munchausen syndrome and munchausen syndrome by proxy in dermatology

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Page 1: Munchausen syndrome and Munchausen syndrome by proxy in dermatology

REVIEW

Munchausen syndrome and Munchausen syndromeby proxy in dermatology

Alan S. Boyd, MD,a,b Coleman Ritchie, MD,a and Sunaina Likhari, MDa

Nashville, Tennessee

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Patients with Munchausen syndrome purposefully injure themselves, often with the injection of foreignmaterials, to gain hospital admission and the attention associated with having a difficult-to-identifycondition. Munchausen syndrome by proxy occurs when a child’s caregiver, typically the mother, injuresthe child for the same reasons. Cases of Munchausen syndrome and Munchausen syndrome by proxy withprimary cutaneous involvement appear to be rarely described in the literature suggesting either thatdiagnosis is not made readily or that it is, in fact, an uncommon disorder. At the center of both conditions issignificant psychological pathology and treatment is difficult as many patients with Munchausen syndromewhen confronted with these diagnostic possibilities simply leave the hospital. Little is known about thelong-term outcome or prognosis of these patients. ( J Am Acad Dermatol http://dx.doi.org/10.1016/j.jaad.2013.12.028.)

Key words: factitious disorders; Munchausen syndrome; Munchausen syndrome by proxy; panniculitis;psychiatric disease; psychoanalysis.

Munchausen syndrome refers to patientswho repeatedly request medical care forfictitious illnesses or conditions and was

coined by Asher1 in 1951. Munchausen syndrome byproxy (MSBP) has been defined as ‘‘the intentionalproduction or feigning of physical or psychologicalsigns or symptoms in another person who is underthe individual’s care for the purpose of indirectlyassuming the sick role.’’2 This syndrome is named forthe 18th century European aristocrat HieronymusKarl Friedrich, Baron von Munchausen (1720-1797),who held entertained audiences rapt with stories ofimpossible and fantastic feats in which he was thecentral character. Table I lists additional terms for thiscondition. Given the proximity of the skin, a searchof the literature revealed surprisingly few reportedcases of primary cutaneous Munchausen syndromeand MSBP leading the authors to suspect this condi-tion might be either rare or uncommonly diagnosed.

Patients with Munchausen syndrome typicallypresent to hospitals with acute, often spectacularillnesses and a history of multiple hospitalizations.3

This presentation tends to be later at night and onweekends, presumably when less experienced staff

the Departments of Medicine (Dermatology)a and

thology,b Vanderbilt University.

ing sources: None.

licts of interest: None declared.

pted for publication December 12, 2013.

ints not available from the authors.

will be working.4 They are willing if not eager tosubmit themselves to extensive and invasive thera-peutic and diagnostic procedures and appear to havea considerable pain threshold. Their initial history isdramatic and plausible but often not completelyconvincing. Specific symptoms and diagnoses areoften claimed.5 These patients tend to bedemanding, insistent on constant attention from themedical staff, and request medications, consulta-tions, laboratory tests, and diagnostic procedures.5

Patients with Munchausen syndrome often refuse toallow procurement of medical records from otherinstitutions. Lyell6 believedmost of these patients aremale, however, virtually every other treatise notedthe majority of these patients are women, typicallyyoung to middle-aged and often with medicaltraining. Patients with Munchausen syndrome arefrequently intelligent, quite interested in their con-dition, and well versed in medical terminology, oftenfrom their repeated admissions and discussions withtheir physicians and other patients.4,7,8 Their meanage is 35 years with a ‘‘career’’ span of about 9 years.4

The number of previous hospitalizations can beastoundinge1 patient having been admitted more

Correspondence to: Alan S. Boyd, MD, Vanderbilt University, 719

Thompson Ln, Suite 26300, Nashville, TN 37204. E-mail: alan.

[email protected].

Published online March 05, 2014.

0190-9622/$36.00

� 2014 by the American Academy of Dermatology, Inc.

http://dx.doi.org/10.1016/j.jaad.2013.12.028

1

Page 2: Munchausen syndrome and Munchausen syndrome by proxy in dermatology

J AM ACAD DERMATOL2 Boyd, Ritchie, and Likhari

than 850 times in 650 hospitals and undergoing 42laparotomies.9 Psychologically they exhibit socio-pathic and psychopathic tendencies includingcriminality, pathologic lying, vagrancy, and impos-tership.3 They may also harbor an element ofmasochism.10

MSBP is almost exclusively seen in children with

CAPSULE SUMMARY

d Dermatology patients with Munchausensyndrome and Munchausen syndromeby proxy present with unusual clinicalfindings and multiple hospitalizations.

