Background
Etymology: Greek: Haïma, meaning Blood; Hïdrös, meaning Sweat.
Hematidrosis is an extremely rare condition in which the patient spontaneously starts sweating blood. Literature and research regarding such cases are not easily available due to their rarity. It often occurs in patients suffering from stress and anxiety. Here, in this case report, we try to understand the clinical presentation, associated fear and stigma, investigations, and treatment options for hematidrosis.
There have been few historical references made to this disease, However, most such references were connected to religious explanations, rather than scientific ones. In the Bible, it is said that Jesus Christ experienced hematidrosis before the crucifixion, as Luke 22:44 says, “…and being in anguish, he prayed more earnestly, and his sweat was like drops of blood falling to the ground.”
One of the earliest mentions of this disease has been done by Leonardo da Vinci in the 15th century when he described a soldier who sweats blood in stressful situations.
Even in today’s modern era, sweating blood without injury often raises fear and distress in patients and their families.
Duan et al. [1] mentioned such a case associated with primary thrombocytopenic purpura and Migliorini has stated a case of Hematidrosis otorrhea with otoerythrosis [2]. Herein, we present a case report of isolated hematidrosis without any other associated systemic manifestations.
Case Presentation
A 10-year-old female presented with complaints of episodes of bleeding from the skin for the last 1 year. The primary areas involved were the trunk - near the umbilicus, forehead, oral cavity, and groin (Fig. 1 and 2). These episodes were intermittent with a frequency of one episode every 1-2 days and occasional multiple episodes in a day as well.
The bleeding was peculiar in three ways:
Bleeding was from intact skin or mucosa.
Bleeding was episodic, spontaneous, and self-limiting, lasting only a few minutes.
After the bleeding stops, there will not be any visible cuts or abrasions or trauma to the skin.
These episodes of bleeding were not associated with or preceded by fear, stress, or other precipitating factors, and were spontaneous. At the beginning of each episode, profuse sweating will start, which will soon be followed by bright red-colored blood coming out of the skin. In most episodes, only a single site was involved, with no multiple areas of bleeding in the same episode. After a few minutes, the bleeding used to stop eventually on its own, with no subsequent symptoms. Each episode will last for 7-8 minutes, and the bleeding was self-limiting. The general condition of the patient remained all right with normal vitals.
There was no history of usage of anti-coagulants or other blood thinners. There was no major medical or surgical history. Family history was as well, non-significant.
Upon the general and systemic examination, no major abnormalities were detected. The area of bleed appeared normal post-bleeding and had no tenderness or visible swelling (Fig. 3). Upon further examination, bleeding would not occur if the concerned area were squeezed or compressed.
The examination of secretion revealed that it was bright red in color, with no clots or clumps in it, and its viscosity was slightly less than the blood. Microscopic examination showed multiple red blood cells with few white blood cells as well. All her blood investigations were normal (Table. 1), with a normal coagulation profile, normal Liver Function Tests (LFTs), normal Hb electrophoresis (Table. 2), normal platelet counts, and Table. 3, 4 and 5, and Peripheral Blood Smear (PBS). (Table. 3, 4 & 5). The patient had a normal psychological evaluation with no signs and symptoms of mental disorders, mental retardation, anxiety, or other psychological illnesses.
The patient was at first treated with tab propranolol 10 mg once a day, which showed partial improvement, as bleeding episode frequency was decreased from once a day to one episode in 3-5 days. The patient was then given atropine sulfate transdermal patches which further improved the symptoms and the bleeding episodes stopped completely.
Discussion
Hematidrosis is also sometimes referred to as hematohidrosis, hemidrosis, or hematofolliculohidrosis. Since hematidrosis cases are quite rare, the available literature is very scarce. Shafique et al. [3] described a few cases of hematidrosis, in which the majority of cases (83%) were of female gender and of age <18 years. Some patients had shown prodromal symptoms like headache, nausea, and local tingling sensation before the bleeding episode. Case series published by Tshifularo [4] and Manonukul et al. [5] noted that psychological stress was an associated condition and was probably a causative factor.
It is extremely crucial that before making the diagnosis of hematidrosis, we must differentiate it from either self-inflicted/otherwise accidental skin injuries or other pathologies with similar presentation such as vasculitis, chromhidrosis (colored sweat), or other connective tissue disorders where vascular pathology leads to bleeding from the skin.
