Scurvy presenting with cutaneous and articular signs and decrease in red and white blood cells

A 23-year-old man was admitted to the hematology clinic for evaluation of perifollicular hemorrhages, arthralgias, myalgias, fatigue, and sudden difficulty to stand and walk, all of 3-weeks' duration. On questioning about his dietary habits, we found that, for the past 10 months, the patient had consumed sandwiches, tea, chocolate, beer, or eaten fast foods and had eaten fresh fruits or vegetables rarely. His purpuric lesions were associated with a hair follicle and follicular hyperkeratosis, some of which were palpable, were particularly prominent over the legs and sporadically on his trunk, distal part of the arms, and abdomen. His gums were edematous, hemorrhagic, erythematous, and eroded. Corkscrew hairs were observed. His right knee was swollen and tender, and the patient was unable to walk because of pain and weakness. The patient's serum ascorbic acid level was 0.2 mg per dL (normal, 0.2 to 2.0 mg per dL). The hemoglobin level was 5.6 g per dL, and the hematocrit 17.1% with microcytic, hypochromic indices; the uncorrected reticulocyte count was 3.3%. The white blood cell count was 2700 cells per μL, with 57% neutrophils, 37% lymphocytes, 1% monocyte, and 5% eosinophils, and the platelet count was 268,000 per μL. The peripheral smear revealed small red cells with pale cytoplasm and polychromasia; basophilic stippling and hyper-segmented polymorphonuclear leukocytes were also noted. A bone marrow aspiration showed erythroid hyperplasia with a normal differential count of the myeloid cell line. Microscopic examination of a 4-mm skin punch biopsy specimen from the left leg showed extravasations of red cells in the upper dermis. There was no inflammation or vasculitis, but focally scattered perivascular deposits of hemosiderin were observed in the dermis. Intrafollicular keratotic plugs and coiled hair sections were also seen. The stool was hemoccult-positive. Analysis of the right knee joint fluid showed many red cells. An upper gastrointestinal endoscopic examination showed multiple mucosal hemorrhagic lesions extending from the distal esophagus to the duodenum. Within 72 hours after beginning therapy with parenteral ascorbic acid (250 mg per day) the patient showed dramatic clinical improvement. The purpuric skin lesions began to fade and the gingival erosions began to heal. The patient's hemoglobin level rose to 14.5 g per dL, the hematocrit to 44.1%, and the white blood count to 5200 cells per μL in 2 months after he was discharged. At this time, ascorbic acid supplementation was discontinued and he was instructed on proper dietary requirements.