Pediatric case of Crohn's disease with preceding vulvitis granulomatosa.

Crohn's disease (CD) of the vulva is a rare and under-recognized condition. Since vulvar lesions may precede the diagnosis of digestive CD in approximately 25% of all cases, the coexistence or future onset of CD should be considered regardless of the gastrointestinal symptoms, even for pediatric patients.

CASE DESCRIPTION

An 8‐year‐old girl with spina bifida and constipation noticed redness and swelling of her vulva, particularly the labia majora (Figure 1). Skin biopsy was performed after 6 months, and epithelioid granulomatous formation with Langerhans‐type giant cell infiltrations was observed in the dermis (Figure 2A,B). After excluding sarcoidosis from the diagnosis, she was diagnosed with vulvitis granulomatosa (VG). She was started on tranilast, and after 6 months, the vulvar swelling and redness almost disappeared. However, the drug was discontinued at the discretion of her parents. At 10 years of age, she only experienced persistent diarrhea and reswelling of the vulva (Figure 3A). Total colonoscopy (TCS) revealed multiple small ulcers from the sigmoid colon to the rectum (Figure 3B), and pathological findings showed epithelioid granulomas (Figure 3C). Abdominal enhanced‐computed tomography scan showed no abnormalities. She was ultimately diagnosed with Crohn's disease (CD). Tranilast and 5‐aminosalicylic acid therapies before infliximab improved her symptoms.

FIGURE 1

Physical appearance of the patient's vulva showing redness and swelling

FIGURE 2

Pathological findings of the skin biopsy showing epithelioid granulomatous formation with Langerhans‐type giant cell infiltrations in the dermis (A, B) (hematoxylin and eosin staining)

FIGURE 3

Physical appearance of the patient's vulva showing exacerbated redness and swelling at 10 years of age (A). Total colonoscopy showing multiple small ulcers from the sigmoid colon to the rectum (B). Pathological findings showing the infiltration of inflammatory cells, decreased goblet cells in the crypts, and epithelioid granulomas (hematoxylin and eosin staining) (C). Black arrow: epithelioid granulomas

Vulvar lesions may precede the diagnosis of digestive CD in approximately 25% of cases. One possible reason for this is the delayed use of TCS in children. When VG is diagnosed, the presence or future onset of CD may have to be considered regardless of gastrointestinal symptoms.

CONFLICT OF INTEREST

The authors state explicitly that there are no conflicts of interest in connection with this article.

AUTHOR CONTRIBUTION

T.K. was involved in all stages of patient management and wrote the manuscript. M.Y., A.T., and M.N‐H. performed the treatment and analyzed the data. M.Y. collaborated as the reviewer. All the authors have read and approved the final manuscript.

ETHICAL APPROVAL

This report was exempted from ethical approval because it is an observation report after the current care.

CONSENT

Written informed consent for publication was obtained from the patient and her parents.