Hisashi Kawabata

Treatment of pustular psoriasis with granulocyte and monocyte adsorption apheresis

We studied the efficacy of granulocyte and monocyte adsorption apheresis in 2 patients with pustular psoriasis, one localized, the other generalized. Treatment with granulocyte and monocyte adsorption apheresis resulted in remarkable clearing of the skin lesions, suggesting that this therapy is a valuable tool for treating patients with intractable skin diseases attributable to activated granulocytes. We present detailed descriptions of these patients and this novel therapy.

MRI studies of spinal visceral larva migrans syndrome

We report serial MR findings in four patients with myelitis caused by visceral larva migrans syndrome due to Toxocara canis or Ascaris suum infection. MR imaging revealed spinal cord swelling with or without gadolinium enhancement in three patients. T2-weighted images showed high signal intensities preferentially located in both lateral and posterior columns. Antihelmintic and corticosteroid treatment yielded improvement in neurologic deficits and spinal lesions. However, one patient with T. canis infection relapsed associated with reappearance of MRI abnormalities.

Treatment of psoriatic arthritis with granulocyte and monocyte adsorption apheresis

Granulocyte and monocyte adsorption apheresis (GCAP) is a new extracorporeal apheresis treatment modality that removes pathogenic granulocytes. Recently, we found that GCAP is useful for treating pyoderma gangrenosum and pustular psoriasis. We thought that this treatment may also be effective for treating other disorders attributable to activated granulocytes and studied the efficacy of GCAP in 4 patients with psoriatic arthritis. Treatment with GCAP resulted in remarkable clearing of joint pain, suggesting that GCAP is valuable for treating arthritis as well as skin disorders. We present a detailed description of these patients and this novel therapy.

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.

A Case of Sarcoidosis Involving the Tongue

Sarcoidosis is a systemic granulomatous disorder which commonly affects the skin. Involvement of the tongue is rare; a review of the previous literature over the last 30 years revealed only six cases of sarcoidosis affecting the tongue. We studied a case of sarcoidosis involving the tongue in a 32‐year‐old Japanese woman with characteristic clinical and pathological findings. She visited our department with a complaint of a tongue lesion of which she had been aware for a month. A diagnosis of sarcoidosis was made for the lesion by clinical and pathological examinations. Oral involvement by sarcoidosis is rare, however this disorder should be considered as a possible cause of intraoral granulomatous lesions.

Hyper nuclear acetylation (HNA) in proliferation, differentiation and apoptosis.

Coactivators such as cyclic AMP-response-element binding protein (CREB)-binding protein (CBP) and p300/CBP associated factor (P/CAF) play a crucial role in coordinating and cointegrating eukaryotic transcription. One of the recent paradigms in the eukaryotic transcription field is the finding of molecular basis of coactivator function. The well characterized coactivators such as CBP and P/CAF have been proposed to coactivate/cointegrate gene expression with many transcription activators through two mechanisms. One is complex formation with the components with basal transcriptional machinery. Another is its intrinsic and associated enzymatic activity, which transfers an acetyl-base to the epsilon ( epsilon ) portion of lysine-residues in histones and certain nuclear proteins (factor acetyltransferases; FATs), such as p53, lymphoid enhancer-binding factor (LEF), and transcription factor IIE (TFIIE), which often results in increased transcriptional activity. Recently, the status of hyper nuclear acetylation (HNA) has been thought to influence proliferation, differentiation and apoptosis. Furthermore, recent reports showed that histone acetyltransferase (HAT) activity is increased in human disease, such as cancer and atherosclerosis, and studies have shown associations between nuclear acetylation/deacetylation and cell proliferation/differentiation.

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.

Thrombomodulin exerts cytoprotective effect on low-dose UVB-irradiated HaCaT cells

Thrombomodulin (TM) is an endothelial cell surface anticoagulant glycoprotein that performs antimetastatic, angiogenic, adhesive, and anti-inflammatory functions in various tissues. It is also expressed in epidermal keratinocytes. We found that a physiological dose (10mJ/cm(2)) of mid-wavelength ultraviolet irradiation (UVB) significantly induced TM expression via the p38mitogen-activated protein kinase (MAPK)/cyclic AMP response element (CRE) signaling pathway in the epidermal keratinocyte cell line HaCaT; this shows that TM regulates the survival of HaCaT cells. SB203580, a p38MAPK inhibitor, significantly decreased TM expression and the viability of cells exposed to UVB. Furthermore, overexpression of TM markedly increased cell viability, and it was abrogated by TM small interfering RNA (siRNA), suggesting that TM may play an important role in exerting cytoprotective effect on epidermal keratinocytes against low-dose UVB.

