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__NOTOC__
__NOTOC__
{{SI}}
{{SI}}
{{CMG}}
{{CMG}}; {{AE}} {{Hudakarman}}


{{SK}} Gammel's disease.
{{SK}} [[Gammel's disease]]




==Overview==
==Overview==
      
      
Erythema gyratum repens is a rare highly specific and characteristic [[Paraneoplastic Syndromes|paraneoplastic s]][[syndrome|yndrome]] that usually affect older people. It is characterized by [[wood]]-[[grain]] scaly skin [[eruption]] with intense [[pruritus]]. The cause of erythema gyratum repens is unknown but many theories suggest [[Immunology|immunologic]] etiology or [[Toxicology|toxicologic]] products that are released by the associated [[tumor]]. The first case of erythema gyratum repens was described by a  [[dermatologist]] named Gammel in the year 1952. For many years after erythema gyratum repens original description, there was little progress in defining the pathogenesis of erythema gyratum repens. [[Erythema]] gyratum repens has no specific [[classification]] but we can classify it based on its association with an internal [[malignancy]] into [[Paraneoplastic Syndromes|para-neoplastic]] and [[Para-|non-para-neoplastic]] erythema gyratum repens. The most common [[malignancies]] associated with erythema gyratum repens are [[lung]] or [[Bronchogenic carcinoma|bronchogenic]] [[cancer]], [[esophageal]] [[cancer]], and [[breast cancer]]. Erythema gyratum repens can also be associated with [[Neoplastic|non-neoplastic]] diseases such as [[tuberculosis]], [[autoimmune]] disorders, or [[CREST syndrome]]. Erythema gyratum repens is characterized by its [[Pathognomonic|pathogonomic]] figurate, gyrate, or annular [[erythematous]] skin [[Eruption|eruptions]]. The intense [[pruritus]] can be debilitating and usually urges the patient to go to the [[emergency department]]. The [[microscopic]] [[histopathological]] features of erythema gyratum repens consist of acanthosis, focal parakeratotic, and spongiosis of the [[epidermis]] with perivascular [[mononuclear]], [[lymphocytic]], and [[histiocytic]] infiltrate in the [[superficial]] [[Plexuses|plexus]] of the [[dermis]]. Erythema gyratum repens is very rare and it mainly affects people in their seventieth decade, the male to female ratio is 2:1. Erythema gyratum repens is [[Diagnosis|diagnosed]] clinically by its characteristic skin [[eruption]] and an [[Urgent care|urgent]] thorough [[paraneoplastic]] workup should be initiated to look for internal [[malignancies]]. Patients with erythema gyratum repens presents with intensely [[Pruritic disorders|pruritic]], gradually progressive, skin lesions that crawl rather than migrate from one body region to the other. It can start in the [[upper trunk]] or upper back and extends to involve the [[extremities]] sparing the [[face]]. The mainstay of the treatment of erythema gyratum repens is finding and treating the underlying [[malignancy]]. [[Symptomatic treatment|Symptomatic]] treatment is not very effective in relieving the [[pruritus]] and its associated pain. The management can be [[surgical]] removal of the [[tumor]], [[chemotherapy]], or [[palliative]] conservative management. The skin [[Eruption|eruptions]] can improve completely after the removal of the underlying [[Tumor cell|tumor]], or can recur especially if the [[Tumor cell|tumor]] recurred or [[metastasized]]. Patients can live a few weeks, months or up to five years depending on when and at what stage the [[malignancy]] was detected.


==Historical Perspective==
==Historical Perspective==
* In 1953, the dermatologist, Dr. John A Gammel who was trained to link skin lesions to internal malignancy was the first one who described and labeled Erythema Granulatum Repens in a 55-year-old patient with poorly differentiated breast adenocarcinoma  <ref name="pmid25996397">{{cite journal| author=| title=Reorganized text. | journal=JAMA Otolaryngol Head Neck Surg | year= 2015 | volume= 141 | issue= 5 | pages= 428 | pmid=25996397 | doi=10.1001/jamaoto.2015.0540 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25996397  }} </ref>
* Up to 1992, there were only 49 cases in the literature, 41 of which (84%) were associated with a neoplasm <ref name="pmidhttps://doi.org/https://doi.org/10.1016/0190-9622(">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=https://doi.org/https://doi.org/10.1016/0190-9622( | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10  }} </ref>
* EGR is associated with internal malignancy in 82% of cases. However, between 1990 and 2010, data was collected from the medical records of patients form dermatology department in University of Genoa and from databases as pubmed and medline, the conclusion of this literature review was that EGR is no longer considered as an obligate paraneoplastic syndrome. More than expected cases of EGR were found with no neoplasm association. Non-paraneoplastic EGR could be:  <ref name="pmidhttps://doi.org/10.1111/j.1468-3083.2012.04663.x">{{cite journal| author=Schmoldt A, Benthe HF, Haberland G| title=Digitoxin metabolism by rat liver microsomes. | journal=Biochem Pharmacol | year= 1975 | volume= 24 | issue= 17 | pages= 1639-41 | pmid=https://doi.org/10.1111/j.1468-3083.2012.04663.x | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=10  }} </ref>
** Idiopathic EGR
** EGR-like eruptions (different dermatologic lesions that mimic EGR)
** Drug-induced EGR


