Abstracts of ‘‘Duel in Dermatopathology’’papers presented at the 41st AnnualMeeting of The American Societyof Dermatopathology

Westin Copley PlaceBoston, Massachusetts, USAOctober 14–17, 2004

The ‘‘Duel in Dermatopathology’’ is a resident abstract competition sponsored by The AmericanSociety of Dermatopathology. These resident abstracts were presented in oral format at the 41stAnnual Meeting of The American Society of Dermatopathology on October 14, 2004. They are listedon the following pages in alphabetical order by the first author’s last name.

J Cutan Pathol 2005: 32: 67–70 Copyright # Blackwell Munksgaard 2005Blackwell Munksgaard. Printed in Denmark

Journal of

Cutaneous Pathology

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EPSTEIN BARR VIRUS (EBV)-ASSOCIATED DIFFUSE

LARGE B-CELL LYMPHOMA (BCL) WITH ISOLATED

SKIN INVOLVEMENT ASSOCIATED WITH COMBINED

IMMUNODEFICIENCY AND MULTIPLE

OPPORTUNISTIC INFECTIONSN. Burkemper and A. BridgesDepartment of Dermatology, Mayo Clinic, Rochester, MN,USA

A 72-year-old man presented with nausea, vomiting, cough, dyspnea,

weight loss and an ulcerated nodule on the right calf of four month’s

duration. CT revealed multiple bilateral pulmonary nodules. Skin

biopsy demonstrated an angiodestructive infiltrate with neutrophils,

CD4þ T-lymphocytes, CD20þ large, atypical lymphocytes and his-

tiocytes in the reticular dermis and subcutis. The atypical lymphocytes

were positive for EBV by ISH. PCR demonstrated a clonal immuno-

globulin gene rearrangement. Lung biopsy showed histoplasmosis

without evidence of a lymphoproliferative disorder or EBV infection.

Further investigation revealed a low CD8 count, IgM deficiency and

candidal esophagitis. Treatment consisted of itraconazole and ritux-

imab. The cutaneous findings in this patient resemble lymphomatoid

granulomatosis (LyG), an angiocentric and angiodestructive lympho-

proliferative disorder involving extra-nodal sites. While LyG can

involve multiple organs, it primarily affects the lungs. The infiltrate

consists of large, atypical EBV-positive B-cells admixed with reactive

T-cells. Although our patient’s skin pathology resembles LyG, we

favor the broader diagnosis of EBV-associated diffuse large BCL

since he had an isolated skin lesion without evidence of lung or

other systemic involvement. We will compare the clinical and

histologic findings in our patient to cases of LyG from our institution

to help classify this entity among other cutaneous lymphoproliferative

disorders.

PRIMARY CUTANEOUS PARAGANGLIOMA (PCP) OF

THE SCALPSarka Cernosek, Ramin Ram, Payam Saadat and Manju VadmalDepartment of Pathology and Dermatology, University ofSouthern California, Los Angeles, CA, USA

We report the first case of PCP in a 12-year-old boy who presented

with a growing, bleeding nodule on his scalp. On physical examina-

tion a 1.5� 1.5� 1.0-cm firm, red nodule on the right temporal scalp

was observed. Histological examination revealed a dermal neoplasm

composed of nests/lobules of uniform, polygonal cells separated by

thin fibrous trabeculae. The cells had distinct cell borders, round

nuclei with evenly dispersed chromatin and abundant fine, eosino-

philic cytoplasm. No mitoses or necrosis were noted. The cells showed

a strong positive immunoreaction for vimentin, NSE, chromogranin,

synaptophysin, keratin AE3; and a negative immunoreaction for

HMB45, melan-A, muscle markers, AE1, CK20 and EMA. S-100

protein stained sustentacular cells. Ultrastructural examination of the

paraffin embedded tissue revealed electron dense neuroendocrine

granules. A complete workup and skin examination for possible

neoplasms elsewhere in the body was negative. Follow-up of the

patient, 6 months and 18 months post-excision revealed no recur-

rence. In conclusion, we report the first case of PCP documented

by histological, immunohistochemical and ultrastructural findings.

