EKBOM SYNDROME

 EKBOM’S SYNDROME

📌DEFINITION

📍A group of neuropsychiatric disorders characterised by the  recurrent & bizzare delusional belief that one’s body is infested by parasites, insects, worms, small organisms or even inanimate materials.

📍The only symptom of psychosis is delusion

📍It cannot be explained by another condition

📍Lasting 1 month or more(DSM-5)/3 months or more(ICD-10)

📌ORIGIN OF THE NAME… "EKBOM SYNDROME"

📍KARL-AXEL EKBOM(1907-1977)-Swedish Neurologist

The disease is named after him

📍EKBOM SYNDROME =Delusional parasitosis

📌EPIDEMIOLOGY

📍Uncommon(1.9-27.3 cases per 1 lakh)

📍Many underdiagnosed

📍Females-2-3 times more affected

📍Bimodal age of onset(20-30yrs,>50yrs)

📍Average age- 57 years

📍More in Caucasians

📌Why is it important?.....

📍Patient’s feel they are not being taken seriously

📍Self infliction of injuries

📍Stigma to psychotherapy

📍Disappointment & depression

📍Financial loss due to repeated consultations

📍Medicolegal issues

📌HISTORY  OF  EKBOM SYNDROME

📍1st case described by : - Lyell in the book, ”Brief Lives”(1626)

Patient-James Harrington

“He began to imagine that his sweat turned into flies & sometimes to bees & other insects .He would cry, ”Don’t you see that these flies come from me” .It was the strangest madness that ever I found in anyone”- the desciption of Ekbom syndrome in this book

📍1st case report-Sir Robert Willan(1799)

📌SYNONYMS

📍Delusional parasitosis- Wilson & Miller(1946)

📍Delusory parasitosis

📍Monosymptomatic hypochondriacal psychosis

📍Chronic tactile hallucinations- Bers & Conrad(1954)

📍Delusional infestation- Freudenmann & Lepping (2009)

📍Parasitophobia

📍Parasitiphobic neurodermatitis- Leon Perrin(1896)

📍Acarophobia- Georges Thibierge(1894)

📍Dermatophobia

📍Morgellons disease

📌NOSOLOGY

📍As per ICD-10 & DSM-5:-

Ekbom’s syndrome is a delusional disorder

📍ICD-10🡪Persistent delusional disorder

📍DSM-5🡪Somatic type of delusional disorder

📌AETIOLOGY

📍PRIMARY DELUSIONAL PARASITOSIS

*No identifiable underlying cause

📍SECONDARY DELUSIONAL PARASITOSIS

*Has a definite cause

*Secondary delusional parasitosis-Means ,as a result of some primary cause

*Psychiatric disorders:-Bipolar affective disorder (BPAD) ,Hypochondriasis, Body dysmorphic disorder (BDD), depression ,anxiety, Schizophrenia

(31-81% have a comorbid psychiatric condition)

*Neurological disorders:-Cerebro vascular accident (CVA) ,Dementia, Delirium. Multiple sclerosis ,Meningitis ,Encephalitis, syphilis, cerebral trauma &neoplasms

*Substance  intoxication:-Amphetamines, coccaine, Hallucinogens, Cannabinoids, synthetic cathinones

*Substance withdrawal:-Alcohol, Benzodiazepines

*Autoimmune disorders:-SLE

*Endocrinological:-Hypothyroidism, Hyper thyroidism ,Pan hypopituitarism, Diabetes mellites

*Neoplasms-Colon cancer, lung carcinoma ,mediastinum cancers ,breast cancer ,lymphoma

*Drugs:-Antibiotics (Clarithromycin, Ciprofloxacin, Erythromycin),L-DOPA, Ropinirole, Pramipexole, Gabapentin, Topiramate, Corticosteroids, interferon, Phenelzine, methylphenidate, amphetamine diet pills

*Other medical conditions:-Hepatic failure ,renal failure ,Leprosy, Hepatitis ,Tuberculosis, Vitamin deficiencies (B12,folate,thiamine)

📌Pathophysiology of Ekbom syndrome:-

📍Poorly understood

📍Increase of dopamine within striatum of brain due to reduced functioning of dopamine transporters

📌The prototype patient of Ekbom’s syndrome…..

