Inguinal syndrome with penoscrotal elephantiasis

Introduction

Lymphogranuloma venereum (LGV) is a sexually transmitted disease of the lymph channels caused by the L-serotype of Chlamydia trachomatis, characterzed by a small fleeting primary lesion followed by the development of suppurative regional lymphadenitis.[1] Esthiomene, a rare late manifestation of LGV, is a primary infection affecting the lymphatics of scrotum, penis or vulva, and may cause chronic progressive lymphangitis, chronic edema and sclerosing fibrosis of subcutaneous tissues[2] which results in induration and enlargement of affected parts and ultimately in ulceration. The male genitalia are affected less commonly by esthiomene. We report a male patient who presented with bilateral inguinal bubos and penoscrotal elephantiasis.

Case Report

A 40-year-old married man had extra-marital contact about one and half years back. About fifteen days after the exposure, he developed a single non-tender, non-discharging ulcerated lesion on the under surface of prepuce which healed with antibiotics. About six months thereafter, the patient developed swellings in the inguinal region, bilaterally. Initially, the swellings were firm but later on, they softened and suppurated. This was followed by firm swellings of the penis and scrotum with thickening of the overlying skin. The onset of swellings was accompained by fever, arthralgia and malaise. There was no history of difficulty in defecation, rectal discharge or constipation.

On examination, oval, lobulated swellings were present in the inguinal folds, bilaterally. The lesions were adherent to the overlying skin and fixed to the subjacent tissues with the overlying skin showing thickening, scaling and a cyanotic hue. The surface was smooth and the consistency soft. Sinus formation was present in the right inguinal swelling with discharge of greyish white, thick, gluey pus and adherent haemorrhagic crusting was present at places.

Elephantiasis of penis and scrotum was present with the overlying skin showing a violaceous hue, thickening and rugosity. The penis was solidified and twisted giving rise to the so--called ′saxophone′. Superficial ulcerated lesions with irregular edges and a shiny yellowish- white base were present on the preputial margin. A diagnosis of LGV inguinal syndrome alongwith penoscrotal elephantiasis was made. Per-rectal examination did not reveal any abnormality.

On investigations, haemoglobin level, total and differential leukocyte counts, total serum proteins, albumin/globulin ratio and blood sugar were within normal limits. Mantoux test was negative. VDRL, ELISA for HIV and HBs Ag were negative. X-ray of the chest did not reveal any abnormality. The repeated midnight peripheral blood smears after provocation by 100mg tablet of diethyl carbamazine were negative for microfilariae. The complement fixation test for LGV could not be done because of non-availability.

Discussion

Inguinal adenopathy in LGV is usually unilateral but may be bilateral in upto one-third of cases.[3] The adenitis may terminate by softening and suppuration (60-70%) or spontaneous resolution (25-30%)or may persist for months as a stationary mass (5%).[1] The suppurative variety may lead to late complications like genital and anorectal syndrome. Late complication of the male inguinal syndrome are rare.[4] Elephantiasis of penis and scrotum characterized by infiltrative, ulcerative and fistular lesions occurs in approximately 4% of cases.[5]

This patient is being reported as he presented with bilateral inguinal adenitis alongwith penoscrotal elephantiasis which, in themselves, are rare presentations of LGV.