Plantar fibromatosis and Dupuytren’s contracture in an adolescent

Abstract

Background. Fibromatosis represents a wide group of benign, locally proliferative disorders of fibroblasts. Dupuytren` s disease is a benign proliferative disease of palmar aponeurosis which usually affects adults between 40 and 60 years of age. Ledderhose`s disease or plantar fibromatosis is plantar equivalent of Dupuyten`s disease most often affecting middle- aged and older men, usually bilateral, represented with painless nodule in the medial division of plantar fascia. Case report. We presented a 19-year old adolescent that turned to a plastic surgeon complaining to his small finger contracture. He noticed palmar thickening with nodule over the metacarpophalangeal joint of small finger of his right hand when he was 16 years old. A year later a finger started to band. During physical checkup we noticed plantar nodule that also had his father and grandmother. Magnetic resonance and tumor biopsy confirmed a suspicion on plantar fibromatosis - Ledderhose`s disease. Clinical exam of the hand clearly led to a conclusion that the patient had Dupuytren`s contracture with pretendinous cord over the small finger flexor tendons and lack of extension of proximal interphalangeal (PIP) joint. On the extensor side of the PIP joints there were Garrod`s nodes. The patient refused surgical treatment of plantar tumor, but agreed to surgical correction of finger contracture. Conclusion. Despite the fact that Dupuytren`s disease and plantar fibromatosis are diseases of adults, the possibility of conjoint appearance of these forms of fibromatosis in adolescent period of life should be kept in mind especially in patients with strong genetic predisposition.Uvod. Fibromatoze predstavljaju sirok spektar benignih, lokalno proliferativnih oboljenja fibroblasta. Dupuytren-ova bolest predstavlja benigno proliferativno oboljenje palmarne fascije koje se najcesce javlja kod odraslih u 4. i 5. deceniji zivota. Ledderhose-ova bolest ili plantarna fibromatoza je plantarni ekvivalent Dupuytren-ove bolesti, i najcesce se javlja kod muskaraca srednjeg i starijeg doba, obicno bilateralno, u vidu zadebljanja u predelu svoda stopala. Prikaz bolesnika. Prikazali smo 19-godisnjeg mladica koji se javio na pregled hirurgu plasticaru zbog kontrakture 5. prsta desne sake, u dobi od 16 godina primetio je potkozni cvoric u dlanu, u nivou metakarpofalangealnog zgloba 5. prsta desne sake. Godinu dana kasnije prst je poceo da se savija. Pri pregledu uoceno je da ima i tumefakt na svodu stopala. Istu promenu na stopalu imali su otac i oceva majka. Magnetna rezonancija stopala i biopsija tumefakta potvrdili su sumnju u plantarnu fibromatozu. Klinicki pregled sake ukazao je na jasnu sliku Dupuytren-ove bolesti sa pretendinoznim zadebljanjem nad tetivama pregibaca 5. prsta i deficitom ekstenzije u proksimalnom interfalangealnom (PIP) zglobu. Na dorzumima PIP zglobova uoceni su Garrod-ovi cvorici. Bolesnik je odbio hirursko lecenje promene na stopalu, ali se odlucio za hirursku korekciju kontrakture 5. prsta. Zakljucak. Bez obzira na karakteristicnu pojavu Dupuytren-ove i Ledderhose-ove bolesti kod starije populacije treba imati na umu mogucnost udruzene pojave ovih formi fibromatoza u ranijem, adolescentnom periodu, posebno kod bolesnika sa pozitivnom porodicnom anamnezom.