|
Dermatopathology
The Dermatolopathologic Manifestations
of Systemic Disease:
Novel manifestations of local and systemic microbial
infection and neoplasia in the skin
Thursday, March 11, 2004 - 7:30 p.m.
Ballroom B
|
Moderator:
A. NEIL CROWSON
Regional Medical Laboratory and University of Oklahoma
Tulsa, OK
|
Clinical histories are printed below.
Click on the case numbers for text and references of each case.
Click on each slide thumbnail image for an enlarged view
submitted by:
SCOTT R. GRANTER
Brigham & Women's Hospital and Harvard Medical School
Boston, MA
The patient is a child who developed raised ulcerated lesions on her hand 2 weeks following a prairie
dog bite.
Case 1 - Figure 1 - The biopsy shows ballooning degeneration of the epidermis. There is a mixed inflammatory infiltrate involving the dermis and showing exocytosis. Abundant karryorhexis is present.
|
Case 1 - Figure 2 - High-magnification shows ballooning degeneration and inflammation of the epidermis. Several keratinocytes show intracytoplasmic viral inclusions that have a tendency to abut the nucleus.
|
Dr. Kurt Reed, Marshfield, Wisconsin, kindly provided this case.
submitted by:
SCOTT R. GRANTER
Brigham & Women's Hospital and Harvard Medical School
Boston, MA
The patient is a 57-year old handyman who presented one day after believing he had been bitten by an
arthropod after working in a ventilation tower. The following day he collapsed and was noted to have
a necrotic lesion on his chest in the approximate area of his alleged bite the day before. This
lesion was biopsied.
Case 2 - Figure 1 - Scanning magnification shows massive dermal edema.
|
Case 2 - Figure 2 - High-power magnification shows mixed acute and chronic inflammation, numerous extravasated red blood cells, and fibrin thrombi in small vessels.
|
This case was kindly provided by provided by Dr. PH McKee, Boston.
submitted by:
A. NEIL CROWSON
Regional Medical Laboratory and University of Oklahoma
Tulsa, OK
A 38 year old man with a 15 year history of Crohn's disease developed an 8 x 5 cm plaque on the right
buttock that was not in continuity with diseased anal or rectal mucosa. A biopsy showed a
psoriasiform pattern of epidermal hyperplasia overlying dilated dermal lymphatic spaces containing
aggregates of banal histiocytes.
Case 3 - Figure 1 - There is a plaque on the left buttock, some 8x5 cm in diameter. It is not in continuity with the anal canal.
|
Case 3 - Figure 2 - The biopsy shows psoriasiform hyperplasia overlying markedly dilated dermal lymphatic spaces.
|
Case 3 - Figure 3 - The dilated lymphatic spaces contain clusters of endovascular histiocytes.
|
Case 3 - Figure 4 - The dilated lymphatic spaces contain clusters of endovascular histiocytes.
|
This is a patient of Dr Michael Wilkerson, Tulsa OK, who kindly provided clinical photographs.
submitted by:
A. NEIL CROWSON
Regional Medical Laboratory and University of Oklahoma
Tulsa, OK
A 54 year old man presented with fatigue and weakness and was found to be seropositive for hepatitis
C. He then developed ulcerating pustular plaques up to 11 cm in diameter on the forearms and shins. A
forearm lesion was biopsied along it's pustule-studded rolled border. The biopsy showed epidermal
hyperplasia with pustulation overlying a dermis heavily infiltrated by neutrophils with lysis of
dermal collagen and a Sweet's-like vascular reaction peripheral to the zones of maximal tissue
pathergy.
Case 4 - Figure 1 - There is an ulcerative and pustular lesion of the forearm with a raised, rolled lesional edge.
|
Case 4 - Figure 2 - There is pseudoepitheliomatous hyperplasia of the epidermis in this biopsy from the lesional edge.
|
Case 4 - Figure 3 - The dermis contains neutrophilic microabscesses and there is neutrophilic dermal connective tissue lysis.
|
Case 4 - Figure 4 - A Sweet's-like vascular reaction is seen around blood vessels peripheral to the zone of maximal tissue pathergy.
|
submitted by:
CYNTHIA M. MAGRO
Ohio State University
Columbus, OH
A 56 year old male presented with progressive tightening of the lower extremities and proximal arms.
A biopsy revealed morphologic changes typical of eosinophilic fasciitis. However he continued to
develop features of progressive sclerosis including evidence of esophageal dysmotility, sclerodactyly,
and progressive dyspnea, the latter prompting an open lung biopsy. The lung biopsy showed an active
paucicellular capillaritis in concert with vascular drop out and concomitant ensuing septal
fibroplasia. Serology showed a myriad of positive connective tissue disease markers including
antibodies to Ro, La, and RNP. The patient's quantitative IgG antibodies to B19 were markedly
elevated although in the absence of discernible IgM antibodies. B19 DNA was discovered in the
patient's lung and skin biopsies.
