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December 1974, Vol 110, No. 6 >
Article | December 1974

Epidermolysis Bullosa Dystrophica With Epidermal Neoplasms

William B. Reed, MD; Joseph College, Jr., MD; Martin J. O. Francis, PhD; Hugh Zachariae, MD; Frederic Mohs, MD; Ian B. Sneddon, MD, FRCP; Mary Ann Sher, BM, BCH
[+] Author Affiliations

Accepted for publication May 21, 1974.

Reprint requests to 1013 W Olive Ave, Burbank, CA 91505 (Dr. Reed).


From the University of California, Irvine (Dr. Reed) and San Diego (Dr. College); the Nuffield Orthopedic Hospital, Oxford, England (Dr. Francis); the University of Aarhus Medical School, Aarhus, Denmark (Dr. Zachariae); the University of Wisconsin Medical School, Madison (Dr. Mohs); University of Johannesburg Medical School, South Africa (Dr. Sher); Rupert Hallam Hospital, Department of Dermatology, Sheffield, England (Dr. Sneddon).


Arch Dermatol. 1974;110(6):894-902. doi:10.1001/archderm.1974.01630120044009
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Patients with epidermolysis bullosa dystrophica (EBD) tend to develop epidermal neoplasms, usually low-grade squamous cell carcinoma of the skin and less commonly that of the mouth. There have been 21 reported cases in the literature, and we are adding seven new cases and providing a further history in a previously reported case.

The average EBD patient develops carcinomas after 30 years of age, and some of these patients live to be 60 years of age before they die from the carcinoma. More than two thirds of the patients die from metastases.

In our patients, carcinoma developed in the mouth, ostium cardiacum, and in the midesophagus, with extension into the lungs. The cause remains unknown, but abnormal collagen scarring is the most likely cause. Carcinoma can probably develop in all the severe dystrophic types of EBD.