The tumor described likely represents an aberrant migration of

neural crest cells in their decent towards the branchial arches. It

also highlights the complex interaction of the neural crest and bran-

chial arches during embryogenesis.

DIFFUSE DERMAL ANGIOMATOSISB. Draper and A. BoydDepartment of Medicine (Dermatology), Vanderbilt University,Nashville, TN, USA

A 53-year-old Caucasian male with peripheral vascular atherosclero-

sis presented with a 6-week history of violaceous, tender, plaques with

focal ulcerations of the mid left lower extremity. A biopsy specimen of

the left leg plaque demonstrated a diffuse proliferation of benign

endothelial cells in the papillary and reticular dermis with some

forming small vascular spaces, occasional extravasating erythrocytes

and no evidence of cholesterol emboli or vasculitis. There were no

atypical endothelial mitoses or cytologic atypia. Immunohistochem-

ical staining using endothelium-specific anti-CD31 antibody was

positive. The clinical and pathological findings were consistent with

diffuse dermal angiomatosis. The patient had a diagnostic angiogram

that revealed a 1.5-cm, focal, 80% stenosis of the midsegment of the

left external iliac artery. He underwent left external iliac artery stent-

ing with an Omnilink 8 mm by 28 mm balloon expandable stent.

Poststent films showed resolution of the stenosis. Within 10 weeks of

the revascularization procedure, he had complete resolution of his

cutaneous manifestations. Diffuse dermal angiomatosis is a rare man-

ifestation of a common co-morbid condition in our ageing population.

Early intervention to correct the ischemia of peripheral vascular disease

results in rapid resolution of this unique clinico-pathologic entity.

PIGMENTED OLFACTORY NEUROBLASTOMA,

A MIMIC OF MELANOMAAmy Han1, Anita C. Gilliam1, Gretta Jacobs2, Rosana Eisenberg2,1Departments of Dermatology and2Pathology, Case Western University, Cleveland, OH, USA

A 23 year-old man presented with a mass in the nasal cavity, initially

diagnosed as malignant melanoma. The diagnosis was based on the

presence of tumor cells arranged in nests of varying sizes with some

cells containing brown pigment and scattered pigmented macro-

phages. Immunostaining revealed focal positivity for S100 and vimen-

tin and a negative HMB-45 stain. The patient was referred to our

institution for further evaluation. Upon reviewing the case, a fibrillary

background within some nests of tumor cells was noted and additional

work-up was initiated. Immunohistochemistry demonstrated positivity

for CD56, synaptophysin, and calretinin. Electron microscopy showed

dendritic processes containing neurosecretory granules and no melano-

somes or premelanosomes in the tumor cells. Given the findings,

a diagnosis of pigmented olfactory neuroblastoma was rendered.

The differential diagnosis of nasopharyngeal neoplasms includes olfac-

tory neuroblastoma and malignant melanoma. The usual problem is in

distinguishing amelanotic melanomas from olfactory neuroblastomas.

The difficulty in our case resided in the fact that the olfactory neuro-

blastoma contained pigment. In either case, close attention to histo-

logic features, use of immunohistochemistry, and in selected cases,

electron microscopy, can lead to the correct diagnosis.

MARGINAL ZONE LYMPHOMA WITH A DUAL

CUTANEOUS AND LEUKEMIC PRESENTATION AND

AN ABERRANT MYELOID-MONOCYTIC PHENOTYPEC. Keehn and H. CualingDepartment of Pathology, University of South Florida College ofMedicine and H. Lee Moffitt Cancer Center & ResearchInstitute, Tampa, FL, USA

A 69-year-old man with heart failure was hospitalized with worsening

dyspnea. At the time of admission he also complained of a ‘‘rash’’ of

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three weeks duration over the left neck. Multiple erythematous pla-

ques were noted. There was no lymphadenopathy or hepatospleno-

megaly. The white blood cell count was elevated at 37,700/uL, with

70% composed of atypical monocytoid lymphocytes with abundant

pale-staining cytoplasm. Skin biopsy showed a superficial and deep

nodular dermal lymphoid infiltrate. Bone marrow aspirate and biopsy

showed nodules of similar cells. Flow cytometry identified a mono-

clonal B cell population with light chain restriction, with positivity for

CD19, CD20, HLA-DR, CD11c, FMC7, CD71, and the myeloid-

monocytic marker CD13. There was no expression of CD5 or CD10.

