|Year : 1992 | Volume
| Issue : 4 | Page : 277-279
Painful bruising syndrome presenting as Persistent haematuria
Ajay Poonia, G Kalla, RP Agrawal, DK Kochar
Source of Support: None, Conflict of Interest: None
A 35-year old hysterical woman had persistent gross haematuria. She started developing painful ecchymosis 4 years after the onset of haematuria. The diagnosis of painful bruising syndrome was confirmed by intracutaneous sensitivity test and the patient responded excellently to cyproheptadine.
|How to cite this article:|
Poonia A, Kalla G, Agrawal R P, Kochar D K. Painful bruising syndrome presenting as Persistent haematuria. Indian J Dermatol Venereol Leprol 1992;58:277-9
|How to cite this URL:|
Poonia A, Kalla G, Agrawal R P, Kochar D K. Painful bruising syndrome presenting as Persistent haematuria. Indian J Dermatol Venereol Leprol [serial online] 1992 [cited 2019 Jun 16];58:277-9. Available from: http://www.ijdvl.com/text.asp?1992/58/4/277/3818
| Introduction|| |
Painful bruising syndrome (PBS) is a rare chronic purpuric state mainly affecting emotionally disturbed adult women. It is characterized by repeated crops of ecchymoses and bleeding from other sites. We report a case.
| Case Report|| |
A 35-years-old housewife presented with 10 days history of mild dull aching pain in both lumbar regions and haematuria, which was not associated with dysuria, urinary frequency, arthralgia, or fever. There was no history of allergy to any drug. Her past history revealed episodes of hysterical fits occurring regularly during the last 4 years.
Urinary sediment revealed 80-100 red cells and 2-3 WBC per high power field. Urine culture was sterile for pyogenic organisms and acid-fast bacilli.
All routine haematological investigations were normal.
On intravenous urography and bilateral retrograde pyelography the collecting system on right side was found to be duplex. Cystoscopy, performed during an episode of haematuria, evinced 2 ureteral openings on the right side and 1 on the left side. Catheterization of ureters showed bloody urine coming from the left ureter. Abdominal aortography and selective renal arteriography demonstrated 2 arteries supplying the right kidney. There was no other abnormality.
A percutaneous renal biopsy was performed. Frozen cryostat sections stained with fluorescein-isothiocynate conjugated antiserum to human IgG, IgM, IgA, C3, and fibrinogen were examined but no significant change was found which could account for haematuria.
Four years later she developed ecchymosis over face, left shoulder, and both lower extremities. The eruptions appeared suddenly with a stinging sensation followed by redness progressing to ecchymosis in 24 to 48 hours [Figure - 1]. The lesions were extremely painful and tender, appeared in crops, and faded spontaneously in 7-10 days leaving hyperpigmentation.
Skin biopsy specimen revealed extravasated red cells with no evidence of a vascular lesion [Figure - 2].
The presence of painful ecchymotic spots, painless idiopathic haematuria, normal routine blood investigations with normal coagulation time in a hysterical lady led to the clinical diagnosis of PBS.
Intracutaneous sensitivity test was performed to confirm the diagnosis. 0.1 ml of autologous washed red blood cells was injected intradermally. The skin was observed immediately, 4 to 12 hours, and then 24 hours after injection for changes in colour, erythema, induration, and oedema. Within 24 hours an 8 cm x 6 cm area of painful erythematous induration with central ecchymosis developed at the site of injection. The eruption was similar to those which had appeared spontaneously. The induration and ecchymosis persisted for 6-8 days and then began to fade. This phenomenon confirmed the diagnosis of PBS and the patient was put on cyproheptadine 4 mg 8 hourly. After a period of 7 days no fresh bruising appeared and during a follow up of 3 months the patient did not have any fresh skin lesion, however, haematuria persisted unabated.
| Comments|| |
Haematuria is not an unusual feature of PBS. Bleeding from other sites like gastrointestinal tract and intracranial bleeding has also been reported. In these cases bleeding was attributed to ecchymotic - like lesion in these areas, possibly because of specific vasculitis associated with red cell sensitivity.  The patients with this disorder are women with profound emotional problems and psychophysiological mechanism has been put forward to explain the bleeding episodes. [z] McIntosh et al (1977) suggested that haematuria was probably because of immune-deposit nephritis. 
There is no specific treatment for this disorder though repeated intramuscular injections of whole blood and cyproheptadine have been tried with varying success. In our case oral use of cyproheptadine gave a very good response.
This case is peculiar in one aspect that gross haematuria was the only feature for 4 years till the skin lesions appeared. The cases of idiopathic haematuria should be screened for this rare disorder by autoerythrocytic sensitivity test.
| References|| |
|1.||Gardner FH, Diamond LK. Autoerythrocyte sensitization. A form of purpura producing painful bruising following autosensitization to red cells in certain women. Blood 1955; 10: 675. [PUBMED] |
|2.||Agle DP, Ratnoff OD. Purpura as a psychosomatic entity: a psychiatric study of autoerythrocyte sensitization. Int Med 1962: 109:685. [PUBMED] |
|3.||McIntosh et al. Nephropathy associated with Gardner diamond syndrome: autologus erythrocyte antigen-antibody immune complex disease. New Eng J Med 1977;296:1265. |
[Figure - 1], [Figure - 2]
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