| PARAMETERS | PATIENT’S VALUES | REFERENCE RANGE |
|---|---|---|
| Hemoglobin (g/dl) | 13.0 | 11.5-15.5 |
| RBC count (× 1012/l) | 4.77 | 4.0-5.2 |
| Hematocrit (%) | 38.0 | 35-45 |
| MCV (fl) | 79.7 | 77-95 |
| MCH (Pg) | 27.3 | 25-33 |
| MCHC (g/dl) | 34.2 | 31-37 |
| RDW (%) | 13.2 | 11.5-14.5 |
| WBC Count (per cmm) | 7,340 | 5,000-13,000 |
| Neutrophils (%) | 39.9 | 30-70 |
| Lymphocytes (%) | 48.2 | 20-50 |
| Monocytes (%) | 6.4 | 0-10 |
| Eosinophils (%) | 5.0 | 0-4 |
| Basophils (%) | 0.5 | 0-1 |
| Immature granulocytes | 0.1 | 0-0.5 |
| Absolute neutrophil count | 2,930 | 1,800-7,000 |
| Absolute lymphocyte count | 3,530 | 1,200-5,000 |
| Absolute monocyte count | 470 | 100-400 |
| Absolute eosinophil count | 370 | 20-300 |
| Absolute basophil count | 40 | 0-100 |
| Neutrophil lymphocyte ratio | 0.83 | 0.1-4.5 |
| Platelet count | 301,000 | 170,000-450,000 |
| MPV | 8.1 | 7.2-11.7 |
| PARAMETERS | PATIENT’S VALUES | REFERENCE RANGE |
|---|---|---|
| HbF level | 0.8% | 0-4 |
| HbA2 | 3.0 | 0-3.5 |
| E- Window | 0.0 | 0-15 |
| D - Window | 0.0 | 0-15 |
| Sickle cell - Window | 0.0 | 0-15 |
| C - Window | 0.0 | 0-15 |
Normal Hb electrophoretic pattern. No evidence of hemoglobinopathies.
| PARAMETERS | PATIENT’S VALUES | REFERENCE RANGE |
|---|---|---|
| Clot retraction test | Good | - |
| C - Window | 0.0 | 0-15 |
| PARAMETERS | PATIENT’S VALUES | REFERENCE RANGE |
|---|---|---|
| Activated partial thromboplastin time | 34.8 seconds | 25.4-38.4 seconds |
| Mean - APTT (Seconds) | 31.3 | 25.4-38.4 |
| Prothrombin time (Seconds) | 11.7 | 10.5-13.5 |
| MNPT (Seconds) | 11.6 | 10.5-13.5 |
| INR | 1.01 | |
| Ristocetin-induced platelet aggregation | Normal platelet aggregation | Normal platelet aggregation |
Normal coagulation profile.
| PARAMETERS | PATIENT’S VALUES |
|---|---|
| CD41 Antibody | 100% Bright intensity |
| CD61 Antibody | 100% Bright intensity |
| CD42b Antibody | 100% Bright intensity |
No phenotypic evidence of deficiency of platelet membrane Gp IIb/IIa (Glanzmann’s thrombasthenia) or Gp Ib (Bernard Soulier Syndrome).
The exact etiopathogenesis of hematidrosis is unknown, but few hypotheses have been proposed. One hypothesis presented by Uber et al. [6] says that sympathetic stimulation causes constriction, thus raising vascular pressure and then dilatation of capillaries surrounding the sweat glands, causing blood to enter sweat ducts and then appear along with sweat. The fact that beta-blocker has been a treatment of choice in our and many other hematidrosis cases, makes this hypothesis more plausible. Another hypothesis, presented by Manonukul et al. [5] says that defects in the dermis cause its direct communication with the vascular spaces filled with blood, thus allowing blood to pass into follicular canals and eventually over the skin surface. Zhang et al. [7] suggested that obstructed peripheral capillaries along with vasculitis are the main pathology in hematidrosis. However, many cases reported by other authors show normal skin biopsies and no features of vasculitis, thus vasculitis as a causative factor for hematidrosis appears less convincing.
Management options range from beta-blockers, atropine patches, anxiolytics, Selective Serotonin Reuptake Inhibitors (SSRIs), tricyclic antidepressants, psychotherapy, etc. Some cases have shown remission without any intervention, while some have not responded at all with treatment options.
A thorough evaluation of the etiopathology of this disease is essential in developing a comprehensive management algorithm for the disease.
Conclusion
Hematidrosis can be a frightening and fearful experience for patients and their families, which leads to a repeated unnecessary and futile exercise of excessive hematological evaluation which mostly comes normal, often making patients, and in some cases, clinicians too, more stressed, unable to find the explanation. Although the condition is benign and has no major local or systemic consequences, the sight of blood in sweat usually makes the patient petrified. The diagnosis of hematidrosis should be done as a diagnosis of exclusion, only after ruling out other possible disease pathologies. Reporting such cases of hematidrosis and discussing them will eventually make disease management more uniform and bring more patient awareness to this frightening disease.
What is new?
Hematidrosis is an extremely rare condition in which the patient spontaneously starts sweating blood. Its pathogenesis, diagnosis, and treatment options are not been very well studied and well discussed so far. Therefore, rather than getting the medical and emotional help these cases need, such cases often end up at the doorsteps of shamans, especially in developing countries.