*The [[Association (statistics)|association]] between [[cutaneous]] manifestations and [[systemic]] [[malignancies]] was first studied in 1925 by Rothman, the Hungarian investigative [[dermatologist]], who wrote a comprehensive review on this subject and since then, cases were added to proof for the relationship between internal [[neoplasm]] and some [[skin lesions]].<ref name="Rothman1925">{{cite journal|last1=Rothman|first1=Stephan|title=Über Hauterscheinungen bei bösartigen Geschwülsten innerer Organe|journal=Archiv für Dermatologie und Syphilis|volume=149|issue=1|year=1925|pages=99–123|issn=0340-3696|doi=10.1007/BF02297811}}</ref><ref name="pmid25373439">{{cite journal| author=Burgdorf WHC, Bickers DR| title=The scientific legacy of Stephen Rothman. | journal=J Invest Dermatol | year= 2015 | volume= 135 | issue= 4 | pages= 954-959 | pmid=25373439 | doi=10.1038/jid.2014.447 | pmc=4366295 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=25373439  }} </ref>
*Erythema gyratum repens was first described by Dr. John A Gammel, the [[dermatologist]], who was trained to link bizarre or recalcitrant [[dermatoses]] to internal [[malignancy]], In 1952, in a 55-year-old patient who had been complaining of [[Pruritic disorders|pruritic]] scaly skin [[eruption]] and [[Diagnosis|diagnosed]] nine months later with [[Adenocarcinoma|poorly differentiated adenocarcinoma]] of the [[breast]] with [[metastasis]] to [[axillary lymph nodes]].<ref name="Gammel1952">{{cite journal|last1=Gammel|first1=John A.|title=ERYTHEMA GYRATUM REPENS|journal=A.M.A. Archives of Dermatology and Syphilology|volume=66|issue=4|year=1952|pages=494|issn=0096-5979|doi=10.1001/archderm.1952.01530290070010}}</ref><ref name="Purdy1959">{{cite journal|last1=Purdy|first1=M. J.|title=Erythema Gyratum Repens|journal=A.M.A. Archives of Dermatology|volume=80|issue=5|year=1959|pages=590|issn=0096-5359|doi=10.1001/archderm.1959.01560230076020}}</ref>
*In 1950, Dr. Gammel presented his case of Erythema gyratum repens before the [[Cleveland Clinic|Cleveland]] [[Dermatological]] Society as Erythema gyratum migrans then he changed the term to erythema gyratum repens because the eruption does not "migrate" from one place to another but "crawls" constantly in the areas involved, like "ants on an anthill".<ref name="Gammel1952">{{cite journal|last1=Gammel|first1=John A.|title=ERYTHEMA GYRATUM REPENS|journal=A.M.A. Archives of Dermatology and Syphilology|volume=66|issue=4|year=1952|pages=494|issn=0096-5979|doi=10.1001/archderm.1952.01530290070010}}</ref>
*In 1973, 45 year old man was [[Diagnosis|diagnosed]] with erythema gyratum repens associated with [[metastatic]], [[Adenocarcinoma|undifferentiated adenocarcinoma]] which was removed following a right- sided [[craniotomy]]. The patient was misdiagnosed with  [[Erythema|erythema perstans]] and the [[malignancy]] was discovered after 8 months of the skin manifestations.<ref name="Skolnick1975">{{cite journal|last1=Skolnick|first1=Marvin|title=Erythema Gyratum Repens With Metastatic Adenocarcinoma|journal=Archives of Dermatology|volume=111|issue=2|year=1975|pages=227|issn=0003-987X|doi=10.1001/archderm.1975.01630140085011}}</ref>
* Up to 1992, there were only 49 cases in the literature, 41 of which (84%) were associated with a [[neoplasm]] and that is why erythema gyratum repens has been considered as a [[paraneoplastic]] syndrome.<ref name="pmid1583177">{{cite journal| author=Boyd AS, Neldner KH, Menter A| title=Erythema gyratum repens: a paraneoplastic eruption. | journal=J Am Acad Dermatol | year= 1992 | volume= 26 | issue= 5 Pt 1 | pages= 757-62 | pmid=1583177 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=1583177  }}</ref>
* Between 1990 and 2010, a literature review was done by collecting [[data]] from the [[Medical record|medical records]] of patients form [[dermatology]] department in University of Genoa and from [[databases]] as [[Pubmed|pubMed]] and [[medline]], to conclude that erythema gyratum repens is no longer considered as an [[obligate]] [[paraneoplastic]] [[syndrome]]. More than expected cases of EGR were found with no [[neoplasm]] association.<ref name="RongiolettiFausti2014">{{cite journal|last1=Rongioletti|first1=F.|last2=Fausti|first2=V.|last3=Parodi|first3=A.|title=Erythema gyratum repens is not an obligate paraneoplastic disease: a systematic review of the literature and personal experience|journal=Journal of the European Academy of Dermatology and Venereology|volume=28|issue=1|year=2014|pages=112–115|issn=09269959|doi=10.1111/j.1468-3083.2012.04663.x}}</ref>


==Classification==
* Erythema gyratum repens has no established system for the [[classification]]. However, we can classify erythema gyratum repens based on its [[Association (statistics)|association]] with systemic [[malignancy]] into: <ref name="RongiolettiFausti2014" /><ref name="pmid28690517">{{cite journal| author=Fukunaga M, Harada K, Mae K, Wakamatsu K, Kiriyama N, Tsuboi R et al.| title=Erythema Gyratum Repens-Like Purpura in a Patient with Sjögren Syndrome. | journal=Case Rep Dermatol | year= 2017 | volume= 9 | issue= 2 | pages= 40-43 | pmid=28690517 | doi=10.1159/000477375 | pmc=5498950 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=28690517  }}</ref><ref name="pmid12168480">{{cite journal| author=Günther R, Nasser S, Hinrichsen H, Fölsch UR| title=[Erythema gyratum repens: drug reaction following azathioprine administration in a patient with type I [[autoimmune]] [[hepatitis]]. | journal=Med Klin (Munich) | year= 2002 | volume= 97 | issue= 7 | pages= 414-7 | pmid=12168480 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=12168480  }}</ref><ref name="RongiolettiFausti2012">{{cite journal|last1=Rongioletti|first1=Franco|last2=Fausti|first2=Valentina|last3=Parodi|first3=Aurora|title=Erythema Gyratum Repens Induced by Pegylated Interferon Alfa for Chronic Hepatitis C|journal=Archives of Dermatology|volume=148|issue=10|year=2012|pages=1213|issn=0003-987X|doi=10.1001/archdermatol.2012.1968}}</ref>


