Koji Uede

Brooke‐Spiegler Syndrome Associated with Cylindroma, Trichoepithelioma, Spiradenoma, and Syringoma

Brooke‐Spiegler syndrome is a rare, autosomally dominant disease characterized by the development of multiple cylindromas, trichoepitheliomas, and occasional spiradenomas. We report herein a case of Brooke‐Spiegler syndrome in a 55‐year‐old woman associated with multiple cylindromas, spiradenomas, trichoepitheliomas, and syringomas that developed on her face. Her 52‐year‐old sister also suffered from multiple trichoepitheliomas. Although there is variable clinical and histological expression in Brooke‐Spiegler syndrome, all of the tumors encountered in our case were related to folliculosebaceous‐apocrine units.

Skin manifestations in acute arsenic poisoning from the Wakayama curry‐poisoning incident

Background  Four people died and 63 others became ill after eating arsenic‐laced curry served at a community festival in Wakayama, Japan, on 25 July 1998. Although dermatological manifestations after the acute ingestion of arsenic have seldom been documented, they were observed in 56% of the victims in the Wakayama curry‐poisoning incident.

Effects of alpha-hydroxy acids on the human skin of Japanese subjects: The rationale for chemical peeling

Alpha‐hydroxy acid (AHA) agents, such as glycolic acid and lactic acid, have been used as therapeutic agents for more than a quarter of a century. Recently, they have been used as agents to rejuvenate photo‐aged skin. It is believed that these AHA agents induce the epidermis to remodel and accelerate desquamation, thus exerting their therapeutic effects. In this study, we investigated the histological differences in skin treated with glycolic, lactic, citric and acetic acids once daily for 6 weeks. The melanin pigments in the basal layer were less prominent in the glycolic and lactic acid‐treated skin than in the citric and acetic acid‐treated skin. The melanin deposits in the horny layers were equal for all AHA. However, the melanin deposits in the squamous layers were less prominent in the glycolic and lactic acid‐treated skins than in the citric and acetic acid‐treated skins; this was analogous to observations of the basal layers. Collagen I and procollagen I were increased after treatment with glycolic, lactic and citric acid in the upper dermis, but were not increased with acetic acid treatment. However, the staining of the epidermis and dermis for matrix metalloproteinase‐1 (MMP‐1) after treatment was not significantly different among the agents. Our data suggest that longer treatment intervals with glycolic and lactic acid can cause improvements in both the epidermal and dermal components and support the usefulness of AHA for rejuvenating photo‐damaged skin.

Susceptibility of T cell receptor-α chain knock-out mice to ultraviolet B light and fluorouracil: a novel model for drug-induced cutaneous lupus erythematosus

The anticancer agent 5‐fluorouracil (FU) frequently induces cutaneous lupus erythematosus (LE) lesions on sun exposed sites. Based on this observation, we have tried to establish a cutaneous LE model of C57BL/6 J (B6) mice, B6 T cell receptor (TCR)‐α–/– mice and B6 TCR‐δ–/– mice treated with FU and/or ultraviolet B light (UVBL) in order to clarify the role of T cells and the cytokine profile of cutaneous lupus lesions. Cutaneous LE‐like skin lesions could be induced in TCR‐α–/– mice with low FU (0·2 mg) plus UVBL, and in B6 mice treated with a high dose of FU (2·0 mg) plus UVBL. In contrast, low FU plus UVBL induced such skin lesions in TCR‐δ–/– mice at a very low incidence. Specifically, the skin lesions of TCR‐α–/– mice with low FU plus UVBL appeared more rapidly and were more severe than lesions in B6 mice. The former had the common characteristic features of human chronic cutaneous LE such as typical histology, positive IgG at the dermoepidermal junction, low antinuclear antibody and low mortality. Furthermore, a Th1 response was induced in the development of drug‐induced cutaneous LE. FU and UVBL‐induced cutaneous LE‐like eruption is an excellent model for better understanding the pathomechanisms of skin lesion development in LE.

Immunohistochemical staining of cutaneous tumours with G-81, a monoclonal antibody to dermcidin

Background  Recently, the novel antimicrobial peptide named dermcidin (DCD) was reported in human eccrine sweat glands.

