Necrolytic migratory erythema

• Migratory circinate erythema/plaques with areas of necrosis and sloughing

• Crusted  Erythematous scaly plaques with centrifugal growth

• 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.

• Obligatory paraneoplastic syndrome
• First manifestation of the rare pancreatic neuroendocrine tumor (glaucagonoma)
• No other association
• Can be misdiagnosed as:
  • Contact dermatitis
  • Intertrigo
  • Inverse psoriasis
  • Zinc deficiency
  • Other nutritional deficiencies

• Paleness and spongiosis of the upper layer of the epidermis

• A perivascular lymphocytic and histiocytic infiltrate

• Necrotic keratinocytes are common and can lead to erosions, crusting and scaling

• Increased glucagon level

• Evaluation of the associated tumor:
  • CT or MRI abdomen

• * Selective visceral angiography to localize the tumor

• * Positron Emission Tomography (PET)

• * Octreotide scintigraphy

• 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
  

• Migratory annular and configurate erythematous

or polycyclic lesions

• 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   

• 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  

• Infections

• Allergic reactions to drugs

• Deep form:
  • Mononuclear, 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 swelling   
  • No epidermal changes   
• Superficial:
  • More non-specific
  • Slight superficial perivascular lympho-histiocytic infiltrate   
  • Focal parakeratosis and mild spongiosis with microvesiculation

• 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 count
• Urinalysis
• Liver function tests
• Kidney function test

• Lesions disappear after the underlying etiology is managed (allergy, infection, malignancy)
• if no underlying cause, lesions can recur after discontinuation of the supportive treatment

• 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
  

• Skin 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)

• Dermatologic conditions:
  • Ichthyosis palmar/plantar hyperkeratosis
• Less frequently EGR co-present with:
  • Pityriasis Rubra Pilaris, Bullous pemphigoid, Pemphigus vulgaris, discoid lupus eythemutosus, psoriusiform lesions, and nonspecific vesicles and bullae
• Tuberculosis

• CREST syndrome

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

• The epidermis:
  • Acanthosis
  • Focal parakeratotosis and spongiosis
• The dermis:
  • Mild focal spongiosis and parakeratosis
  • Moderate perivascular mononuclear, lymphocytic, and histiocytic infiltrate
  • Eosinophils and melanophages have also been reported in the infiltrate
• Diffuse to moderate edema of the connective tissue can be seen

• There are no diagnostic laboratory findings associated with erythema gyratum repens
• Eosinophilia is observed in 60% of cases^[3]
• Decreased T lymphocytes and increased B lymphocytes observed in an erythema gyratum repens patient with increased luteinizing hormone and follicle-stimulating hormone^[1]
• Decreased serum levels of C3^[1]
• Normal percentages of B and T lymphocytes and normal T-cell function were reported in an EGR patient without cancer^[1]

• 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.