d Compiled here are reported cases, theirproximate causes, and therapeuticinterventions.

d Clinicians should consider these entitiesin patients with unusual cutaneouslesions that defy diagnosis and exhibitnormal findings with customaryinvestigations.

the parent or caregiverresponsible for their injuriesand may be a cause of thebattered child syndrome.2

When occurring in adults thepatients are usually elderly ormentally unstable.11 Long-term sequela occur in 8% ofcases including mental retar-dation, joint damage, and sur-gical complications and themortality is estimated at 9% to12%.12-14 The most commonpresentation involves hemor-rhage or bleeding and 9% ofcases exhibit some form ofskin manifestation.2,14 Long-term skin and soft-tissue in-

Table I. Synonymous terms for Munchausensyndrome and patients with Munchausensyndrome12

Hospital toxicomania syndrome

Peregrinating problem patientsHospital hoboesFrater HospitalisMythomaniaHospital black-book patientsHospital addition syndromeThick-chart syndromeHospital-hopper syndromeAhasuerus syndromeVan Goh syndrome

fections are uncommon in MSBP.15 Three subtypeshave been describedecaregivers actually causing theinjury to the patient, caregivers inventing the patient’ssymptoms, and caregivers inventing symptoms withmanipulation of blood and urine samples to supportthe credibility of the patient’s condition.15 Infants andpreschool-age children aremost often affected and in40% of cases a sibling is involved.15

Mothers are overrepresented in MSBP as thechildren’s father is usually absent, disinterested, ordistant and it has been suggested that the physicianbecomes the surrogate husband/father providing theattention and support for which the mother yearns.16

There is often a background of abuse in theperpetrator with an element of repressed anger.14

The mother is usually an excellent historian, familiarwith medical terminology, and often has a back-ground in the medical field. Intense and sometimesbizarre attentiveness to the child occurs, the motherrarely leaving the patient’s room for longer than afew minutes. The child may be dressed inappropri-ately with lavish clothing or have excessive numbersof toys/stuffed animals in the hospital room. Whenconfronted by physicians and nurses about a sus-pected diagnosis of MSBP, typically the parents reactangrily and leave the hospital.11

It is important to distinguish Munchausen syn-drome and MSBP from other entities. Munchausensyndrome is incompatible with suicidal ideation asthe patient has no desire to die from their condition.

Although Munchausen syndrome is by definitiona factitial disorder (International StatisticalClassification of Diseases, 10th Revision, F68.10), incases of somatoform disorder the patient is unawareof the psychological motivations for their behavior orthat their illness is functional.14 Hysteria or ‘‘conver-sion’’ symptomsmay be reported but disappear upon

confrontation.4 Malingeringpatients are seeking someform of secondary gain,often administration of nar-cotics and only wish to behospitalized in furtherance ofthat pursuit. In Munchausensyndrome external incentivesfor disease, such asmonetary gain, are absent.2

Apotemnophilia is a condi-tion in which patients pursueamputations of digits or limbsseeking the satisfaction ofoverachieving despite theirhandicap.17 These patientswill often use ligatures to pro-duce bizarre cellulitis-like

presentations or infect their skin hoping for anamputation of the affected digit or limb but are notnecessarily interested in hospitalization.

Cutaneous Munchausen syndrome should beconsidered when skin findings are spectacular,difficult to diagnose, and demonstrate normal ornegative features using routine investigative tech-niques. Table II lists cases reported in the English-language literature.3,4,7,8,18-27 Most patients injectforeign material(s) in the skin and soft tissue (Fig 1).Patients on presentation may be quite ill with, forinstance, fever, hypotension, and sepsis, dependingon what they have injected.25,27 The cutaneousfindings are typical for the intracutaneous presence

Page 3: Munchausen syndrome and Munchausen syndrome by proxy in dermatology

Table II. Patients with Munchausen syndrome and cutaneous disease

Age, y Sex Site of involvement Culprit Results of cutaneous cultures Outcome

3518 F Buttocks, LEx Feces Streptococcus faecalis,Bacillus proteus,Escherichia coli,Citrobacter freundii, others

Resolved with psychiatriccounseling

2119 F* Bilateral hips Unknown NA Left AMA1520 F Thigh, buttocks Turpentine Enterococcus, Staphylococcus

aureus, Escherichia coli,Klebsiella species

NA

2121 F UEx, LEx Milk Cultures negative NA277 M Left thigh Saliva a-Hemolytic streptococci,

diphtheroids, bacteroides,Neisseria, micrococci

Agreed to psychiatrictreatment; ultimatelyLTF

3622 F* Hips, thighs Pentazocine NA NA5822 F* Left LEx Talc NA NA1823 F Face, upper aspect of

chestAir NA NA

373 M Abdomen ‘‘Blue, homogenousmaterial’’

NA NA

244 M Gasoline NA NA368 F Right abdominal wall Unknown NA Left AMA4524 F UEx Blood and bacteria NA NA1525 F UEx, LEx Milk Escherichia coli Psychiatric counseling6026 F Face Wood splinters NA LTF3327 F* Left arm, right buttock Fermented beans

and fecesNAy NA

AMA, Against medical advice; F, female; LEx, lower extremities; LTF, lost to follow-up; M, male; NA, not available or not done; UEx, upper

extremities.

