Intended for healthcare professionals

Filler

A very rare bleeding disorder

BMJ 1998; 317 doi: https://doi.org/10.1136/bmj.317.7170.1437 (Published 21 November 1998) Cite this as: BMJ 1998;317:1437
  1. Sundaram V Ramanan, associate professor of clinical medicine
  1. University of Connecticut, Connecticut, USA.

    As a haematologist, I am often asked to evaluate patients for a possible bleeding disorder. A common story is that the patient has begun to notice bruising for which no antecedent cause can be identified. Many of the patients are young women who often present in early summer when they are more concerned about cosmetic disfigurement than a serious blood disorder. Such a patient came to me a few years ago. A secretary in her mid 20s, she had noticed the appearance of spontaneous bruises on her limbs, but none on her face or torso. A careful history and physical examination yielded no clue, and all screening tests for a possible bleeding disorder were returned with normal values.

    Fortunately, the textbook provided me with a diagnosis. Purpura simplex, also known as “Devil's Pinches,” is a curious ill defined disorder affecting young women. No underlying cause can be identified, and it is said that the Devil is attracted to these subjects, and expresses his affection by pinching them during his nocturnal forays. Recent studies have suggested that some patients have abnormal platelet function, but the disorder still remains poorly understood. I went through the all too familiar routine, concluding with my masterly diagnosis. This was cheerfully accepted by the patient, and rather reluctantly by the referring physician who would have preferred to have a more solid diagnosis. And that, so to speak, was that.

    A few years later, I called a surgical colleague to refer him a patient. “Hi, Doctor Ramanan, how are you?” was the warm greeting from the receptionist. After assuring her that my health continued to be excellent, I warily inquired if I had ever met her before. “Don't you remember me?” Rather shamefully, I admitted that I did not, attributing the apparent lapse in my memory to the advanced age of 40. “I am the one who came with all the bruising.” “And what happened?” I cautiously inquired. You are reluctant to probe too deeply into your diagnostic failures. “They've all gone,” was the response. I must have uttered some curious sound because she went on, “You won't believe this, Doctor Ramanan. When I got my glasses they all went away.” “Got what?” I responded. “You won't believe this,”she repeated, “but I was as blind as a bat, bumping into everything because I couldn't see where I was going. Once I got my glasses, no more bruises.”

    “Curiouser and curiouser,” as Alice said. I had seen my first (and only)patient with Myopia Haemorrhagica.