[Familial epithelioma of Ferguson-Smith about two familial cases].
Two cases of multiple self-healing epithelioma (Ferguson-Smith) are reported in a French family. This dermatosis has been present since the age of 30 in both father and son. The father aged 66 years, farmer, had suffered since 1939 from recurrent spontaneously healing tumors of the face and neck, with deeply pitted scars on the face. He was treated during fifteen years in a cancer institute for multiple well-differentiated " squamous cell carcinoma " on the neck, face, and tongue; local recurrence did not occur after treatment of the active tumors and there was no clinical evidence of secondary lymphnode involvement. His son, aged 30, teacher, came in July with a papular active tumour on the cheek, which was surgically removed; his face and ears soon became affected. In the two cases, numerous biopsies showed in the early stage a great down growth of the epidermis with a central horny plug, like in keratotic papules or small kerato-acan-thomas and in more active lesions, typical appearances of squamous cell epithelioma grade I. The familial incidence is proved in the majority of the 30 cases reported since the first description by Ferguson-Smith in 1934. Only caucasians are concerned, most of them being Scottish. There is no sex predominance. First changes generally occur in young adults on light exposed areas but mucous membranes can be affected. The individual lesions last for several months, but fresh ones appear continuously, so that the disease remains extensive. The cause of these tumors is still unknown; histological and ultrastructural findings cannot explain this condition. Our ultrastructural study is parallel to previous works and fails to find viral particles in keratinocyte nuclei or cytoplasma.
D002294:Carcinoma, Squamous Cell / Q000235:genetics* / Q000473:pathology; D002610:Cheek; D004428:Ear Neoplasms / Q000473:pathology; D005153:Facial Neoplasms / Q000235:genetics; D006258:Head and Neck Neoplasms / Q000473:pathology; D006801:Humans; D008297:Male; D012878:Skin Neoplasms / Q000235:genetics* / Q000473:pathology; D014062:Tongue Neoplasms / Q000473:pathology
Epithéliomatose familiale de Ferguson-Smith. A propos de 2 cas familiaux