Secondary syphilis presenting with “crown of Venus” alopecia

Abstract

Since the era of antibiotics, the frequency of secondary syphilis manifestations has declined. During the last decade, there has been a resurgence of syphilis cases. We describe a case of a 28-year-old man with various secondary syphilis symptoms including alopecia with the well-described characteristic “crown of Venus” pattern not commonly seen during this decade, as well as mucosal plaques, pustules, and palmoplantar macular rash. This case suggests that syphilis should be included in the differential diagnosis of hair loss for a correct screening, diagnosis, and early treatment.

Alopecia is an uncommon manifestation of secondary syphilis, with a prevalence between 2.9% and 7%.¹ Syphilitic alopecia has two well-known patterns: a moth-eaten pattern, which is considered pathognomic, and essential alopecia. With penicillin treatment, hair regrowth is observed within approximately 6 months.²

CASE DESCRIPTION

A 28-year-old Hispanic man with no previous medical history presented with a 6-month history of hair loss, shedding scalp, progressive vision loss, body aches, loss of balance, numbness and tingling of the lower extremities, and headache. He had a history of sex with men but no prior sexually transmitted disease diagnosis. Physical examination revealed scaly and diffuse scalp alopecia with rough texture involving the frontotemporal area and associated with eyebrow hair loss (Figure 1a), as well as an irregular erythematous rash involving the palms and webs of fingers (Figure 1c), brown macules located on the soles of the feet (Figure 1d), multiple ulcerated painless lesions in the penis, conjunctival hyperemia with decreased visual acuity, an erythematous lesion on vermilion of the bottom lip, two whitish tender lesions on the roof of the mouth, and dysphonia. Computed tomography of the head and magnetic resonance imaging of the brain were within normal limits. Serology was reactive for syphilis, with a rapid plasma reagin titer of 1:2048; the Cryptococcus neoformans antigen test showed a titer of 1:2 in the cerebrospinal fluid, and the HIV polymerase chain reaction test viral load was 307,000 copies/mL. The CD4 count was 215 cells/mm³ (9%), the CD8 count was 1682 cells/mm³ (70%), and the CD4/CD8 ratio was 0.1 (1/8). Cerebrospinal fluid studies revealed a reactive Venereal Disease Research Laboratory test with a titer of 1:2, a white blood cell count of 72 mm³, lymphocytes 68 mm³, monocytes 7 mm³, glucose 39 mg/dL, and protein 67 mg/dL.

Figure 1.

(a) Secondary syphilitic alopecia “crown of Venus.” (b) Improvement of scaly rash 2 days after penicillin was initiated. (c) Palmar maculo-erythematous irregular lesions. (d) Hyperpigmented macular plantar lesions.

The patient was diagnosed with HIV/AIDS, secondary syphilis, neurosyphilis, and cryptococcal meningitis and was started on amphotericin B 300 mg intravenously every 24 hours for 14 days for Cryptococcus meningitis and penicillin G 24 million units intravenously every 24 hours for 14 days for neurosyphilis. While on antibiotic therapy during his hospitalization, the patient had dramatic symptomatic improvement (Figure 1b).

DISCUSSION

Even though alopecia is not a common manifestation of secondary syphilis, about 2.9% to 7% of patients experience it, and it can be the only manifestation and mimic other conditions.¹ Secondary syphilitic alopecia can be divided into two groups: uncommon symptomatic type, found in association with a secondary lesion on the scalp, and essential syphilitic alopecia, which is hair loss with the absence of visible syphilic scalp lesions.²

In previous literature, secondary syphilis symptoms were described as partial alopecia, crown of Venus, syphilitic papules, indurated lymphadenopathy, and mucosal plaques, but these symptoms have declined since the antibiotic era. In the described case, one of the patient’s chief complaints was alopecia, demonstrating the importance of a thorough history and physical examination to guide the differential diagnosis. This case is one of the first in recent times showing syphilitic manifestations from the pre-antibiotic era. The patient presented with the characteristic crown of Venus, mucosal plaques and syphilitic pustules, the classic palmoplantar macular rash, vision loss associated with ocular syphilis, and neurosyphilis, with concomitant HIV/AIDS and cryptococcal meningitis.

Syphilis has been called the great imitator due to the wide spectrum between usual and unusual manifestations.³ Detection of spirochetes and a peribulbar lymphocytic infiltrate pattern are characteristics observed in secondary syphilis for the diagnosis of alopecia associated with syphilis.⁴ In this case, we did not have a scalp biopsy, but improvement was observed after initiation of treatment. The current hypothesis of pathogenesis is vasculitis of peribulbar capillaries causing perifollicular lymphocytic infiltration with scattered plasma cells, which stops the hair cell cycle.⁵

Another study found that syphilitic alopecia occurs in only 4% of patients, located in the parieto-occipital area of the scalp and described as multiple, irregular patches with erythematous scaling lesions. Hair loss is reversible and generally completely resolves 8 to 12 weeks after the initiation of treatment.⁶

Penicillin was first reported as an effective treatment for syphilis in 1943; since then, there have been no known resistant cases. HIV coinfection in 2017 was found to be present in 46% of men who have sex with men.⁷ C. neoformans dermatologic manifestations are found in 6% of AIDS patients, usually as a result of expression of the hematogenous dissemination of the fungus presented as papules, pustules, vesicles, nodules, and ulcers.⁸

Urticarial reactions and thrombophlebitis are well known side effects of amphotericin B caused by liposomal activation of the complement cascade releasing anaphylotoxins.⁹ No association between alopecia and C. neoformans infection or its treatment has been described in the literature.