An AIDS Patient with Recurrent Multiple Skin Crusted Ulcerations

Background

Malignant syphilis is an unusual ulcerative variation of secondary syphilis that is normally found in immunosuppressed patients. These ultimately form ulcers with raised edges and central necrosis and may be followed by rupioid crusts. ¹

This study describes the case report of a 22-year-old man who discovers acquired immune deficiency syndrome (AIDS) and syphilis at the same time for presenting of fever and skin disseminated brown–black rupioid crusts compatible with malignant syphilis.

Case Report

A 22-year-old homosexual male presented with fever, maculopapules, and blisters on his face, trunk, and extremities just after removing the nasal prosthesis 40 days before admitted into hospital. He had been diagnosed as chickenpox at an early stage, without special treatment, and the rash had not subsided. He had been treated with levofloxacin for a week, the temperature gradually decreased to normal, but the rash did not improve 20 days before admission. When the blisters broke, the skin ulcerated and then scabbed with overlying brown–black rupioid crusts within 20 days. When he was admitted to hospital, there were multiple skin crusted ulcerations on the trunk (Fig. 1a) and extremities (Fig. 1b). There was a small ulcer with yellow discharge in the perineum, swelling of bilateral subaxillary lymph nodes. He had a history of men who have sex with men for 5 years.

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FIG. 1.

Multiple skin ulcerations scabbed with overlying brown–black rupioid crusts on the trunk (a) and extremity (b). The biopsy pathology of right forearm lesion (hematoxylin and eosin; original magnification × 200) (c). There are scars on the trunk after treatment with benzathine penicillin for 3 weeks (d). Color images are available online.

The examinations showed white blood cell (WBC) 11.98 × 10⁹/L, neutrophils 9.82 × 10⁹/L, hemoglobin (Hb) 130g/L, C-reactive protein (CRP) 152 mg/dL, serum immunoglobulins, and antineutrophil cytoplasmic antibodies were normal, cultures of fungi, bacteria were negative. He was confirmed human immunodeficiency virus (HIV) infection and his CD4⁺ T cell counts were 117/μL, HIV-RNA 4.22 × 10⁵ copies/mL. The biopsy pathology of right forearm lesion (Fig. 1c) showed an ulcer, dense inflammatory cells infiltrated in the dermis, including neutrophil, lymphocytes, and histocytes, there was deep perivascular infiltration of lymphocytes and plasma cells. AIDS was confirmed. Malignant secondary syphilis was diagnosed according to the biopsy pathology, positive serum syphilis enzyme immunoassay (EIA) screening and high serum rapid plasma regain (RPR) test titer (1:64). After application of benzathine penicillin for 3 weeks, the rashes were in complete remission (Fig. 1d). Then the patient began with highly active antiretroviral therapy (zidovudine, lamivudine, and nevirapine). But zidovudine was changed to tenofovir dipivoxil for severe anemia (Hb 54 g/L).

In the follow-up, the RPR titer decreased (1:4) 8 months later, HIV-RNA was negative and CD4⁺ T cells were significantly increased (522/μL). But 13 months later, the patient appeared with fever and rashes again. The rashes was also multiple skin crusted ulceration, accurately the same with rashes before, but more intensive. The RPR rose to 1:64, and then 1:128 in 2 weeks, WBC was 8.59 × 10⁹/L, CRP was 99.5 mg/dL, CD4⁺ T cells were 657/μL. It was likely to be reinfection of syphilis after frequent sexual activity. Since penicillin skin test was positive, doxycycline was applied for 4 weeks, and RPR decreased to 1:4, rashes disappeared again.

Discussion

Malignant syphilis is an uncommon form of destructive secondary syphilis. A multicentric retrospective study found that 1.33% (151/11,368) of HIV-infected individuals in Germany had syphilis, of which 7.3% (11/151) were malignant syphilis. ² HIV-infected patients were 60 times more likely to present with malignant syphilis compared with general population. ³

The clinical manifestations of syphilis might be altered with impairment of both humoral and cell-mediated immunity by HIV, ⁴ which may limit the host's defenses against treponema pallidum. The progressive destructive course of malignant syphilis may be due to an immunocompromised condition of the host, a virulent strain, or even an inappropriate immune response. ^5,6

Malignant syphilis is characterized by occlusion of the blood vessels with resultant fibrinoid necrosis at the dermal-subcutaneous junction. ⁷ The skin lesions of malignant syphilis are just like this patient, start as papules and evolve into pustules, then the lesion centre undergoes necrosis, resulting in ulcers and scabbed with overlying rupioid crusts.

In this patient, the diagnosis of malignant syphilis was confirmed by the positive RPR/EIA titer, characteristic clinical nodular lesions, a plasma cell-rich inflammatory infiltrate on pathology, and good response to benzathine penicillin. However, a year later, the patient developed fever again, the appearance of the rash was the same as before, and the RPR increased again. Is it a relapse or reinfection? Since the RPR of the patients had been at a low level during the nearly 1-year follow-up, and the RPR rose to 1:64, and then 1:128 in 2 weeks, it was considered that the patients had a high possibility of “syphilis reinfection,” for the patient admitted to having frequent sex in recent months. Fortunately, the patient responded well to doxycycline treatment. Despite its name, malignant syphilis is usually treated well after accurate diagnosis and regular treatment. ⁸

Conclusion

Malignant syphilis is a rare form of secondary syphilis, but tends to occur in AIDS patients because of poor immunity. Although the clinical manifestations of malignant syphilis are severe, the response to the therapy of both penicillin and doxycycline are excellent.