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Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e31820bcff9
Case Reports

Asymptomatic Granulomatous Vulvitis and Granulomatous Cheilitis in Childhood: The Need for Crohn Disease Workup

Nabatian, Adam S*; Shah, Kara N; Iofel, Elizaveta; Rosenberg, Stephen*; Javidian, Parisa§; Pappert, Amy||; Milgraum, Sandy S||

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*UMDNJ-Robert Wood Johnson Medical School, USA

Department of Pediatric Dermatology, Children's Hospital of Philadelphia, Philadelphia, PA, USA

Department of Pediatric Gastroenterology, USA

§Department of Pathology, USA

||Department of Dermatology, UMDNJ-Robert Wood Johnson Medical School, New Brunswick, NJ, USA.

Received 19 October, 2010

Accepted 15 December, 2010

Address correspondence and reprint requests to Adam S. Nabatian (e-mail: adam.nabatian@gmail.com).

The authors report no conflicts of interests.

Granulomatous vulvitis (GV) and granulomatous cheilitis (GC) are inflammatory conditions characterized by painless swelling of the vulva and lips, respectively (1,2). When a patient presents with GV and/or GC, a granulomatous infectious process and disease must be ruled out (2,3). The differential diagnosis of GV and GC must include rosacea, lupus vulgaris, mycobacterial infections, sarcoidosis, Anderson-Fabry disease, metastatic Crohn disease, and foreign body granuloma (2). Other causes of chronic vulval edema include angioneurotic edema, malignant tumor, and infectious etiologies such as syphilis, actinomycosis, and lymphogranuloma venereum (1,3,4). We report here the case of an 8-year-old girl who presented with swelling of the genitalia and lips and was found to have Crohn disease.

An 8-year-old white girl presented with an 8-month history of swelling of the genitalia. The initial episode was associated with left cheek erythema and swelling. Antibiotic treatment for impetigo by the pediatrician resulted in resolution of the cheek swelling, but the swelling of the genitalia remained unchanged and was asymptomatic. The patient denied any gastrointestinal or constitutional symptoms.

On physical examination, the labia majora were grossly enlarged, erythematous, and indurated, with the right side more affected than the left (Fig. 1). The lips appeared mildly swollen and indurated (Fig. 2). In addition, induration of the anal ring and immediate surrounding area was also noted. There were no signs of any other perianal disease, and stool was guaiac negative. A 3.5-mm punch biopsy of the labia majora was consistent with granulomatous vulvitis (Fig. 3). No acid-fast bacilli or fungi were identified. The patient had a negative PPD and normal angiotensin-converting enzyme levels. Comprehensive metabolic panel, complete blood count, iron studies, erythrocyte sedimentation rate, liver function tests, and alkaline phosphatase were normal. Her original albumin was 4.4 g/dL, but the repeat a few weeks later was 3.7 g/dL. Her weight was 31.4 kg (10%) and height was 146.5 cm (25%). Her previous growth chart revealed growth along 25% to 50%.

Figure 1
Figure 1
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Figure 2
Figure 2
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Figure 3
Figure 3
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Treatment was started with topical clobetesol 0.05% cream twice daily to the vulva only. The patient was referred to pediatric gastroenterology for further evaluation. EGD was performed and revealed mild erythema of the stomach, but otherwise was visually unremarkable. Biopsies of the duodenum, stomach, and esophagus were normal. Colonoscopy was also performed and revealed few superficial aphthous ulcerations in the transverse colon. However, biopsies revealed acute cryptitis and granulomas in the cecum, as well as granulomas in the right, transverse, left, and sigmoid colon (Fig. 4). Terminal ileum biopsies were unremarkable. IBD serology was sent at the time of colonoscopy and was positive for anti-CBir1 with titers of 92.5 EU/mL (normal is <21). The patient was then started on oral prednisone. After several weeks of treatment there was notable clinical improvement.

Figure 4
Figure 4
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DISCUSSION

Granulomatous vulvitis (GV) is a rare inflammatory condition affecting children and adults that is clinically characterized by painless swelling of the vulva (1). Granulomatous cheilitis (GC) is a rare inflammatory disease that is characterized by diffuse, nontender, soft-to-firm swelling of 1 or both lips (2). Histological examination of both GV and GC demonstrates noncaseating granulomas extending into the deep dermis, composed of histiocytes and giant cells, associated with a lymphomonocytic infiltrate (2,3).

