in sclerosing lymphangitis.^[3] In addition, a biopsy reveals obstruction of the lumen of the vein indicative of Mondor’s disease while sclerosing lymphangitis is characterized by lymphatic vessel hypertrophy and sclerosis.^[1] Non-venereal sclerosing lymphangitis is usually a self-limiting condition and management includes sexual abstinence and reassurance with or without non-steroidal anti-inflammatory drugs.^[3]