Skin nontumor

Dermal perivascular and vasculopathic reaction patterns

Erythema annulare centrifugum



Last staff update: 4 May 2022

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PubMed Search: Erythema annulare centrifugum

M. Suzanne Bloomquist, M.D.
Silvija P. Gottesman, M.D.
Page views in 2021: 1,381
Page views in 2022 to date: 656
Cite this page: Bloomquist MS, Gottesman SP. Erythema annulare centrifugum. PathologyOutlines.com website. https://www.pathologyoutlines.com/topic/skinnontumorerythemaannularecentrifugum.html. Accessed May 20th, 2022.
Definition / general
  • Annular, erythematous, well circumscribed, mildly pruritic eruption with classic trailing scale (scale is seen behind the advancing erythematous edge)
  • Histopathology shows tightly cuffed or coat sleeve perivascular lymphohistiocytic inflammation
  • Classified into superficial and deep type, based on depth of inflammation; superficial type is more likely to have epidermal changes, such as spongiosis and mounds of parakeratosis (Calonje: McKee’s Pathology of the Skin, 5th Edition, 2019)
Essential features
  • Clinically, annular erythematous plaques with central clearing and trailing scale, with a superficial or deep coat sleeve perivascular lymphohistiocytic inflammation, with or without spongiosis and parakeratotic scale
  • Clinicopathologic correlation is necessary, since the superficial variant may be indistinguishable from pityriasis rosea (Patterson: Weedon’s Skin Pathology, 4th Edition, 2015)
  • Erythema annular centrifugum (EAC) is often recurrent (Ann Dermatol 2016;28:257)
Terminology
ICD coding
  • ICD 10: L53.1 - erythema annulare centrifugum
Epidemiology
Sites
Pathophysiology
  • Regarded as a hypersensitivity type reaction in response to a variety of triggers but the true pathophysiology remains unknown (J Dermatol 2002;29:61)
Etiology
Clinical features
  • Lesions start as urticarial smooth erythematous papules that enlarge centrifugally in annular erythematous plaques; these clear centrally and have a characteristic trailing scale
  • Advancing erythematous edges are typically raised
  • Superficial type may have pruritus and scale
  • Deep type may have indurated borders and no scale
  • Often recurrent, with relapsing / remittent course, including some cases showing seasonal or annual recurrences (Calonje: McKee’s Pathology of the Skin, 5th Edition, 2019)
Diagnosis
  • Easily made on examination of a punch biopsy specimen taken from the edge of the lesion with trailing scale
Laboratory
  • Dependent on the underlying association, nonspecific
Prognostic factors
  • One study showed that the superficial type may show shorter duration of skin lesion and better response to therapy but may have higher rate of recurrence (Ann Dermatol 2016;28:257)
Case reports
Treatment
Clinical images

Contributed by Silvija P. Gottesman, M.D.

Annular eruption with scale

Microscopic (histologic) description
Microscopic (histologic) images

Contributed by Silvija P. Gottesman, M.D.

Perivascular lymphohistiocytic inflammation

Subtle epidermal changes

Virtual slides

Images hosted on other servers:

Perivascular coat sleeve inflammation

Negative stains
  • PAS special stain in search of hyphae should be performed on all EAC biopsies to exclude dermatophytosis
Sample pathology report
  • Skin, right upper leg, punch biopsy:
    • Superficial perivascular lymphohistiocytic inflammation with spongiosis and parakeratotic scale crust (see comment)
    • Comment: Multiple levels were examined showing a tightly cuffed perivascular lymphohistiocytic infiltrate with subtle basilar spongiosis with parakeratosis. The histopathologic differential includes erythema annulare centrifugum and pityriasis rosea. Submitted clinical image was reviewed; annular erythema with trailing scale is noted and a diagnosis of erythema annulare centrifugum is favored. PAS stain for fungal organisms is negative.
Differential diagnosis
Board review style question #1
A 55 year old man presents with a 2 week history of circular rash on his bilateral thighs. The notes mention that although he had a positive fecal occult blood test (FOBT) last year, he has not been to the gastroenterologist for follow up, due to concerns regarding cost. On exam, the dermatologist notes the lesions are annular and raised, with trailing scale and central clearing. A punch biopsy is sent for histopathologic evaluation. Histology shows a well demarcated perivascular lymphohistiocytic infiltrate in the superficial dermis with subtle spongiosis and scale. What is the most likely diagnosis and appropriate next step?

  1. Erythema annulare centrifugum; include comment regarding association with malignancy among other triggers and need for further clinical evaluation
  2. Pityriasis rosea; inquire about recent upper respiratory symptoms and monitor for resolution in 2 - 6 weeks
  3. Secondary syphilis, the great mimicker, can have various histopathology outside the classic; perform an RPR
  4. Tinea corporis; perform a PAS stain to confirm
Board review style answer #1
A. Erythema annulare centrifugum; include comment regarding association with malignancy among other triggers and need for further clinical evaluation. The most likely diagnosis is erythema annulare centrifugum (EAC); a clinicopathologic diagnosis combining the findings of annular clinical eruption with scale and a perivascular lymphohistiocytic infiltrate on histopathologic sections. EAC will sometimes show an association with underlying malignancy (sometimes called paraneoplastic erythema annulare centrifugum or PEACE). Given the patient’s history of positive FOBT and this new onset of rash, a comment or phone call to the clinician may be warranted to ensure this patient receives appropriate follow up care (Calonje: McKee’s Pathology of the Skin, 5th Edition, 2019).

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Reference: Erythema annulare centrifugum
Board review style question #2


A 35 year old woman, with a history of COVID-19 infection 2 months prior, presents to the dermatologist with 1 month history of a raised circular rash. Pathology is shown in the images above. What is the most likely diagnosis?

  1. Annular elastolytic giant cell granuloma
  2. Erythema annulare centrifugum
  3. Erythema elevatum diutinum
  4. Granuloma annulare
Board review style answer #2
B. Erythema annulare centrifugum. There are many similar sounding names in dermatopathology, which can be confusing. Of the available choices, erythema annulare centrifugum is the only one that fits the histologic and clinical picture. Erythema elevatum diutinum is a vasculitic process presenting with papules / nodules on extensor surfaces with histologic features similar to leukocytoclastic vasculitis and onion skin fibrosis. Granuloma annulare (GA) and annular elastolytic giant cell granuloma (AEGC) may present with annular lesions but histology will show granulomatous inflammation with degenerating elastic fibers in the case of AEGC and central necrobiotic collagen and mucin in the case of GA (Calonje: McKee’s Pathology of the Skin, 5th Edition, 2019).

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Reference: Erythema annulare centrifugum
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