B cell receptor gene rearrangement was identified. A diagnosis of

extranodal marginal zone lymphoma was made. He was treated for

congestive heart failure and discharged. The patient has had an

indolent course without chemotherapy, with waxing and waning of

the skin lesions. This case is unusual, as leukemic involvement is rare

in marginal zone lymphoma and is typically only seen in patients with

severe widespread disease with an aggressive clinical course. This is

also the first reported case with aberrant expression of myeloid-

monocytic markers.

SECONDARY SYPHILIS PRESENTING AS CUTANEOUS

T-CELL LYMPHOMA IN AN HIV-POSITIVE PATIENTA. Laungani, J. McDonnell, W. Bergfeld and K. SellheyerDepartment of Dermatology, The Cleveland Clinic Foundation,Cleveland, OH, USA

We present the case of an HIV-positive 38-year-old Caucasian male

with a history of fevers, chills, and disseminated nonpruritic erythe-

matous papules that began on his abdomen. An initial skin biopsy

revealed a lymphohistiocytic infiltrate at the dermoepidermal junc-

tion, exocytosis of atypical lymphocytes, and minimal spongiosis.

Immunohistochemistry showed increased CD8-positive cells but

only scattered CD30-positive cells. PCR analysis demonstrated

T-cell receptor gamma gene rearrangement. RPR, blood and tissue

cultures were all negative. The patient was thought to have a cytotoxic

cutaneous T-cell lymphoma. Over time, the lesions progressed to the

palms and soles and the patient remained febrile. Repeat biopsy

demonstrated a lichenoid interface dermatitis with a superficial and

deep perivascular, interstitial, and periadnexal lymphohistiocytic infil-

trate and the formation of epithelioid granulomas throughout the

dermis. The epidermis showed blurring of the dermoepidermal junc-

tion, spongiosis, and exocytosis of lymphocytes and neutrophils. Few

spirochetes were demonstrated by Steiner stain. Repeat RPR and

FTA-Abs serologies were positive. The patient was diagnosed with

late secondary syphilis and was successfully treated with benzathine

penicillin. This case demonstrates that atypical lymphoid infiltrates

can simulate mycosis fungoides in an HIV-positive patient with sec-

ondary syphilis and also reiterates that syphilis is a great mimicker of

other entities.

PRIMARY CUTANEOUS IMMUNOCYTOMA

PRESENTING WITH DIFFUSE SUBCLINICAL

INVOLVEMENT AND DEMONSTRATING KAPPA AND

LAMBDA LIGHT CHAIN RESTRICTIONSC. Lorenzo and D. BrenemanDepartment of Dermatology, University of Cincinnati MedicalCenter, Cincinnati, OH, USA

A 37 year-old man with primary cutaneous immunocytoma with

lambda light chain restriction involving the left shoulder was treated

with Rituxan with clinical resolution. Four years later, he presented

with a six-month history of diffuse asymptomatic erythema. Physical

examination revealed three grouped papules on the right upper arm

and an irregular sclerotic patch with slight erythema superiorly on the

left shoulder at the site of the initial tumor. There was no diffuse

erythema. Biopsies were obtained from a papule on the right upper

arm, the sclerotic patch on the left shoulder, and clinically uninvolved

skin on the right anterior thigh. All three specimens showed a variably

dense, predominantly periadnexal and perivascular dermal infiltrate

of plasma cells and lymphocytes. The specimen from the right arm

demonstrated kappa light chain restriction. The specimen from the

right thigh showed lambda light chain restriction. Physical examina-

tion six weeks later demonstrated mottled erythema on the anterior

thighs. Two biopsies were obtained from the right thigh. One showed

immunocytoma. The other was unremarkable. The patient’s primary

cutaneous immunocytoma demonstrated two unusual findings: (1)

histologic presence of tumor in clinically uninvolved and minimally

involved skin; and (2) the presence of two distinct monoclonal popu-

lations.

