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{{CMG}} {{Hudakarman}}


==Overview==
==Overview==
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;Associated symptoms
;Associated symptoms
[[Weight loss]], [[anemia]] , [[mild diabetes]], [[diarrhea]] and [[glossitis]] are associated. Liver metastasis is often present.
[[Weight loss]], [[anemia]] , [[mild diabetes]], [[diarrhea]] and [[glossitis]] are associated. Liver metastasis is often present.
==Differentiating necrolytic migratory erythema from Other Diseases==
*Necrolytic migratory erythema has to be differentiated from Reactive gyrate erythematous eruptions, such as:
**[[Erythema annulare centrifugum]] (EAC)
**[[Erythema gyratum repens]] (EGR)
*Reactive (figurate or gyrate) [[Erythema|erythemas]] that are associated with [[malignancy]] include:'''<ref name="pmid8339188" /><ref name="pmid861171" />''' <br />
{| style="border: 0px; font-size: 90%; margin: 3px;" align="center"
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF| Disease}}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF|Erythema Characteristics}}
! colspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF|Signs and Symptoms}}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF|Associated Conditions}}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF|Histopathology}}
! colspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF|Lab finding 
&
Other evaluation}}
! rowspan="1" style="background: #4479BA; padding: 5px 5px;" |{{fontcolor|#FFFFFF| Prognosis}}
|-
| 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]]
*<nowiki>* </nowiki>[[Visceral angiography|Selective visceral angiography]] to localize the tumor
*<nowiki>* </nowiki>[[Positron Emission Tomography]] (PET)
*<nowiki>* </nowiki>[[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
*Necrolytic migratory erythema usually resolved after the resection and treatment of the [[Pancreatic tumor|pancreatic tumor,]] eg.10 days after tumor resection
*Early recognition is crucial for better diagnosis and prognosis <br />
|-
| 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;" |'''[[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" />
| 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.
|}
==References==
{{reflist|2}}


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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1] Huda A. Karman, M.D.

Overview

Necrolytic migratory erythema (NME) is a classical symptom observed in patients with glucagonoma and is present in 80% of cases. Associated NME is characterized by the spread of erythematous blisters and swelling across areas subject to greater friction and pressure, including the lower abdomen, buttocks, perineum, and groin.

Diagnosis

Symptoms

It consists of serpiginous (slow progressing) erythematous plaques. Where the migratory edge has an "eroded" appearance. It usually starts in the Perineum.

Associated symptoms

Weight loss, anemia , mild diabetes, diarrhea and glossitis are associated. Liver metastasis is often present.

Differentiating necrolytic migratory erythema from Other Diseases

Disease Erythema Characteristics Signs and Symptoms Associated Conditions Histopathology Lab finding

& Other evaluation

Prognosis
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
Erythema annulare centrifugum (EAC) [1]
  • 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
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.



References


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