Retroperitoneal round‐cell liposarcoma associated with paraneoplastic pemphigus presenting as lichen planus pemphigoides‐like eruption
Authors: Krunic Aleksandar L.J. , Kokai Djerdj , Bacetic Branko , Kesic Vesna , Nikolic Milos M. , Petkovic Spasoje , Clark Robert E. 
Published: August 2010

A 59‐year old Caucasian woman refugee from Bosnia had been in good health until a year before admission, when she developed painful erosions and ulcerated lesions in the mouth, anogenital area, hands, and feet. Treatment with topical and systemic antibiotics, acyclovir, and antifungal creams was ineffective. Three months before admission, her skin and mucosal lesions had worsened, resulting in the limitation of walking and her daily activities. She had also experienced significant weight loss.

On admission to the Department of Dermatology, University of Belgrade, the patient was noted to be thin, distressed, and quite depressed. Physical examination reveaied ulceration that involved the floor of the mouth, ventral and laterai tongue, buccai mucosa, and hard and soft palate (Fig. 1). The vermiiion surfaces were fissured, eroded, and crusted with shiny vioiaceous scaiy papules dispersed across the cutaneous edge of both upper and iower lip (Fig. 1). Skin examination disciosed scattered vioiaceous scaiy lesions on the fiank, wrists, dorsa of the hands and feet, and lower legs. There were numerous erosions with livid erythema on the palms and soles, fingers and toes, with fissured naiis, partial and totai onycholysis, and pterygium formation. On the vulvar and vaginai mucosa, as well as the anal and perianal area, numerous erosions with erythema and white exudate were aiso present. On the skin of the anterior abdomen, both axillas, and right groin, large tense blisters were noticed. In the right lower quadrant of the abdomen, a firm, painful, ill‐defined mass was palpated, approximateiy 15 cm beiow the right costal margin. A pelvic examination confirmed a hard, immobile, painful, abdominai mass, with the suggested origin from the right adnexai (tubo‐ovarial) region. Abdominal and peivic sonogram, as well as CT scan revealed a 10x9 cm, right‐sided retroperitoneal tumor. Chest rentgenogram, and CTscan of the lungs were within normal limits.

Laboratory studies revealed normocytic, normochromic anemia with hypoalbuminemia. The following analyses were negative or within normal limits: antinuclear antibodies, cryogiobulins, VDRL, cultures for Candida and Herpes virus from oral and perianal mucosa.

A biopsy specimen from the orai mucosa demonstrated acantholytic blister with keratinocyte necrosis and vacuolar interface changes. A biopsy of the violaceous papules from the wrist, finger and lower legs showed hyperkeratosis, hypergranulosis, dyskeratotic keratinocytes, prominent vacuolar interface changes, lichenoid infiltrate with exocytosis of mononuclear celis and superficial, subcorneal clefting in one specimen (Fig. 2). Biopsy of the tense blister from the anterior abdomen disciosed subepidermal bulla consistent with bullous pemphigoid. Direct immunofluorescence (DIF) of perilesional skin and oral mucosa revealed intercellular binding of IgG as well as diffuse deposition of IgG and C3 along with dermoepidermal junction. Indirect immunofluorescence (IIF) studies of the patient serum revealed strong intercellular binding on human skin (titer 1:640) as well as on transitional epithelium of rodent urinary biadder (titer 1:40) (Fig. 3). Immunoprecipitation studies were not available at the time of hospitalization and were not performed.

Two weeks after the admission, the tumor was resected. Final pathoiogicai diagnosis was consistent with encapsulated round‐oeii liposarcoma. No metastatic spread was observed through the tumor capsule into the surrounding tissue.

After tumor resection, the patient was treated with prednisone (0.75–1.0 mg/kg) and azathioprine (100 mg/day) with significant improvement of skin lesions in the following 2 weeks. The mucosal lesions were however still active with slow, but progressive, epitheliazation of the erosions. At the time of discharge, late in November 1993, her prednisone dose was tapered to 0.5 mg/kg, with azathioprine 50 mg/day, with almost complete healing of the oral lesions. Her general health condition was satisfactory, and no signs of metastatic diseases were evident. Since discharge, the patient has never returned to our department, and was subsequently lost to follow‐up.