Clinical effects of undershirts coated with borage oil on children with atopic dermatitis: A double-blind, placebo-controlled clinical trial

It has been reported that γ‐linolenic acid contained in borage oil is effective against atopic dermatitis. The clinical effects of undershirts coated with borage oil rich in γ‐linolenic acid on atopic dermatitis were evaluated. Thirty‐two children, aged 1–10 years, were involved in the clinical control study. Sixteen had worn undershirts coated with borage oil everyday for 2 weeks, and 16 had worn non‐coated undershirts as a placebo. Their symptoms were assessed on a 4‐point scale. Those children who had worn undershirts coated with borage oil for 2 weeks showed improvements in their erythema and itch, which were statistically significant. Transepidermal water loss from the back was decreased. In the placebo group, there were no statistically significant differences. The undershirts coated with borage oil were found to be statistically effective, and had no side‐effects on children with mild atopic dermatitis.

A New Infant Case of Nakajo-Nishimura Syndrome with a Genetic Mutation in the Immunoproteasome Subunit: An Overlapping Entity with JMP and CANDLE Syndrome Related to PSMB8 Mutations

Nakajo-Nishimura syndrome (NNS) is a very rare hereditary autoinflammatory disorder that generally has its onset in infancy with pernio-like rashes and gradually develops into partial lipodystrophy. A distinct homozygous PSMB8 mutation encoding an immunoproteasome subunit has recently been identified as its genetic cause. Here, we report a new case of a patient with NNS who developed exudative erythemas on his face and extremities at 2 months of age, along with high fever, elevated serum hepatic aminotransferase levels and hepatosplenomegaly. Massive infiltration of inflammatory cells was observed histologically in the dermis and subcutis without apparent leukocytoclastic vasculitis. These symptoms improved with oral corticosteroids but recurred periodically, and a thin angular face with long clubbed fingers gradually developed. Identification of the PSMB8 mutation finalized the diagnosis of NNS at 5 years of age. Understanding a variety of clinicopathological features at the developmental stages is necessary to make an early diagnosis of NNS.

Effects of sarpogrelate hydrochloride on skin ulcers and quality of life in patients with systemic sclerosis

5‐Hydroxytryptamine 2A serotonin receptor (5‐HT2A) is associated with the contraction of vascular smooth muscle, platelet aggregation and thrombus formation and coronary artery spasms. Sarpogrelate hydrochloride (sarpogrelate) is a selective 5‐HT2A antagonist and was supposed to be effective for Raynaud’s phenomenon with collagen disease. Sarpogrelate has not been investigated regarding the effects, safety and quality of life (QOL) in patient with skin ulcers of collagen disease. Eleven patients with skin ulcers and systemic sclerosis (SSc) were administrated sarpogrelate p.o. three times a day for 3–6 months. The area (mean ± standard error) of skin ulcer at the pretreatment, and after 3 and 6 months of sarpogrelate intake was 2.1 ± 0.8, 0.2 ± 0.1 and 0.1 ± 0.1 mm2, respectively. The reduction of skin ulcer area was significant after 3 months of sarpogrelate intake. In assessment of QOL, scores of symptoms and emotions but not of functioning were significantly improved after sarpogrelate intake. The global score (mean ± SE) of Skindex‐16 at pretreatment, and after 3 and 6 months of sarpogrelate intake was 31.8 ± 8.7, 23.7 ± 8.3 and 10.9 ± 4.6, respectively. The score was significantly improved after 6 months of sarpogrelate intake. There were no obvious side‐effects during this study. Sarpogrelate was considered to be a useful drug to improve skin ulcers and QOL in patients with SSc.

Expression patterns of proliferating cell nuclear antigen in trichloroacetic acid peeled skin

This study was designed to investigate whether trichloroacetic acid (TCA) peeling induces cellular proliferation in human skin using an immunohistochemical method. A 40% TCA peel resulted in a greater number of proliferating cell nuclear antigen (PCNA)‐immunopositive cells in the whole epidermis as compared with 60% TCA or phenol peels. This finding suggests that long‐term and frequent TCA peelings of low concentration would require special attention for unexpected cutaneous lesions such as skin tumors.

A case of symmetrical leukemia cutis on the eyelids complicated by B-cell chronic lymphocytic lymphoma

We describe herein a case of symmetrical bilateral leukemia cutis on the eyelids, especially on the lower lids, in a patient with B‐cell chronic lymphocytic leukemia (CLL). The patient was a 63‐year‐old Japanese man who had been diagnosed with CLL. Eyelid swelling is well known to result from local skin or systemic diseases including tumors, hyperthy‐roidism, nephritic syndrome, and hypoalbuminemia. To our knowledge, symmetrical, bilateral, flesh‐colored leukemia cutis on the eyelids has not been previously reported.