Case Analysis Of Hematohidrosis A Lacrimation Sweat Disorder

Hematohidrosis/hemidrosis is a well-known clinical condition, dating back to a report of Leonardo Da Vinci who described a soldier who had bloody lacrimation when he was asked to go to battle. Jesus Christ experienced hematohidrosis while praying in the garden of Gethsemane before his crucifixion as mentioned in the Defenders Bible by Physician Luke as “and being in anguish he prayed more earnestly and his sweat was like drops of blood falling to the ground”.

In this rare clinical condition, human being sweats blood under conditions of extreme physical or emotional stress, it may be associated with bloody otorrhea, bloody lacrimation or bleeding from salivary glands. In this condition, there is a spontaneous painless bleeding through unbroken skin in any part of the body or from salivary, lacrimal or perifollicular sweat gland.

The diagnosis of hematohidrosis is based upon the presence of bloody discharge without any obvious cause through intact skin, witnessed and confirmed by health professional/doctor/nurse and the presence of blood components on biochemistry studies of the discharge. Treatment with propranolol is effective in patients with hematohidrosis, but psychoanalysis and antianxiety medications are also needed in most cases. In this study, we described two cases of hematohidrosis and reviewed the literature. The purpose is to raise awareness of hematohidrosis among hematologists, pediatricians and psychiatrists.

Case 1

An eight year-old-girl presented with bleeding from intact skin of forearm, leg and face for a period of 6 months. The patient had no history of any previous bleeding disorders or coagulation disorders in her family. She was fully investigated for bleeding disorders, but all tests including PT, PTT, BT, Factor XIII, Von Willebrand Factor and Fibrinogen were within normal limits. Her mother has taken some photos of her during bleeding episodes. She was asked to go to the toilet while the interview with her parents was continuing and we saw bleeding from her face, forearms, legs and abdomen when she came back to her parents. Detailed history revealed that most of her bleedings were associated with a fear of aloneness or when she goes to the toilet, without any sign of self-injury or abrasion on skin.

A sample from bleeding site was sent to the coagulation laboratory which showed 30% hematocrit and peripheral blood smears revealed all components of the blood cells. Propranolol (non-cardioselective beta-adrenergic antagonist) 10 mg, oral, twice per day was started causing partial improvement, but she had significant improvement after starting pediatric psychiatric consultation including psychotherapy and taking tricyclic antidepressant (nortriptyline 10 mg, oral, once per day at night) as antianxiety medication. She is under close follow up with no complaint of bleeding event. Written informed consent was taken from her parents.

Case 2

A 12 year-old girl was referred to hematologist due to bleeding episode from ears, eyes, nostrils and sublingual area without history of trauma. All coagulation profiles were within normal limits with no previous history of bleeding disorders in the patient or her family. Her mother has taken many photos during her bleeding episodes without any sign of self-inflicting. Although no anxiety or fear history was taken, psychoanalytic consultation was requested and a conflict between this girl and her obsessive mother was detected. Propranolol 10 mg, oral, twice per day was started with minimal response but additional treatment with antianxiety (nortriptyline 10 mg, oral, once per day at night) medication along with psychotherapy was associated with significant improvement .She is under regular follow up with no complaint of bleeding event. Written informed consent was taken from patient and her parents.

Discussion

Hematohidrosis is a rare clinical condition that presents with bleeding diathesis. It manifests with episodes of spontaneous discharge of bloody secretion through intact skin or sweat gland orifices, with an unknown cause. Due to response to propranolol in some cases with this history, a theory has been suggested. In this hypothesis, increased vascular pressure or sympathetic hyperactivity leads to the blood cell passage through the perifollicular ducts of the sweat glands, causing periglandular vessel contraction and consequent expansion, allowing the passage of blood cells into ducts.6-8 Skin biopsies during remission period revealed no blood cells within sweat glands in some reported cases which supports this theory. Under the stress condition or sympathetic hyperactivity, the vessels contract. Consequently, as the anxiety leads to dilation of blood vessels, the blood goes into the sweat glands, which pushes the blood to the surface, manifesting as droplets of blood mixed with sweat. In our report, anxiety was strongly associated with both cases, causing bleeding from intact skin which had good response to anti-anxiety medication and psychotherapy.

This approach concentrates on alerting physicians, nurses and health professionals to consider different aspects of psychological problems in such rare bleeding manifestations along with normal coagulation tests. High suspicion to this disease in pediatric age group is more important than adults because of the presumption of absence of history of psychological issues taken from parents like our second case report. Response to antianxiety or beta-adrenergic blocker medicines and psychotherapy could be a good strategic approach which is both diagnostic and therapeutic when all coagulation profiles are normal as we observed in our cases and other case reports.2

In conclusion, hematohidrosis should be taken into consideration when faced with emotional stress, depression or anxiety in cases that present with bleeding manifestations from sweat glands and negative hemostatic coagulation tests.