Child who presented with hematohidrosis (sweating blood) with oppositional defiant disorder.

Hematohidrosis is a very rare condition of sweating blood. A child's case who presented to us with hematohidrosis is reported. There are only few reports in the literature. A 10-year-old boy presented to our hospital with a history of repeated episodes of oozing of blood from navel, eyes, ear lobules, and nose. During the examination, it disappeared as soon as it was mopped leaving behind no sign of trauma only to reappear within a few seconds. Bleeding time, clotting time, and prothrombin time were normal. Patient was diagnosed with hematohidrosis and oppositional defiant disorder clinically. Management of this condition at our center is discussed below.

INTRODUCTION

Hematohidrosis is a rare condition in which a human being sweats blood.[1] The term “hematofolliculohidrosis” was proposed by Manonukul et al. because it appeared along with sweat-like fluid and the blood exuded via the follicular canals.[2] Various causative factors have been suggested by Holoubek and Holoubek, like component of systemic disease, vicarious menstruation, excessive exertion, psychogenic, psychogenic purpura, and unknown cause. Acute fear and intense mental contemplation are the most frequent causes.[3]

CASE REPORT

A 10-year-old boy studying in 4th grade, belonging to middle socioeconomic strata was referred to Vijayawada Institute of Mental Health and Neuro Sciences (VIMHANS) Hospital, Vijayawada by a dermatologist in December 2013. Detailed history was collected. The history dated back to February 2013, when he first suffered from a bout of hemoptysis while in school, following which his parents were called to school and he was taken to a local physician who then referred him to a multispecialty hospital, where he was thoroughly investigated.

Following this episode over the next few months, the patient had repeated bouts of hemoptysis, hematemesis, epistaxis, and hematochezia along with hematuria. From February 2013 to May 2013, he was thoroughly investigated for all types of blood dyscrasias and all investigations were found to be normal. From May 2013, he started having repeated episodes of oozing of blood from navel, eyes, ear lobules, and nose. These episodes occurred while he was at home as well as in school. Due to this, parents stopped sending him to school and he was being taken to various doctors.

When he was brought to VIMHANS Hospital, a detailed assessment was done, and it was found that the child was temperamentally adamant, stubborn and defiant by nature. The child was living in a joint family with his grandparents, paternal uncles, and his cousins. As the patient was the only male child in the entire family, he was pampered by all the other members of the family. The child was average in studies, and there were episodes of child not going to school previously too especially before exams, but the child was persuaded to go to school. When the child presented to us in December 2013, he was not going to school for the last 10 months due to the above symptoms. The episodes of bleeding from various parts of the body, especially the eyes were preceded by issues such as upcoming exams, fight with parents, and parents not satisfying his demands. Before coming to our hospital, complete blood count, liver function test, kidney function test, random blood sugar, BT/CT, chest X-ray, endoscopy/colonoscopy, urine examination, prothrombin time, activated partial thromboplastin time, international normalized ratio, fibrogen, and platelet function analysis were done and found to be normal. At our hospital, we did peripheral smear of the blood sample collected from the eye during an episode. The result showed red blood cell and no abnormal cells.

Diagnosis - Oppositional defiant disorder with hematohidrosis

Patient had already been treated with the following in the past:

Antibiotics

Saline nasal drops

Proton pump inhibitors

Mouth gargles.

Management plan at our hospital

The plan we adopted for management of this case combined pharmacotherapy and nonpharmacological methods of treatment. After doing a literature search, we started treatment with tablet lorazepam 1 mg at bedtime and tablet propranolol 10 mg twice a day.

The main focus of our treatment was nonpharmacological management that consisted of behavioral interventions for the child and counseling and psychoeducation to the parents, as it was clear that the stress precipitated episodes of bleeding from orifices. Relaxation exercises were taught to the boy to reduce anxiety. Parents were provided information about the illness; their undue concerns about the seriousness of the condition were addressed. They were explained about the antecedents of the bleeding episodes and how they should respond to such episodes.

Symptoms such as adamant and stubborn behavior, defiant behavior were targeted using graded behavioral interventions like normalization, decreasing contact between parents and child, cutting down secondary gain and empowering the parents to deal with such situations. Parents were taught the use of positive reinforcement, use of time out and token economy through which desired behavior would be encouraged and undesired behavior could be ignored.

Enhancement of child's self-confidence through simulated exposure to the school environment and practice tests were conducted. At discharge after 20 days, child's condition had significantly improved. Bleeding episodes had stopped, child's behavior also showed positive change. Patient was discharged on the tablet propranolol 10 mg twice a day. Lorazepam was tapered and stopped. At the first follow-up after 1 month of discharge, child was going to school regularly, and behavioral symptoms have reduced. At the time of writing this report, the child has been attending school regularly and also appeared for his final exams.

DISCUSSION

Hematohidrosis is a very rare condition in which a human sweats blood. Blood usually oozes from the forehead, nails, umbilicus, and other skin surfaces. In addition, oozing from mucocutaneous surfaces causing nose bleeds, blood stained tears, and vicarious menstruation are common.[1] The episodes are usually self-limiting. In some condition, the secreted fluid is more dilute and appears to be blood tinged, while others may have darker bright red secretions resembling frank blood.[3] This disease has been described in various terms and has been often tied to religious belief as stigmatization. It may occur when a person is suffering extreme levels of stress. Several historical references have been described; notably by Leonardo da Vinci describing a soldier who sweated blood before battle, and men unexpectedly given a death sentence, as well as descriptions in the Bible, that Jesus experienced hematidrosis when he was praying in the garden of Gethsemane.

Proposed etiopathogenesis is that multiple blood vessels, which are present in a net-like form around the sweat gland constrict under pressure of stress. As the anxiety increases, the blood vessels dilate to the point of rupture. The blood goes into the sweat glands, which push it along with sweat to the surface, presenting as droplets of blood mixed with sweat. The extravasated blood has identical cell components as that of peripheral blood. The severe mental anxiety activates the sympathetic nervous system to invoke the stress-fight or flight reaction to such a degree as to cause hemorrhage of the vessels supplying the sweat glands into the ducts of the sweat glands.[4] Aggravating factors known to-date include emotional stress and excitement. This condition is very rare but reports of successful treatment with beta blockers[24] with a significant reduction in the frequency of spontaneous blood oozing are described in the medical literature. In the above case too, propranolol was found to be effective in controlling the bleeding episodes. The successful use of beta blockers supports the theory that the condition is induced by stress and anxiety yet this etiology is not established yet as the high prevalence of stress and anxiety in the modern era did not change the incidence of this extremely rare disease suggesting that other co-abnormality also play a key role in this disease.[2] Symptoms of oppositional defiance were targeted through nonpharmacological interventions as described above.

The key to successful treatment also includes convincing the parents about the nature of this illness, its aggravating factors and the possible treatment of this condition.