A case of a primary cutaneous plasmacytoma presenting in adolescence

Primary cutaneous plasmacytoma is defined as monoclonal proliferation of plasma cells that arises primarily in the skin without evidence of systemic disease. We present an extremely rare case of a young adult diagnosed with solitary plasmacytoma. A 20-year-old woman presented with a pruritic erythematosquamous indurated plaque on the inner aspect of her right thigh. She had undergone a biopsy 5 years ago, and under the diagnosis of Nekam disease, she was treated with topical steroids followed by intralesional injections of triamcinolone acetonide. A new skin biopsy revealed infiltration of the epidermis by small T lymphocytes while plasma cell accumulations were found in the dermis. Immunostains for light and heavy chains [kappa, lambda, immunoglobulin (Ig) G, IgA, and IgM] demonstrated IgG/κ monoclonality of the plasma cells. On molecular analysis, T-cell receptor and immunoglobulin heavy chain rearrangements were polyclonal. Serum protein electrophoresis, immunofixation, and quantitative assessment of serum Igs were normal. Bone marrow biopsy, skeletal survey, and body computed tomography scan were unremarkable. A diagnosis of primary solitary cutaneous plasmacytoma was made. The lesion was removed surgically, and the patient remains in remission up to now. Primary cutaneous plasmacytoma represents only 2%-4% of extramedullary plasmacytomas. The rarity and the nonspecific presentation of cutaneous plasmacytomas does not allow a definite clinical diagnosis. Only histopathology reveals the typical pattern of a dense monomorphic dermal plasmacytic infiltrate, whereas immunohistochemistry shows monoclonality of the neoplastic cells.     

Cutaneous plasmacytomas. A review and presentation of an unusual case.

Recent reviews separate four types of plasma cell tumor: multiple myeloma, extramedullary plasmacytoma (without multiple myeloma), solitary myeloma of bone, and plasma cell leukemia. Cutaneous plasma cell tumors may arise from lymphatic or vascular spread of tumor (metastatic cutaneous plasmacytoma) or by direct extension from bone lesions. The former, metastatic cutaneous plasmacytomas, are quite rare. Specific malignant plasmacyte cutaneous tumors can also be seen in extramedullary plasmacytoma, solitary myeloma of bone, and plasma cell leukemia. We present a patient with multiple myeloma and lymphedema of the right arm, who developed a pathologic fracture of the right humerus and subsequently developed numerous metastatic cutaneous plasmacytomas localized to the lymphedematous arm. Direct immunofluorescence of frozen sections and enzymatically released cells from tumor nodule failed to reveal cell-associated immunoglobulins.     

Cutaneous manifestations of multiple myeloma

We report the cutaneous manifestations of multiple myeloma, using a retrospective review of 115 patients' records obtained from tumor registry files. Five patients were found to have biopsy-proved extramedullary plasmacytomas without extension from an underlying bony focus. Twelve patients had ecchymoses without thrombocytopenia; two of them had biopsy-proved amyloidosis. One patient presented with pyoderma gangrenosum and was subsequently diagnosed with multiple myeloma. The last patient initially presented with what clinically appeared to be leukocytoclastic vasculitis, and, in the course of a standard workup, he was diagnosed with multiple myeloma. These findings are discussed with regard to the current literature on the cutaneous manifestations of multiple myeloma.     

Pyoderma gangrenosum or cutaneous lymphoma: a difficult clinical diagnosis

Pyoderma gangrenosum is frequently associated with an underlying condition such as ulcerative colitis or a myeloproliferative syndrome. However, lymphoproliferative malignancies have only rarely been seen concurrently with pyoderma gangrenosum. We present the case of a patient with small lymphocytic lymphoma who noted a slowly enlarging skin ulcer that was clinically consistent with pyoderma gangrenosum. Examination of a biopsy specimen showed infiltration of the skin with lymphoma cells as well as deeper necrotic material and thrombosis of vessels that were infiltrated by lymphoma. This case illustrates the difficulty of differentiating pyoderma gangrenosum from cutaneous lymphoma clinically.     

