Hirohisa Ishibuchi

Induction of matrix metalloproteinase-1 by small interfering RNA targeting connective tissue growth factor in dermal fibroblasts from patients with systemic sclerosis.

Please cite this paper as: Induction of matrix metalloproteinase‐1 by small interfering RNA targeting connective tissue growth factor in dermal fibroblasts from patients with systemic sclerosis. Experimental Dermatology 2010; 19: e111–e116.

Successful treatment with cyclosporin administration for persistent benign migratory glossitis

We herein describe a 54 year‐old female patient with a 5‐year history of persistent and painful benign migratory glossitis (BMG), which was remarkably improved by systemic administration of cyclosporin. She had noted some white patches leaving smooth denuded red areas with whitish elevated borders on the dorsum of her tongue, and finally felt strong pain. The lesion was refractory to the previous treatment with topical corticosteroid treatment for the last 2 years. Because clinicopathological findings were compatible with BMG, systemic administration of 20 mg/day prednisolone and topical 0.1% dexamethasone application were started, however, she suffered a severe relapse after tapering the dosage of predonisolone to 10 mg/day. Because some investigations have suggested that BMG is an oral manifestation of psoriasis, we introduced cyclosporin administration. The systemic treatment of cyclosporin microemulsion pre‐concentrate, 3 mg/kg/day, resulted in a satisfactory improvement. Two months later, we could reduce cyclosporin microemulsion pre‐concentrate dosage to 1.5 mg/kg/day for maintenance therapy, and the disease has been well controlled so far.

Clinical usefulness and patient satisfaction for treatment with low-dose cyclosporin administration in patients with moderate psoriasis vulgaris

The Japanese guidelines for psoriasis therapy with cyclosporin microemulsion preconcentrate (CyA MEPC) has been revised, and the clinical application of CyA MEPC is being expanded to include mild to moderate psoriasis. In this study, we aimed to confirm the clinical efficiency of low‐dose cyclosporin therapy in patients with moderate psoriasis vulgaris. After informed consent was obtained, 19 patients with psoriasis vulgaris were enrolled in this study. Each patient basically administrated CyA MEPC, 2.5 mg/kg/day, orally over 12 weeks. When the psoriasis area and severity index (PASI) score showed a 75% reduction from the initial value, the dosage of CyA MEPC was reduced to 1.5 mg/kg/day and added a topical application of active vitamin D3 ointment. We interviewed the patients as to their satisfaction for the usefulness and cost of the treatment. All patients obtained improvement within 12 weeks. In 10 patients whose PASI score reduced over 75%, we could reduce CyA MEPC dosage. No adverse effects were noted in any patients during the treatment. It is of note that the cost for 1.5 mg/kg/day administration of CyA MEPC was accepted by all the patients. In conclusion, this preliminary study suggests that the CyA MEPC is effective, safe and would provide patients with acceptable costs.

Extramammary Paget's Disease with Bowenoid Histologic Features Accompanied by an Ectopic Lesion on the Upper Abdomen

We present a 79‐year‐old man who suffered from extramammary Pagets disease (EMPD) with bowenoid histological features accompanied by an ectopic EMPD lesion on his abdomen. He had had an erythematous plaque on his genital region for three years. Based on a biopsy specimen, he was referred to our hospital with the histological diagnosis of Bowens disease. The histological findings of the genital lesion obtained by surgical resection showed typical areas of Pagets cells adjacent to areas characteristic of Bowens disease. Immunohistochemical findings showed CEA and CK7 positive tumor cells in both areas, so the atypical cells showing the bowenoid pattern could be regarded as tumor cells of Pagets disease. Immunohistochemical staining for CEA and CK7 along with multiple biopsies can be helpful in making the diagnosis of Pagets disease with bowenoid histologic features.

Successful treatment with dapsone for skin lesions of amyopathic dermatomyositis.

