Circinate balanitis: Early manifestation of reactive arthritis? A case series

Abstract

Circinate balanitis, although a common cutaneous manifestation of reactive arthritis (ReA), is usually an associated finding present along with the triad of arthritis, conjunctivitis, and urethritis. It is rarely seen as the only or the preceding manifestation of ReA. Here, we report three cases of circinate balanitis as stand-alone and initial presentation of probable ReA without any other symptoms of arthritis and conjunctivitis at the time of presentation.

Introduction

Circinate balanitis presents as annular erythematous erosive plaques with polycyclic margins on the glans penis and prepuce, commonly associated with reactive arthritis (ReA).

ReA is a genetically orchestrated immune response focused on the skin and joints.[1] It is characterized by the triad of urethritis or cervicitis, conjunctivitis, and arthritis, which closely follows lower urogenital or enteric infection.[2] It is usually seen in individuals with histocompatibility antigen B27 (human leukocyte antigen [HLA]-B27). Mucocutaneous findings such as circinate balanitis (50%), keratoderma blennorrhagicum, oral ulcers, and dystrophy of nails are associated with ReA.[3] Here, we report three cases of circinate balanitis as the sole clinical manifestation at presentation, a condition typically linked with ReA but seldom observed as an initial or stand-alone manifestation.

Case Reports

Case 1

A 25-year-old unmarried male presented with mildly itchy and burning lesions on the glans penis for 3 months. Examination revealed three well-defined erythematous, round to oval superficial erosions (0.5–2.0 cm) with surrounding scaling, forming a circinate pattern on the glans and corona [Figure 1a]. The patient reported multiple similar episodes over the past year, partially relieved by topical steroids.

Figure 1.

(a) Circinate lesions over the glans and corona suggestive of circinate balanitis. (b) Psoriasiform hyperplasia (H and E, ×10)

Laboratory investigations showed positive herpes simplex virus 1 (HSV1) immunoglobulin G (5.61 units), negative HSV1 immunoglobulin M, and negative HSV2 serology. Other serologies (human immunodeficiency virus [HIV], hepatitis B surface antigen [HBsAg], hepatitis C virus [HCV], and serum Venereal Disease Research Laboratory [VDRL]) were nonreactive, and routine blood tests were within the reference range. He had been treated with oral antivirals, antibiotics, antifungals, and topical steroids without long-term relief. The patient had no history of genital discharge, systemic symptoms including any gastrointestinal (GI) complaints, or any other skin lesions, and he had no high-risk behaviors.

Histopathology revealed psoriasiform changes, parakeratotic hyperkeratosis, neutrophilic collection suggestive of Munro’s abscess, and dermal lymphocytic infiltrate with occasional plasma cells [Figure 1b]. Periodic acid–Schiff (PAS) stain was negative for fungal hyphae.

HLA-B27 was ordered in view of psoriasiform histology and was positive via polymerase chain reaction method.

Circinate balanitis was diagnosed based on clinical, investigative, and histopathological findings.

Case 2

A 32-year-old male presented with asymptomatic lesions on the glans penis for 1.5 months. Examination revealed a well-defined erythematous plaque with a scaly border on the dorsal glans and a superficial erosion on the ventral penile shaft [Figure 2a]. The patient had no itching or burning and reported occasional lower back pain without morning stiffness. The patient was married and he had no history of genital discharge, systemic symptoms including GI complaints, or high-risk sexual behavior.

Figure 2.

(a) Superficial erythematous plaque with well-defined border over the penile shaft and glans. (b) Histopathology suggestive of hyperkeratosis with psoriasiform hyperplasia (H and E, ×10)

Laboratory tests for HIV, VDRL, HBsAg, and HCV were negative. Magnetic resonance imaging of the lumbosacral spine and hip indicated sacroiliitis. Despite treatment with multiple antibiotics and topical antifungals, the lesions persisted. HLA-B27 by flow cytometry was positive, and a biopsy of the genital lesion showed hyperkeratosis with psoriasiform hyperplasia, leading to a diagnosis of circinate balanitis [Figure 2b]. PAS stain did not reveal any hyphae.