Pyoderma gangrenosum and cranial osteolysis: case report and review of the paediatric literature

Pyoderma gangrenosum is a poorly understood, ulcerating cutaneous disorder which is rarely seen in the paediatric age-group. We report a 3-year-old boy who developed an ulcer over the left frontoparietal scalp at the age of l year. A 9-cm area of'underlying cranial bone was destroyed. The appearance on radiographs and CT scun was suggestive of eosinophilic granuloma, osteomyelitis. or other destructive processes. Biopsics of the scalp lesion and calvaria showed granulation tissue and degenerating bone. After the biopsies the scalp lesion increascd in size, and wound dehiscencc occurred, Ulceration developed al the site of a PPD skin test, which on biopsy was consistent with the diagnosis of pyoderma gangrenosum. Pyoderma gangrenosum should be added to the differcntial diagnosis of cutaneous disorders which can result in osleolylic/osteonecrotic defects.     

Superficial Granulomafous Pyoderma

Superficial granulomatous pyoderma, recently described as a variant of pyoderma gangrenosum, would be better termed pathergic granulomatous cutaneous ulceration as the seven previously described cases, as well as our own two cases, have significant dermal involvement histologically and heal with scarring. In contrast to pyoderma gangrenosum, lesions of superficial granulomatous pyoderma respond to less toxic anti-inflammatory agents.     

Extramedullary plasmacytoma: cytological and genotypic studies

A 62-year-old woman had multiple plasmacytomas in the skin and lymph nodes, without Bence-Jones protein or a monoclonal peak of serum immunoglobulins. Infiltrating plasmacytoid cells expressed cytoplasmic IgG (λ) and surface CD38, without any B-cell markers. There was no visceral or bone marrow involvement suggestive of multiple myeloma. Southern blot analysis of extracted DNA from the cutaneous lesions showed two rearranged bands with an immunoglobulin, but not a T-cell receptor, gene probe. The patient showed a poor response to chemotherapy, and died of bronchopneumonia. The clinical course and cytological features differentiate multiple cutaneous extramedullary plasmacytomas from solitary cutaneous extramedullary plasmacytoma and cutaneous lesions associated with tnultiple myeloma.     

Primary cutaneous extramedullary plasmacytoma secreting λ IgG

An adult male was described, who developed a tumour on his left arm, which was diagnosed as a primary cutaneous extramedullary plasmacytoma after histopathological, electron microscopic and immunocytochemical studies. Further studies ruled out the presence of multiple myeloma, extramedullary plasmacytoma of another site, or paraproteinaemia/paraproteinuria. During a relapse of the tumour 21/2 years after initial treatment, a new immunocytochemical study demonstrated that the tumour cells expressed monoclonal λ IgG at the intra-cytoplasmic level, an unusual finding.     

Pustular pyoderma gangrenosum

A 79-year-old woman was admitted to our hospital with pustular pyoderma gangrenosum and an associated IgG kappa monoclonal gammopathy. The patient is currently being evaluated for possible multiple myeloma. IgG multiple myeloma and IgG monoclonal gammopathies are very rare in patients with pyoderma gangrenosum. The skin lesions are improving with the use of prednisone.     

Infliximab for treatment of resistant pyoderma gangrenosum associated with Crohn's disease

We present the case of an 18-year-old woman with Crohn's disease manifested by diffuse abdominal pain, bloody diarrhea accompanied by arthralgia, and swelling of large joints. On the lateral aspect of her right ankle there was an hemorrhagic, necrotic bullous lesion measuring 3 x 4 cm, surrounded by cutaneous inflammation and erythema. Biopsy showed a neutrophilic abscess-like ulcerative skin inflammation, which was diagnosed as pyoderma gangrenosum. The patient was treated with high doses of parenteral methylprednisolone, but her condition failed to improve and infliximab, a TNF-alpha blocking agent, was instituted. An immediate response of Crohn's disease was observed and, over the next 5 weeks, the ulcer on her right ankle also healed completely.     

Pyoderma gangrenosum associated with ulcerative colitis

We report the case of a 45-year old man with non-healing ulcers located on his chest, lumbal, sacral, retroauricular areas and forehead. Both clinical and histopathological examinations suggested pyoderma gangrenosum (PG). For six months the diagnosis of ulcerative colitis was established. PG in our patient was presented as a rapidly enlarging, painful ulcer with purple, undermined edges and a necrotic, haemorrhagic base. Initially, he was treated with a high dosage of peroral glucocorticosteroid, sulfasalazine, and systemic antibiotics, together with daily wound care. Ulceration partially regressed. Total colonoscopy showed pancolitis. When the dose of glucocorticosteroids was tapered down to 35 mg, new ulcerations on his right thigh and abdomen were formed. He also developed E. coli sepsis and flare up of bowel disease. Azathioprine, together with two pulse doses of glucocorticosteroids and antibiotics, were administered. He was scheduled for a total colectomy. The management of PG continues to be a therapeutic challenge.     