Case 5 - Figure 1 - There is a striking sclerodermoid tissue reaction involving the dermis characterized by widened collagen bundles arranged parallel to the long axis of the epidermis.
|
Case 5 - Figure 2 - C5-C9 - There is prominent deposition of C5b-9 within the microvasculature of the skin corroborative of in vivo activation of the complement cascade sequence and hence indicative of a humorally mediated microangiopathy syndrome mediated by anti-endothelial cell antibodies. RT in situ PCR shows parvovirus B19 and TNF transcript expression in endothelia and in pervascular mononuclear cells (arrows).
|
Case 5 - Figure 3 - A lung biopsy shows homogeneous septal expansion with associated vascular drop out.
|
submitted by:
CYNTHIA M. MAGRO
Ohio State University
Columbus, OH
A 49 year old woman presented with a 2 week history of fever and vasculitic lesions on the lower
extremities. In addition she subsequently developed shortness of breath. Bronchoalveolar lavage
revealed extensive hemorrhage. Other laboratory investigations revealed a low haemoglobin along with
moderate thrombocytopenia and lymphopenia, a positive rheumatoid factor, and depressed complement.
Cytomegalovirus antibodies of both IgM and IgG isotype were positive. In addition CMV DNA was
isolated from the blood. A skin biopsy was performed and revealed a striking pandermal vascular
reaction characterized by nodular aggregates of neutrophils and mononuclear cells surrounding and
permeating the cutaneous vasculature accompanied by extensive fibrin deposition. Many of the
endothelial cells appeared proplastic, manifesting a hobnailed nuclear contour. In situ PCR studies
for CMV revealed staining of endothelial cells for CMV. A few scattered cells within the interstitium
were also positive. Although initially she was given exclusively immunsuppressive therapy which lead
to worsening of her symptoms eventually she was given a combination of gancylcovir and prednisone.
This combined therapy over the course of several weeks lead to resolution of her symptoms.
Case 6 - Figure 1 - There is a necrotizing neutrophil rich vasculitis involving the skin.
|
Case 6 - Figure 2 - An open lung biopsy shows a necrotizing capillaritis
|
Case 6 - Figure 3 - In situ hybridization studies for CMV RNA transcript expression reveals positive staining of endothelial cells.
|
submitted by:
J. ANDREW CARLSON
Albany Medical College
Albany, NY
Healthy 22yo male presented with a three day history of flu-like symptoms- fatigue, sore throat,
cough, headache, and chest congestion and a low grade fever 99°F. Cefzil (Cephalexin™) was prescribed
for presumptive sinusitis. One week later, he complained of an earache and non-pruritic, burning
rash. The rash, morbilliform with erythematous and purpuric macules and papules, began on the face
then spread to the trunk, arms, legs and feet including palms and soles. His soft and hard palate
exhibited petechiae, the tonsils were enlarged and covered by an exudate, and cervical lymphadenopathy
was present. Clinical differential diagnosis: Rocky Mountain spotted fever, erythema multiforme, vs.
infectious mononucleosis.
Case 7 - Figure 1 - There is a superficial and deep perivascular mononuclear cell infiltrate with exocytosis.
|
Case 7 - Figure 2 - There is a pronounced lymphocytic interface dermatitis that obscures the dermoepidermal juncttion.
|
Case 7 - Figure 3 - There is a pronounced lymphocytic interface dermatitis that obscures the dermoepidermal juncttion.
|
Case 7 - Figure 4 - Nuclear debris is present in the dermis.
|
Case 7 - Figure 5 - The infiltrate comprises an admixture of CD4- and CD8-expressing lymphocytes and CD68-expressing histiocytes.
|
Case 7 - Figure 6 - The infiltrate comprises an admixture of CD4- and CD8-expressing lymphocytes and CD68-expressing histiocytes.
|
submitted by:
J. ANDREW CARLSON
Albany Medical College
Albany, NY
40yo female. Asymptomatic truncal rash. Clinical impression: granuloma annulare/rheumatoid nodule.
Case 8 - Figure 1 - There are cord-like cutaneous lesions with plaques on the trunk.
|
Case 8 - Figure 2 - There is a mid- and deep-dermal perivascular and interstitial mononuclear cell infiltrate.
|
Case 8 - Figure 3 - There is a mid- and deep-dermal perivascular and interstitial mononuclear cell infiltrate.
|
Case 8 - Figure 4 - The infiltrate comprises interstitial histiocytes associated with necrosis of collagen bundles.
|
Case 8 - Figure 5 - The infiltrate comprises interstitial histiocytes associated with necrosis of collagen bundles.
|
Case 8 - Figure 6 - The infiltrate comprises interstitial histiocytes associated with necrosis of collagen bundles.
|
submitted by:
MICHAEL MORGAN
University of South Florida College of Medicine
Tampa, FL
A 67 year old man with abdominal pain and recent onset of diabetes mellitus presented with a rash
involving the face, intertriginous areas and genitals.
Case 9 - Figure 1 - There is psoriasiform hyperplasia of the epidermis with a pattern of abrupt epidermal keratinization, pallor and necrolysis.
|
Case 9 - Figure 2 - There is psoriasiform hyperplasia of the epidermis with a pattern of abrupt epidermal keratinization, pallor and necrolysis.
|
submitted by:
MICHAEL MORGAN
University of South Florida College of Medicine
Tampa, FL
A 70 year old man has a history of gradually expanding and ulcerating nodules of the neck areas.
Case 10 - Figure 1 - There are confluent nodular histiocytic infiltrates in the mid and deep reticular dermis
|
Case 10 - Figure 2 - There is an irregular palisading granulomatous process involving the deep dermis; granulomas include giant cells with Touton forms as well as foam cells. Cholesterol clefts completed the picture (not illustrated).
|
|
|
|
|
