Kenjiro Terasaki

Efficacy of topical tacrolimus for treating the malar rash of systemic lupus erythematosus

Summary The rash of systemic lupus erythematosus (SLE) is usually treated with topical corticosteroids, but prolonged use causes adverse cutaneous side‐effects. We assessed the efficacy of topical tacrolimus for treating the skin lesions of SLE. Three patients with SLE affecting their facial skin applied 0·1% tacrolimus ointment on one side of their face twice daily for 3 weeks, in conjunction with a sunscreen cream. After 3 weeks, erythema on the treated side was ameliorated in all three patients compared with the untreated side. Although the study is preliminary, the results demonstrate that topical tacrolimus may be useful for treating the malar rash of SLE.

SUCCESSFUL TREATMENT OF SCLERODERMA WITH PUVA THERAPY

PUVA therapy was carried out on four patients with scleroderma; three of them had cutaneous manifestations of progressive systemic sclerosis and one other exhibited generalized morphea. PUVA therapy was given with daily doses of 0.25 J/cm2 or 0.4 J/cm2 for 3–8 weeks, resulting in total doses between 3.5 J/cm2 and 9.6 J/cm2. All four patients responded well to this treatment; improvements of hand closure, skin sclerosis index, and flexion of fingers or knee joints were obtained. Thus, PUVA appeared to be beneficial for treating scleroderma.

Effects of Recombinant Human Tissue Inhibitor of Metalloproteinases‐2 (rh‐TIMP‐2) on Migration of Epidermal Keratinocytes In vitro and Wound Healing In vivo

Tissue inhibitors of metalloproteinases (TIMP), common inhibitors of matrix proteinases, have cell‐promoting activity. We studied the effects of recombinant human tissue inhibitor of metalloproteinases‐2 (rh‐TIMP‐2) on the migration of normal human epidermal keratinocytes (NHEK). An in vitro migration assay revealed that rh‐TIMP‐2 enhanced random migration (up to 170%, p<0.05) in a dose‐dependent manner. When we applied rh‐TIMP‐2 solution (20 μg/20 μl/wound) daily to full‐thickness wounds made with an 8‐mm punch on the backs of healthy (n=8), aged (n=9), and diabetic (n=15) rodents, we observed faster wound closure (p<0.05) than in vehicle‐treated controls. Accelerated wound closure was dose‐dependent (0–20 μg/wound) in diabetic mice (n=6), and the optimal concentration was 10–20 μg of rh‐TIMP‐2/wound. Histological examinations performed on days 0, 5, 10, 15, and 20 in diabetic mice revealed faster migration of epidermal keratinocytes from wound edges. These results suggest that rh‐TIMP‐2 plays an important role in wound healing.

Improvement of adult Still's disease with granulocyte and monocyte adsorption apheresis.

Adult Stills disease is characterized by a high spiking fever, transient skin rash, and polyarthralgia. Joint pain is one of the major complaints and is often intractable. We assessed the efficacy of granulocyte and monocyte adsorption apheresis (GCAP) therapy for treating arthralgia in adult Stills disease. A 33‐year‐old woman with adult Stills disease who suffered from recalcitrant arthralgia resistant to systemic corticosteroids was treated with GCAP therapy. She underwent five GCAP treatments at 5‐day intervals. Her joint pain responded dramatically to the GCAP therapy, suggesting that GCAP may be useful for treating adult Stills disease. We present a detailed description of the patient and this novel therapy.

Gianotti-Crosti syndrome associated with endogenous reactivation of Epstein-Barr virus.

A 6-year-old girl with Gianotti-Crosti syndrome, which appeared to be caused by a reactivation of Epstein-Barr virus (EBV), is presented. The patient had had infectious mononucleosis at the age of 3 years. Since the titer of anti-EBV capsid antigen antibody was high at 1,280 and the titer of early antigen DR IgG, which increases during the early stage or reactivation, was high at 80 during the recovery stage, the patient was diagnosed as having Gianotti-Crosti syndrome associated with reactivation of EBV. Its clinical symptoms associated with reactivation of EBV were similar to those of that associated with primary EBV infection, in that the present patient had acrolocated papulovesicular dermatitis, superficial lymph node enlargement and mild hepatopathy. This patient provides valuable information in that reactivation was also observed with EBV like other types of herpesvirus.

