—  SPECIALTY CONFERENCE  — Dermatopathology Sunday, March 8, 2009, 7:30 PM Convention Center 302 Skin Disorders with Involvement of the Dermal - Epidermal Interface Moderator: STEVEN R. TAHAN Beth Israel Deaconess Medical Center, Boston, MA Disclosure: In accordance with ACCME guidelines regarding disclosure, the USCAP policy requires that faculty members who have a significant financial or other relationship with a commercial company, entity, or service (which will be discussed in this Symposium) must disclose this to attendees. The Academy also requires that speakers disclose any products that are not labeled for the use under discussion. The speakers listed below have indicated they have nothing to disclose. Panelists: MARSHALL E. KADIN, Roger Williams Medical Center, Providence, RI CYNTHIA M. MAGRO, Weill Cornell Medical School, New York, NY GEORGE F. MURPHY, Brigham and Women's Hospital and Harvard Medical School, Boston, MA STEVEN R. TAHAN, Beth Israel Deaconess Medical Center and Harvard Medical School, Boston, MA Clinical histories are displayed below. For the fastest viewing of virtual slides, click: under each thumbnail image below. You must have Aperio ImageScope installed on your PC. If you do not already have Aperio ImageScope, Windows users with administrator privileges may download and install a free version in order to view USCAP Virtual Slides. Click the icon on the right to get your free copy:   Or, click on slide thumbnail images to view each slide in a Web-based slide viewer, which is somewhat slower. If you have any difficulties viewing these slides, email or call George Clay at +1.724.449.1137. Case 1a Submitted by: Cynthia M. Magro - Weill Cornell Medical School, New York, NY Clinical Summary: CC in ER: Rash for two weeks all over body HPI: 43yo Asian male with painful rash of skin, mouth, eyes, and urethral meatus with sloughing Rash began on his face, neck and arms about 2 weeks previously as small circular spots resulting in an initial visit to his PMD; placed on topical steroids and due to worsening returned started on prednisone 60mg PO qd and azithromycin however over the ensuing days he worsened and was sent to the NY hospital ER. In ER, patient reported pain with eating. Overall he felt his skin was burning. No GI pain or pain with urination. PMH: none SH: furniture maker Meds: prednisone 60mg PO qd for three days, claritin, xyzal, temovate lotion bid, and azithromycin all started 1 week after rash began. Patient, Patient’s daughter, niece, co-worker, and PMD reported no other medications including OTC pain relievers, herbal/homeopathic teas etc were used before or after rash began. Interestingly his coworker developed a similar rash concurrent with his(please see pictures) Labs: CBC 11.8/18.1/198 Initial LFTs ALT 106, LDH 304 ESR 5, CRP 0.16 PE: Head, face, neck, chest, back, abdomen, arms, legs, and groin with Numerous erythematous targetoid plaques (arms, chest, back) most with dusky centers and some with central bullae, confluent erythematous patches and plaques with overlying denuded skin or widespread bullae/sloughing. Conjuctival erythema. Hemorrhagic crusting of lips. Erythematous papules on lower legs/ankles. Denuded/sloughing BSA >10<30%. +Nikolsky sign Case 1a - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 1a - Figure 1a Case 1a - Figure 1b Case 1a - Figure 2a Case 1a - Figure 2b Case 1a - Figure 3a Case 1a - Figure 3b Case 1a - Figure 4 Case 1a - Figure 5a Case 1a - Figure 5b Case 1a - Figure 6a Case 1a - Figure 6b Case 1a - Figure 7a CD8 Case 1a - Figure 7b Granzyme Case 1b Submitted by: Cynthia M. Magro - Weill Cornell Medical School, New York, NY Clinical Summary: 22 month old female admitted to NY Hospital with a brief illness characterized by fever, malaise. Peripheral blood smear (PBS) assessment on October 11 was highly suggestive of acute leukemia with a WBC of 56000, hemoglobin of 11 and platelet count of 156000. In addition the PBS showed many anaplastic T cells highly suggestive of leukemic form of T cell dyscrasia. Subsequently the patient developed an erythematous eruption on her thighs clinically suspicious for leukemia cutis. Case 1b - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 1b - Figure 1a Case 1b - Figure 1b Case 1b - Figure 2a Case 1b - Figure 2b Case 1b - Figure 3a Case 1b - Figure 3b Case 1b - Figure 4a CD3 Case 1b - Figure 4b CD3 Case 1b - Figure 5a Case 1b - Figure 5b Case 1b - Figure 6 Case 1b - Figure 7a Case 1b - Figure 7b Case 1b - Figure 8 Case 1b - Figure 9 CXCR3 Case 1b - Figure 10 CD3 Case 1b - Figure 11 CD8 Case 1b - Figure 12 Granzyme Case 1c Submitted by: Cynthia M. Magro - Weill Cornell Medical School, New York, NY Clinical Summary: The patient was a 31 yo male who initially presented to NYH in May 2004 with fevers, rash, neutropenia, malaise, and lymphadenopathy.  