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New-Onset Vulvar Edema in a Pediatric Patient | Dermatology | JAMA Dermatology | ÌÇÐÄvlog

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JAMA Dermatology Clinicopathological Challenge
September 25, 2024

New-Onset Vulvar Edema in a Pediatric Patient

Author Affiliations
  • 1Dermatology Department, Hospital Universitari Germans Trias i Pujol, Badalona, Barcelona, Spain
JAMA Dermatol. 2024;160(11):1243-1244. doi:10.1001/jamadermatol.2024.2561

A female patient aged 12 years was referred for asymptomatic vulvar edema of 1 month’s duration that was previously treated with topical antifungals and corticosteroids with no improvement. She did not report recent travel or sexual relationships. On examination, swelling, erythema, and palpable induration on the labia minora and majora of the vulva were noted. No lymphadenopathy was palpated in the inguinal region (Figure 1A). A 4-mm punch biopsy specimen was obtained for histopathological examination from the right-sided labia majora (Figure 1B).

D. Metastatic Crohn disease

The medical history of the patient included growth delay, several months of constipation treated with laxatives, and hemorrhoids. Histopathological examination revealed aggregated nodules of epithelioid histiocytes in the superficial and deep dermis without necrosis. Some granulomas were poorly compact and had ill-defined borders, which is associated with a lymphocytic and eosinophilic infiltrate, at the periphery of the granuloma and within it (Figure 2). Results of routine blood tests detected a slight increase in erythrocyte sedimentation rate and normal values of angiotensin-converting enzyme, and interferon γ release assay results were negative. The fecal calprotectin was 297 μg/g (normal laboratory values <50 μg/g). Stool culture and parasite study results were negative. The imaging studies, including chest radiography and intestinal and pelvic magnetic resonance imaging, showed results within normal ranges. The endoscopic study revealed ulcers in the duodenal bulb, rectum, sigmoid colon, terminal ileum, and cecum, along with segments of erythematous and edematous mucosa with some aphthous ulcers in the sigmoid and rectal region. Granulomas were detected in the lamina propria through gastric and intestinal endoscopic biopsy specimens, and they were not linked to active crypt injury. Thus, a final diagnosis of metastatic Crohn disease (MCD) was made.

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