📍A Middle aged woman

📍Claims that she is being infected by some type of parasite, which she occasionally sees & usually feels on/under her skin, head ,eyes etc

📍May describe as a larva OR worm OR flea OR fungus OR simply a small organism

📍The belief qualifies for the definition of delusion

📍Onset-insidious usually ,rarely abrupt

📍Some might have had a real infestation earlier to which they attribute the current symptoms

📍Intense pruritus & dermatitis artefacta

📍Repeated scratching causing excoriations & injuries

📍Overuse of disinfectants /insecticides

📍Excavasations in search of parasites

📍Self mutilation-Self inflicted burn marks and scabs

📍Pits , ulcers , bruises or scars may be seen

📍Hair pulling, alopecia, Onychotillomania, Doctor shopping are usually seen in the patients

📍Repeated bathing & washing of clothes

📍Fear of contaminating other households

📍All the symptoms won't fit into one single organic disease

📍Matchbox sign/Specimen sign(bring specimens/skin particles/hair samples in small boxes)/wrapped in a paper/in plastic bags-for  evidence

📍Photos/self recorded videos will be shown by patient as evidences

📍May have hallucinations coexistent(formications-substance abuse, Cocaine bugs)

📍Other than the delusion of parasites, she reasons normally& appear normal

📌VARIANTS OF EKBOM SYNDROME

1)Shared delusions(Lasegue Falret syndrome):-

*Folie a deux-Most common

*Folie a trios

*Folie a quatre

*Folie a cinq

*Folie a famille

*Folie a plusieurs

*Folie a deux

*Folie communique

📍Folie a deux is seen in 15-25% cases- it is between 2 close people ; Mostly husband & wife, sharing the same delusion

📍Dew hurst&Todd criteria of folie a deux:-

A)Definite evidence that the partners were in intimate association

B)High degree of commonality in the content of delusion, although the formal psychosis may differ

C)Unequivocal support that the partners share support & accept each other’s delusions

📍Subtypes of folie à deux

a)Folie imposée

A dominant person (known as the 'primary', 'inducer' or 'principal') initially forms a delusional belief during a psychotic episode . Then, he will imposes it on another person (the 'secondary', 'acceptor', or 'associate') with the assumption that these secondary person might not have become deluded if left alone.

If the parties are admitted to hospital separately, then the delusions in the person with the induced beliefs usually resolve without the need of medication.

b)Folie simultanée

Both suffers independently from psychosis. They influence the content of each other's delusions so they

become identical or strikingly similar

2)Ekbom syndrome By proxy:-

📍Patient projects the delusion onto a 3rd party who usually cannot share the delusion(eg: pet/young child/Mentally retarded person)

3)Morgellons disease:-

📍Described by Thomas Browne(1674)

📍A bizzare belief that strange materials like fibres are emerging from the skin,accompanied by perceived/real skin lesions

📍1st case-child experienced “harsh hairs on the back”

4)Delusory cleptoparasitosis:-

📍She believes the infestation is in her dwelling, rather than on/in her body

📍History of frequent home change and furniture change will be there

📌Differential diagnoses of Ekbom syndrome:-

📍A genuine infestation.

📍Causes of generalized pruritus -Scabies, Chronic liver disease etc

📍Immunobullous disease

📌COMPLICATIONS AND CO‐MORBIDITIES:-

📍Coexistent affective disease (anxiety, depression or both)

📍Considerable risk of suicide.

📍Some - severe ulcers and extensive erosions

📍Bacterial superinfection

📌Approach to such patients…..

📍It is extremely challenging to diagnose & manage Ekbom syndrome

📍Exclude real infestation(Travel history, contact with infested patients, examination)

📍Find if its primary or secondary delusional parasitosis

(CBC,AEC,TFT,VDRL,LFT,RFT,HIV,VM, Vitamin levels, tox screen, neuroimaging)

📍If secondary,address the underlying cause

📍If primary, treat with empathy by associating with psychiatrist

📍Trying to covince them that they are wrong, will not work

📍Antihistamines ,topical medications for the injuries

📍Specific pharmacotherapy:-Antipsychotics(60-100% remission)

*1st line :- 

-Earlier drug of choice🡪Pimozide 1 mg to 10mg(Side effects-Extra pyramidal side effects,Cardiotoxic)

-Now a days, second generation anti psychotics are the first line of management

Risperidone(0.5-6mg)

Quetiapine(25-600mg)

Aripiprazole(2-30mg)

Olanzapine 5mg

*Long acting injectable anti psychotics if poor compliance

📌Conclusion

Ekbom syndrome or Delusional parasitosis is a psychocutaneous disorder which is difficult to manage. Need a holistic approach by a team of dermatologist and Psychiatrist