Both these patients were treated with oral doxycycline (100 mg bid for 21 days) and topical steroids. While lesions resolved temporarily, partial recurrence occurred over 6 months, but neither developed any other systemic signs of ReA.

Case 3

A 27-year-old male presented with asymptomatic genital lesions for 7 days. He denied high-risk sexual behavior, had no history of urethral discharge or GI complaints, and had no significant medical history. Examination revealed erythematous, well-defined papules and coalescent annular plaques with white, raised polycyclic margins on the glans penis, along with finger and toe nail changes in the form of onycholysis, ridging, hyperkeratosis, discoloration, and brittle nails for 3 months [Figure 3a and b].

Figure 3.

(a) Superficial erythematous plaques with polycyclic lesions on the penile shaft and glans. (b) Multiple eczematous, hyperpigmented, scaly plaques on the extremities, accompanied by significant nail changes and joint involvement in proximal and distal interphalangeal joint swelling

In view of nail changes with asymptomatic scaly lesions, psoriasis was considered differential and was prescribed topical mometasone furoate cream for 10 days, which was ineffective. The patient was lost to follow-up and presented 3 months later, with scaly, psoriasiform lesions on the extremities, sausage-shaped swelling of the right index finger, severe joint pain over the left wrist, right knee, both ankles, lower back, and conjunctivitis. He was unable to walk or fully extend his fingers. His HLA-B27 was positive, and laboratory investigations showed elevated C-reactive protein and erythrocyte sedimentation rate, with normal liver and renal function. Serologies for antinuclear antibody, rheumatoid factor, hepatitis B and C, HIV, and VDRL were negative.

Diagnosed as ReA, he was treated with nonsteroidal anti-inflammatory drugs, antibiotics, and injectable methotrexate (15 mg/week for 4 weeks), leading to significant improvement in skin lesions and joint pain. This case highlights circinate balanitis as a potential precursor to ReA.

Discussion

ReA also known as “oculo-urethro-synovial syndrome” was described by Hans Reiter and simultaneously by French physicians Fiessinger and Leroy in 1916. It is a multisystem autoimmune disease of young males with associated HLA-B27 gene in 80% of cases.[4,5]

Our three cases highlight a rare situation, where young male patients presented with circinate balanitis as the predominant feature of heralding ReA along with HLA-B27 positivity. Kumar et al. and Carney et al. reported cases, where circinate balanitis was the presenting symptom of sexually acquired ReA.[5,6] The appearance of cutaneous lesions early in the course of the disease may herald a poor prognosis, especially in the rare epidemic form of the disease.[7]

Treatment for circinate balanitis includes topical steroids like hydrocortisone, topical calcineurin inhibitors like pimecrolimus 1% and tacrolimus 0.1%, and topical keratolytics like salicylic acid 10% along with topical steroids.[5]

HLA-B27 genetic testing of patients presenting with circinate balanitis could help predict future prognosis. In our cases, all the patients with circinate balanitis tested positive for HLA-B27. This suggests that circinate balanitis along with HLA-B27 positivity may be interpreted as an early sign of ReA, and such patients should be observed for the potential development of further signs of the disease.[8] Gaurav et al. described a case of circinate balanitis as an independent manifestation of ReA, noting that the patient was negative for the HLA-B27 antigen.[9]

In addition, the histopathological results indicate genital psoriasis. Combined with the mentioned clinical signs and the presence of HLA-B27, the diagnosis leans toward ReA and regular follow-up of such patients is essential.

The classic triad of arthritis, urethritis, and uveitis, or tetrad with balanitis, in ReA often develops over time and may be incomplete. Diagnosis is challenging due to the lack of definitive tests and the transient nature of symptoms in young males.[10]

Conflicts of interest

There are no conflicts of interest.

Funding Statement

Nil.