We have demonstrated for the first time that a second-generation antihistamine ameliorates nocturnal scratching behavior in atopic dermatitis patients using a modified wristwatch-type acoustic scratching counting system that we have recently developed. We also analyzed the sleep quality by simultaneous recording of electroencephalogram, and found that sleep quality was unaffected.
Primary cutaneous apocrine carcinoma (PCAC) is a rare neoplasm of skin appendages. To determine the differentiation of apocrine carcinoma, we studied the expression of epithelial keratins and filaggrin immunohistochemically using 10 anti-keratin antibodies againt keratin (K) 1, 7, 8, 10, 14, 15, 16, 17, 18, 19 and the anti-filaggrin antibody. PCAC demonstrated strong positivity for K7, K8, K18 and K19. These keratins are distributed in secretory cells of normal apocrine glands. The tumor cells were negative for K14 and K17. The two keratins exist in myoepithelial cells in normal apocrine glands. Results suggest that PCAC shows differentiation into secretory cells of apocrine glands, although it does not differentiate into myoepithelial cells. K14 is also known as undifferentiated keratin, whereas K17 is considered to be a hyperproliferative keratin. Absence of the expression of K14 and K17 may reflect an indolent clinical course of PCAC.
Abstract Pathogenic variants in the HIBCH gene cause HIBCH deficiency, leading to mitochondrial disorders associated with valine metabolism. Patients typically present with symptoms such as developmental regression/delay, encephalopathy, hypotonia and dystonia. Brain magnetic resonance imaging (MRI) shows bilateral lesions in the basal ganglia with/without brainstem involvement. Here, we report a case of a Japanese patient with Leigh-like syndrome caused by novel HIBCH variants. Long-term follow-up MRI revealed progressive cerebellar atrophy, which expands the phenotypic spectrum of HIBCH deficiency.
Recent advances in bioengineering have introduced materials that enhance wound healing. Even with such new tools, some deep ulcers surrounded by avascular tissues, including bone, tendon, and fascia, are resistant to various therapies and easily form deep cavities with loss of subcutaneous tissue. Atelocollagen sponges have been used as an artificial dermis to cover full-thickness skin defects. Topical recombinant human basic fibroblast growth factor has been introduced as a growth factor to induce fibroblast proliferation in skin ulcers. We applied these materials in combination in two patients with deep resistant wounds: one with a cavity reaching the mediastinum through a divided sternum and one with deep necrotic wounds caused by electric burns. These wounds did not respond to the topical basic fibroblast growth factor alone. In contrast, the combination therapy closed the wounds rapidly without further surgical treatment. This combination therapy is a potent treatment for resistant wounds with deep cavities.
Abstract Azathioprine (AZA) is an immunosuppressant commonly used for organ transplantation and autoimmune diseases. Allergic side effects of AZA are rare, and reported allergic skin eruptions from AZA are very limited in Japan. We report AZA‐induced drug eruption that developed in two cases of systemic scleroderma with polymyositis. One case presented with Stevens‐Johnson syndrome, and the other had systemic papular erythema. The stimulation indices of the drug‐induced lymphocyte stimulation test (DLST) for AZA in these two patients were as high as 2,180% and 430%, respectively, but those of healthy volunteers were under 120% without nonspecific suppression of lymphocyte proliferation. Other drugs used simultaneously were ruled out by patch and challenge tests. The challenge test for Stevens‐Johnson syndrome type drug allergy is very risky. DLST is a good diagnostic tool for AZA allergy, especially for severe drug allergy cases.
We report the use of topical application of recombinant human basic fibroblast growth factor (rhbFGF) to successfully treat therapy-resistant, chronic leg ulcers in scleroderma. Endothelial cell FGF receptors are directly stimulated by bFGF; also, bFGF promotes the regeneration of capillary-rich granulation tissue. We conclude that topical bFGF may be a powerful new pharmacologic tool for treating severe skin ulcers.
Abstract A 42–year‐old woman developed severe erythema with exfoliative scaling on the bilateral palms and soles and erosive dermatitis on the axillae and groin eight days after an autologous peripheral blood stem cell transplantation for the treatment of non‐Hodgkin's lymphoma. She also developed exanthema; however she did not show intestinal, hepatic, or renal involvement. The skin biopsy revealed characteristic apoptotic cell death of the epidermis with eosinophilic necrosis, and she was diagnosed with acute graft‐versus‐host disease (GVHD). The cutaneous lesions responded to topical corticosteroid treatments and improved within a month without systemic immunosuppressing therapies. The cutaneous GVH reaction did not recur. However, she was treated with an intermittent thrombocyte transfusion because of persistent thrombocytopenia. On day 130, she developed intestinal pneumonia and died due to respiratory dysfunction. Unlike an allo‐bone marrow graft, GVHD after an autologous stem cell transplantation is not common. Even for an autologous transplantation, GVH may develop with less characteristic clinical manifestations.
Extraocular sebaceous carcinoma of the skin is uncommon neoplasm. We report a case of sebaceous carcinoma with gastric diffuse large B cell Lymphoma. A 76year old man had a ulcerated tumor measuring 15×15mm in size on his cheek. He was pointed out gastric diffuse large B cell Lymphoma at the same time. Two times of local excision had been performed, but he had a local recurrence. We tried radiotherapy that resulted in a complete remission of the skin region. [Skin Cancer (Japan) 2001; 16: 89-92]
Epidermotropic metastatic malignant melanoma (EMMM) is a form of metastatic malignant melanoma that has dermal cell nests with epidermotropism and specific histopathological features. We report a patient with eight nodular lesions of the scalp with histopathological findings compatible with EMMM. The tumors developed one year before consultation and increased in size simultaneously. The histopathological findings of all eight tumors were very similar. The tumor cells were located mainly in the dermis and partly in the basal layer of the epidermis. They contained melanin pigments and were positive for anti-HMB45 antibody. The tumors did not respond to combination chemotherapy with dacarbazine, nimustine, vincristine, and interferon-beta. Therefore, all the tumors were surgically removed. No local relapse, distant metastasis or re-elevation of plasma 5-S-cysteinyldopa was identified during nine months of follow-up. Histopathologically, all eight tumors lacked apparent vascular invasion, which may be related to a slow clinical course of the present case.