==Classification==
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
* There is no established system for the classification of EGR.  
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Types of Erythema gyratum repens}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Characterestics}}
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Paraneoplastic]] EGR'''
| style="padding: 5px 5px; background: #F5F5F5;" |
* Erythema gyratum repens is associated with internal [[malignancy]] in 82% of cases<ref name="RongiolettiFausti2014" />
*The most common [[neoplasms]] are [[Lung cancer|Lung]]/[[Bronchogenic carcinoma|bronchogenic]], [[breast cancer]], and GI tract ([[Stomach Cancer|stomach,]] [[Esophageal Cancer|esophageal]]) cancer.
*The other associated neoplasms are: [[Urinary bladder cancer|Urinary bladder,]] [[Prostate Cancer|prostate]], [[Uterine cancer|uterine]] and/or [[cervix]], and [[anal cancer]]
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="8;" | Non-[[paraneoplastic]] EGR
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;"|[[Idiopathic]] EGR
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*Erythema gyratum repens with no underlying [[malignancy]], associated conditions, or precipitating cause
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;"| <nowiki>|</nowiki>EGR-like [[Eruption|eruptions]] <ref name="pmid28690517" />
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
* Can appear in various [[autoimmune]] conditions
 
* Characterized by [[Lesions|annular lesions]] with expanding concentric pattern and coalescing to form a zebra-like pattern or [[grain]] [[of wood pattern]]
*EGR-like eruption in [[Sjögren syndrome]] (SS) is extremely rare
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;"| EGR with concomitant [[skin disease]]
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
**[[Pityriasis rubra pilaris]], [[psoriasis]], [[ichthyosis]], [[CREST|CREST (calcinosis, Raynaud phenomenon, esophageal motility disorder, sclerodactyly, and telangiectasia) syndrome]], virginal breast hypertrophy, [[rheumatoid arthritis]], [[tuberculosis]], [[bullous pemphigoid]], [[linear]] [[IgA]] [[disease]], and [[hypereosinophilic syndrome]], [[cryptogenic organizing pneumonia]]<ref name="pmid26765132">{{cite journal| author=Samotij D, Szczech J, Bencal-Kusinska M, Reich A| title=Erythema gyratum repens associated with cryptogenic organizing pneumonia. | journal=Indian J Dermatol Venereol Leprol | year= 2016 | volume= 82 | issue= 2 | pages= 212-3 | pmid=26765132 | doi=10.4103/0378-6323.173594 | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=26765132  }}</ref>
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="1;"|[[Drug-induced]] EGR
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*''[[Azathioprine]]'' with [[type I]] [[autoimmune]] [[hepatitis]]<ref name="pmid12168480" />
 
*''[[Interferon]]'' given for [[hepatitis C]] virus–related [[chronic hepatitis]]<ref name="RongiolettiFausti2012" />
|-
|}


==Pathophysiology==
==Pathophysiology==
* The cause of EGR has not been identified.
* The [[pathogenesis]] of erythema gyratum repens is unclear<ref name="pmid3390794">{{cite journal| author=Appell ML, Ward WQ, Tyring SK| title=Erythema gyratum repens. A cutaneous marker of malignancy. | journal=Cancer | year= 1988 | volume= 62 | issue= 3 | pages= 548-50 | pmid=3390794 | doi=10.1002/1097-0142(19880801)62:3<548::aid-cncr2820620318>3.0.co;2-h | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=3390794  }}</ref><ref name="pmid22224159" />
* Many theories suggest that EGR is due to immunologic mechanisms
* Many [[Immunology|immunologic]] theories have been implicated in its [[pathogenesis]].
* The immunologic mechanism theory is evidenced by the observed immunofluorescence patterns of IgG, C3, and C4 at the basement membrane  
*The [[Immunology|immunologic]] mechanism theory is evidenced by the observed [[immunofluorescence]] patterns of [[IgG]], C3, and C4 at the [[basement membrane]]: <ref name="pmid22224159">{{cite journal| author=Gore M, Winters ME| title=Erythema gyratum repens: a rare paraneoplastic rash. | journal=West J Emerg Med | year= 2011 | volume= 12 | issue= 4 | pages= 556-8 | pmid=22224159 | doi=10.5811/westjem.2010.11.2090 | pmc=3236141 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=22224159  }}</ref>
** Theory 1 the tumor induces antibodies that cross-react with the basement membrane of skin
** Theory 1: the [[tumor]] induces [[antibodies]] that cross-react with the [[basement membrane]] of skin.
** Theory 2 the tumor produces polypeptides that bind skin antigens and render them immunogenic 
** Theory 2: the [[tumor]] produces [[polypeptides]] that bind skin [[antigens]] and render them [[Immunogenicity|immunogenic]]. 
** Theory 3 deposition of tumor antigen-antibody complexes onto the basement membrane causes the reactive dermatitis seen in EGR
** Theory 3: deposition of tumor antigen-antibody complexes onto the [[basement membrane]] causes [[Dermatitis|reactive dermatitis]] seen in erythema gyratum repens.
*The [[gross]] appearance of the unique [[Eruption|eruptions]] are:
** Wavy [[erythematous]] [[concentric]] [[bands]] that can be figurate, gyrate, or annular
** The [[bands]] are arranged in parallel rings and lined by a fine trailing edge of scales, a pattern often described as “wood grained”.
** The distinctive [[wood]][[grain|-grain]] appearance of the eruption is [[pathognomonic]].
** The [[rash]] typically involves large areas of the [[body]] but tends to spare the [[face]], [[hands]], and [[feet]] and it can expand as fast as 1 cm a day.
** Bullae can also form from within the areas of [[erythema]].
* The [[microscopic]] histologic features of erythema gyratum repens are not characteristics but the following are the [[biopsy]] specimen findings that are compatible with the [[diagnosis]]:<ref name="Gammel1952" /><ref name="Skolnick1975" /><ref name="pmid8339188">{{cite journal| author=Tyring SK| title=Reactive erythemas: erythema annulare centrifugum and erythema gyratum repens. | journal=Clin Dermatol | year= 1993 | volume= 11 | issue= 1 | pages= 135-9 | pmid=8339188 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=8339188  }}</ref>
** The [[epidermis]] has thin [[atrophic]] patches with areas of acanthosis, focal parakeratotic horny layers, and spongiosis.
**The [[dermis]] contains a moderate perivascular [[mononuclear]], [[lymphocytic]], and [[histiocytic]] infiltrate in the [[superficial]] [[plexus]] as well as mild focal spongiosis and parakeratosis.
**[[Eosinophils]] and melanophages have also been reported in the [[dermal]] infiltrate.
**Diffuse to moderate [[edema]] of the [[Connective tissues|connective tissue]] can be seen.
 