*Patient with medical or paramedical background.yPatient’s blood cultures grew Pseudomonas aeruginosa, Enterococcus faecalis, and Bacillus.

J AM ACAD DERMATOL Boyd, Ritchie, and Likhari 3

of foreign material, namely erythema, swelling, ne-crosis, and tissue breakdown. Depending on thesubstance in question the degree of tissue loss can beextensive. One patient was found to be applying bluetextile dye to her fingers to simulate Raynaud syn-drome,10 another decorated her skin with pigmentmimicking purpura,28 and a third used a mixture ofpetroleum jelly, hair sheen, and cookie crumbs toincite a recalcitrant vegetative cheilitis.23 Two young

Fig 1. Munchausen syndrome. After 3 admissions forthese suppurative cutaneous nodules, the patient ulti-mately admitted to injecting paint thinner into his skin.

women were reported with recurrent angioedemathought to be from hymenoptera envenomation butwere ingesting aspirin and nuts, respectively, towhich both knew they were highly allergic.29 Aspecific subset of Munchausen syndrome is that ofself-induced traumatic panniculitis (Secretan syn-drome or Charcot oedeme bleau) in which thehand is repeatedly traumatized with tourniquetapplication or blows designed to induceinflammation.4

There have been fewer reported cases of cuta-neous MSBP2,11,13,15,30,31 (Table III) and only 1involving the injection of exogenous material.15

Interestingly, in this case, the mother, after beingconfronted about her actions, committed suicide.Four cases involved contact with chemical or ther-mal sources,2,11,31 1 involved traumatic nail avul-sion,2 1 involved purpura and coagulopathysecondary to brodifacoum administration,13 and 1involved a child with disseminated granuloma an-nulare in which the mother insisted the lesions wereexquisitely painful and demanded their excision.30

Tamay et al31 reported the only fatality, that of a2-year-old who succumbed to an oral hemorrhage

Page 4: Munchausen syndrome and Munchausen syndrome by proxy in dermatology

Table III. Patients with Munchausen syndrome by proxy and cutaneous disease

Age Sex Site of involvement Culprit Outcome

24 mo13 M Bruises on legs, trunk, face Brodifacoum Mother admitted for psychiatricevaluation; child recovereduneventfully

10 y30 M ‘‘Painful’’ GA on LEx Multiple surgeries and skingrafts

Mother referred for psychiatrictherapy; child recovereduneventfully

20 mo15 F Abscess left deltoid withEnterobacter cloacaecultured; asystole secondaryto injected benzodiazepines

Presumed fecal injection of animmunization site

After confrontation, mothercommitted suicide; childrequired multiple deep-tissue debridements and skingrafting

24 mo31 F* Oral and conjunctival erosionsand scarring; respiratorydistress while hospitalized

NaOH-containing householdcleaner

Child exsanguinated from anoral ulceration

6 y31 M* Swelling of the lips and oralerosions; esophagealerosions

NaOH-containing householdcleaner

Mother’s disposition not stated;child removed from hismother and recovereduneventfully

3 y2 M Nail shedding ‘‘during sleep’’with pain and bleeding; 16fractures at multiple sitesover 11 mo

Presumed forceful nail avulsion Parents counseled; childremoved from their care andrecovered uneventfully

15 mo2 F Widespread painful erythemaprogressing to bullae withnecrosis

Presumed contact with heatedobjects (curling iron?)

Parents evaluatedpsychiatrically, found to be‘‘normal’’; child experiencedno further episodes

23 y11 F Painful perineal and medialthigh eruption withwell-demarcated bordersand focal ulcerations

Presumed contact withmultiple agents incitingirritant/allergic contactdermatitis

Parents confronted andbecame ‘‘distraught’’; patienttreated with limited contactwith mother and recovereduneventfully

F, Female; GA, granuloma annulare; LEx, lower extremities; M, male; NaOH, sodium hydroxide.

*Brother and sister.

Fig 2. Munchausen syndrome. Histologic features of thecutaneous wounds from the patient depicted in Fig 2. Notethe diffuse necrosis of the subcutaneous fat with numerousneutrophils and lipophages.

