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Case of the Week #71
Clinical history
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 images: image #1-low
power; #2-low power; #3-high
power; #4-high
power
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?
(scroll
down to continue)
Diagnosis:
Consistent
with erythema gyratum repens
Discussion
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.
References: eMedicine
Nat Pernick, M.D., President
PathologyOutlines.com, Inc.
Bingham Farms,
Telephone: 248/646-0325
Fax: 248/646-1736
Email: NPernick@PathologyOutlines.com