==Causes==
==Causes==
The cause of erythema gyratum repens has not been identified.  
* The exact cause of erythema gyratum repens is unknown.
* Various [[Immunology|immunologic]] mechanisms suggest that erythema gyratum repens [[etiology]] is stemmed from an [[Immunological|immunologic]] reaction.
*The [[Association (statistics)|association]] between erythema gyratum repens and systemic [[malignancy]] is evidenced by the disappearance of the [[Pruritic disorders|pruritic]] [[Eruption|eruptions]] after the treatment of the underlying [[neoplasm]].
*The [[Association (statistics)|association]] doesn't necessarily mean causation.
 
==Differentiating Erythema Gyratum Repens from Other Diseases==
*EGR has a narrow [[differential diagnosis]] and it has to be differentiated from reactive (figurate or gyrate) erythematous skin eruptions.
*[[Differential diagnosis]] of reactive (figurate or gyrate) erythematous skin [[Eruption|eruptions]] based on their association with underlying systemic [[malignancy]]:
 
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Types}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|examples}}
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="3;"|With underlying [[malignancy]]''' '''<ref name="pmid8339188" /><ref name="pmid861171">{{cite journal| author=Holt PJ, Davies MG| title=Erythema gyratum repens--an immunologically mediated dermatosis? | journal=Br J Dermatol | year= 1977 | volume= 96 | issue= 4 | pages= 343-7 | pmid=861171 | doi= | pmc= | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=861171  }}</ref>'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*Erythema gyratum repens (EGR)
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Erythema annulare centrifugum]] (EAC)
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Necrolytic migratory erythema]] (NME)
|-
| style="padding: 5px 5px; background: #DCDCDC; font-weight: bold;" rowspan="6;" | Without underlying [[malignancy]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Erythema marginatum rheumaticum
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Erythema chronicum migrans]] 
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Familial|Familial annular erythema]]
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
* The [[carrier]] state of [[chronic granulomatous disease]]
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Subacute]] [[cutaneous]] [[lupus erythematosus]]
|-
| style="padding: 5px 5px; background: #F5F5F5;" |
* [[Neonatal lupus erythematosus]]
|-
|}
 
 
*[[Differential diagnosis]] of reactive (figurate or gyrate) erythematous skin [[Eruption|eruptions]] associated with underlying [[malignancy]]:'''<ref name="pmid8339188" /><ref name="pmid861171" />''' <br />
 
{| style="border: 0px; font-size: 90%; margin: 3px;" align=center
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Disease}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Erythema Characteristics}}
! style="background: #4479BA; padding: 5px 5px;" colspan=1 | {{fontcolor|#FFFFFF|Signs and Symptoms}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Associated Conditions}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF|Histopathology}}
! style="background: #4479BA; padding: 5px 5px;" colspan=1 | {{fontcolor|#FFFFFF|Lab finding 
&
Other evaluation}}
! style="background: #4479BA; padding: 5px 5px;" rowspan=1 | {{fontcolor|#FFFFFF| Prognosis}}
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Erythema gyratum repens|Erythema gyratum repens (EGR)]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Migratory]] annular and configurate erythematous bands that form concentric rings
* Wood grain scaly appearance
*[[scales]] follows the leading edge of the bands
*[[Eruption]] migrates more rapidly, 1cm/d<br />
| style="padding: 5px 5px; background: #F5F5F5;" |
* Skin [[Eruption|eruptions]]
* Severe Generalized  itching ([[pruritus]])
*[[Scaly]] erythematous patches over  trunk and proximal extremities, sparing the hands, feet, and face, can eventually involve the face.   
*[[Weight loss]]
*[[Malaise]] and [[fatigue]]
*[[Fever]]
*[[Anorexia]]
*[[Lymphadenopathy]]
*[[Headache]] and [[convulsion]] (intracranial metastasis)
*[[Shortness of breath]] ([[Bronchogenic carcinoma|bronchogenic carcinoma)]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Dermatologic conditions:
**[[Ichthyosis]] palmar/plantar [[hyperkeratosis]]
* Less frequently EGR co-present with:
**[[Pityriasis Rubra Pilaris]], [[Bullous pemphigoid]], [[Pemphigus vulgaris|Pemphigus vulgaris,]] [[Discoid lupus erythematosus|discoid lupus eythemutosus]], [[psoriusiform]] lesions, and nonspecific vesicles and bullae
*[[Tuberculosis]]
 
*[[CREST syndrome]]
 
([[Calcinosis]], [[Raynaud's phenomenon|Raynaud’s phenomenon,]] [[Esophageal dysmotility]], [[Sclerodactyly]], and [[Telangiectasia]])
| style="padding: 5px 5px; background: #F5F5F5;" |
* The [[epidermis]]:
**[[Acanthosis]]
**Focal [[parakeratotosis]] and [[spongiosis]]
*The [[dermis]]:
**Mild focal spongiosis and parakeratosis
**Moderate [[perivascular]] [[Mononuclear cells|mononuclear]], [[lymphocytic]], and [[histiocytic]] infiltrate
**[[Eosinophils]] and [[melanophages]] have also been reported in the infiltrate
*Diffuse to moderate [[edema]] of the [[Connective tissue|connective tissue c]]<nowiki/>an be seen
| style="padding: 5px 5px; background: #F5F5F5;" |
*There are no diagnostic laboratory findings associated with erythema gyratum repens
*[[Eosinophilia]] is observed in 60% of cases<ref name="pmid22224159" />
*Decreased [[T lymphocytes]] and increased [[B lymphocytes]] observed in an erythema gyratum repens patient with increased [[luteinizing hormone]] and [[follicle-stimulating hormone]]<ref name="pmid8339188" />
*Decreased serum levels of [[Complement system|C3]]<ref name="pmid8339188" />
*Normal percentages of B and T [[lymphocytes]] and normal T-cell function were reported in an EGR patient without [[Cancer (disease)|cancer]]<ref name="pmid8339188" />
 
