Abstract Leg ulcers are rarely due to congenital spherocytic anemia but they may sometimes be the presenting manifestation of the disease. Related skin lesions other than ulcer are virtually unknown. This report deals with an unusual case of an erythematous dermatosis of prolonged duration without ulceration resistant to therapy until a splenectomy was performed, together with a summary of the natural history of the ulcers known to be associated with congenital spherocytic anemia. Report of a Case A 46-year-old white housewife presented an eruption of both legs above the ankles of six years' duration. During the preceding year there had been progressively increasing local pruritus and generalized fatigue. Inspection of the legs disclosed a mild erythematous, indurated area involving both legs above the malleoli. At the periphery was a brownish pigmentation as in stasis dermatitis, but no varicosities were present. On closer inspection the involved area revealed punc References 1. Barlow, T., and Shaw, H. B.: Inheritance of Recurrent Attacks of Jaundice and of Abdominal Crises, with Hepatosplenomegaly , Tr. Clin. Soc. London 35:155, 1902. 2. Gendel, B. R.: Chronic Leg Ulcers in Diseases of the Blood , Blood 3:1283 ( (Nov.) ) 1948. 3. The Congenital Hemolytic Anemias: I. Hereditary Spherocytosis , in Dacie, J. V.: The Hemolytic Anemias , New York, Grune & Stratton, Inc., 1954, Chap. 2, pp. 48-93. 4. Meulengracht, E.: Der chronische hereditäre hämolytische Ikterus , Leipzig, Dr. Werner Klinkhardt, 1922. 5. Vaughan, J. M.: The Anaemias , Ed. 2, London, Oxford University Press, 1936. 6. Taylor, E. S.: Chronic Ulcer of the Leg Associated with Congenital Hemolytic Jaundice , J. A. M. A. 112:1574 ( (April 22) ) 1939.Crossref 7. Wintrobe, M. M.: Clinical Hematology , Ed. 4, Philadelphia, Lea & Febiger, 1956. 8. Young, L. E.: Hereditary Spherocytosis , Am. J. Med. 18:486 ( (March) ) 1955.Crossref 9. Leger, L. H., and Orr, T. G.: Chronic Leg Ulcerations in Congenital Hemolytic Jaundice , South. M. J. 33:463 ( (May) ) 1940.Crossref 10. Pascher, F., and Keen, R.: Chronic Ulcers of the Leg Associated with Blood Dyscrasias , A. M. A. Arch. Dermat. & Syph. 66:478 ( (Oct.) ) 1952. 11. Mackee, G. M., and Cipollaro, A. C.: Cutaneous Cancer and Precancer: A Practical Monograph , New York, American Journal of Cancer. 1937, p. 85. 12. Gänsslen, M.; Zipperlen, E., and Schüz, E.: Hemolytic Constitution , Deutsches Arch. Klin. Med. 146:1 ( (Jan.) ) 1925. 13. Gänsslen, M.: Unterschenkelgeschwüre bei hämolytischem Ikterus , Klin. Wchnschr. 9:1308 ( (July 12) ) 1930. 14. Dedichen, H. G.: Ictère hémolytique et ulcère de la jambe , Acta med. scandinav. 77:411, 1932.
A.M.A. Archives of Dermatology – American Medical Association
Published: May 1, 1957