A pediatric case of sclerodermatous chronic graft-versus-host disease.

Abstract: We report a rare case of sclerodermatous chronic graft‐versus‐host disease (GVHD) in a 6‐year‐old boy that occurred after bone marrow transplantation for his aplastic anemia. The clinical manifestation and histopathologic findings were typical of scleroderma. Although various kinds of treatment have been tried for scleroderma, no established therapy exists. Furthermore, treating this disease is even more difficult in children. In the future, clarification of the pathogenesis of chronic GVHD and establishment of therapy will be necessary.

Classical juvenile pityriasis rubra pilaris in a patient with Down syndrome

We report a case of classical juvenile pityriasis rubra pilaris (CJPRP) in a 15‐year‐old boy with Down syndrome. The rash was extensive, severe, accompanied by intense itching and responded to low‐dose etretinate. Xerosis and cheilitis were also present, suggesting that keratinization defects associated with Down syndrome may be involved in the pathogenesis of CJPRP. PRP is a rare dermatological disorder and to the best of our knowledge, this is the first reported case of CJPRP in a patient with Down syndrome.

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).

Facial edema as a clue to superior vena cava syndrome associated with lung cancer

ejd.2012.1743 Auteur(s) : Kenjiro Terasaki1 ken091362@ybb.ne.jp, Koichi Ohkubo2, Takuro Kanekura3 1 Terasaki Dermatological Clinic, 7-9 Nishioote, Kanoya 893-0008, Japan 2 Department of Radiology, Imakiire General Hospital, Kagoshima 892-0852, Japan 3 Department of Dermatology, Kagoshima School of Medicine, Kagoshima 890-8520, Japan A 76-year-old man presented with a 20-day history of edematous erythema of the face and bilateral eyelids, that was more severe on awakening and subsided during the [...]

Mondor's disease on the neck

Dear Editor, Mondor’s disease, first described by Mondor in 1939, is an inflammatory vascular disorder characterized clinically by a linear subcutaneous nodule most commonly affecting the thoracoepigastric, lateral thoracic or superior epigastric region. There is currently no consensus whether this disorder is of venous or lymphatic vessel origin. We recently encountered a rare case of Mondor’s disease developed in a cervical lymphatic vessel. This 20-year-old man was referred to our department for the evaluation of a subcutaneous induration on his left neck that was resistant to antibiotics prescribed by his internal medicine practitioner. On physical examination, a 5-cm long, Y-shaped, cord-like, moderately tender subcutaneous induration was detected (Fig. 1). Laboratory examinations of his blood including coagulation tests returned normal results. A biopsy specimen revealed that subcutaneous vessels were obstructed by organized thrombi and that the vessel walls were replaced by fibroblasts. Narrow recanalized lumens were seen in the area of organized thrombi (Fig. 2a). Weigert staining detected few elastic fibers in the affected vessels (Fig. 2b). Cells lining recanalized lumens and lymphatic vessels in the stroma were positive for D2-40, a specific marker for lymphatic endothelial cells (Fig. 3). Based on our clinical and histopathological findings, we made a diagnosis of Mondor’s disease arising from lymphatic vessels. Most patients with Mondor’s disease present with lesions on the chest wall; for example, the thoracoepigastric, lateral thoracic or superior epigastric region. Our review of the published work detected only two cases of Mondor’s disease of the neck. Although the etiology of this disorder is unclear, it may reflect the response of the local vasculature to trauma, muscular strain, febrile illness or contact dermatitis around the affected vessel. As our patient is a carpenter who regularly carries heavy loads on his left shoulder that put strong pressure on the affected site on his neck, we posit that this occupational factor may underlie his development of Mondor’s disease at this unusual site. Whether Mondor’s disease arises from veins or lymphatic vessels remains unclear. In our case, immunohistochemical study showed positive