As previously explored in this journal, the investigation of bleeding symptoms involves
a systematic investigative approach that includes investigation of patient and family
history, physical examination, and laboratory testing.[1 ] Although this is also true of an investigation into pediatric bleeding, there are
special considerations required, including the reduced period of capture for personal
history, and fewer hemostatic challenges.[2 ] Sometimes, such investigations prove fruitless and do not uncover a true bleeding
diathesis. Hematidrosis is one such example.
Hematidrosis is a fascinating disorder characterized by blood oozing from the intact
skin and mucous membranes in the absence of a bleeding problem. It frequently affects
young girls (i.e., typically between 9 and 13 years) under stressful situations, and
probably suffering from an underlying anxiety disorder. These patients usually undergo
a thorough and expensive hematology evaluation aimed to identify a possible underlying
bleeding disorder, but which fails to provide the answer. Here, we report a case of
a 9-year-old girl exposed to bullying at school, which leads to recurrent bleeding
episodes.
The young patient had no prior medical or psychological illness and presented to our
clinic following a 5-month history of recurrent spontaneous blood loss from skin,
scalp, ear, mouth, and eyes ([Fig. 1 ]). Her parents claimed that the episodes happen twice weekly on average, mostly in
the evening, and with no clear precipitating factors. Each episode lasted for 1 or
2 minutes and was usually self-limited. Family history was negative for any bleeding
disorders. She was evaluated by her family physician and a local hematologist, and
a workup for common bleeding disorders was unrevealing ([Table 1 ]). The patient also underwent upper gastrointestinal endoscopy because some of these
episodes were accompanied by bleeding from her mouth. The endoscopy and biopsy were
consistent with celiac disease, which was later confirmed by serological testing.
Nevertheless, no clinical signs of celiac disease could be recorded. Upon her evaluation
in our clinic, her vital signs and growth chart were within normal limits, and physical
examination was unrevealing; specifically, there were no signs of self or secondary-inflicted
injuries (scars, fresh cuts, scratches, or wounds). On further questioning, the parents
reported that these episodes did not occur on holidays and usually happened on the
days she attended school. The patient admitted that she always felt intimidated and
emotionally abused by her school colleagues. A sampling of the fluid discharge confirmed
the presence of red and white blood cells along with epithelial cells.
Table 1
Summary of the relevant investigations performed as a part of the initial bleeding
workup
Test/analyte
Patient result (normal range)
Platelets
241×109 /L (150–400 × 109 /L)
Prothrombin time
12.9 s (12.3–14.2 s)
Activated partial thromboplastin time
31.4 s (30.5–40.4 s)
Factor VIII activity
85% (60–150%)
Factor IX activity
90% (60–150%)
von Willebrand factor: Ristocetin cofactor activity
100 IU/dL (50–200 IU/dL)
von Willebrand factor: Antigen
70 IU/dL (50–150 IU/dL)
Platelet aggregation (adenosine diphosphate, collagen, arachidonic acid, thrombin,
epinephrine, ristocetin) recorded by light transmission
Normal
PFA-100 (C/Epi cartridge)
112 s (78–199 s)
Abbreviations: C/Epi, collagen/epinephrine; PFA-100, platelet function analyser-100.
Fig. 1 Spontaneous blood loss from skin, scalp, ear, mouth, and eyes during one of the bleeding
episodes.
Hematohidrosis or hematidrosis is a rare and largely mysterious phenomenon, with only
a few case reports having been published.[3 ]
[4 ]
[5 ]
[6 ]
[7 ]
[8 ]
[9 ]
[10 ]
[11 ]
[12 ]
[13 ]
[14 ] Reported patients are usually young girls,[3 ]
[5 ] and present with blood oozing from intact skin or mucous membranes in the setting
of stress and anxiety.[11 ] The episodes last for few minutes,[3 ] and resolve almost spontaneously.[5 ] The workup for bleeding disorders is usually normal, and the examination of the
bloody fluid demonstrates the presence of blood elements. It is important to carefully
examine the skin to rule out self-inflicted or secondary injuries (e.g., potentially
pointing to Munchausen's syndrome or Munchausen's by proxy) and to also differentiate
this entity from chromhidrosis (color pigment in sweat), vasculitis, scurvy, or other
connective tissue disorders (where vascular fragility can lead to easy bleeding).
Some of the reported cases underwent biopsy of the oozing areas with active bleeding
and did not show any histopathological findings.[4 ]
[5 ] Although the frightening presentation of hematidrosis leads to immediate medical
evaluation in most of the cases, the diagnosis is often delayed because the condition
is a rare and frequently overlooked phenomenon. Frequently, patients will have extensive
evaluations including hematology consultation before reaching a correct diagnosis.
The etiology remains mostly unknown. Stress-induced vasoconstriction (through adrenergic
stimulation) in the small blood vessels around the sweat glands and eventually leading
to vascular rupture and blood extravasation with the sweat is the proposed cause.[10 ]
[11 ] The fact that most cases respond to β adrenoreceptor blockers and/or anxiolytics
makes this theory plausible.[4 ]
[10 ] Our patient was treated with propranolol 10 mg twice daily and her symptoms resolved.
As in all the reported cases, an underlying cause usually precipitates the episodes
of anxiety that lead to bleeding. The trigger in our case was identified as bullying
at school; the patient and her parents were referred to psychiatry and social services
for further help. The association of hematidrosis with celiac disease in our case
might or might not be significant.
To conclude, hematidrosis is an interesting bleeding phenomenon where a psychological
trigger (rivalry, scolding, punishment, bullying, etc.) leads to a sequence of events
culminating in bleeding from intact skin and mucous membranes. This can be a frightening
experience for the patient and family and often leads to extensive unnecessary testing
and sometimes even to dangerous therapeutic intervention (e.g., a case was treated
with fresh frozen plasma several times[4 ]). Reporting such enigmatic cases might help educate the public as well as the medical
society about a relatively simple yet frightening phenomenon that can exhaust resources
and even lead to unnecessary therapeutic or diagnostic interventions. In the end,
we decided not to pursue additional invasive investigations above those already undertaken
([Table 1 ]) based on our evaluation of this case, successful treatment with propranolol, and
our conclusion that the case was indeed that of the rare condition hematidrosis.
