Yuji Mochitomi

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.

STEVENS-JOHNSON SYNDROME CAUSED BY A HEALTH DRINK (EBERU) CONTAINING OPHIOPOGONIS TUBER

Stevens‐Johnson syndrome is considered to be a severe type of erythema exsudativum multiforme. It is characterized by erythema with bullous and eroded lesions of skin and mucous membranes. We report a case of Stevenjohnson syndrome following consumption of a health drink containing ophiopogonis tuber. A 66‐year‐old female took an O.T.C. health drink for fever. The next morning, she noted erythema and swelling of her face, neck, and chest. She started to develop bullous and eroded lesions on the skin of her entire body and the mucous membranes of her oral cavity, conjunctiva, and cornea, and she became feverish. She had high degrees of corneal erosion and liver dysfunction. Skin biopsy showed diffuse necrosis of the epidermis. After admission to the hospital, steroid pulse therapy (1000 mg/day of methylprednisolone sodium succinate) was continued for 5 days. The health drink induced a positive drug lymphocyte stimulation test (DLST) and patch test. A challenge test was done with a one hundredth dose, and it was positive. We did patch tests with all components of the drink and found that Mai‐Meu‐Dong‐Tang (ophiopogonis) alone was positive at 72 hours. There is no previous report of Stevens‐Johnson syndrome caused by a health drink or Mai‐Meu‐Dong‐Tang. Even though it is a health drink, we should be aware of the possibility of a severe reaction.

Multicentric castleman disease with cutaneous manifestations : Report of 2 cases and comparison with systemic plasmacytosis

We report 2 patients with multicentric Castleman disease. Both presented with multiple, indurated, hyperpigmented plaques, generalized lymphadenopathy and polyclonal hypergammaglobulinemia. Biopsy specimens showed infiltration of mature plasma cells and lymphocytes in the dermis and lymph nodes. Skin specimens were negative for human herpesvirus 8, latent nuclear antigen 1 and Epstein-Barr virus by in situ hybridization. PCR disclosed clonal T-cell receptor gene rearrangement in the bone marrow cells of 1 patient. We discuss the possible relationship between multicentric Castleman disease and systemic plasmacytosis as well as plasma cell proliferation.

Pseudoxanthoma Elasticum (PXE)‐like Calcification in Adult Dermatomyositis

A case of pseudoxanthoma elasticum (PXE)‐like calcification in adult dermatomyositis (DM) is described. The patient was a 38‐year‐old woman with a history of dermatomyositis for 3 months. Yellowish, hard, papulo‐plaque lesions, which looked like those of pseudoxanthoma elasticum, were noted on her left axilla. Calcium deposition was confirmed by X‐ray, histopathological, and electron microscopic examinations. Histopathological and histochemical examinations showed acicular calcium deposition in the middle and deep dermis surrounded by mucin. Electron microscopic examination revealed that the calcium deposition was not on collagen fibers. These morphological features were distinct from those of PXE. We proposed the possibility that degenerated mucin or degenerated elastic fiber might result in subsequent calcium deposition in reticular calcinosis in adult DM. The calcification clinically disappeared without any specific treatment except for prednisolone and cyclophosphamide.

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

A Case of Mastocytosis with Chorea

To the Editor: Mastocytosis is a disease in which the population of mast cells is increased in various tissues. The skin is most frequently involved in this disorder, with or without systemic involvement. The most common cutaneous symptoms are generalized eruptions consisting of multiple reddish brown macules and papules distributed over the trunk and occasionally over the extremities and head. In systemic mastocytosis, mast cells infiltrate into various organs including the liver, spleen and those in the skeletal, gastrointestinal, and lymphatic systems (1, 2). While it is uncommon for mastocytosis to affect the central nervous system, headache, dizziness, seizures, and alterations in cognitive function have been described previously (3, 4). However, the occurrence of chorea is extremely rare. Recently, we encountered a patient with mastocytosis who developed choreic movements. A review of the literature revealed only one such previous case (5). A 38-year-old man with multiple pruritic lesions on his trunk and extremities was referred to our department. He had first noticed them 19 years earlier. They were reddish brown macules or papules which varied between 0.5 and 1.0 cm in diameter (Fig. 1). A biopsy specimen obtained from the upper right arm revealed a dermal infiltration of mast cells (Fig. 2). Based on clinical and histopathological findings, a diagnosis of mastocytosis was made. For the previous 18 months, he had also suffered from involuntary movements mainly involving his extremities. Results of hematological and biochemical examinations were all within normal limits. CT and MRI of his brain showed no abnormalities. His early development had been normal. There was no family history of involuntary movements or any other neurological disease. The patient had not been treated with drugs which were known to induce involuntary movements. Thus, intensive examinations failed to provide a particular diagnosis for the involuntary movements such as Huntington chorea, chorea acanthocytosis, neurovascular disorders, cerebellar ataxia, Wilson disease and other diseases which could develop involuntary movements and a clinical diagnosis of chorea was made. In the previously reported case, because choreic movements developed after administration of a local anesthetic (mepivacaine 1%) for a skin biopsy and mepivacaine is a well-known activator of mast cell secretion, the choreic movements were considered to be caused by the mastocytosis. The authors suggested that mastocytosis might induce The Journal of Dermatology Vol. 28: 451–452, 2001