Hemophagocytic syndrome induced by diaminodiphenylsulfone.

Dear Editor, Hemophagocytic syndrome (HPS) is characterized by fever, hepatosplenomegaly and jaundice. Phagocytosis of erythrocytes, leukocytes, platelets and their precursors by macrophages in bone marrow (BM) and other tissues is the definitive pathological finding. HPS is often diagnosed in association with malignant, genetic or autoimmune diseases; it is prominently associated with Epstein–Barr virus (EBV) infection. and there are few reports of HPS as a result of drug therapy. We encountered a patient who developed HPS in response to the administration of diaminodiphenylsulfone (DDS). A 40-year-old man was referred to our clinic in 1997 with diffuse pruritic, scaly erythematous lesions over his entire body. He had no history of drug allergy or collagen disease. A biopsy of abdominal skin showed perivascular and interface infiltration by atypical lymphoid cells with hyperchromatic convoluted nuclei. Electron microscopic examination of his peripheral blood showed a few abnormal lymphocytes with indented nuclei and high nuclear contour indices. We made a diagnosis of Sézary syndrome and started treatment with topical and systemic corticosteroids. His physical symptoms were improved by prednisolone (PDN) at 15 mg/day but not at lower doses. To control his disease with tapered PDN doses we tried various drugs including cyclophosphamide, methotrexate and etretinate, however, his response was limited. In March 2001, we started therapy with DDS (50 mg/day) and PDN (10 mg/day). After 1 week, he manifested general fatigue and cervical lymphadenopathy. These symptoms worsened in the course of the next week; his body temperature rose to 40°C and blood cell examination disclosed pancytopenia (300 leukocytes/μL), 6.5 g/dL hemoglobin and 6 × 10/μL platelets. His C-reactive protein and lactate dehydrogenase levels were elevated to 14.05 mg/dL and 496 IU/L, respectively. Examination of BM smears revealed decreased cellularity and abundant hemophagocytes (Fig. 1).

The prevalence of human T‐cell lymphotropic virus type I infection in patients with cutaneous malignancies

Sir, Langerhans cell histiocytosis (LCH) includes a spectrum of disorders with overlapping clinical features, i.e. Letterer±Siwe disease, Hand±SchuÈ ller±Christian syndrome, eosinophilic granuloma and congenital self-healing reticulohistiocytosis. The typical cutaneous findings comprise seborrhoeic dermatitis-like lesions, reddish-brown purpuric papules and nodules, oozing erosions, and ulcerations. A child with LCH is described, who presented with an uncommon pattern of pustular lesions on the forehead, hands, feet and genital region, following an atypical clinical course. One week after birth, a 14-month-old girl developed multiple, sharply demarcated itchy pustules on the forehead, together with red papules, and pustules partly covered with haemorrhagic crusts, which were predominantly on the soles of the feet (Fig. 1a). Some pustules occurred on the hands and in the genital and gluteal region. No lymphadenopathy was detectable. During topical treatment with lotio zinci (lotion with zinc oxide, talcum, glycerol and distilled water) the cutaneous lesions regressed slowly, but new lesions developed during the following months. After 5 months, an ulcer developed in the maxillary region. There was no specific family history of skin diseases. A skin biopsy of a pustule from the gluteal region revealed a dense upper dermal infiltrate of large histiocytes with a reniform nucleus, and intraepidermal collections of histiocytic cells (Fig. 1b). Immunohistochemical stains were positive for CD1 and for S-100 antigen. Electron microscopy revealed intracytoplasmic Birbeck granules within the histiocytic cells, thus confirming the diagnosis of LCH. Bacteriological and mycological examinations from skin lesions were negative. Full blood count, liver and kidney function were normal. Scintiscanning and radiography showed distinct involvement of the maxilla. Abdominal sonography was normal. Based on the occurrence of new cutaneous lesions and the subsequent involvement of the osseous part of the maxilla, chemotherapy was initiated with oral prednisolone 40 mg m daily for 4 weeks; then reduction of dose, vinblastine 0 ́2 mg kg once weekly for 6 weeks, then once monthly; and 6-mercaptopurine 50 mg m daily. Cutaneous lesions on the scalp, hands and feet, and the osseous lesion of the maxilla healed during the chemotherapy, which was performed for 1 years. In 1955, Lichtenstein summarized the three entities Letterer±Siwe disease, Hand±SchuÈ ller±Christian disease and eosinophilic granuloma under the term histiocytosis X. The cause of histiocytosis X (LCH) is unknown, but it is believed to be a proliferative process of Langerhans cells. Lesional histiocytes in the child described here were positive for S-100 antigen and CD1, as well as for intracytoplasmic Birbeck granules. The skin lesions did not clear completely