Syphilis d' emblee.

A 28-year-old male patient presented to Skin, V.D. and Leprosy outpatient with a single gray white plaque on the left side of the lower lip for last 8 months and multiple papulosquamous lesions all over the body for last 6 months. There was history of blood transfusion for anemia 1 year back. Histopathology of lip lesion and reactive VDRL and TPHA tests confirmed the diagnosis as syphilis. We report this rare case of Syphilis d' emblee.

Introduction

Syphilis is an infectious disease caused by Treponema pallidum. Syphilis, ‘the great imitator’, is among the most fascinating of skin disease. It may present to the dermatologist as a sexually acquired, contagious disease or as a congenitally acquired infection. Syphilis can be transmitted by blood transfusion, but it is very rare now and in this condition, no primary lesion appears. This is called Syphilis d’ emblee. We present a patient with secondary syphilis who acquired the infection during blood transfusion.

Case Report

A 28-year-old unmarried male, office worker, presented to the Skin, V.D. and Leprosy department with a grayish white painless plaque on the left side of the lower lip for last 8 months and multiple papulosquamous non-pruritic lesions all over the body including palm and sole.

There was history of transfusion of 3 U of stored blood in to the patient for severe anemia 1 year back, in a local nursing home. The patient did not have history of any type of sexual contact. There was no history of hypertension, diabetes, tuberculosis or drug eruption. Similarly there was no history in the family members. On examination, lip lesion was grayish white in color, oval in shape and size was about 4 cm in diameter, soft and non-tender [Figure 1]. There was generalized lymphadenopathy and lymph nodes were palpable, mobile, non-tender and rubbery. There were also multiple papulosquamous non-pruritic lesions all over the body including trunk [Figure 2], upper and lower limbs, palms and soles but sparing oral mucosa and the genital. There was no loss of scalp hair. There was no scar of any previous lesions on the genital.

Figure 1

Lesion of secondary syphilis-flat papule (condyloma lata) on lip

Figure 2

Lesions of secondary syphilis-papulosquamous lesions on trunk

Hematological examination showed moderate anemia (8.2 gm%) and all biochemical parameters were within normal range. Serological tests for syphilis were positive (VDRL in 1:32 dilution and reactive TPHA) and ELISA for HIV-1 and 2 were negative. Incisional biopsy of the lip lesion was sent for histopathological study, which showed dense infiltration of plasma cells and few lymphocytes in the dermis, in and around blood vessels in the form of perivasculitis and intimal proliferation in few of the arteries and veins (endarteritis obliterans) [Figure 3]. Silver staining of the lip lesion showed multiple spirochetes.

Figure 3

Histopathology of lip lesion(condyloma lata) showing plasma cell infiltration in dermis and endarteritis obliterance (H and E Stain ×400)

The patient showed hypersensitive reaction during intradermal testing with benzathine penicillin. Then patient was put on oral azithromycin 1 gm stat and doxycycline 100 mg twice daily and partial regression of lesion was observed after nearly 3 weeks of treatment.

Discussion

Syphilis is a chronic disease with a waxing and waning course, the manifestations of which have been described for centuries. The causative organism is T. pallidum. The rate of primary and secondary syphilis, the most infectious stages of the disease decreased throughout the 1990s and in 2000 reached an all time low.[1] The primary mode of transmission is by sexual contact, and the next most common is transfer across the placenta.[2] Kissing, blood transfusion and accidental inoculation have also been reported as routes of transmission, but are of minor importance today. The risk of transmission through blood is negligible due to improved donor selection, uniform serological testing of all blood donor, and a shift from transfusion of fresh blood to transfusion of refrigerated blood components.[34] Transmission via blood products is nonetheless theoretically possible since organism may survive for up to 5 days in refrigerated blood.[5] Needle sharing probably does not play a significant role in syphilis, but remains unclear.[6] Our patient developed lesions of secondary syphilis after transfusion of stored whole blood.

The disease has been arbitrarily divided into three stages. The primary stage is defined by a chancre at the site of inoculation. The secondary stage is characterized by a polymorphic rash, lymphadenopathy and other systemic manifestations. The tertiary stage is the most destructive and is marked by cardiovascular and neurologic sequelae and gummatous involvement of any organ system. Our patient presented with lesions of secondary syphilis including soft, non-tender plaque on the left side of lower lip, papulosqumaous lesions on the trunk, palms and soles and lymhadenopathy. The confirmed diagnosis of primary, secondary or early congenital syphilis are made by demonstration of organism by dark ground microscopy.[7] Serological test for syphilis include VDRL, T. pallidum immobilization test, fluorescent treponemal antibody absorption(FTA-ABS) test, T. pallidum hemagglutination assay (TPHA) test and EIA (Treponemal enzyme immunoassay) test.[8-11] In this case VDRL and TPHA tests were reactive. The characteristic histopathological findings of syphilis are perivascular infiltration of plasma cells and lymphocytes and intimal proliferation of both arteries and veins (endarteritis obliterans), which was seen in the biopsy of lip lesion of our case. The differential diagnosis of our case were Chancre, Squamous cell carcinoma lip, Lichen planus, Actinic granuloma, Leukoplakia, Psoriasis, Drug eruption and Graft versus Host Disease. Theoretically, the possibility of chancre of lip could not be ruled out but strongly denied of any type of sexual contact by the patient and history of blood transfusion indicate syphilis d’ emblee.

Many antibiotics, with notable exceptions of the aminoglycosides and sulphonamides, have some treponemicidal activity.[12] Benzathine penicillin is the recommended first-line therapy for syphilis.[13] In patients who are hypersensitive to penicillin, regimens based on tetracycline, doxycycline, erythromycin, azithromycin, ceftriaxone and chloramphenical have all been used. The patient in this case showed hypersensitivity to penicillin so he was put on azithromycin 1 gm single dose and doxycyclin 100 mg twice daily and he had been responding to it.