Malignant syphilis (Leus maligna) in a HIV infected patient

Introduction

Venereal syphilis was defined by Stokes as an "infectious disease due to Treponema pallidum, of great chronicity, systemic from the outset, capable of involving practically every structure of the body in its course, distinguished by florid manifestations on the one hand and years of complete asymptomatic latency on the other, able to simulate many diseases in the fields of medicine and surgery, transmissible to offspring in man, transmissible to certain laboratory animals, and treatable to the point of presumptive cure.[1]

Unnatural methods of sexual acts, widespread use of antibiotics and corticosteroids and, most importantly the advent of HIV infection have all resulted in the development of unusual manifestations of syphilis.[2] Leus maligna, or malignant syphilis was first reported by Allen in 1983 where he described an indurated genital ulcer in a patient followed by multiple pustular lesions on the face and trunk.[3] Leus maligna is a type of secondary syphilis with large ulcerative lesions. The ulcers are covered with thick crust and heal slowly. The lesions are commonly seen over the face and extremities.[1] These lesions were reported frequently in the preantibiotic era, and have now re-emerged with the advent of HIV infection.[2]

Case Report

A 45-year-old farmer attended the out patient department with complaints of fever and multiple nodular and ulcerative skin lesions all over the body of 20 days duration. Patient also gave a history of genital sore which healed partially 10 days prior to the onset of skin lesions. He was promiscuous and had genital discharge and inguinal swelling in the past which was treated. The patient′s wife died 5 years before and he was living with another destitute woman.

Examination revealed multiple papulo nodular lesions over the face, trunk and extremities. Some of these nodules on the face, and forearms were ulcerated, covered with slough and were tender. Palms, soles, hair and nails were spared. Oral mucosa was normal whereas on the genital mucosa there was a large ulcer on the coronal sulcus measuring one centimeter in size, covered with slough. There were non tender moist papules on the scrotal skin suggestive of condylomata lata. The inguinal and epitrochlear lymph nodes were enlarged, and rubbery in consistency and non tender.

Investigations revealed blood hemoglobin 10.6 gms %, normal white cell count, and ESR 66 mm at 1 hour. Liver function tests were normal. VDRL was reactive in 64 dilutions. HIV test was positive (ELISA method). Lymph node aspirate revealed Treponema pallidum under dark-field microscope. Pus culture from the ulcerative cutaneous lesions grew Klebsiella.

Patient was given injection benzathine penicillin 2.4 mega units per week for 3 weeks. He was also given a course of ciprofloxacin for the secondary bacterial infection. On the 5th day the genital ulcers healed and the cutaneous lesions started resolving. On the 21st day all cutaneous lesions healed completely. Patient′s contact was also examined. She was found to have a single ulcer on the inner aspect of right labium majus. Her blood VDRL was reactive in 32 dilutions and she was also HIV positive. Patient′s contact was treated with a single dose of benzathine penicillin.

Follow up after 1 year revealed no serological or clinical relapse of syphilis. But, interestingly patient showed multiple hypo pigmented macules distributed over the neck and trunk suggestive of "leukoderma colli".

Discussion

It is well known that one third of patients with secondary syphilitic rash can still have chancre.[4] In a study conducted by Hutchinson it was found that HIV infected patients were found to present more often in the secondary stage and 43% of these patients were more likely to have chancres.[5]

A rare form of destructive syphilide, with deeply ulcerating lesions and severe toxemia, which may end in death, was described under the name malignant syphilis (Leus maligna). It resembled the first out break of syphilis in the fifteenth century.[4] A screening done from 1969-1989 by Mindel et al[6] in 854 patients with secondary syphilis did not reveal nodulo ulcerative lesions. There were other sporadic reports of this unusual condition.[7],[11] Petrozzi reported a case of malignant syphilis in a homosexual man whose workup revealed him to be anergic to the standard battery of skin tests.[9] These atypical or severe forms of syphilis in the presence of co-infection with HIV predisposes to accelerated neuro syphilis. This results from HIV-induced suppression of cellmediated immunity as reflected in the CD4 T-lymphocyte count.[2]

This case is highlighted for the presence of ′leus maligna′ in an HIV positive patient. Interestingly most of the skin lesions resolved with ′leukoderma colli′. This is the name given for hypopigmented macules in a resolved case of secondary syphilis around the neck.[10] With the current global increase in cases of HIV infection and syphilis, there seems to be a rise in the frequency of rare manifestations.[11] Allen′s postulates that a secondary viral infection might give rise to malignant pustular syphilis holds true even after hundred years.[3]