REFERENCES

1 +
Klausner E:  Zungenkrebs als Folgezustand bei einem Falle von Epidermolysis bullosa . Arch Dermatol Syphilol 116:71-78, 1913;.
2 +
Halpern LK:  Development of squamous cell epithelioma in epidermolysis bullosa: Report of a case . Arch Dermatol Syphilol 56:517-522, 1947;.
3 +
Rasponi L:  II Cancro sull'epidermolisi bullosa distrofica . Arch Ital Dermatol Sifilogr Venereol 23:19-37, 1950;.
4 +
Garner VC:  Discussion of a case of epidermolysis bullosa . Arch Dermatol Syphilol 62:767, 1950;.
5 +
Calnan CD, Porter AD:  Recessive epidermolysis bullosa with unusual features . Trans St Johns Hosp Dermatol Soc 33:62-65, 1954;
6 +
Wetteland P, Hovding G:  Squamous cell carcinoma in dystrophic epidermolysis bullosa . Acta Derm Venereol 36:27-36, 1956;.
7 +
Leinbrock A:  Epidermolysis bullosa dystrophica et albo-papuloidea (Pasini) et ulcero-vegetans (cum carcinoma) mit diffuser Hämangiomatose bei Schilddrüsen-dysfunktion; wesentlich veränderte Blut-und-Stoffwechselbefunde . Hautarzt 7:395-407, 1956;.
8 +
Rockl H:  Carcinom bei Epidermolysis bullosa dystrophica . Hautarzt 7:463-464, 1956;.
9 +
Schiller F:  Zungencarcinom bei Epidermolysis bullosa dystrophica . Arch Klin Exp Dermato 209:643-651, 1960;.
10 +
Goltz R, Good RA:  Benign hyperglobulinemic purpura: Relation to Mikulicz's disease, sicca syndrome, and epidermolysis bullosa dystrophica . Arch Dermatol 83:26-39, 1961;.
11 +
Sonneck HJ, Hantzchel K:  Über einen Fall von Epidermolysis bullosa dystrophica mit Oesophagusstenose, und Kardiocarcinom . Hautarzt 12:124-125, 1961;.
12 +
Edland RW:  Dystrophic epidermolysis bullosa: Tolerance of the bed and response of multifocal squamous cell carcinomas to ionizing radiation: Report of a case . Am J Roentgenol Radium Ther Nucl Med 105:644-647, 1969;.
13 +
Wechsler HL, Krugh FJ, Domonkos AN, et al:  Polydysplastic epidermolysis bullosa and development of epidermal neoplasms . Arch Dermatol 102:374-380, 1970;.
14 +
Eastwood DS:  Autographing in the treatment of squamous cell carcinoma in epidermolysis bullosa dystrophica . Plast Reconstr Surg 49:93-95, 1972;.
15 +
Cornelius CE, Shelley WB:  Syndactylism, dyschromia, and the arsenical dilemma . Arch Dermatol 98:107-110, 1968;.
16 +
Sneddon I:  Carcinoma with epidermolysis bullosa . Read before the British Association of Dermatology, Sheffield, England, 1970 .
17 +
Francis MJO, MacMillan DC:  The extraction of polymenic collagen from biopsies of human skin . Biochem Biophys Acta 251:236-245, 1971;.
18 +
Lazarus GS:  Collagenase and connective tissue metabolism in epidermolysis bullosa . J Invest Dermatol 58:642-648, 1972;.
19 +
Gedde-Dahl T Jr: Epidermolysis Bullosa: A Clinical Genetic and Epidermiological Study . Baltimore, Johns Hopkins Press, 1971;.
20 +
Pearson R: The Mechanobullous Diseases: Dermatology in Medicine . New York, McGrawHill Book Co Inc, 1971;, pp 621-643.
21 +
