Naoki Ohtake

Encapsulated fat necrosis in a patient with Ehlers-Danlos syndrome

Mobile encapsulated lipoma, first described by Sahl in 1978 (1), is characterized by its great mobility within subcutaneous adipose tissue and presentation with or without tenderness when the nodules are manipulated. This lipomas histopathology is very characteristic; i.e., it consists of mature viable or degenerative lipocytes encapsulated by fibrous tissue. We report a case of this so‐called mobile encapsulated lipoma involving a patient with Ehlers‐Danlos syndrome. This case is the first report of mobile encapsulated lipoma in a patient with a disease of this type. We discuss its pathogenesis and the nomenclature for these lesions.

Membranocystic lesions in a patient with cytophagic histiocytic panniculitis associated with subcutaneous T-cell lymphoma

A 51-year-old Japanese woman had erythematous, tender subcutaneous nodules on her lower legs and buttocks, as well as fever, pancytopenia, and liver dysfunction. Histopathologic examination of the nodules showed subcutaneous T-cell lymphoma with cytophagocytosis and membranocystic lesions. Our patient was unique in having the combination of cytophagic histiocytic panniculitis due to subcutaneous T-cell lymphoma and membranocystic lesions.

Leiomyoma of the Scrotum

Leiomyoma is a benign tumor derived from smooth muscle. Leiomyoma of the scrotum arising from the tunica dartos is exceedingly rare. We describe the case of a 51-year-old Japanese man with this disease entity.

A Case of Adult T‐cell Leukemia/Lymphoma (ATLL) with Angiocentric and Angiodestructive Features

This report describes a case of adult T‐cell leukemia/lymphoma (ATLL) with angiocentric and angiodestructive features. The patient was a 66‐year‐old Japanese woman who began developing widespread skin lesions ten months prior to admission. The diagnosis of ATLL was made on the basis of her having an antibody to human T‐cell lymphotropic virus type ‐1 (HTLV‐1) and typical flower cells (ATLL cells) in peripheral blood smears. Once hospitalized, the course of her disease was very acute and severe, as is seen with angiocentric lymphoma. Based on histological features, this case was judged not to be angiocentric lymphoma; however, it may lie within the spectrum of angiocentric immunoproliferative lesions (AIL). The findings in this case strongly suggest that HTLV‐1 can be a pathogenic factor in the expression of angiocentric and angiodestructive features in ATLL, as is Epstein‐Barr virus (EBV) (1–4). To our knowledge, the present case is the sixth reported in the literature of lymphoma in which these features are associated with HTLV‐1 infection (5–7).

A Case of Squamous Cell Carcinoma Arising in a Pilonidal Sinus

Malignant degeneration is a rare complication of pilonidal sinus. It has a high rate of recurrence and a poor prognosis compared with other nonmelanoma skin cancers. We report a Japanese male patient with carcinoma arising from a neglected sacrococcygeal pilonidal sinus. A 36-year-old male first had symptoms of pilonidal sinus 8 years prior to admission. In 1995, an exophytic tumor arose rapidly in an area of his sinus tracts. He presented in 1997 with a large tumor as a sacral lesion (Fig. 1). A biopsy revealed a moderately differentiated squamous cell carcinoma. A wide local excision with 1.5-cm margins, including excision of the gluteal and presacral fasciae, was performed. Histologic examination showed a sinus tract lined by atypical epidermal cells containing many hair shafts (Fig. 2). The tumor nests showed the cytologic and architectural patterns of a squamous cell carcinoma, so the diagnosis of squamous cell carcinoma arising in a pilonidal sinus was made. He was treated with adjuvant radiation of 50 Grey. However, in 1998, he presented with left inguinal lymphoadenopathy, and squamous cell carcinoma was found in the lymph node. Despite combined chemotherapy with mitomycin C, vincristine, epirubicin, carboplatin and 5fluorouracil, there was local invasion into his left iliac bone, and he died three years later. Malignant degeneration is estimated to occur in approximately 0.02–0.1% of pilonidal sinuses (1, 2). The origin of this disease appears to be the same as that of chronically inflamed wounds such as burns, The Journal of Dermatology Vol. 29: 757–758, 2002

