Hematidrosis: A rare entity - case series and review of literature.

INTRODUCTION

Hematidrosis is an exceedingly rare and peculiar clinical disorder characterized by spontaneous exudation of blood-mixed fluid from the intact skin or mucosa.[12] Though fewer than hundred cases have been reported so far,[12345] its mention dates back to the era of Leonardo da Vinci and notably in Bible too.[3] It has been mainly cited in dermatological texts,[123] whereas management involves mostly psychotropics, beta blockers, and psychotherapy. Hematidrosis still remains a diagnosis of exclusion as there is no corroborative test available yet owing to its unknown pathophysiology.[13] It requires ruling out of conditions such as bleeding diathesis, connective tissue disorders, vasculitis, and self-inflicted or secondary wounds. Ailments like chromhidrosis or pseudochromhidrosis need to be precluded by ascertaining presence of erythrocytes in bloody discharge, thus establishing hematidrosis.[3] Herein, we present a case series of three such patients of hematidrosis where stressor-related symptomatology was found in all and who could be successfully treated with psychotropics and beta blockers.

CASE PRESENTATION

Case 1

An otherwise well 18-year-old female presented with spontaneous bleeding from eyes, ear, nose, and mouth for 2 weeks, which happened even during sleep or eating [Figure 1]. She could sense her episodes prior to onset, as she used to get tingling sensation over the areas. She had several episodes of hemoptysis 2 years back, for which she underwent multiple noncontributory investigations and even got admitted several times under a chest physician, but to no favorable response till the present time when she visited psychiatry outpatient department (OPD) of our institute. On detailed evaluation, it was found that she was academically sound and bleeding episodes frequently preceded her examination schedules. Her physical examination was unremarkable. Blood parameters, coagulation profile, autoimmune profile (antinuclear antibody, rheumatoid factor, anti- cyclic citrullinated peptide), IgE, and Aspergillus- specific IgE were all within normal limits. Invasive examinations including nasal endoscopy, laryngoscopy, bronchoalveolar lavage (BAL), upper gastrointestinal (GI) endoscopy, fiberoptic bronchoscopy (FOB), and guided biopsy were done and all came out to be nonconclusive. Computed tomography (CT) scan of paranasal sinus and thorax revealed no abnormality. The smear made from her secretion during the bleeding episode showed numerous red blood cells on microscopy. Multidisciplinary consultations with hematology, dermatology, otorhinolaryngology, ophthalmology, and immunology were done with dismal results. Hence, a diagnosis of hematidrosis was made after proper exclusion of other clinical conditions [Table 1]. She was administered fluoxetine 10 mg once daily (OD) along with clonazepam 0.5 mg in tapering dose over 2 weeks and propranolol 10 mg OD. Subsequently, her frequency of episodes diminished but persisted. Hence, a low-dose antipsychotic, olanzapine 2.5 mg, was added, which could be stopped within 2 months on successful complete cessation of episodes.

Figure 1

Picture taken during a bleeding episode. Arrow shows bleeding from the eye

Table 1

Different tests performed to rule out organic causes of bleeding

Investigations	Reason
Smear of exudate	To ascertain presence of blood corpuscles, thus excluding chromhidrosis or pseudochromhidrosis
History of injury	To exclude self-harm or secondary wound
Coagulation screening	To exclude bleeding diathesis
Autoimmune profile (ANA, RF, anti-CCP)	To rule out connective tissue disorder or vasculitis
Blood parameters (CBC, RFT, LFT)	To ascertain the functioning status of bone marrow, kidney, liver
IgE and Aspergillus-specific IgE	To exclude allergic bronchopulmonary aspergillosis
Nasal endoscopy, laryngoscopy, BAL, upper GI endoscopy, FOB, and guided biopsy	To exclude local pathologies (tumor, infective, inflammatory, traumatic)
CT scan of paranasal sinus and thorax	To exclude local pathologies (tumor, infective, inflammatory, traumatic)

BAL=bronchoalveolar lavage, CBC=complete blood count, CT=computed tomography, FOB=fiberoptic bronchoscopy, GI=gastrointestinal, LFT=liver function test, RFT=renal function test

