Development of Squamous Cell Epithelioma in Epidermolysis Bullosa

Recessive Dystrophic Epidermolysis bullosa and Multiple Squamous Cell Carcinomas

“…Since the first contribution by Klausner [1] in 1913 who described a tumor located on the tongue of a patient with dystrophic epidermolysis bullosa, it seems that no other cases have been reported until 1947 when Halpern [2] described a new case of bilateral squamous cell carcinoma involving the legs of a male, with a process similar to that of our patient.…”

Metastatic Squamous Cell Carcinoma Resembling Angiosarcoma Complicating Dystrophic Epidermolysis bullosa

“…Malignant skin tumours are a well-recognized complication of various types of epidermolysis bullosa [5][6][7][8][9][10] but most frequently are associated with generalized RDEB 11] as in our patient. A list of characteristic fea tures is given in table 1.…”

Clinical Criteria for Differentiating between Recessive and Dominant Forms of Dystrophic Epidermolysis Bullosa, Elaborated from an Analysis of 119 Cases

“…Dental lesions: In RDEB, (+), (-), and N.D. are 17, 2, and 9, respectively. Detailed descriptions were caries in 6 (4,8,15,16), discoloration in 3 (6,19), falling in 2 (22,23), enamel defect in 2 (6), and decay in 1 (17). In DDEB, (+), (-), and N.D. are 8, 70, and 13, respectively.…”

“…In DDEB, (+), (-), and N.D. are 12,67, and 12, respectively. Detailed descriptions were as follows: improvement was seen at the age of 8 (13), 10 (lQ), 12 (13), 18 (25), 26 (13), at the time of puberty (9,21), and at the time of pregnancy on three occasions (23).…”

“…There were 15 kinds of complications in 24 patients with RDEB. Esophageal stenosis in 7 (7,8,(17)(18)(19)22), iron deficiency anemia in 4 (4,17,23), corneal opacities and/or erosion in 6 (6-8, 15, 16), squamous cell carcinoma in 5 (left hand, right hand, right leg, pleura, esophagus) (16)(17)(18)(19)23), keratoconjunctivitis in 3 (6-8), dysphagia in 2 (17,19), severe constipation in 2 (17,19), perforation of the nasal septum in 2 (16,17), dystrophic vertebral column in 1 (4), poor nutrition in 1 (4), amyloidosis (heart, thyroid gland, kidney) in 1 (5), pneumonia in 1 (5), dry conjunctiva in 1 (5), generalized demineralized appearance of bone in 1 (18), renal calculus in 1 (16), hyperglobulinemia in 1 (16), change of voice in 1 (19), noisy breathing in 1 (19), impairment of hearing due to eruptions in the external auditory canals in 1 (15), sepsis in 1 (5), esophageal ulcer in 1 (16) (18). In DDEB, (+), (-), and N.D. are 1, 17, and 73, respectively.…”