Akira Gushi

Extramammary Paget's Disease/Carcinoma Successfully Treated with a Combination Chemotherapy: Report of Two Cases

We have treated two patients with extramammary Pagets disease/carcinoma (EMPD/C), a 62‐year‐old woman and a 78‐year‐old man. In both patients, lymph nodes in the areas of the bilateral inguinal, external iliac arteries, and abdominal aorta were affected. After surgery, they underwent 5 courses of systemic combination chemotherapy at 4‐week intervals to residual or recurrent lymph node metastasis. Each course consisted of 3.5 mg mitomycin C and 50 mg epirubicin (day 1), 0.6 mg vincristine (days 1 and 7), 30 mg cisplatin (days 1, 2, and 3), and 350 mg 5‐fluorouracil (days 3, 4, 5, 6, and 7). The affected lymph nodes in both patients subsided in response to the chemotherapy. Positron emission tomography (PET) scans confirmed the complete remission of lymph node metastasis in Case 1. In Case 2, they were reduced by more than 90% on computed tomography (CT) scans. Adverse effects included leukocytopenia, vomiting, hypesthesia, and constipation, all of which disappeared after the completion of chemotherapy. While surgery with wide local excision is the treatment of choice in patients with EMPD/C, there is currently no standardized treatment for advanced cases with metastasis. We describe two patients with EMPD/C whose metastatic lesions responded well to this combination of chemotherapy.

Three Cases of Polyarteritis Nodosa Cutanea and a Review of the Literature

We describe three cases of polyarteritis nodosa cutanea (PNC) showing necrotizing arteritis and only cutaneous lesions without systemic symptoms or visceral involvement for eleven, six, and three years after the onset of the disease. Since it was first described, there has been continuous controversy as to whether PNC progresses to systemic PN. Some cases have been described which had begun with a cutaneous lesion and progressed to the systemic form 19 and 18 years after the onset of the disease, so we believe that long term follow‐up of this disease is essential.

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.

A Case of Localized Pemphigus Foliaceus

Pemphigus foliaceus (PF) is most commonly observed on the face, scalp, chest and back at the onset of the condition. The case described here is that of an 81‐year‐old female with a single PF lesion localized to the right cheek. A review of the literature published in English and Japanese disclosed only 3 cases of PF in which the patient presented with a single lesion, and 2 of these cases were referred to as “localized pemphigus foliaceus”.

The 3G5 antigen is expressed in dermal mast cells but not pericytes

Background:  It has been shown that the 3G5 antigen recognized by monoclonal antibody 3G5 (mAb 3G5) is a useful marker of pericytes in normal human skin. However, most 3G5 antigen‐expressing cells in capillary vessels were stained negatively for α‐smooth muscle actin (α‐SMA), a prominent pericyte marker. This study was designed to determine whether the expression of the 3G5 antigen is restricted to specific stages of pericyte development, or if it is expressed in other cells rather than pericytes in capillary vessels.

The Discrepancy in Hardness between Clinical and Histopathological Findings in Localized Scleroderma Treated with PUVA

Topical PUVA therapy was applied to a patient with localized scleroderma. Her localized scleroderma responded very well to the topical PUVA therapy, i.e., her sclerotic skin softened to normal skin texture. However, despite this dramatic clinical change the histopathological findings did not change at all and were still “hard”.

Contact dermatitis due to orchids (Cymbidium and Oncidium)

A 64‐year‐old male who cultivated orchids as a hobby, had noticed itchy erythematous lesions on both hands since 6 months earlier. His eruptions had gradually worsened and scales and fissures had appeared. Although he was treated with topical corticosteroids by a doctor, his erythema showed no improvement. Patch tests with the orchids he cultivated, fertilizers, antiseptics and insecticides showed positive reactions to the stems of Cymbidium and Oncidium orchids. A diagnosis of contact dermatitis attributable to the cultivated orchids was made.