• May be associated with perineal disease or vulvar fistulas
• May be separated from anal lesions by normal tissue
• Vulvar involvement in Crohn's may be contiguous (direct extension of intestinal involvement) or non-contiguous (no connection between the vulva and bowel)

• 8 year old girl with vulvar inflammation as only clinical manifestation (Pediatrics 2010;125:e1518)
• 37 year old woman with isolated Crohn's disease of vulva (Indian J Dermatol Venereol Leprol 2011;77:342)

• Medical treatment for initial stages (metronidazole alone or with steroids)
• Surgical treatment (local excision or vulvectomy) for advanced disease

Multiple fleshy ulcers

• Hyperemia, edema, ulceration, "knife-cut" ulcers (resemble lacerations, almost pathognomonic of Crohn's disease but also reported in herpetic infections in immunocompromised and in cutaneous tuberculosis), "apthous-like" ulcer

• Noncaseating granulomas, ulcerations, lymphatic lesions and even dysplasia and carcinoma (Am J Dermatopathol 2011;33:588)

Ulcerated epidermis

Skin biopsy

Terminal ileum

• Risk factors: diabetes mellitus (greatest risk factor), hypertension, immunocompromise, increasing age, obesity, peripheral vascular disease, radiation exposure
  
• Primary site of infection: surgical incision (50% cases), skin damaged by infection or trauma

• Manifests as 2 types
  
  
• Type 1:
  • Forms majority of vulvar necrotizing fasciitis infections
  • Synergistic polymicrobial infection of both aerobes and anaerobes
  • Most commonly seen in setting of: diabetes, peripheral vascular disease, postoperative patients
  • Most common bacteria: B. fragilis, Clostridia, E. coli, Enterocci, S. aureus, Streptococci (J Bone Joint Surg Am 2003;85:1454)
  
  
• Type 2:
  • Monomicrobial infection of group A streptococcus (Streptococcus pyogenes) and less frequently methicillin-resistant Staphylococcus aureus (MRSA)
  • Seen in patients without underlying comorbidities

Criteria

• Mortality up to 50% (Am J Obstet Gynecol 1992;166:1324)

• Wide excision with broad spectrum antibiotics (Hawaii Med J 2010;69:13)

• Associated with avitaminoses, blood dyscrasias, diabetes, malnutrition, uremia

• Giant cell arteritis and polyarteritis nodosa Mod Pathol 1994;7:610)

• Inflammation of vestibular glands in vulvar vestibule submucosa
• Produces small ulcerations

• Surgical removal of mucosa

• Common cause of superficial dyspareunia in young women, athough vulvar pain likely represents many disorders (J Sex Med 2008;5:5)
• Chronic inflammation of vulvar vestibular mucosa, lamina propria and periductal connective tissue of vestibule
• May see mild exocytosis of lymphocytes in vestibular glands and ducts (Ann Dermatol Venereol 2008;135:367)

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