J AM ACAD DERMATOL4 Boyd, Ritchie, and Likhari

after household cleaner application containingsodium hydroxide.

The histologic features of the cutaneous lesions inMunchausen syndrome have been reported, how-ever, none of the cases of MSBP reported biopsieshaving been performed. These pathologic changesare nonspecific, most having been described inpatients injecting material into their skin, fascia,and muscles. The inflammatory infiltrate is poly-morphouswith numerous neutrophils, lymphocytes,histiocytes, foreign body giant cells, and lipophageswithout primary vasculitic changes (Fig 2).3 Thesubcutaneous fat is almost always necrotic with fewor no changes noted in the dermis and epidermis.Hemorrhage in the fat may be seen.8 Organicsolvents including mineral spirits, soya alkyds, andother hydrocarbons evoke the most inflammatoryreactions, which would be expected given that theyessentially ‘‘dissolve’’ the subcutaneous fat.32,33

Vasoconstriction with tissue necrosis may also play

a role.4 Foreign material such as paint, wood, andother vegetable matter may be appreciated, particu-larly when viewed under polarized light.4,21 Gramstains may be positive if infection is taking place.

Page 5: Munchausen syndrome and Munchausen syndrome by proxy in dermatology

J AM ACAD DERMATOL Boyd, Ritchie, and Likhari 5

In patients suspected of exhibiting Munchausensyndrome or MSBP, some helpful diagnostic tipsexist. Contact with previous treating clinicians/institutions, family members, and friends may yieldvaluable information.5 Cross-checking the patient’sSocial Security number and medical record numberswith other hospitals and clinics may reveal thepatient to have sought medical care under differentaliases. Hospital room and personal belongingsearches can yield evidence of methods for symptomcreation and video monitoring or a bedside sitter canbe beneficial, often noting a reduction or absence ofmorbidities during observation. Searches and moni-toring, however should be undertaken after theinstitution’s legal counsel has approved. Clues todiagnosis include cultured organisms from cuta-neous wounds that are uncommonly found in theskin (mouth and gastrointestinal tract),7 sparing ofthe skin decorated with tattoos,8 involvement of thenondominant side of the body, and the patient beingable to predict where the next lesion will arise.18

Treatment of Munchausen syndrome isdifficult, however, medications can be useful inpatients experiencing a major depressive episode.34

Confronting the patient may be effective when donein a nonaggressive and accepting manner althoughapproximately 5% of patients will remain resistant toany form of confrontation.35 Hospitalization in apsychiatric treatment center is the best means forinstituting therapy. Supportive psychotherapy,analytical psychotherapy, and if warranted, psycho-analysis appear to provide the best chance for recov-ery.35 Outpatient or ‘‘interval’’ therapy can also besuccessful but risks thepatient being lost to follow-up.Whatever course is undertaken, the danger of thepatient breaking off therapy is greatest in the earlystages.36 Unfortunately, there is almost no informa-tion on the long-term prognosis of patients withMunchausen syndrome as the majority will leave thehospital only to move on to another one and repeatthe process. Of the 16 reported cases with primarycutaneous findings only 7 noted any outcome ofthe hospitalization. Four undertook psychiatric ther-apy,7,18,25 1 noting improvement in the condition.18

Two patients left against medical advice8,19 and 1 waslost to follow-up.26 Feldman34 reported an interestingfollow-up on a patient with Munchausen syndrome.By thiswoman’s estimation shehadbeenhospitalized30 to 40 times andundergonenumerous surgeries anddiagnostic procedures. She entered psychotherapy,was eventually given the diagnosis of an avoidantpersonality disorder, and over time improved. Shealso stated that she had undergone a spiritual reawak-ening and that her faith and the support of her churchcongregation had sustained her recovery.

There are some, albeit it limited, long-term follow-up data on victims of MSBP. McGuire and Feldman37

noted psychological problems including feedingdisorders, behavioral problems, and conversionsymptoms and fabrications in a group of 6 olderchildren. Libow38 evaluated 10 adults who wereself-reported victims of MSBP. Most experiencedsignificant psychological and in some instancesphysical disease throughout much of adult-hood including eating disorders, problems withgrowth, suicidal ideation, anxiety, depression, lowself-esteem, and posttraumatic stress disorder.Interestingly, 7 patients believed they had ‘‘probably’’engaged in Munchausen syndrome at some point intheir lives. None of the parents admitted the abusewhen directly confronted by their adult children.There have been reports of psychiatric interventionin the offending parents resulting in sufficientmental health to allow for a re-establishment ofthe family unit.39,40 A comprehensive treatise onidentification and treatment of MSBP has recentlybeen published.41

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