A rare case of lues maligna with ocular involvement after immune reconstitution syndrome

Secondary syphilis, has a rare and severe form called Lues Maligna (LM).1 Long recognized to afflict debilitated persons, recent cases have been reported in HIV patients. Anti-Retroviral Therapy (ART) in HIV patients can result in restoration of immune response against opportunistic infections.2 Rarely, this can lead to paradoxical worsening of infection or unmasking subclinical infection, termed immune reconstitution inflammatory syndrome (IRIS).2 We present a case of LM in a 71-year-old gentleman with ocular involvement manifesting as part of IRIS. Clinically, he had disseminated scaly erythematous papules and ulcerated plaques involving the torso, limbs, soles and palms with no lymphadenopathy. This had developed two weeks after initiating ART. It was associated with visual loss in the left eye, myalgia, fatigue, fever and night sweats. At the time of HIV diagnosis, syphilis serology was negative, HIV-1 Viral Load was 177,828 copies/ml. Two weeks after initiating ART, HIV-1 Viral load decreased to 65 copies/ml. At time of presentation, syphilis serology became positive. Vitreous tap detected Treponema Pallidum DNA. He had significant improvement with benzylpenicillin and supportive steroid treatment to prevent Jarisch-Herxheimer reaction. This presentation is rare and as far as the authors are aware, it is the first reported case to have Lues Maligna with ocular involvement as a manifestation of IRIS.
Physicians should be aware of IRIS and repeating serology after immune restoration in the setting of advanced immunosuppression or early disease. We discuss the role of IRIS and how it impacts dermatological conditions.
1. 1. Haslund A. Syphilis maligna. Ach Dermatol Syphiligr 1987; 38.
2. 2. Martyn A H French. Immune reconstitution inflammatory syndrome: immune restoration disease 20 years on. Med J Aust 2012; 196: 318-21

Dr. Francis Yap

Royal Perth Hospital