Infrequently, GC may be present in a patient with GV (Table 1). There is 1 report in the literature of a 10-year-old girl who presented with both GC and GV in the context of Crohn disease (5); however, our case would appear to be the youngest reported. Our case is also unique because GV and GC presented simultaneously, and in the prior pediatric case report there was a 2-year delay in the appearance of the GV after the GC was first evident (5). Both cases were asymptomatic in terms of Crohn disease at the time of initial presentation. Interestingly, 3 reported adult patients who developed GV and GC were not found to have an underlying cause, including Crohn disease (2–4). Based on these findings, if a child presents with both GV and GC, this should be considered as an absolute indication for gastroenterological workup for Crohn disease, even if asymptomatic.

Table 1
Table 1
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Our patient was referred for gastroenterology evaluation owing to the presence of granulomatous inflammation in the labial biopsy, with negative workup for sarcoidosis and other possible causes. At presentation the patient did not have any GI or systemic complaints. Detailed nutritional assessment was not done for this patient. However, dietary recall did not reveal any obvious dietary restrictions or deficiencies. We did not measure vitamin levels in this patient. Given the decline in our patient's weight percentiles across the years, the mild decline in albumin, and findings on physical examination, decision was made to proceed with the workup for Crohn disease.

We cannot be certain that our patient had GC because we did not take a biopsy of her lip; however, she appeared to have GC based on physical assessment. GV and GC are classically chronic conditions typically manifested by persistent painless erythema and edema with progressive induration of affected areas (2). The etiologies of GV and GC are unknown, but the histological features are similar to those of Crohn disease (1–3).

Our patient, although otherwise asymptomatic, was diagnosed with Crohn disease on the basis of intestinal biopsy. However, it has been reported that cutaneous findings can often precede bowel symptoms in Crohn disease by months to years (5,6). Patients with persistent, nontender, anogenital or lip lesions with edema and induration should be followed by their physician because these lesions may be a cutaneous sign for the future development of Crohn disease. Sexual abuse should be considered in any child with chronic anogenital lesions such as GV; however, early recognition of lesions typical of GV, such as cutaneous Crohn disease, can prevent unnecessary evaluations (6,7).

Treatment for GV and GC, although of variable and limited efficacy, includes metronidazole; topical, intralesional, and systemic corticosteroids; sulfasalazine, mesalazine; danazol; clofazamine; and antimalarial drugs (1,2,5). Thalidomide and infliximab have also been used to treat refractory vulval Crohn disease (8). N-[3,4-dimethoxycinnamol]-anthranilic acid has also been used to treat GV (9).

To our knowledge this is the youngest reported case of Crohn disease presenting as both granulomatous vulvitis and granulomatous cheilitis. Despite being asymptomatic our patient was diagnosed as having Crohn disease on the basis of intestinal biopsy. The only other child presenting with both GV and GC that was reported in the literature was also diagnosed as having Crohn's disease. However, the etiology of GV and GC in the 3 adult cases discussed was not discovered. Based on our experience with this patient and a review of the literature, when a child presents with both GV and GC, despite being asymptomatic, an aggressive workup including colonoscopy may be advised searching for Crohn disease. This is especially true if additional, even subtle signs such as a falloff in weight or low albumin are present.

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REFERENCES

1. Rowan DM, Jones RW. Idiopathic granulomatous vulvitis. Australas J Dermatol 2004; 45:181–183.

2. Sbano P, Rubegni P, Risulo M, et al. A case of idiopathic granulomatous cheilitis and vulvitis. Int J Dermatol 2007; 46:720–721.

3. Guerrieri C, Ohlsson E, Ryden G, et al. Vulvitis granulomatosa: a cryptogenic chronic inflammatory hypertrophy of vulvar labia related to cheilitis granulomatosa and Crohn's disease. Int J Gynecol Pathol 1995; 14:352–359.

4. Knopf B, Schaarschmidt H, Wollina U. Monsymptomatisches Melkersson-Rosenthal-Syndrom mit nachfolgender vulvitis and perivulvitis granulomatosa. Hautartz 1992; 43:711–713.

5. Ploysangam T, Heubi JE, Eisen D, et al. Cutaneous Crohn's disease in children. J Am Acad Dermatol 1997; 36:697–704.

6. Vaid RM, Cohen BA. Cutaneous Crohn's disease in the pediatric population. Pediatr Dermatol 2010; 27:279–281.

7. American Academy of Pediatrics, Committee on Child Abuse and Neglect. Guidelines for the evaluation of sexual abuse of children: subjective review. Pediatrics 1999;103:186–91.

8. Khaled A, Ezzine-Sebai N, Fazaa B, et al. Vulvoperineal Crohn's disease: response to metronidazole. Skinmed 2010; 8:240–241.

9. Tsuboi H, Masuzawa M, Katsuoka K. A case of vulvitis granulomatosa. J Dermatol 2005; 32:831–834.

Copyright 2011 by ESPGHAN and NASPGHAN

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