Erythema migrans arciforme et palpabile L53.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 01.04.2021

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Synonym(s)

EMAP; Erythema arciformis et palpabile migrans; Palpable migratory arciform erythema (engl.); PMAE

History
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Clark, 1974

Definition
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Cutaneous T-cell pseudolymphoma of unknown genesis, presumably variant of lymphocytic infiltration of the skin. A relationship to lupus erythematosus is also discussed.

Manifestation
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Occurs mainly in adult males.

Localization
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Back, arms, thighs. In one case, facial involvement was reported.

Clinical features
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Isolated, pale, homogeneously disc-shaped, also anular or large (10 cm or larger), arched, red, more rarely blue-red, not scaly (or only slightly scaly!), clearly and firmly palpable, flat papules and plaques with elevated margins. Centrifugal progression is common to all foci, and peripheral growth may result in large scale annular or circulatory formations. Important: Scaling on the surface is absent (DD: Erythema anulare centrifugum) or minimal. There is also no relevant itching (delimitation to the tinea corporis).

Histology
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Normal epidermis that is orthokeratotic keratinized by a stratum grnulosum. Spot-shaped, homogeneous, perivascular and periadnexial, superficial and deep, small cell, cuff-shaped, small cell T-cell infiltrate (CD4+/CD8+ reactivity =4:1) without epidermal involvement. No indication of a granulomatous component. Identical with lymphocytic infiltration of the skin.

Direct Immunofluorescence
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The immunofluorescence tests performed with the routine programs are generally non-reactive. In particular, there is no immunoglobulin ablation at the dermo-epidermal junction zone.

Differential diagnosis
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Malignant lymphoma of the skin: histological differentiation possible

Lupus erythematodes integumentalis: positive direct immunofluorescence

Erythema anulare centrifugum: Histologically similar picture possible. Clinically, there are usually raised scales.

Lupus erythematosus tumidus: this distinction is clinically as well as histologically very difficult.

Erythema anulare centrifugum-like psoriasis: also here anular structures. However, surface scaling, also pustular formations, localizations not limited to the trunk. Histology with granulocytic microabscesses(Munro microabscess).

Tinea corporis: scaling, itching, cultural and microscopic evidence of dermatophytes possible.

Therapy
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The therapy depends on the underlying pathomechanism. Symptomatically, UVA1 light therapy can be initiated.

External therapy
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Glucocorticoid externa.

Case report(s)
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Medical history: The 48-year-old previously skin-healthy administrative clerk reported a moderately itchy "skin rash" on the back which disappeared after some 14 days during the maritime summer vacation, but persisted for about 2 months with peripheral size growth. The allergological history was completely blank. No other relevant general diseases were found. No febrile infections in the last months. No medication except occasional ASA for headaches.

Findings: The otherwise completely healthy patient showed disseminated red, scaly papules, homogeneously flat, size-progressive, angular and large (up to 10 cm in diameter), arch-shaped, medium red plaques. The consistency of the lesions was surprisingly firm.

Histology: Normal epidermis orthokeratotic keratinized by a stratum granulosum. Fleck-shaped, homogeneous, perivascular and periadnexial, superficial and deep, small cell, cuff-shaped, small cell T-cell infiltrate (CD4+/CD8+ reactivity =1:4) without epidermal involvement. No indication of a granulomatous component. Direct immunofluorescence: no indication of patholgic reactivity.

Laboratory: completely inconspicuous. ANA, DNA-AK. neg. normal complement fractions, BSG: 10/20mm n.d.; CRP: <0,5mg/dl

Imaging procedures (sonography; X-ray thorax): o.B.

Therapy: The changes were sensitive to local therapy with a class II glucocorticoid. However, recurrences occurred within 4 days after discontinuation. UVA1 light therapy showed a significant improvement after 4 weeks of treatment. In a subsequent therapy break after 3 weeks, recurrence occurred again. A systemic therapy with azathioprine (150mg/day p.o.) in combination with initial 50mg prednisolone and a later maintenance dose of 7.5 mg resulted in a freedom from symptoms. In the meantime, the treatment was carried out for 1 year with good tolerance. Several attempts to reduce the steroidal therapy below the threshold of 7.5 mg led to recurrences.

Literature
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  1. Abeck D et al (1997) Palpable migratory arciform erythema. Clinical morphology, histopathology, immunohistochemistry, and response to treatment. Arch Dermatol 133: 763-766
  2. Clark WH et al (1974) The lymphocytic infiltrates of the skin. Human Pathol 5: 25-43
  3. Dantas FL et al(2015) Possiblydrug-induced palpable migratory arciform erythema. An Bras Dermatol 90(3 Suppl 1):77-80.

  4. Lohrisch I et al (1990) Erythema migrans arciforme et palpabile. dermatologist 41: 78-82

  5. Muche JM et al (2004) Palpable arciform migratory erythema in an HIV patient, a CD8+ pseudolymphoma. J Cutan catheter 31:379-382.

  6. Poenitz N et al (2003) Jessner's lymphocytic infiltration of the skin: a CD8+ polyclonal reactive skin condition. Dermatology 207: 276-284

  7. Quay ER et al.(2014) A variant of palpable migratory arciform erythema. J Drugs Dermatol 13:1288-1289.

  8. Steinmann A et al (1999) Erythema migrans arciforme et palpabile and lymphocytic infiltration of the skin - different manifestations of the same entity? dermatologist 50: 270-274

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Last updated on: 01.04.2021