NK/T-CELL LYMPHOMA PRESENTING AS

SUBCUTANEOUS NODULES IN A PREVIOUSLY

HEALTHY YOUNG WOMAN: AN UNUSUAL

GRANULOMATOUS COMPONENT FOUND ON

BIOPSYA. Rubin and S. HusainDepartment of Dermatology, Columbia University College ofPhysicians and Surgeons, New York, NY, USA

A 27-year-old previously healthy woman presented with an acute

onset of multiple tender subcutaneous nodules and nasal congestion.

Biopsy of an arm nodule revealed an atypical, dense, diffuse cellular

infiltrate, predominantly involving the deep dermis and the subcuta-

neous fat lobules. The infiltrate consisted of a spectrum of small,

medium to large sized lymphocytes with the large lymphocytes pre-

dominating. The lymphoid cells showed irregular nuclear contours,

fine chromatin, and indistinct nucleoli. Mitoses were frequent.

A significant granulomatous component was present. In foci the

infiltrate revealed an angiotropic and angiodestructive pattern.

Large areas of tumor cell necrosis were noted. The subcutaneous

infiltration was reminiscent of a lobular panniculitis, with areas

resembling subcutaneous panniculitis-like T-cell lymphoma. Special

stains for infectious organisms were negative. The neoplastic cells

were CD2þ, CD7þ, CD3-, CD5-, CD8þ, CD56þ, CD52þ, TIA-

1þ, perforinþ, granzyme-Bþ, and LMP-1-. The infiltrate was

EBVþ. Flow cytometry showed 93% of cells to be NK cells. Workup

revealed many nodules in the abdominal wall, lungs, and liver as well

as sinusitis. The patient failed CHOP and ICE chemotherapy. The

nodules resolved after three cycles of L-aspariginase, vincristine, and

dexamethasone. NK/T-cell lymphoma usually affects middle-aged

and older adults. This case contains an unusual granulomatous com-

ponent on biopsy.

A CASE OF KAPOSIFORM

HEMANGIOENDOTHELIOMA AND KASABACH-

MERRITT PHENOMENONK. Shackelford, J. Tamburro and S.D. BillingsDepartments of Dermatology and Pathology, Indiana UniversitySchool of Medicine, Indianapolis, IN, USA

Kaposiform hemangioendothelioma (KH) is a vascular tumor of

intermediate malignancy that occurs in childhood, of which 50%

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are present at birth. The tumor has a predilection for the trunk,

extremities, and retroperitoneum. It is commonly associated with

Kasabach-Merritt phenomenon, a consumptive coagulopathy char-

acterized by profound thrombocytopenia and hypofibrinogenemia.

The histologic differential diagnosis of kaposiform hemangioendothe-

lioma includes infantile hemangioma, tufted angioma, Kaposi’s sar-

coma, and spindle cell hemangioendothelioma. We present a case of

a neonate with a soft tissue mass encompassing the proximal two-

thirds of the right thigh. After delivery by Ceasarean section for

failure to progress, the patient developed repiratory failure, consump-

tive coagulopathy and intracranial hemorrhage. The initial clinical

differential diagnosis included KH, infantile hemangioma, and infan-

tile fibrosarcoma, all entities which have been associated with Kasa-

bach-Merritt phenomenon. An biopsy demonstrated a nodular

vascular proliferation composed of grouped, spindled endothelial

cells forming irregular, slit-like vascular lumens. There were asso-

ciated fragmented red blood cells and hemosiderin deposits. The

tumor cells were focally positive for D2–40, an antigen specific for

lymphatic endothelium and expressed by KH, and were negative for

GLUT-1, an antigen expressed in infantile hemangiomas but not

KH. The histologic and immunohistochemical findings confirmed

the diagnosis of KH.