<br />
| style="padding: 5px 5px; background: #F5F5F5;" |
* Skin manifestations can be improved within 48 hours of the resection of the underlying [[tumor]] with on of the following:
** Complete cure of the skin [[eruption]] and [[pruritus]]
** Temporary improvement then recurrence of the [[eruption]] (specially in cases of [[metastasis]])
** No effect of the tumor treatment on the course of EGR
*** Death can occur few weeks after the detection of the [[malignancy]], few months, or four years as in Gammel's patient.
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Erythema annulare centrifugum]] ([[Erythema annulare centrifugum|EAC]]) <ref name="pmid8339188" />'''
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Migratory]] annular and configurate erythematous
 
or [[polycyclic]] lesions     
 
*[[Urticaria|Urticarial]] in appearance, ringed, [[arcuate]] figures
 
*[[Eruption]] migrate at a slower rate (2 -3 mm/d) reaching up to 10 cm in diameter with central clearing
 
* Cover only a small percentage of the total body surface    
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Annular]] or [[polycyclic]] [[lesions]] which may begin as [[urticaria]]-like [[Papules|papule]]
* Eventually old lesions can spontaneously resolve in several days to a few weeks while new eruptions develop.
* The [[deep]] form of erythema annulare centrifugum has a firm, indurated border, is rarely [[Pruritic disorders|pruritic]], and has no scale
* The superficial type of erythema annulare centrifugum has an indistinct scaly border and is usually [[Pruritic disorders|pruritic]]  
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Infections]]
 
*[[Allergic Reaction|Allergic reactions]] to drugs
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Deep form:]]
**[[Mononuclear cells|Mononuclear]], [[Perivascular cell|perivascular]] [[infiltrate]] in the middle and lower portions of the [[dermis]] ([[coat sleeve-like configuration]])
**[[Infiltrate]] is primarily of [[lymphocytes]], but [[eosinophils]] are occasionally present
**Extravasation of [[erythrocytes]] is associated with [[Endothelial|endothelial swelling]]   
** No epidermal changes   
* Superficial:
** More non-specific
** Slight superficial perivascular [[Lymphocyte|lympho-]][[Histiocyte|histiocytic]] infiltrate   
** Focal parakeratosis and mild spongiosis with microvesiculation
| style="padding: 5px 5px; background: #F5F5F5;" |
* No specific laboratory changes
 
*[[Eosinophilia]] of the peripheral blood, as well as tissue, can be observed in erythema annulare centrifugum associated with a [[drug reaction]] or [[parasitic]] [[infection]]   
 
* Evaluation for possible [[infection]] or [[drug reaction]] (prescribed and non-prescribed)
 
*[[Complete blood counts|Complete blood count]]
*[[Urinalysis]]
*[[Liver function tests]]
*[[Renal function tests|Kidney function test]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Lesions disappear after the underlying etiology is managed ([[allergy]], [[infection]], [[malignancy]])
* if no underlying cause, lesions can recur after discontinuation of the supportive treatment
|-
| style="padding: 5px 5px; background: #DCDCDC;" | '''[[Necrolytic migratory erythema|Necrolytic migratory erythema (NME)]]'''
| style="padding: 5px 5px; background: #F5F5F5;" |
* Migratory circinate [[erythema]]/[[plaques]] with areas of [[necrosis]] and [[sloughing]]
 
*[[Crusted]]  [[Erythematous]] scaly plaques with centrifugal growth
 
*
| style="padding: 5px 5px; background: #F5F5F5;" |
* Red [[erythematous]] scaly [[plaques]] over [[Perineum]], distal [[extremities]], lower [[abdomen]], and [[face]]
* Spontaneous exacerbation and [[remission]] periods without knowing what the trigger is
*[[Weight loss]]
*[[Anemia]]
*[[Diabetes]]
*[[Diarrhea]]
*[[Stomatitis]].
| style="padding: 5px 5px; background: #F5F5F5;" |
* Obligatory [[paraneoplastic]] [[syndrome]]
*First manifestation of the rare [[pancreatic neuroendocrine tumor]] ([[Glucagonoma|glaucagonoma]])
*No other association
* Can be misdiagnosed as:
**[[Contact dermatitis]]
**[[Intertrigo]]
**[[Psoriasis|Inverse psoriasis]]
**[[Zinc deficiency]]
** Other [[nutritional deficiencies]]
| style="padding: 5px 5px; background: #F5F5F5;" |
*[[Paleness]] and spongiosis of the upper layer of the [[epidermis]]
 
* A [[Perivascular cell|perivascular]] [[lymphocytic]] and [[histiocytic]] infiltrate
 
*[[Necrotic]] [[Keratinocyte|keratinocytes]] are common and can lead to erosions, crusting and [[Scaling skin|scaling]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Increased [[glucagon]] level
 
* Evaluation of the associated [[tumor]]:
**[[CT-scans|CT]] or [[MRI]] [[abdomen]]
 
* * [[Visceral angiography|Selective visceral angiography]] to localize the tumor
 
* * [[Positron Emission Tomography]] (PET)
 
* * [[Octreotide]] [[scintigraphy]]
| style="padding: 5px 5px; background: #F5F5F5;" |
* Due to the difficulty of necrolytic migratory erythema recognition, and its association with [[glucagonoma]], diagnosis is usually delayed


==Differentiating ((Page name)) from Other Diseases==
* Necrolytic migratory erythema usually resolved after the resection and treatment of the [[Pancreatic tumor|pancreatic tumor,]] eg.10 days after tumor resection
* EGR has a narrow differential diagnosis and it has to be differentiated from skin lesions with gyrate erythematous eruptions, such as:
 
** Necrolytic migratory erythema (NME)
* Early recognition is crucial for better diagnosis and prognosis <br />
** Erythema annulare centrifugum (EAC)
|}
** Erythema migrans
 
==Epidemiology and Demographics ==
* Erythema gyratum repens is a [[rare]], characteristic, and [[paraneoplastic syndrome]] with the following [[demographics]]:<ref name="pmid22224159" />


==Epidemiology and Demographics==
* EGR is a rare dermatologic disease
'''Age'''
'''Age'''
* The average age of onset of EGR is in the seventh decade of life
* The [[average]] age of onset of erythema gyratum repens i is in the seventh decade of life (65 years old).
 
'''Gender'''
'''Gender'''
* The male to female ratio is 2:1
* The male to female ratio is 2:1.
 
'''Race'''
'''Race'''
* EGR commonly affects Caucasians
* EGR commonly affects Caucasians.