Case 2

Another 21-year-old female with no significant past or family history presented with painless bleeding from eyes and facial skin for 2 months, which used to stop spontaneously after a few minutes [Figure 2]. The onset of symptoms coincided with untimely demise of her father, following which she started feeling low and often would worry about trivial issues. The episodes used to follow stressors at home. The frequency of bleeding episodes further intensified as she remained preoccupied with thoughts of having serious illness. She was also subjected to series of futile elaborative investigations. Upon diagnosing a case of hematidrosis after careful exclusion, she was started on fluoxetine 20 mg OD along with clonazepam 0.5 mg in tapering dose over 2 weeks and olanzapine 2.5 mg. She attained complete resolution of symptoms within 2 months, following which olanzapine was stopped.

Figure 2

Arrow shows bleeding from the eye and facial skin

Case 3

The friend of above-described lady (Case 2), who was a 20-year-old female, developed similar bleeding instances from ear and facial skin upon knowing symptoms of Case 2 lady [Figure 3]. No physical injury was associated with it. In this case, limited investigations were done to exclude other illnesses. She was put on short course of benzodiazepines, which could alleviate her symptoms effectively within 1 month.

Figure 3

Arrow shows bleeding from the perioral facial skin

DISCUSSION

Hematidrosis, also referred as H(a) ematohidrosis, hematofolliculohidrosis,[6] or simply bloody sweat, is a notoriously scarce and fascinating condition illustrated by excretion of bloody discharge from noninjured skin/mucosa, with appearance of few case reports and fewer case series[7] in existing literature. Due to extreme paucity of cases, detailed prospective study has not been performed yet. Hence, a few systematic reviews of available cases are all that we have got.[1389] The first such comprehensive review was done by Holoubeks[9] back in 1996, which compiled 76 such cases from the existing literature and classified them as per the inciting factors. Commonest causative factors were acute fear and intense mental strain. Legitimacy of the included cases was questioned as majority dates back before the 20th century when detailed laboratory tests were not available to exclude other bleeding disorders. Shafique et al.[3] analyzed 36 cases of hematidrosis, of which 83% were females and majority (83%) belonged to <18 years age group. Similarly, in our case series, all three individuals were females and young. However, the condition has also been observed in extreme of ages, that is, from 2 months[10] to 72 years.[11] Asian dominance, particularly from India and Pakistan, has been noted in the majority of cases.[1] Our three cases further enrich existing literature from India. Prodromal symptoms like headache, vomiting, pain, local tingling, or soreness have been prominently seen in many patients (28%–30% cases in reviews by Kluger et al.[1] and Shafique et al.,[3] respectively). Likewise, one of our index cases could predict her episodes as she used to develop local tingling prior to bleeding episodes. Face is the most affected part in patients (96%), followed by eyes, ears, nose, umbilicus, palms, and soles.[1] Alike distribution has also been noted in our cases; in addition to it, hemoptysis was also seen in one of our presented cases. Bleeding episodes triggered by physical or mental stresses were evident in a majority of cases,[13] as has also been noticed in all our presented cases. Not surprisingly, almost 11 patients were found to have antecedent psychiatric illnesses including anxiety, depression, conversion disorder, and oppositional defiant disorder.[13] Management invariably consisted of medications like beta blockers, anxiolytics, tricyclic antidepressants (TCAs), and selective serotonin reuptake inhibitors (SSRIs) with or without psychotherapy.[134] In a few cases, symptoms resolved on its own without any therapy. In our index cases, besides the above-mentioned medications, low-dose atypical antipsychotic was also administered. Fortunately, everyone responded well to treatment, as has been previously noted in cases described in literature.[134]

CONCLUSION

Hematidrosis appears to be a phenomenon of exudation of bloody fluid from the intact mucosa or skin in those experiencing physical or emotional stresses. It has a benign and transient clinical course. The particular observation of resolution of symptoms with psychotropics and psychotherapy precludes hematidrosis to be an organic disease.

Consent to participate and publication

Consent was taken from the cases to publish the report.

Abbreviations

OPD- outpatient department

FOB- fiberoptic bronchoscopy

BAL- bronchoalveolar lavage

CT scan- computed tomography scan

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.