Pyoderma gangrenosum in a thoracotomy wound associated with a pulmonary cavitating lesion

We present a patient with both pyoderma gangrenosum in a thoracotomy wound and a pulmonary cavitating lesion with the histological features of Wegener's granulomatosis. An ulcer with blue undermined edges developed in our patient's thoracotomy scar after a lobectomy was carried out for the cavitating lung lesion and c Antineutrophil Cytoplasmic Antibodies (cANCA), which is highly specific for active Wegener's granulomatosis but was negative at the time of her skin lesion. This suggests that our patient had cutaneous pyoderma gangrenosum rather than cutaneous Wegener's granulomatosis, although the two conditions may occasionally have similar clinical and histological features.     

Primary cutaneous plasmacytoma. A case report and review of the literature

Primary extramedullary plasmacytoma of the skin is a very rare disease; to our knowledge, only a few cases have been published in the world literature. In this paper, a patient who had a primary cutaneous plasmacytoma of the lower lip for 20 years is described. Serological, radiological and histological examinations excluded the possibility of an underlying multiple myeloma. The position of cutaneous plasmacytomas within the group of malignant plasma cell neoplasias is discussed and the relevant literature is reviewed.     

继发性皮肤浆细胞瘤

报告1例继发于多发性骨髓瘤（multiple myelonm,MM)的皮肤桨细胞瘤，患者女，44岁，皮损为多发性，来源于MM直接蔓延和血行转移，组织病理检查示瘤细胞呈结节状浸润，免疫组化染色结果示IgG阳性表达，患者于皮损出现后78d死亡。     

Histopathologic and immunopathologic study of pyoderma gangrenosum

Sixty-three patients with pyoderma gangrenosum were seen and studied at the Mayo Clinic from 1971 to 1980. Biopsies from the erythematous border or necrotic edge of the pyoderma gangrenosum lesions usually demonstrated a characteristic pathogenic morphologic evolution. The early lesions revealed mild to moderate perivascular lymphocytic infiltrate associated with endothelial swelling. The fully developed lesions demonstrated necrosis in addition to a dense lymphocytic infiltration surrounding as well as involving the blood vessels. Extravasation of erythrocytes and thrombosis sometimes were seen. Ulceration, infarction, and abscess formation were found in the later stages of evolution. Direct immunofluorescence results were positive in the blood vessels of 36 of 65 (55%) specimens. IgM, C3, and fibrin were found in the papillary and reticular dermal vessels. IgG and IgA were only occasionally present. Pyoderma gangrenosum appears to be a reactive process that is manifested as a vasculitis. Biopsy material from the advancing active erythematous border has early characteristic dermatopathologic findings of lymphocytic vasculitis. Cutaneous vascular immune deposits suggest an immune pathogenesis of either an immune complex disease or lymphocytotoxic reaction.     

CD8-positives,CD30-negatives kutanes T-Zell-Lymphom unter dem klinischen Bild eines Pyoderma gangraenosum

Ulcerated primary cutaneous lymphomas are not rare, but the clinical manifestation as a pyoderma gangrenosum look-alike is extraordinary. CD8-positive lymphomas are rare, unclassifiable tumours with variable prognosis. We report on a 49-year-old patient with a large ulcerated primary cutaneous lymphoma on the left chest wall presenting as pyoderma gangrenosum. With immunohistochemical staining, most lymphocytes were shown to be CD8-positive. The CD30 antigen was not expressed. After radiotherapy with complete skin irradiation, the lymphoma regressed completely. The patient has been free of relapse for 28 months so far.     

