Genital aphthae

Genital aphthae 1

Genital aphthae (nonsexually acquired genital ulceration) are benign and recurrent lesions characterized by necrotic ulceration of the mucosa of the genital organs that appear and disappear spontaneously. The ICD-10 codes for this condition are N48.5 and N76.6.

This condition is more common in adolescent girls and young women aged 12 to 25 years, and it is very rare in men. The etiology and pathogenesis are not clear. Idiopathic genital aphthae, which account for up to 80% of cases, and secondary aphthae associated with Behçet's disease, cyclic neutropenia, ulcerative colitis, Crohn's disease, HIV infection, myeloproliferative disorders, erythema multiforme, MAGIC syndrome (mouth and genital ulcers with inflamed cartilage), and PFAPA syndrome (periodic fever, aphthous stomatitis, pharyngitis, and adenitis) are distinguished. Some studies have shown an association with certain infections, such as Epstein-Barr virus, cytomegalovirus, and mycoplasmas, but their definitive etiologic role has not been established.

Many authors consider Lipschütz's vulvar ulcers, acute vulvar ulcers, and vulvar aphthae to be the same condition. In some cases, simultaneous occurrence of oral and genital aphthae is observed. This condition has been proposed to be called aphthous complex or complex (bipolar) aphthosis.

The disease starts acutely with the appearance of an erythematous spot that quickly transforms into a vesicle, which then ruptures, forming a round or irregularly shaped, deep, sharply painful "stamped" erosive-ulcerative defect with an inflammatory rim and a base covered with gray-yellowish coating. Multiple lesions usually occur. In some cases, the eruption of aphthae is accompanied by moderate fever, malaise, and gastrointestinal and/or respiratory symptoms.

The following types are distinguished:

  1. Minor aphthae: These are smaller than 1 cm and often superficial, healing without scarring.
  2. Major aphthae: These are larger than 1 cm and usually deep, often resulting in scarring during healing.
  3. Herpetiform aphthae: These are grouped small aphthae with more than 10 elements.

Minor aphthae typically heal within 5-10 days without scarring, while major aphthae take 2-4 weeks to heal, often with scarring. The course of the disease is chronic, with most cases experiencing recurrent episodes at intervals of 1 to 6 months, decreasing in frequency with age.

Vulvar aphthae occur primarily in the vaginal vestibule and less commonly on the labia majora, labia minora, and perineum. They can be up to 2 cm in diameter and 1 cm in depth. In most cases, more than two aphthae are observed, often coalescing into extensive, irregularly shaped lesions. The base of the ulcers is bright red and almost always covered with a necrotic layer, sometimes forming a black crust. Extreme pain is often noted.

Aphthae on the penis are rare and occur in the scrotal area, glans penis, and shaft of the penis. The clinical presentation is indistinguishable from vulvar aphthae, but they are often smaller, more numerous, and tend to cluster.

The diagnosis of idiopathic genital aphthae is based on the clinical presentation. Histological and laboratory findings are nonspecific and cannot confirm or exclude the disease. Microbiological, serological, and molecular tests should be done to rule out genital herpes, syphilis, HIV infection, and chancroid. Differential diagnosis with Behçet's syndrome can be challenging, and in such cases, pathergy testing should be considered.

  • Behçet's disease
  • Cyclic neutropenia
  • Ulcerative colitis
  • Crohn's disease
  • HIV infection
  • Myeloproliferative disorders
  • Systemic lupus erythematosus
  • MAGIC (Mouth and Genital Ulcers with Inflamed Cartilage) syndrome
  • PFAPA (Periodic Fever, Aphthous stomatitis, Pharyngitis, and Adenitis) syndrome
  • Celiac disease
  • Erosive lichen planus
  • Erosive balanoposthitis
  • Chancroid
  • Primary syphilis
  • Chemotherapy-induced mucositis
  • Herpes simplex
  • Herpes zoster
  • Candidiasis
  • Pemphigus vulgaris
  • Genital amebiasis
  • Cicatricial pemphigoid
  • Squamous cell carcinoma
  • Deficiency of iron, zinc, folic acid, vitamins B1, B2, B6, B12
  • Sweet's syndrome
  • Pyoderma Gangrenosum
  • Scrotal ulcers caused by trans-retinoic acid.

Topical treatment:

  • 5% lidocaine ointment or cream
  • Cauterization with silver nitrate solution
  • 0.05% clobetasol cream or ointment, applied twice daily for 5 days
  • Intralesional injection of triamcinolone (3-5 mg/ml)

Systemic treatment:

  • Prednisone 40 mg orally for 7-10 days
  • Doxycycline or minocycline 100 mg twice daily
  • Pentoxifylline 400 mg three times daily
  • Colchicine 0.6 mg 2-3 times daily
  • Dapsone 100-150 mg per day