==Risk Factors==
==Risk Factors==
* There are no established risk factors for EGR.  
* There are no established [[risk factors]] for erythyma gyratum repens.
*Many patients with erythyma gyratum repens and [[malignancy]] had a history of [[tobacco smoking]].
*Some patients with erythyma gyratum repens and [[malignancy]] have a family history of [[neoplasm]].


==Screening==
==Screening==
* There are no screening tests for EGR.
* There are no [[screening]] tests for erythema gyratum repens.
* Screening for internal malignancy should be done immediately after EGR is diagnosed.  
*[[Screening]] for internal [[malignancy]] should be done immediately after erythema gyratum repens is [[Diagnosis|diagnosed]].


==Natural History, Complications, and Prognosis==
==Natural History, Complications, and Prognosis==
* The majority of patients with EGR presents with severely pruritic erythematous skin lesions that appear several months prior to the malignancy diagnosis.
* The majority of patients with erythema gyratum repens presents with severely [[Pruritic disorders|pruritic]] [[erythematous]] skin lesions that appear several months prior to the [[malignancy]] diagnosis<ref name="pmid22224159" />
* If the underlying malignancy left untreated, the debilitating pruritus could persist until the patient dies.
* If the underlying [[malignancy]] left untreated, the debilitating [[pruritus]] could persist until the patient dies<ref name="pmid22224159" />
*Prognosis depends on the type of the underlying tumor and the probability of its treatment.  
*[[Prognosis]] depends on the type of the underlying [[tumor]] and the probability of its treatment. It depends on the time of the erythema gyratum repens onset and the [[neoplasm]] discovery. The course and [[prognosis]] of erythema gyratum repens can be one of the following: 
** Complete [[cure]] of the skin [[eruption]] and [[pruritus]] after removal and treatment of the internal [[neoplasm]].
** Temporary improvement then recurrence of the [[eruption]] (specially in cases of [[metastasis]]).
** No effect of the [[tumor]] treatment on the course of erythema gyratum repens.
** Death can occur few weeks after the discovery of the [[malignancy]], few months, or four years as in Gammel's patient.


==Diagnosis==
==Diagnosis==
===Diagnostic Study of Choice===
===Diagnostic Study of Choice===
* EGR is mainly diagnosed clinically by its characteristic skin lesions.  
* Erythyma gyratum repens is mainly [[Diagnosis|diagnosed]] [[Clinical|clinically]] by its characteristic [[skin lesions]].
* It is considered as a [[cutaneous]] [[marker]] of [[malignancy]] with high [[specificity]] so physicians shouldn't miss its unique [[clinical]] skin presentation.
 
===History and Symptoms===
===History and Symptoms===
* The universal symptoms of EGR are:
* The universal symptoms of erythema gyratum repens are:
** Skin eruptions  
**[[Eruption|Skin eruptions]]
** Intense pruritus  
**[[Pruritus|Intense pruritus]]
* Other symptoms related to the associated internal malignancy are:
* Other symptoms related to the associated internal [[malignancy]] may include:
** Weight loss
** [[Weight loss]]
** Anorexia
** [[Fatigue]]
** Fatigue
**[[Anorexia]]
** Fever      
** [[Vomiting]]
** [[Fever]]
**[[Headache]]
**[[Convulsion]]
**[[Shortness of breath]]
**[[Abdominal distention]]


===Physical Examination===
===Physical Examination===
* Patients with EGR presents with a rash consisting of wavy erythematous concentric bands that can be figurate, gyrate, or annular.
* Patients with erythyma gyratum repens usually are ill-appearing and [[lethargic]]
* The bands are arranged in parallel rings and lined by a fine trailing edge of scale, a pattern often described as “wood grained.  
*[[Physical examination]] may be remarkable for:
* The rash typically involves large areas of the body but tends to spare the face, hands, and feet and it can expand as fast as a cm a day.  
**[[Wood]]-[[grain]] erythematous scaly skin [[eruption]].
* Bullae can also form from within the areas of erythema.
**Bullae can also form within the areas of erythema.
**Typically involves large areas of the [[body]] but tends to spare the [[face]], [[hands]], and [[feet]] and it can expand as fast as 1 cm a day.<ref name="pmid22224159" />
**[[Signs]] of [[malignancy]] can be seen based on the [[neoplasm]] location such as:
***[[Lymphadenopathy]]
***[[Palpable]] [[mass]]
***[[Abdominal]] [[ascites]]
***[[Pleural effusion]]
***[[Papilloedema]]


===Laboratory Findings===
===Laboratory Findings===
* There are no diagnostic laboratory findings associated with EGR.
* There are no [[diagnostic]] [[laboratory]] findings associated with erythema gyratum repens.
* Eosinophilia is observed in 60% of cases  
* [[Eosinophilia]] is observed in 60% of cases.<ref name="pmid22224159" />
 
*Decreased [[T lymphocytes]] and [[increased]] [[B lymphocytes]] observed in an erythema gyratum repens patient with increased [[luteinizing hormone]] and [[follicle-stimulating hormone]].<ref name="pmid8339188" />
*Decreased serum levels of [[C3 (complement)|C3]].<ref name="pmid8339188" />
*Normal percentages of [[B lymphocyte|B]] and [[T lymphocytes]] and normal [[T-cell|T-cell function]] were reported in an erythema gyratum repens patient without [[cancer]].<ref name="pmid8339188" />


=== Imaging Findings===
=== Imaging Findings===
* There are no imaging findings associated with EGR.
* There are no [[imaging]] findings associated with erythyma gyratum repens.
* Imaging of the chest and abdomen could show malignancy findings.  
*[[Imaging]] to look for systemic [[neoplasms]] are:<ref name="pmid22224159" />
*<nowiki>* </nowiki>[[Computed tomography]] of the [[head]], [[neck]], [[chest]], [[abdomen]], and [[pelvis]].
**[[Positron emission tomography]]/[[computed tomography]]
** Upper and lower [[gastrointestinal]] [[endoscopy]]
*The abnormal findings that heightened concern for systemic or widespread [[malignancy]] are:
**[[Brain]], [[chest]], [[lung]], [[abdominal]], [[peritoneal]], or [[Pelvic masses|pelvic mass.]]
**[[Lymphadenopathy]].
** Enhanced bone lucencies suggestive of diffuse [[metastasis]].


===Other Diagnostic Studies===
===Other Diagnostic Studies===
* Direct immunofluorescence in some cases shows patterns of IgG, C3, and C4 at the basement membrane. 
 