Unusual polyp-like structures in lobular panniculitis of a patient with Behcet's disease

Unusual histopathologic structures were observed in lobular panniculitis of a patient with Behçets disease. These were polyp-like in shape and protruded into cavities made of lysed fat cells. Various stages of development were observed. To determine the origin of cells making up these structures, we applied various histochemical and immunohistochemical techniques and found the cells to be histiocytes (macrophages) engaged in phagocytizing degenerated fat cells and cell membranes which had formed into membranocystic lesions.

Melkersson‐Rosenthal Syndrome with Malignant Pharyngeal Lymphoma

To the Editor: We recently treated a patient who had been suffering from MelkerssonRosenthal syndrome for more than 10 years. He subsequently developed non-Hodgkins type malignant lymphoma of the pharynx. To our knowledge, there has been only one case of this association reported in the literature (1). Case Report: A 49-year-old man visited our clinic on February 17, 1995, with a complaint of diffuse edema of his lips which had developed 10 years earlier (Fig. 1). On examination, his tongue was found to be enlarged and fissured (Fig. 2), but no facial paralysis was present. Microscopic examination of a biopsy specimen from the lower lip showed dilated lymph vessels, edema, and infiltration of lymphocytes in the vicinity of capillaries in the thickened lamina propria (Fig. 3). However, as there was no apparent granuloma formation, we diagnosed this patient as having cheilitis granulomatosa, based on the clinical and histopathological findings. He was found to have developed a pharyngeal tumor several months previously which was revealed by histopathological examination to be a non-Hodgkins type malignant lym-

Differentiation of Malignant Lymphoma and Non‐Lymphoma by an Occlusive Dressing Method Employing Bromodeoxyuridine

Although lymphoma cells can proliferate in skin tissue, lymphocytes which have infiltrated the skin due to inflammatory changes are generally unable to do so. It may be possible to differentiate malignant lymphomas from benign lymphocytic infiltrations in skin tissue by detection of cell cycle‐related antigens. We developed a novel in vivo method for studying the cell kinetics of human skin using bromodeoxyuridine (BrdU) with an occlusive dressing. Following the application of BrdU, BrdU‐labeled lymphoid cells were counted in the dermis. The patients studied included 22 with various types of lymphomas (lymphoma group) and 22 with different forms of inflammatory skin disease (non‐lymphoma group). Skin specimens were obtained after applying occlusive adhesive plasters containing 2% BrdU dissolved in 0.9% sodium chloride solution and were then immunostained with anti‐BrdU monoclonal antibody. The average percentages of labeled cells were 15.9 ± 7.6% for the lymphoma group and 4.7 ± 2.6% for die non‐lymphoma group. This difference was statistically significant (p<0.0001). Using this method, malignant lymphomas and benign lymphocytic infiltrations of the skin could be differentiated by counting BrdU‐labeled lymphoid cells, and this method could help in determining a prognosis.

2 cases of Cole-Engman syndrome.

We report two patients with Cole-Engman syndrome (dyskeratosis congenita) .Case 1: A 48-year-old Japanese man had noticed reticulated skin hyperpigmentation on his whole body from 11-year-old. He came to our dental hospital with acomplain of a tumor in the oral cavity. It was diagnosed as leukoplakia and squamous cell carcinoma arising from leukoplakia of the mucous menbranes on the left mandibular gingiva. He had also dental anomalies, nail dystrophy and lacrimal duct obstruction. He was diagnosed as Cole-Engman syndrome, comlete type.Case 2: A 49-year-old Japanese man visited our hospital with a complain of nummular eczema and prurigo-like eruption with itching. He also had reticulated skin hyperpigmentation, nail dyatrophy, hyperkeratosis of palms and soles, angular stomatitis and leukoplakia on right lateral side of tongue.We diagnosed him as Cole-Engman syndrome, comlete type.We followed up them very carefully.