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A spontaneous hematoma in a healthy young woman

      Keywords

      1. Case description

      A 40-year-old woman presented herself with a large hematoma of her left lower limb which appeared spontaneously three weeks prior to her visit. The hematoma was responsible for a symptomatic anemia, so she was transfused with two packed red blood cells. Her past medical history was marked only by a depressive syndrome. She was currently taking neither medication nor drugs.
      She had visited a doctor during the past week for this hematoma, a purpura involving both of her legs for six months, and a possible diagnosis of erythema nodosum. She had reported occasional epistaxis. Nothing else had been noted during the clinical examination. Laboratory results had come back with a normal blood count except for mild anemia, normal coagulation tests, normal renal and hepatic functions, no evidence for an auto-immune disease or a viral infection. A skin biopsy of the purpura had been made but the results were still in progress at the time of hospitalization.
      Our examination revealed a perifollicular distribution of purpura and hair dystrophies (Fig. 1).
      Fig. 1
      Fig. 1Right leg. Perifollicular purpura (panel A). Hair dystrophies (panel B): corkscrew (arrows) and swan-neck (arrow heads) dystrophies.
      What is the diagnosis?

      2. Diagnosis

      A spontaneous hematoma alongside normal coagulation tests and those findings were consistent with scurvy. We thought that past erythema nodosum were misdiagnosed spontaneous hematomas. Detailed questioning revealed a diet restricted to pasta. Dental hygiene was poor without hemorrhagic gingivitis. A blood sample for vitamin C serum level dosing was taken and the patient immediately treated with ascorbic acid infusions followed by oral supplementation one week after. Nutritional evaluation brought out B9 vitamin and iron deficiencies. Skin biopsy analysis showed perifollicular red blood cells extravasation consistent with scurvy as well. Vitamin C serum level was low (0,6 mg/l; N: 5–17). At week three, purpura has vanished and hematoma was improving.
      Scurvy's dermatological manifestations are due to collagen alteration [
      • Khonsari H.
      • Grandière-Perez L.
      • Caumes E.
      Scurvy, a re-emerging disease.
      ]. Corkscrew hair dystrophy is well known. Swan-neck hair dystrophy is less known but just as evocative. Oral involvement is characteristic but not systematic because of its late onset. Diagnosis of scurvy is clinical, thought histological analysis of a skin biopsy may help. Asymptomatic low vitamin C level is frequent [
      • Colacci M.
      • Gold W.L.
      • Shah R.
      Modern-day scurvy.
      ].
      Risk factors for scurvy are not always obvious, as in our patient: clinicians should be aware of it and openly ask for diet restrictions. Clinical manifestations need to be known to avoid such serious complications. Treatment is simple and risk-free. Other deficiencies must be screened for, especially iron, because of vitamin C implications in its metabolism [
      • Colacci M.
      • Gold W.L.
      • Shah R.
      Modern-day scurvy.
      ].

      Declaration of Competing Interest

      None.

      Appendix. Supplementary materials

      References

        • Khonsari H.
        • Grandière-Perez L.
        • Caumes E.
        Scurvy, a re-emerging disease.
        Rev Med Interne. 2005; 26: 885-890
        • Colacci M.
        • Gold W.L.
        • Shah R.
        Modern-day scurvy.
        CMAJ. 2020; 192: E96