* The histopathologic features of EGR is non-specific.  
* The [[histopathologic]] features of EGR is non-specific.
* Biopsy specimens show the following:  
* [[Biopsy]] specimens show the following:<ref name="pmid22224159" />
** Acanthosis, mild hyperkeratosis, focal parakeratosis, and spongiosis confined to the epidermis and superficial dermis.
**Acanthosis, mild [[hyperkeratosis]], focal parakeratosis, and spongiosis confined to the [[epidermis]] and superficial [[dermis]]
** Mononuclear, lymphocytic, and histiocytic perivascular infiltrate in the superficial plexus can also be seen.  
** Mononuclear, [[Lymphocyte|lymphocytic]], and [[histiocytic]] perivascular infiltrate in the superficial [[plexus]] can also be seen
**[[Eosinophils]] and melanophages have also been reported in the dermal infiltrate
**Diffuse to moderate [[edema]] of the [[connective]] [[tissue]] can be seen
**Direct [[immunofluorescence]] can show patterns of [[IgG]], [[C3 (complement)|C3]], and C4 at the [[basement membrane]]
* Thorough paraneoplastic and systemic workup includes:<ref name="pmid22224159" /><ref name="pmid31111084">{{cite journal| author=Ridge A, Tummon O, Laing M| title=Response to "Transformation from pityriasis rubra pilaris to erythema gyratum repens-like eruption without associated malignancy: A report of 2 cases". | journal=JAAD Case Rep | year= 2019 | volume= 5 | issue= 5 | pages= 461-462 | pmid=31111084 | doi=10.1016/j.jdcr.2019.03.012 | pmc=6510971 | url=https://www.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&tool=sumsearch.org/cite&retmode=ref&cmd=prlinks&id=31111084  }}</ref>
**[[Computed tomography]] of [[head]], [[neck]], [[chest]], [[abdomen]], and [[pelvis]].
**[[Positron emission tomography]]/[[computed tomography]].
** Upper and lower [[gastrointestinal]] [[endoscopy]].
**Complete [[blood]] [[chemistry]] ([[CBC]]).
**[[Comprehensive metabolic panel]] (CMP).
**[[Urinalysis|Urinanalysis.]]
**[[Rapid plasma reagin]] test [to exclude [[syphilis]]].
**[[Anti-nuclear antibody]] test [to exclude [[autoimmune]] disorders as [[systemic lupus erythematosus]] ([[SLE]])].
**Guaiac stool test.
**[[Serum protein electrophoresis]] [to exclude [[cancers]] as [[multiple myeloma]]].
**[[Lactate dehydrogenase]] [tissue damage, [[kidney disease]], [[liver disease]]].
**[[QuantiFERON]] [to exclude [[tuberculosis]]].
**[[Tumor markers]].


==Treatment==
==Treatment==
'''Medical Therapy'''
=== Medical Therapy ===
* There is no treatment for EGR; the mainstay of therapy is supportive care and treating the underlying condition.
*Treatment of erythema gyratum repens, and its associated intense [[pruritus]] depends on the recognition and treatment of the underlying [[malignancy]]<ref name="pmid22224159" />
* Various dermatologic and immunosuppressive therapies have been used to treat EGR.  
*[[Symptomatic treatment|Symptomatic management]]:
* Systemic steroids are frequently ineffective.  
**[[Hydroxyzine]] for itching, [[ibuprofen]] and [[oxycodone]] for pain, and t[[riamcinolone]] 0.1% [[cream]] for the rash.
* Topical steroids, vitamin A, and azathioprine have also failed to relieve skin manifestations.
*Management of the [[neoplasm]] depends on its type, location, stage, and time of its discovery and on patient preference:
* Improvement of EGR, and its associated intense pruritus depends on recognition and treatment of the underlying malignancy.
**[[Surgical]] removal
* Chemotherapy can be used to treat the internal malignancy.  
** [[Chemotherapy]]
** [[Conservative]] [[palliative]] management
*Various [[Dermatological|dermatologic]] and [[immunosuppressive]] therapies have been used to treat erythema gyratum repens.
*Systemic [[steroids]] are frequently ineffective.
* Topical [[steroids]], [[vitamin A]], and [[azathioprine]] have also failed to relieve skin manifestations
 
===Surgery ===
* [[Surgical]] [[resection]] of the discovered [[malignancy]] could be recommended as part of the management of Erythyma gyratum repens.
 
=== Prevention ===


===Surgery===
*[[Primary prevention]]:
* Surgical resection of the internal tumor could be recommended as part of the management of EGR.
** There are no [[Primary prevention|primary preventive]] measures available for erythema gyratum repens
'''Prevention'''
*[[Secondary prevention]]:
* There are no primary preventive measures available for [disease name].
** If the thorough screening after Erythyma gyratum repens diagnosis detected the [[malignancy]] in its earliest stages.
* Tertiary [[prevention]]:
** If the thorough screening after Erythyma gyratum repens [[diagnosis]] detected the [[malignancy]] in its late stages or with widespread [[metastasis]].
** Tertiary [[prevention]] aims to improve the [[quality of life]] and [[life expectancy]].


==References==
==References==
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Huda A. Karman, M.D.