Pyoderma gangraenosum Erfolgreiche topische Therapie mit Tacrolimus (FK506)

Pyoderma gangrenosum is a distinct clinical entity characterized by chronic, recurring, destructive ulcerations. Although the pathogenesis of pyoderma gangrenosum is unknown, immunologic aberrations of neutrophil granulocytes seem to be important. Systemic steroids and macrolide lactones such as cyclosporin A and tacrolimus have been reported to be useful in the clinical management of disease. Pyoderma gangrenosum has been found to be associated with several systemic diseases.The association with chronic ulcerative colitis is well known, but the diagnosis may be complicated by early administration of systemic steroids.Therefore, local immunosuppression with topically applied agents could be an efficient therapeutic alternative especially for mild or early cutaneous lesions.We describe the successful topical treatment of a patient with multiple lesions of pyoderma gangrenosum with 0,1% tacrolimus (FK506) ointment which is known to have better dermal penetration and higher immunosuppressive potency than topical cyclosporin A. In addition, other indications for topical tacrolimus are discussed.     

Psoriasis vulgaris als Trigger eines superfiziellen multilokulären Pyoderma gangraenosum?

We report on a 60-year-old man with psoriasis vulgaris who had developed disseminated ulcerations of different sizes over the complete integument over a period of 3 months. The initial examination revealed approximately 150 clearly defined ulcerations, which were mostly reported to be painless. The histopathology report of a biopsy taken from the border area of an ulceration showed a nonspecific inflammation with a predominantly neutrophilic infiltrate. In the synopsis of all results, after excluding relevant differential diagnoses, we diagnosed a superficial multilocular pyoderma gangrenosum that had possibly developed in the areas of the preexisting psoriasis vulgaris. Reports of superficial multilocular pyoderma gangrenosum, a variation of pyoderma gangrenosum, are extremely rare. This condition is characterised by multiple superficial, chronically persistent ulcerations. These ulcerations are often described as indolent and usually develop from a pustule in the area of a preexisting condition or trauma. Up to now, psoriasis vulgaris has seldom been described as a trigger for pyoderma gangrenosum. The treatment is very difficult and is based on local and/or systemic immunosuppression. In this patient's case, considerable improvement was attained with a topical polyhexanide gel in combination with moist wound therapy, with no systemic immunosuppression. Our case report underscores that physicians should keep in mind the rare variation of superficial multilocular pyoderma gangrenosum in the differential diagnosis of multiple disseminated, painless ulcerations over the complete integument in patients with psoriasis vulgaris.     

坏疽性脓皮病诊疗研究进展

坏疽性脓皮病(PG)是一种罕见的嗜中性粒细胞皮病,由触发炎症级联后的异常中性粒细胞聚集引起的,主要表现为无菌性中性粒细胞浸润和全身性炎症。最新研究表明药物会引起坏疽性脓皮病,但是相关报道较少,临床工作者对其认识不足。临床中PG易被误诊为难愈合性溃疡而进行清创术治疗,导致病变区扩散恶化,因此正确诊断PG对于疾病的治疗和预后十分重要。本文就PG的诊疗进展进行综述。     

Skin manifestations of refractory anemia with excess of blasts (RAEB)

The authors describe three cases of refractory anaemia with an excess of myeloblasts in the bone marrow (RAEM), associated with pyoderma gangrenosum (PG) and vasculitis. The first patient was an 85-year old man whose RAEM had begun in 1979. In 1985, he developed pyoderma gangrenosum in the popliteal fossa and on the right heel. Histology confirmed the diagnosis. A direct immunofluorescence test on the biopsy specimen was negative. Apart from the haematological syndrome, there were no laboratory abnormalities. The cutaneous lesions disappeared after 7 weeks of treatment with prednisolone 20 mg per day. The blood disease remained unchanged. The second patient was a 71-year old woman with RAEM since 1982. In 1984, she presented with lesions of cutaneous vasculitis located on the anterior aspect of the upper third of her left leg. There was neither arthralgia nor fever, and no history of drug toxicity or infection. Beside RAEM, polyclonal hyperglobulinaemia was present. Histological examination of the skin showed evidence of vasculitis with fibrinoid necrosis of vascular walls and perivascular lympho-histiocytic infiltrate with granulocytes and slight leucocytoclasia. Direct immunofluorescence testing of the skin demonstrated intravascular complement deposits. There were no circulating immune complexes; measurements of complement and complement fractions gave normal values; no cryoglobulin was found. The cutaneous lesions recurred on two occasions in 6 months, although no drug toxicity or infection could be elicited and the haematological syndrome was unaffected. The third patient was a 67-year old man with RAEM since 1982. In 1983, an ulcero-necrotic lesion spontaneously developed on his right leg.(ABSTRACT TRUNCATED AT 250 WORDS)