Painful bruising syndrome

Introduction

Painful bruising syndrome (PBS) is a chronic, seldom disabling condition, apparently due, in many cases, to allergic sensitivity to red cells in the tissues. Minor extravasation of blood is followed by a severe inflammatory reaction. The condition occurs in young adult females who are nearly always psychiatrically abnormal. They present with recurrent painful erythematous lesions on the extremities, face or trunk, after trauma or emotional stress. Treatment has been unsatisfactory in most cases.

We report a case of this uncommon entity who showed good response to cyproheptadine.

Case Report

A 1 3-year-old girl presented with history of recurrent painful bruise-like discolouration over the lower extremities of six months duration. The lesions appeared suddenly and were heralded by tingling or burning sensation, followed by multiple areas of redness progressing to ecchymoses in a couple of days. Lesions occurred at intervals of 2-3 weeks. They were non pruritic and disappeared spontaneously in a week or so, leaving a bruise-like discolouration. The patient was never disturbed or disabled by the lesions and it was parental concern which brought the case to notice. There was no preceding or concurrent history of injury, psychiatric illness, drug intake, nutritional imbalance or irregularity, bleeding from natural orifices, arthralgia, respiratory infection, central nervous system or gastrointestinal symptoms. No family history of similar illness was present.

Examination revealed multiple tender, faintly erythematous, non oedematous and ecchymotic macules 3-8cm in diameter over the thighs and legs. Mucosae showed no evidence of petecheal/purpuric lesions. General, systemic and psychiatric examination were within normal limits.

Based on the above history and findings on examination, a provisional diagnosis of PBS was made.

Intracutaneous sensitivity test was done to confirm the diagnosis. 0. 5ml of the patient′s own blood as source of RBCs was taken and injected into her forearm. After a period of 24 hours, a rounded area of ecchymosis of diameter 5cm was seen at the area of injection, confirming the diagnosis of PBS. Skin biopsy showed dermal extravasation of RBCs along with haemosiderin around blood vessels surrounded by a lymphohistiocytic infiltrate suggestive of a diagnosis of painful bruising syndrome. Other relevant investigations including platelet count, bleeding, clotting, prothrombin times, liver function tests and serum proteins were within normal limits.

The patient was managed with tables cyproheptadine in a dose of 4mg per day along with tab vitamin C 200mg. There was almost complete regression of lesions in a period of 14 days. No recurrence of lesions was seen during a follow up period of six months.

Discussion

PBS, also called autoerythrocyte sensitization, was first described by Gardner and Diamond in 1955. [1] Lesions are most commonly located over the arms and legs. They are heralded by a burning or stinging sensation, followed a few hours later, by oedema and erythema. Bruising occurs after a day or so. Bleeding from internal organs such as the gastrointestinal tract, genitourinary tract or Intracranially, may occur. [2],[3] However, this was not present in our case. The condition is so bizarre that emergency departments should be aware of its features and differential diagnoses, which include injuries caused by violence, factitious lesions, platelet disorders, drug or chemical induced lesions, coagulation disorders, gangrene and purpura simplex. Though there is no satisfactory treatment of PBS, plasmapheresis, psychotherapy and oral cyproheptadine have been successfully tried in isolated cases. [4],[5]