At that point he had both discoid and malar rashes.  A full work-up ensued with biopsy of lymph nodes, bone marrow, and skin.  Serologic studies included a positive ANA at 1:80, +smith, +RNP, +SSA; dsDNA was negative and APL was negative; HHV6 IgG and IgM titers were positive.  Skin, lymph node and bone marrow biopsies were done The patient that he missed his plaquenil he would have terrible arthralgias. The patient was well until Feb 2007 when he presented again to the ER with a 3 to 4 week history of productive cough, mild dyspnea, 15 lb weight loss, increased rashes, oral and nasal ulcers. He had fevers for one week up to 102 PE showed oral and nasal ulcers, malar erythema, DLE of face, scalp, ears, chest, back , arms and legs,and lymphadenopathy. Cardiopulmonary exam was normal Responded to antibiotics, prednisone, increased doses of plaquenil Case 1c - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 1c - Figure 1a Case 1c - Figure 2 Case 1c - Figure 3a Case 1c - Figure 3b Case 1c - Figure 4a Case 1c - Figure 4b Case 1c - Figure 5 Case 1c - Figure 6a Case 1c - Figure 6b Case 2 Submitted by: George F. Murphy - Brigham and Women's Hospital and Harvard Medical School, Boston, MA Clinical Summary: A 28 year-old male who had recently undergone induction therapy for acute myelocytic leukemia developed malaise and a vague maculopapular exanthem seven days after allogeneic hematopoietic stem cell transplantation. The rash was described as mildly pruritic. He had a history of atopic disease and had once developed a similar rash after taking an antibiotic. There was no clinical evidence of hepatic or gastrointestinal dysfunction. Because of the clinical circumstances that resulted in a broad differential, a biopsy was performed. Case 2 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 2 - Figure 1 Case 2 - Figure 2 Case 2 - Figure 3 Case 2 - Figure 4 Case 3 Submitted by: Marshall E. Kadin - Roger Williams Medical Center, Providence, RI Clinical History: 64 year old Caucasian woman with 4 year history of pruritic/burning skin lesions on breasts and trunk. Repeated skin biopsies revealed chronic dermatitis. Treatment with medium-strength topical steroids and anti-histamines gave only temporary improvement. Physical exam revealed atrophic red-brown scaly patches with telangiectasia. There was minimal enlargement of axillary and inguinal lymph nodes. Biopsy of an abdominal lesion was performed. Case 3 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 3 - Figure 1 Case 3 - Figure 2 Case 3 - Figure 3 Case 3 - Figure 4 Case 3 - Figure 5 Case 3 - Figure 6 Case 3 - Figure 7 Case 3 - Figure 8 Case 3 - Figure 9 Case 3 - Figure 10 Case 4 Submitted by: Steven R. Tahan - Beth Israel Deaconess Medical Center, Boston, MA Clinical Summary: 22 year old male with a several month history of pruritic erythematous scaly plaques initially on the posterior and sides neck and spreading to the anterior neck, upper chest, and later face, upper trunk, and arms. Lesions were well demarcated and sharply cut-off at lines of clothing. Eruption worsened in summer. He was ot taking drugs, but reported a vague history of endogenous eczema. The eruption persisted for years. Case 4 - Slide 1 Click to view with ImageScope Click to view with a Web-Based Viewer Case 4 - Figure 1 Case 4 - Figure 2 Case 4 - Figure 3 Case 4 - Figure 4 Case 4 - Figure 5 Case 4 - Figure 6 Case 4 - Figure 7 Case 4 - Figure 8 Case 4 - Figure 9a CD4 Case 4 - Figure 9b CD8 Case 4 - Figure 9c CD5 Case 4 - Figure 9d CD7 If there are glass slides for a conference, they will be available for study in the microscope room in the Convention Center Room 313 for participants who wish to review them prior to the evening session. Handouts for all Specialty Conferences will be accessible via the "Educational Materials" section on the homepage the morning after each respective conference. Printed copies of the handout will not be available at the meeting. A booklet containing an agenda for each Specialty Conference symposia and a list of speakers is provided at the meeting.