Perianal skin plaque in a child. Crohn's disease

Metastatic Cutaneous Crohn’s Disease in Children: Case Report and Review of the Literature

“…Metastatic Crohn’s disease, first described in 1965 by Parks et al (1), is the least common dermatological manifestation (8). Although well recognized in adults, an exhaustive review of the English literature revealed only 28 cases, including the present case, of metastatic cutaneous Crohn’s disease in children <18 years of age (2,3,5,8–25) (Table 1). Among the 28 pediatric cases, the clinical presentation ranged from genital swelling/induration (16) (2,3,5,9–19) to plaques (four) (5,8,20,21), nodules (two) (22,23), papules (three) (12,24,25), abscesses (two) (5), and ulcers (one) (15).…”

“…Of the 27 previously documented pediatric cases of metastatic cutaneous crohn’s disease, perianal disease was noted on presentation in 22 of the 27 pediatric patients (2,5,8–18,20,22–24), not mentioned in 4 of the 27 patients (5,19,21,25), and absent in 1 of the 27 patients (3). Similarly, gastrointestinal symptoms were present in 19 of the 27 patients (5,9–12,14–16,19,21–23,25), not mentioned in 3 of the 27 patients (5,17,20), and absent in 5 of the 27 patients (2,3,8,13,17) at initial presentation. Only one child lacked both perianal disease and gastrointestinal symptoms (3).…”

“…Among the 28 pediatric cases, the clinical presentation ranged from genital swelling/induration (16) (2,3,5,9–19) to plaques (four) (5,8,20,21), nodules (two) (22,23), papules (three) (12,24,25), abscesses (two) (5), and ulcers (one) (15). Overall, in children, the genitalia was the most commonly involved location for metastatic cutaneous Crohn’s disease representing 21 of the 28 cases with 16 vulvar (2,3,5,9–17) and five penile/scrotal (5,18–21) lesions.…”

“…In adults, metastatic cutaneous Crohn’s disease most frequently occurs in the setting of well documented intestinal disease with reports of only 20% of cases occurring prior to its development (5). Review of metastatic cutaneous Crohn’s disease in children, however, reveals 86% of cases (24 of 28) occurring in patients without a known diagnosis of intestinal Crohn’s disease (2,3,5,9,10,12–17,19–25). Nevertheless, all 24 children without a history of intestinal Crohn’s disease were subsequently diagnosed with intestinal disease within 6 years of initial presentation.…”

Granulomatous lymphangitis of the scrotum and penis

“…In the setting of chronic lymphedema, intra‐ and perilymphatic sarcoidal (non‐caseating) granulomas described herein are most accurately classified as granulomatous lymphangitis. In children, granulomatous lymphangitis is a rare cause of genital swelling and in the majority of cases are associated with concomitant or subsequent development of Crohn’s disease 3–15 . Clinically and pathologically, this presentation is remarkably similar to orofacial granulomatosis (cheilitis granulomatosis and Melkersson‐Rosenthal syndrome) 16,17 .…”