Synonyms and keywords: Gammel's disease


Overview

Erythema gyratum repens is a rare highly specific and characteristic paraneoplastic syndrome that usually affect older people. It is characterized by wood-grain scaly skin eruption with intense pruritus. The cause of erythema gyratum repens is unknown but many theories suggest immunologic etiology or toxicologic products that are released by the associated tumor. The first case of erythema gyratum repens was described by a dermatologist named Gammel in the year 1952. For many years after erythema gyratum repens original description, there was little progress in defining the pathogenesis of erythema gyratum repens. Erythema gyratum repens has no specific classification but we can classify it based on its association with an internal malignancy into para-neoplastic and non-para-neoplastic erythema gyratum repens. The most common malignancies associated with erythema gyratum repens are lung or bronchogenic cancer, esophageal cancer, and breast cancer. Erythema gyratum repens can also be associated with non-neoplastic diseases such as tuberculosis, autoimmune disorders, or CREST syndrome. Erythema gyratum repens is characterized by its pathogonomic figurate, gyrate, or annular erythematous skin eruptions. The intense pruritus can be debilitating and usually urges the patient to go to the emergency department. The microscopic histopathological features of erythema gyratum repens consist of acanthosis, focal parakeratotic, and spongiosis of the epidermis with perivascular mononuclear, lymphocytic, and histiocytic infiltrate in the superficial plexus of the dermis. Erythema gyratum repens is very rare and it mainly affects people in their seventieth decade, the male to female ratio is 2:1. Erythema gyratum repens is diagnosed clinically by its characteristic skin eruption and an urgent thorough paraneoplastic workup should be initiated to look for internal malignancies. Patients with erythema gyratum repens presents with intensely pruritic, gradually progressive, skin lesions that crawl rather than migrate from one body region to the other. It can start in the upper trunk or upper back and extends to involve the extremities sparing the face. The mainstay of the treatment of erythema gyratum repens is finding and treating the underlying malignancy. Symptomatic treatment is not very effective in relieving the pruritus and its associated pain. The management can be surgical removal of the tumor, chemotherapy, or palliative conservative management. The skin eruptions can improve completely after the removal of the underlying tumor, or can recur especially if the tumor recurred or metastasized. Patients can live a few weeks, months or up to five years depending on when and at what stage the malignancy was detected.

Historical Perspective

Classification

Types of Erythema gyratum repens Characterestics
Paraneoplastic EGR
Non-paraneoplastic EGR Idiopathic EGR
  • Erythema gyratum repens with no underlying malignancy, associated conditions, or precipitating cause
|EGR-like eruptions [8]
EGR with concomitant skin disease
Drug-induced EGR

Pathophysiology


Causes

Differentiating Erythema Gyratum Repens from Other Diseases

Types examples
With underlying malignancy [14][15]
  • Erythema gyratum repens (EGR)
Without underlying malignancy
  • Erythema marginatum rheumaticum


Disease Erythema Characteristics Signs and Symptoms Associated Conditions Histopathology Lab finding

& Other evaluation

Prognosis
Erythema gyratum repens (EGR)
  • Migratory annular and configurate erythematous bands that form concentric rings
  • Wood grain scaly appearance
  • scales follows the leading edge of the bands
  • Eruption migrates more rapidly, 1cm/d

(Calcinosis, Raynaud’s phenomenon, Esophageal dysmotility, Sclerodactyly, and Telangiectasia)


  • Skin manifestations can be improved within 48 hours of the resection of the underlying tumor with on of the following:
    • Complete cure of the skin eruption and pruritus
    • Temporary improvement then recurrence of the eruption (specially in cases of metastasis)
    • No effect of the tumor treatment on the course of EGR
      • Death can occur few weeks after the detection of the malignancy, few months, or four years as in Gammel's patient.
Erythema annulare centrifugum (EAC) [14]
  • Migratory annular and configurate erythematous

or polycyclic lesions

  • Eruption migrate at a slower rate (2 -3 mm/d) reaching up to 10 cm in diameter with central clearing
  • Cover only a small percentage of the total body surface   
  • Annular or polycyclic lesions which may begin as urticaria-like papule
  • Eventually old lesions can spontaneously resolve in several days to a few weeks while new eruptions develop.
  • The deep form of erythema annulare centrifugum has a firm, indurated border, is rarely pruritic, and has no scale
  • The superficial type of erythema annulare centrifugum has an indistinct scaly border and is usually pruritic  
  • No specific laboratory changes
  • Lesions disappear after the underlying etiology is managed (allergy, infection, malignancy)
  • if no underlying cause, lesions can recur after discontinuation of the supportive treatment
Necrolytic migratory erythema (NME)
  • Due to the difficulty of necrolytic migratory erythema recognition, and its association with glucagonoma, diagnosis is usually delayed
  • Necrolytic migratory erythema usually resolved after the resection and treatment of the pancreatic tumor, eg.10 days after tumor resection
  • Early recognition is crucial for better diagnosis and prognosis

Epidemiology and Demographics

Age

  • The average age of onset of erythema gyratum repens i is in the seventh decade of life (65 years old).

Gender

  • The male to female ratio is 2:1.

Race

  • EGR commonly affects Caucasians.

Risk Factors

Screening

Natural History, Complications, and Prognosis

  • The majority of patients with erythema gyratum repens presents with severely pruritic erythematous skin lesions that appear several months prior to the malignancy diagnosis[13]
  • If the underlying malignancy left untreated, the debilitating pruritus could persist until the patient dies[13]
  • Prognosis depends on the type of the underlying tumor and the probability of its treatment. It depends on the time of the erythema gyratum repens onset and the neoplasm discovery. The course and prognosis of erythema gyratum repens can be one of the following:
    • Complete cure of the skin eruption and pruritus after removal and treatment of the internal neoplasm.
    • Temporary improvement then recurrence of the eruption (specially in cases of metastasis).
    • No effect of the tumor treatment on the course of erythema gyratum repens.
    • Death can occur few weeks after the discovery of the malignancy, few months, or four years as in Gammel's patient.

Diagnosis

Diagnostic Study of Choice

History and Symptoms

Physical Examination

Laboratory Findings

Imaging Findings

Other Diagnostic Studies

Treatment

Medical Therapy

Surgery

Prevention

References

  1. Rothman, Stephan (1925). "Über Hauterscheinungen bei bösartigen Geschwülsten innerer Organe". Archiv für Dermatologie und Syphilis. 149 (1): 99–123. doi:10.1007/BF02297811. ISSN 0340-3696.
  2. Burgdorf WHC, Bickers DR (2015). "The scientific legacy of Stephen Rothman". J Invest Dermatol. 135 (4): 954–959. doi:10.1038/jid.2014.447. PMC 4366295. PMID 25373439.
  3. 3.0 3.1 3.2 Gammel, John A. (1952). "ERYTHEMA GYRATUM REPENS". A.M.A. Archives of Dermatology and Syphilology. 66 (4): 494. doi:10.1001/archderm.1952.01530290070010. ISSN 0096-5979.
  4. Purdy, M. J. (1959). "Erythema Gyratum Repens". A.M.A. Archives of Dermatology. 80 (5): 590. doi:10.1001/archderm.1959.01560230076020. ISSN 0096-5359.
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