Pers M:  Skin grafting in a case of epidermolysis bullosa . Acta Chir Scand 129:333-339, 1965;.
22 +
Briggaman RA, Dolldorf FG, Wheeler CE Jr:  Formation and origin of basal lamina and anthormy fibrils in adult human skin . J Cell Biol 51:381-392, 1971;.
23 +
Briggaman RA:  Epidermolysis bullosa . Read before the Academy of Dermatology, Chicago, 1973 .
24 +
Eisen AZ:  Human skin collagenase relationship to the pathogenesis of epidermolysis bullosa dystrophica . J Invest Dermatol 52:449-453, 1969;.
25 +
Hashimoto K, Yamanishi Y, Maeyeus E, et al:  Collagenenolytic activities of squamous cell carcinoma of the skin . Cancer Res 33:2790-2801, 1973;.
26 +
Lazarus GS, Daniels JR, Brown RS, et al:  Degradation of collagen by human granulocyte collagenolytse system . J Clin Invest 47:2622-2629, 1968;.
27 +
Michaeli D, Fudenberg HN:  Incidence of antibodies to denatured collagen in rheumatoid arthritis . Arthritis Rheum 14:404, 1971;.
28 +
Treves N, Pack GT:  The development of cancer in burn scars: An analyses and report of 34 cases . Surg Gynecol Obstet 51:749-782, 1930;.
29 +
Willis RA:  Further studies on the mode of origin of carcinomas of the skin . Cancer Res 5:469-479, 1945;.
30 +
Lawrence EA, Moore TC:  Carcinoma arising in the scars of thermal burns . Surg Gynecol Obstet 95:579-588, 1952;.
31 +
Cruickshank AH, McConnell EM, Miller DG:  Malignancy in scars, chronic ulcers, and sinuses . J Clin Pathol 16:573-580, 1963;.
32 +
Lund HZ:  How often does squamous cell carcinoma of the skin metastasize? Arch Dermatol 92:635-637, 1965;.
33 +
Abels D, Reed WB:  Fanconi-like syndrome . Arch Dermatol 107:419-423, 1973;.
34 +
Pindborg J: Atlas of Diseases of the Oral Mucosa . Philadelphia, WB Saunders, 1968;, pp 115-118.
35 +
DeMoragas JM, Castillo R:  Anomalias de la coagulacion en la epidermolysis ampollosa , in: Acta. del VI Congreso Ibero-Latino-Americano de Dermatologia . Barcelona, Cientifico-Medica, 1970;, pp 247-251.
36 +
Crikelair GF, Hoehn RJ, Domonkos AN, et al:  Skin homografts in epidermolysis bullosa dystrophica . Plast Reconstr Surg 46:89-92, 1970;.
37 +
Gwinn SL, Lee FA, Sugarman GI:  Radiological case of the month: Epidermolysis bullosa dystrophica . Am J Dis Child 123:231-232, 1972;.
38 +
Brownstein MH, Helwig E:  Systemic amyloidoses complicating dermatoses . Arch Dermatol 102:1-7, 1970;.
39 +
Roenigk HH, Ryan IG, Bergfeld WF:  Epidermolysis bullosa acquista . Arch Dermatol 103:1-10, 1971;.
40 +
Isobe T, Osserman EF:  Patterns of amyloidosis and their association with plasma cell dyscrasia, monoclonal immunoglobulins and Bence-Jones protein . N Engl J Med 290:473-477, 1974;.
41 +
Elliot GT:  Two cases of epidermolysis bullosa . J Cut Genitocium Dis 13:10-18, 1895;.
42 +
Almeyda J:  Epidermolysis bullosa associated with neoplasm of the bronchus . Br J Dermatol 87:70-71, 1972;.
43 +
Didokar MS, Grener RE, Moore GE:  Epidermolysis bullosa dystrophica and epithelioma of the skin . Cancer 33:198-202, 1974;.