NECROLYTIC ACRAL ERYTHEMAM. Sharp1, M. Abdallah2, C. Hull1 and T.D. Horn1

1Department of Dermatology, University of Arkansas for MedicalSciences, Little Rock, AR, USA,2Department of Dermatology and Venereology, Ain ShamsUniversity, Cairo, Egypt

A 46-year-old hepatitis C-positive African American woman pre-

sented with a several month history of worsening, well-marginated,

erythematous, dusky plaques on the dorsum of her feet and ankles.

After an initial skin biopsy showed spongiotic dermatitis, she was

treated with emollients, high potency topical steroids, and kerato-

lytics, all of which were of no benefit. A punch biopsy from the

dorsum of her foot showed acanthosis, individual keratinocyte

necrosis, confluent upper epidermal necrosis, and a superficial

and deep perivascular lymphocytic infiltrate. Given the patient’s

positive hepatitis C status and clinicopathological correlation, a

diagnosis of necrolytic acral erythema was rendered. Empiric

therapy with oral zinc sulfate was initiated despite a normal plasma

zinc level, and the skin lesions resolved with post-inflammatory

hyperpigmentation. Necrolytic acral erythema, considered one of

the necrolytic erythemas, is a cutaneous manifestation of hepatitis

C virus infection. A total of nine cases have been reported in

the literature, seven from Egypt and two from the United States.

All reported patients have been hepatitis C positive. This case

represents the third reported patient from the US with necrolytic

acral erythema.

RELAX, ITS ONLY A BLAP!K. Wagamon and C. JaworskyDepartment of Dermatology, University Hospitals of Cleveland/Metro Health Medical Center

A 67 year old female with past history of breast cancer treated with

lumpectomy and post surgical radiation presented for evaluation of

new papules over the breast. She was sent by her oncologist to rule

out breast cancer metastases to the skin. Punch biopsy of one of the

papules showed dilated vascular spaces located in the papillary dermis

lined with plump endothelial cells with hyperchromatic nuclei and a

hobnail like appearance. The lumen of the vascular spaces was filled

with pink amorphous eosinophilic material and mitoses were not

present. The stroma contained a moderately dense lymphohistiocytic

infiltrate with a variable number of plasma cells. Given these features

a diagnosis of benign lymphangiomatous papules of the skin (BLAP)

was made. BLAPs are well reported in both the general pathology

and surgical literature but have yet to be publicized in the dermato-

pathological literature. It is important that dermatopathologists be

aware of and able to differentiate this condition from other vascular

lesions, most notably well differentiated angiosarcomas.

VIRUS-ASSOCIATED TRICHODYSPLASIA SPINULOSAAJ Wyatt1, D Sachs2, D Began1, J Shia3, R Delgado3 andKJ Busam3

1Department of Dermatology, Weill Medical College of CornellUniversity,2Dermatology Service, and3Department of Pathology, Memorial Sloan-Kettering CancerCenter, New York, NY, USA

Background: Virus-associated trichodysplasia spinulosa (VATD) is a

relatively new entity associated with a distinctive histologic picture of

distended follicles with excessive production of inner root sheath

material. Only two patients have thus far been reported. Both had

developed the condition after a kidney transplant. Observation: We

report two additional cases of VATD. One is a nineteen year-old man

with a history of acute lymphocytic leukemia, who developed a

papular facial eruption as well as madarosis. The other is a nine

year-old boy, who presented with facial papules after a kidney trans-

plant. Histologic examination of both cases reveals characteristic

findings for trichodysplasia spinulosa, but also indicates a spectrum

of histologic alterations. Ultrastructural studies confirm the presence

of a variable number of intranuclear viral particles. Conclusions: Our

cases illustrate the histologic spectrum seen in association with

VATD. The severity of histologic alterations corresponds to the

viral load seen by electron microscopy. This report documents for

the first time that VATD can be found in patients without a solid

organ transplant. Increased awareness of the distinct histologic pic-

ture associated with VATD will likely lead to more frequent diagnosis

of this under-recognized entity.

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