Torrential epistaxis with symmetrical facial-skin ulceration in sickle-cell anaemia.

Epistaxis and skin ulcerations are just two of the protean manifestations of sickle-cell anaemia. Epistaxis is common (Hughes et al., 1940; Wintrobe, 1951), even to the extent of one-third of all patients with a sickle-cell anaemia and a sickle-cell/haemoglobin-C disease (Hook and Cooper, 1958), and can be very severe (Mabayoje, 1956; Hook and Cooper, 1958). Chronic leg ulcers are the commonest skin lesions in sickle-cell anaemia. They were described in-all the earliest reports The most common site for the appearance of ulcers is the lower third of the leg, particularly on the medial side just above the ankle (McGavack and Nussbaum, 1942), but no area of the skin is really exempt. One-third of the ulcers occur bilaterally (McGavack and Nussbaum, 1942); they are usually discreet and rather punched out, and can be of all sizes. In some cases, as in the one described below, ulcers may be covered with a dry serous or seropurulent crust (Cummer and LaRocco, 1940), and may heal only slowly, leaving a scar which is thin, atrophic, smooth, glossy, unpigmented, and surrounded by a pigmented areola (Steinfield and Klauder, 1927). The histo-logical picture of the ulcer is that of a chronic granuloma (Ormsby and Montgomery, 1954). The association in a sickle-cell crisis of severe epistaxis with symmetrical facial-skin necrosis has not been described before. A 16-year-old schoolgirl was admitted very early one morning in January 1961 to Korle Bu Hospital, Accra, with profuse epistaxis which had occurred on and off for the previous week. Facial ulceration was noted about the same time as the epistaxis. She was not in the habit of nose-picking and denied having had recurrent joint pains in the past. On examination she was undersized for her age, and was breathing through her mouth because of profuse nose-bleeding. There was slight icterus with generalized soft lymphadenopathy. Her temperature was 100' F. (37.8' C.), pulse 118/min., B.P. 100/60. The liver was three and the spleen four fingerbreadths palpable, but not tender. The apex beat was not displaced, but a systolic bruit was heard over the whole of the praecordium. The face was covered from the hairline to a horizontal line drawn just beneath the lower lip, and from ear to ear, with symmetrically arranged shallow ulcers, nearly all of which had a dry seropurulent crust. Apart from pallor of her palms and conjunctivae there were no other physical signs. Epistaxis continued intermittently for a …