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1 +
Klausner E:  Zungenkrebs als Folgezustand bei einem Falle von Epidermolysis bullosa . Arch Dermatol Syphilol 116:71-78, 1913;.
2 +
Halpern LK:  Development of squamous cell epithelioma in epidermolysis bullosa: Report of a case . Arch Dermatol Syphilol 56:517-522, 1947;.
3 +
Rasponi L:  II Cancro sull'epidermolisi bullosa distrofica . Arch Ital Dermatol Sifilogr Venereol 23:19-37, 1950;.
4 +
Garner VC:  Discussion of a case of epidermolysis bullosa . Arch Dermatol Syphilol 62:767, 1950;.
5 +
Calnan CD, Porter AD:  Recessive epidermolysis bullosa with unusual features . Trans St Johns Hosp Dermatol Soc 33:62-65, 1954;
6 +
Wetteland P, Hovding G:  Squamous cell carcinoma in dystrophic epidermolysis bullosa . Acta Derm Venereol 36:27-36, 1956;.
7 +
Leinbrock A:  Epidermolysis bullosa dystrophica et albo-papuloidea (Pasini) et ulcero-vegetans (cum carcinoma) mit diffuser Hämangiomatose bei Schilddrüsen-dysfunktion; wesentlich veränderte Blut-und-Stoffwechselbefunde . Hautarzt 7:395-407, 1956;.
8 +
Rockl H:  Carcinom bei Epidermolysis bullosa dystrophica . Hautarzt 7:463-464, 1956;.
9 +
Schiller F:  Zungencarcinom bei Epidermolysis bullosa dystrophica . Arch Klin Exp Dermato 209:643-651, 1960;.
10 +
Goltz R, Good RA:  Benign hyperglobulinemic purpura: Relation to Mikulicz's disease, sicca syndrome, and epidermolysis bullosa dystrophica . Arch Dermatol 83:26-39, 1961;.
11 +
Sonneck HJ, Hantzchel K:  Über einen Fall von Epidermolysis bullosa dystrophica mit Oesophagusstenose, und Kardiocarcinom . Hautarzt 12:124-125, 1961;.
12 +
Edland RW:  Dystrophic epidermolysis bullosa: Tolerance of the bed and response of multifocal squamous cell carcinomas to ionizing radiation: Report of a case . Am J Roentgenol Radium Ther Nucl Med 105:644-647, 1969;.
13 +
Wechsler HL, Krugh FJ, Domonkos AN, et al:  Polydysplastic epidermolysis bullosa and development of epidermal neoplasms . Arch Dermatol 102:374-380, 1970;.
14 +
Eastwood DS:  Autographing in the treatment of squamous cell carcinoma in epidermolysis bullosa dystrophica . Plast Reconstr Surg 49:93-95, 1972;.
15 +
Cornelius CE, Shelley WB:  Syndactylism, dyschromia, and the arsenical dilemma . Arch Dermatol 98:107-110, 1968;.
16 +
Sneddon I:  Carcinoma with epidermolysis bullosa . Read before the British Association of Dermatology, Sheffield, England, 1970 .
17 +
Francis MJO, MacMillan DC:  The extraction of polymenic collagen from biopsies of human skin . Biochem Biophys Acta 251:236-245, 1971;.
18 +
Lazarus GS:  Collagenase and connective tissue metabolism in epidermolysis bullosa . J Invest Dermatol 58:642-648, 1972;.
19 +
Gedde-Dahl T Jr: Epidermolysis Bullosa: A Clinical Genetic and Epidermiological Study . Baltimore, Johns Hopkins Press, 1971;.
20 +
Pearson R: The Mechanobullous Diseases: Dermatology in Medicine . New York, McGrawHill Book Co Inc, 1971;, pp 621-643.
21 +
Pers M:  Skin grafting in a case of epidermolysis bullosa . Acta Chir Scand 129:333-339, 1965;.
22 +
Briggaman RA, Dolldorf FG, Wheeler CE Jr:  Formation and origin of basal lamina and anthormy fibrils in adult human skin . J Cell Biol 51:381-392, 1971;.
23 +
Briggaman RA:  Epidermolysis bullosa . Read before the Academy of Dermatology, Chicago, 1973 .
24 +
Eisen AZ:  Human skin collagenase relationship to the pathogenesis of epidermolysis bullosa dystrophica . J Invest Dermatol 52:449-453, 1969;.
25 +
Hashimoto K, Yamanishi Y, Maeyeus E, et al:  Collagenenolytic activities of squamous cell carcinoma of the skin . Cancer Res 33:2790-2801, 1973;.
26 +
Lazarus GS, Daniels JR, Brown RS, et al:  Degradation of collagen by human granulocyte collagenolytse system . J Clin Invest 47:2622-2629, 1968;.
27 +
Michaeli D, Fudenberg HN:  Incidence of antibodies to denatured collagen in rheumatoid arthritis . Arthritis Rheum 14:404, 1971;.
28 +
Treves N, Pack GT:  The development of cancer in burn scars: An analyses and report of 34 cases . Surg Gynecol Obstet 51:749-782, 1930;.
29 +
Willis RA:  Further studies on the mode of origin of carcinomas of the skin . Cancer Res 5:469-479, 1945;.
30 +
Lawrence EA, Moore TC:  Carcinoma arising in the scars of thermal burns . Surg Gynecol Obstet 95:579-588, 1952;.
31 +
Cruickshank AH, McConnell EM, Miller DG:  Malignancy in scars, chronic ulcers, and sinuses . J Clin Pathol 16:573-580, 1963;.
32 +
Lund HZ:  How often does squamous cell carcinoma of the skin metastasize? Arch Dermatol 92:635-637, 1965;.
33 +
Abels D, Reed WB:  Fanconi-like syndrome . Arch Dermatol 107:419-423, 1973;.
34 +
Pindborg J: Atlas of Diseases of the Oral Mucosa . Philadelphia, WB Saunders, 1968;, pp 115-118.
35 +
DeMoragas JM, Castillo R:  Anomalias de la coagulacion en la epidermolysis ampollosa , in: Acta. del VI Congreso Ibero-Latino-Americano de Dermatologia . Barcelona, Cientifico-Medica, 1970;, pp 247-251.
36 +
Crikelair GF, Hoehn RJ, Domonkos AN, et al:  Skin homografts in epidermolysis bullosa dystrophica . Plast Reconstr Surg 46:89-92, 1970;.
37 +
Gwinn SL, Lee FA, Sugarman GI:  Radiological case of the month: Epidermolysis bullosa dystrophica . Am J Dis Child 123:231-232, 1972;.
38 +
Brownstein MH, Helwig E:  Systemic amyloidoses complicating dermatoses . Arch Dermatol 102:1-7, 1970;.
39 +
Roenigk HH, Ryan IG, Bergfeld WF:  Epidermolysis bullosa acquista . Arch Dermatol 103:1-10, 1971;.
40 +
Isobe T, Osserman EF:  Patterns of amyloidosis and their association with plasma cell dyscrasia, monoclonal immunoglobulins and Bence-Jones protein . N Engl J Med 290:473-477, 1974;.
41 +
Elliot GT:  Two cases of epidermolysis bullosa . J Cut Genitocium Dis 13:10-18, 1895;.
42 +
Almeyda J:  Epidermolysis bullosa associated with neoplasm of the bronchus . Br J Dermatol 87:70-71, 1972;.
43 +
Didokar MS, Grener RE, Moore GE:  Epidermolysis bullosa dystrophica and epithelioma of the skin . Cancer 33:198-202, 1974;.

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