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Dermatopathology: Practical & Conceptual January - March 2008
6. New Heights: Erythema annulare centrifugum (deep type), palpable migratory and arciform erythema, erythema figuratum, and lymphocytic infiltration of Jessner and Kanof: pattern or disease?
Almut Böer, M.D.
Mihaela Costache, M.D.
erythema annulare centrifugum
tumid lupus erythematosus
Based on our reassessment in historical perspective of all the conditions mentioned above, the following statements seem to be reasonable:
Lupus erythematosus tumidus
is generally accepted as a distinctive clinicopathologic variant of lupus erythematosus typified by superficial and deep lymphocytic infiltrates accompanied by deposits of mucin in the reticular dermis. Slight epidermal changes in the form of vacuolar alteration may be found sometimes, deposits of mucin in the dermis are variable, immunofluorescence is usually negative, and the clinical course is often favorable and devoid of systemic signs and symptoms.
Lymphocytic infiltration of Jessner and Kanof is
lupus erythematosus tumidus because criteria for diagnosis as they were set forth by Jessner and Kanof and later by Clark et al. are indistinguishable from those used today for diagnosis of lupus erythematosus tumidus. The authors had separately designated the condition from lupus erythematosus because, at the time of their publications, the concept of lupus erythematosus tumidus was not yet widely known and lupus erythematosus was considered to be associated invariably with epidermal changes. Today, the term lymphocytic infiltration of Jessner and Kanof should no longer be used and should be replaced entirely with the designation lupus tumidus.
3) What today is called
erythema annulare centrifugum
has long been referred to as "erythema annulare centrifugum (Darier)superficial type." It is a distinctive disease in clinicopathologic correlation characterized by a spongiotic dermatitis similar to pityriasis rosea, [
] and it is completely unrelated to the condition long referred to as "erythema annulare centrifugum (Darier)deep type." Nomenclature that distinguishes between superficial and deep type, implying that both are variants of one and the same condition, should be abandoned because it is confusing.
Erythema annulare centrifugum (Darier)deep type
has been referred to over the decades by numerous different names including "deep gyrate erythema," "deep figurate erythema," "palpable migratory and arciform erythema" (Clark),[
] "erythema figuratum" (Ackerman).[
] This disease has been conventionally regarded as a distinctive disease, but a report on a larger series of patients in clinicopathologic correlation has ever been published.
Criteria for diagnosis of this latter condition are meager; they consisting of moderately dense superficial and deep infiltrates of lymphocytes without epidermal changes and unaccompanied by deposits of mucin in the dermis. Can such an infiltrate be diagnosed with specificity?
In our practice we have observed this pattern in a broad variety of conditions:
examples of lupus erythematosus tumidus, in which deposits of mucin in the dermis were sparse or lacking
erythema migrans, in which not uncommonly plasma cells are lacking entirely in the infiltrate
herpetic infection, especially folliculotropic zoster infections early in the course of an eruption when typical changes in epithelial structures are not yet apparent
polymorphous light eruption, in which edema and spongiosis may at times be subtle only
specific infiltrates of chronic lymphocytic leukemia, in which lymphocytes were only slightly polygonal.
Unfortunately, neither deposits of mucin in the dermis nor slight vacuolar changes at the dermoepidermal junction permit a diagnosis of lupus erythematosus tumidus with surety. Vacuolar changes can be seen in erythema migrans as well as in herpetic infections.[
] Deposits of mucin can be observed in erythema migrans as well as in specific infiltrates of chronic lymphocytic leukemia.[
] Plasma cells are often absent in erythema migrans but they may be present in some cases of lupus tumidus.[
In the cases studied by us, specific diagnosis was possible because another "outside measure" apart from morphology was available: elevated antinuclear antibodies in the case of lupus tumidus; positive polymerase chain reaction for borreliosis in the cases of erythema migrans and for varicella zoster viruses in the papular manifestation of herpetic infection; response of the condition with UV hardening therapy in polymorphous light eruption; and previous diagnosis of lymphocytic leukemia in the bone marrow in the case of leukemic infiltration of the skin.
In sum, what has been described as erythema annulare centrifugum (Darier)deep type, deep gyrate erythema, deep figurate erythema, palpable migratory and arciform erythema, and erythema figuratum is nothing more than a pattern that may be formed by infiltrates of various conditions, all of which may sometimes present clinically with annular, arcuate, gyrate, or figurate lesions and at other times not. Therefore, this is not a distinctive clinicopathologic entity. In any patient who presents with annular or figurate macules, papules, or plaques that show superficial and deep lymphocytic infiltrates, additional examinations and investigations need to be performed in order to diagnose lupus tumidus, erythema migrans of borreliosis, herpetic infection, polymorphous light eruption, or even leukemia cutis, with specificity. They include clinicopathologic correlation, serology for antinuclear antibodies, PCR for herpes viruses and/or borrelia on paraffin embedded biopsy specimens, and lymphocyte clonality investigations.
The terms deep gyrate erythema, deep figurate erythema, palpable migratory and arciform erythema, and erythema figuratum should no longer be used because they do not convey a specific diagnosis. When a specific diagnosis cannot be made because findings in a biopsy specimen consist of lymphocytic infiltrates superficial and deep without any further significant finding, that should be stated frankly with a set of differential diagnoses given to enable the referring clinician to manage the patient properly. The condition should not be designated to be "deep figurate erythema," which implies specificity, when in truth it is not a distinctive disease entity but just a pattern seen in a variety of conditions.
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