Metastatic crohn’s disease in a child: rare extraintestinal manifestation

Abstract

Extraintestinal manifestations occur in every second child with Crohn’s disease, can affect any organ system, and determine the type of therapy and prognosis of the disease. The skin is the most frequent “target organ” of all extraintestinal manifestations, the spectrum of skin lesions is extremely clinically and pathogenically heterogeneous. Metastatic Crohn’s disease (MBC) is a rare extraintestinal manifestation of the disease, approaches to the diagnosis and management of which are not standardized in either adults or children. The most frequent description of MBC in the literature is skin damage, although interstitial lung damage, bone and muscle damage, and ENT organs, could be destructive. MCD lesions vary in morphology and can arise anywhere on the skin, rarely undergo reverse development, require special approaches to therapy. The primary diagnosis of such elements is extremely difficult, often delated, biopsy is required for a definitive diagnosis - the detection of epithelioid granuloma and its elements outside the gastrointestinal tract. Therapeutic approaches to MCD are not standardized now, they are carried out according to the protocols of CD management, and the effect of the use of oral steroids, a-TNFa drugs is shown. A case of metastatic Crohn’s disease in a 16-year-old teenager with an unusual localization - the nasal septum is described. The destructive nature of the lesion with the formation of perforation of the nasal septum determined the difficult path of differential diagnosis with Wegener’s granulomatosis. Morphological examination of the biopsy of the nasal mucosa revealed epithelioid cells of sarcoid-like granuloma. Systemic glucocorticosteroids use (GCS) led to epithelialization of the nasal septum defect and a decrease in the activity of CD in the intestine mucosa.