1 Nofal A, Assaf M, Ghonemy S, Nofal E, Yosef A. Generalized eruptive keratoacanthoma: a diagnostic and therapeutic challenge. Int J Dermatol 2015; 54: 160–167. 2 Haas N, Schadendorf D, Henz BM. Fuchsn PG Nine-year follow-up of a case of Grzybowski type multiple keratoacanthomas and failure to demonstrate human papillomavirus. Br J Dermatol 2002; 147: 793– 796. 3 Dessoukey MW, Omar MF. Abdel-Dayem H Eruptive keratoacanthomas associated with immunosuppressive therapy in a patient with systemic lupus erythematosus. J Am Acad Dermatol 1997; 37: 478– 480. 4 Snider BL, Benjamin DR. Eruptive keratoacanthoma with an internal malignant neoplasm. Arch Dermatol 1981; 117: 788–790. 5 Consigli JE, Gonz alez ME, Morsino R et al. Generalized eruptive keratoacanthoma (Grzybowski variant). Br J Dermatol 2000; 142: 800– 803.

Transitory pigmented purpuric dermatoses in a young Japanese female

We report a 23‐year‐old female patient with a 4‐month history of transitory pigmented purpuric dermatoses (PPD). She was otherwise healthy and reported no history of previous medication intake and none of her family members had any disorders. Clinical examination revealed well‐demarcated, brownish hyperpigmented, reticulated pigmentation with pinhead‐sized purpura. The histopathological specimen was characterized by a mild epidermal hyperkeratosis, elongated rete ridges, papillomatosis and mild mononuclear cell infiltration in the superficial dermis with focal extravasations of red blood cells without siderophage. Despite prominent extravasations of red blood cells and edema both in the papillary dermis and the subpapillary layer, no definite capillaritis was seen. Based on these clinicohistopathological findings, the diagnosis of transitory PPD was considered to be most compatible. Clinicians should recognize the unique but rarely acknowledged disease as a subtype of pigmented purpuric dermatoses.

Localized cutaneous immunoglobulin light chain kappa-positive amyloidosis associated with juvenile dermatomyositis

Dear Editor, A 19-year-old Japanese woman was referred to our department with a 17-year history of erythema on the face, neck, hand and back. When she was 2 years old, she was diagnosed with infantile dermatomyositis based on skin manifestations and muscle weakness. Systemic corticosteroid (20 mg/day) was started. Because symptoms were improved, systemic corticosteroid was discontinued at 6 years old. Although topical corticosteroid was applied for several years, her skin lesions did not improve. Physical examination revealed dark diffuse erythema in the face, posterior neck and lower back (Fig. 1a,b). Hypopigmented areas were associated with the lichenoid eruption. Periungual erythema, Gottron’s papules and Gottron’s sign were present (Fig. 1c). Muscle weakness and pain were not detected. Results of laboratory examination included normal levels of creatine kinase and C-reactive protein. KL-6, a lung fibrosis marker, was 197 U/mL (normal, <500 U/mL). Antinuclear antibody was negative. Anti-TIF1 antibody was detected. AntiRNP, anti-Sm anti-dsDNA, anti-SS-A/Ro, anti-SS-B/La, antiJo-1, anti-aminoacyl-transfer RNA synthetase autoantibodies and anti-melanoma differentiation-associated gene 5 antibody were not detected. No interstitial lung disease and internal malignancy were detected. Histopathological examination of the posterior neck showed hyperkeratosis, acanthosis and thick deposits of an eosinophilic, homogeneous substance in the papillary dermis (Fig. 1d), which were positively stained with Congo red and direct fast scarlet (Fig. 1e). Immunohistochemical staining of the amyloid deposited lesion resulted in positive staining with anti-immunoglobulin light chain kappa (Fig. 1f) and antiserum amyloid P antibodies, but negative staining with antikeratin (Fig. 1g) and anti-transthyretin antibodies. Direct immunofluorescence was negative. Multiple myeloma was excluded by normal levels of urinary Bence Jones protein and the ratio of j/k immunoglobulin free light chain (0.52; normal, 0.31–1.56), no serum M-protein, and no submucosal amyloid deposits by rectal biopsy. Diagnosis was established of secondary localized cutaneous amyloidosis associated with juvenile dermatomyositis. Localized cutaneous amyloidosis includes primary cutaneous amyloidosis, which is the absence of systemic involvement, and secondary cutaneous amyloidosis, which is accompanied by skin neoplasms and systemic diseases, such

