UPMC Dermatopathology "Case of the Month" Presentations
UPP - Department of Dermatology, Dermatopathology Unit
Pooja Khera, MD; Joseph English, MD; Larisa Geskin, MD; Drazen Jukic, MD, PhD
OCTOBER 2007 CASE OF THE MONTH
CLINICAL FINDINGS
A 27-year-old Caucasian female was admitted to Presbyterian Hospital in May 2007 for a fever and a left groin mass. A CT scan revealed a soft tissue swelling suspicious for an abscess complicated by left hip osteomyelitis. An incision and drainage of the abscess was performed, and the patient was treated with IV vancomycin. One month later, she was re-admitted to the hospital with fever, fatigue, a twenty-pound unintentional weight loss, an ulcer, and excruciating pain of the left hip and back.
On physical examination, the patient was febrile (T=38.3° C). She was in great distress secondary to the pain of the left hip and back. Examination of the left upper thigh revealed a 24-cm plaque composed of coalescing, multicentric, irregular ulcerations with a surrounding indurated erythematous border. Numerous pink nodules and bullae were adjacent to the plaque (Figure I).
Labs on admission were: WBC 27.9 (74% neutrophils, 20% lymphocytes), ESR 80, Bun/Cr/LFTs WNL
Dermatology was consulted on the day of admission. The clinical differential diagnosis included: pyoderma gangrenosum, atypical Sweet’s syndrome, vasculitis, infection, and malignancy (primary versus metastatic). Wound cultures and punch biopsies were taken from the edge of the lesion. The wound cultures for bacteria, fungi, HSV/VZV remained negative throughout the hospital admission.
Histopathology: A punch biopsy revealed a diffuse infiltrate with sheets of large pleomorphic, hyperchromatic CD30+ and Ki67+ lymphoid cells throughout the dermis. The cells were positive for EMA, CD4, and CD43 and were negative for ALK, CD57, and CD8. There were also changes of secondary vasculitis. Histochemical stains were negative for microorganisms. The histological differential diagnosis was ALK negative anaplastic large cell lymphoma versus lymphomatoid papulosis, type C. (Figures II, III, IV, V)
As part of the staging workup, a CT scan was performed which revealed massive retroperitoneal lymphadenopathy. There were lytic lesions of the clavicle, sternum, thoracic and lumbar vertebrae, and hip. In the pelvis, there was a poorly defined 6 cm soft tissue mass (Figure VI). The soft tissue mass and iliac bone were biopsied and were positive for CD30+, ALK negative cells consistent with anaplastic large cell lymphoma. Flow cytometry on peripheral blood showed no involvement. The patient was diagnosed with systemic anaplastic large cell lymphoma and was treated with CHOP. Unfortunately, the patient expired one month after the diagnosis from neutropenic sepsis.
.
GO TO FIGURE AND IMAGE REVIEW PAGE