Pyoderma Gangrenosum and Sterile Multifocal Osteomyelitis Preceding the Appearance of Takayasu Arteritis

Abstract

A 9-month-old infant had pyoderma gangrenosum (PG) and sterile osteomyelitis. Three years later the patient developed Takayasu arteritis (TA). Sterile osteomyelitis was reported in approximately 30 patients with different skin lesions, but never together with pyoderma gangrenosum. The association of PG and TA has been reported rarely, and then mainly in adults or in children over age 9 years. This rare association should alert physicians with a case of infantile PG of unknown etiology to consider the possible diagnosis of TA.

Chronic Recurrent Multifocal Osteomyelitis

“…Palmoplantar pustulosis is one of the most common diseases associated with CRMO, occurring in 20-50% of cases [3, 5, 8-10, 12, 19] and frequently recurring synchronously along with the osseous exacerbations [20]. Other dermatologic manifestations include psoriasis vulgaris [5, 8-12, 19, 21], acne [22], Sweet syndrome [23,24], pyoderma gangrenosum [11,25,26], dermatomyositis [4] and tumor calcinosis [27]. Gastrointestinal associations include Crohn disease [5,11,[28][29][30], ulcerative colitis [5] and celiac disease [5].…”

“…Gastrointestinal associations include Crohn disease [5,11,[28][29][30], ulcerative colitis [5] and celiac disease [5]. The spectrum of diseases associated with CRMO is large, also including sclerosing cholangitis [11,29], granulomatosis with polyangiitis [11], Takayasu's arteritis [9,10,26], Ollier disease (multiple enchondromatosis) [11] and parenchymal lung disease [31,32].…”

An Unusual Association of Chronic Recurrent Multifocal Osteomyelitis, Pyoderma Gangrenosum, and Takayasu Arteritis

“…Occurrence of the 3 conditions together is rare: In the literature to date, 2 similar cases have been reported. In the study by Dagan, et al with a similar pattern of occurrence 4 and in a study by Ghosn, et al 5 , a similar clinical spectrum of TA, malignant PG, and relapsing polychondritis was reported. Both these patients' treatments with steroids and immunomodulators resulted in dramatic response.…”

Infantile pyoderma gangrenosum

“…It has been estimated that 4% of PG cases are in infants and children 1 and, to our knowledge, there are only 12 cases reported in the literature of PG in infants (\1 year of age). [2][3][4][5][6][7][8][9][10][11][12][13] Eight of the reported patients with infantile PG had an underlying medical condition (Table I). Two infants had Takayasu's arteritis, 2,3 one of whom also had sterile osteomyelitis 3 and one further patient had chronic recurrent multifocal osteomyelitis.…”