Distinct clinical and histological features in dermatomyositis with anti-aminoacyl-tRNA synthetase antibodies.

pacitating right foot pain. She presented with bullous eruption with red halo on her nose, anterior chest and abdomen (Fig. 1a). Also, her right thigh was swelling with a band of bullous eruption into near-circumferential erythema (Fig. 1b). She was diagnosed as having disseminated herpes zoster with atypical features. Computed tomography showed no abnormality although she complained of acute pain in her right upper thigh. Clinical laboratory investigation revealed no inflammatory response. She was treated with oral valacyclovir 500 mg three times daily for 7 days. The serological study of anti-varicella zoster virus (VZV) antibody during the acute stage revealed slight elevation of immunoglobulin (Ig)M (3.02 mL; normal, <0.8 mL) and high elevation of IgG (>128.0 mL; normal, <2.0 mL). Fifteen days after treatment, she complained of soreness and numbness in her left leg. We consulted an orthopedic surgeon for advice on her complaint. The magnetic resonance imaging (MRI) found that she suffered from a ruptured disk in the lower spine (Fig. 1c,d). Her symptoms were those of lumbar disc hernia (LDH) in the left L4 and L5 dermatomes. Herein, we reported a rare case of LDH in the left L4 and L5 dermatomes after herpes zoster infection in the right L2 and L3 dermatomes. It was reported that intervertebral disc degeneration may be caused by VZV. It seemed that our patient had pre-existing spinal canal stenosis between L3 and L4, and also L4 and L5 spinal column, by using X ray and MRI of the spine. She was affected by the reactivation of latent VZV in the right L2 lesion in this course. The distance between the L2 and L3 posterior root ganglia decreased because she had spinal canal stenosis in the region innervated by nerves running from the spinal column. As a result, L2 nerves inflamed by VZV spread to the L3 nerves lesion. Therefore, the swelling on the right side of the inflamed nerves increased stress on the left side of the spine. As a result, the right upper side of the spine was overloaded with the left lower side of the spine. For these reasons, we considered that LDH in the left L4 and L5 dermatomes was caused by herpes zoster infection in the right L2 and L3 dermatomes.

Methotrexate-associated lymphoproliferative disorder: Sequential development of angioimmunoblastic T-cell lymphoma-like lymphoproliferation in the lymph nodes and diffuse large B-cell lymphoma in the skin in the same patient.

A 66-year old Japanese woman was diagnosed with polymyalgia rheumatica in October 2012 and was treated with methotrexate (MTX) at a dose of 10mg/week. Swelling of the inguinal lymph nodes appeared in February 2013. Histological examination of the lymph nodes revealed numerous, medium-to-large, atypical mononuclear cells with irregular nuclei (figure 1A). Increased numbers of CD21+ follicular dendritic cells were observed around the high endothelial venules (figure 1B). The neoplastic cells stained [...]

A perianal erythematous plaque: a quiz. Secondary extramammary Paget's disease from adenocarcinoma of the anorectal region.

An 83-year-old man with a 4-month history of an itchy perianal lesion was referred to our hospital. A physical examination revealed a round, well-demarcated, erythematous, erosive plaque involving the perianal skin (Fig. 1a). The histopathological examination of the patient’s lesion (Fig. 1b) revealed the presence of atypical tumour cells with an abundant pale-staining cytoplasm and large atypical nuclei in all layers of the epidermis. Immunohistochemically, the tumour cells were positive for carciA Perianal Erythematous Plaque: A Quiz