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Case of the Week #71
A 64 year old woman had pruritic, erythematous plaques on her abdomen and back that appeared and disappeared randomly. She also had a long history of smoking. The clinical concerns were mycosis fungoides or morphea (localized scleroderma). An ellipse of skin on the abdominal wall was excised.
Micro description: The skin biopsy shows epidermis, dermis and subcutis. The epidermis shows focal acanthosis, parakeratosis and spongiosis. There are no blisters or intraepithelial microabscesses, and exocytosis (inflammatory cells in the epidermis) is minimal. The dermis shows a patchy and perivascular infiltrate which consists of small, round, mature appearing lymphocytes, as well as some histiocytes and eosinophils.
What is your diagnosis?
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Consistent with erythema gyratum repens
This case illustrates a problematic area for many pathologists - interpreting an inflammatory dermatosis. In these cases, input from the dermatologist is crucial in providing the correct diagnosis.
Erythema gyratum repens is very rare. The diagnosis is important because it is associated with existing or subsequent internal malignancy in 80% of cases (Am J Med Sci 2001;321:302). It typically causes concentric and parallel bands of erythema and scale (“wood grain pattern”) on the trunk and extremities (gross image #1; #2; #3). The rash migrates at the rate of 1 cm per day and is very pruritic. It is associated with ichthyosis (16%) and hyperkeratosis (10%).
The histology is non-specific, with mild acanthosis and spongiosis and focal parakeratosis. The dermis shows a superficial perivascular infiltrate of lymphocytes and histiocytes, with variable eosinophils and melanophages. There may be exocytosis of neutrophils and eosinophils.
The differential diagnosis include erythema annulare centrifugum. This is a hypersensitivity reaction that also grows over a period of weeks, is mildly pruritic and affects the trunk and proximal extremities. Most cases resolve within six weeks. Histologically, there may be a superficial perivascular infiltrate, but it shows tight cuffing of the inflammatory cells around swollen endothelial cells, and focal extravasation of red blood cells into the papillary dermis. The deep variant shows a “coat-sleeve-like” pattern of perivascular infiltrate in the middle to deep dermis.
The cause of erythema gyratum repens is unknown, but may have an immunological basis due to the occasional presence of C3, C4, and IgG at the basement membrane zone with direct immunofluorescence. It may also be considered a paraneoplastic syndrome. Treatment of the underlying malignancy may cause remission.
Nat Pernick, M.D., President