You-Won Choi

The expression of RAGE and EN-RAGE in leprosy

Background  Extracellular newly identified RAGE‐binding protein (EN‐RAGE) is a ligand of the receptor for advanced glycation endproducts (RAGE) and has been termed S100A12. The ligation of EN‐RAGE with RAGE on the endothelium, mononuclear phagocytes and lymphocytes triggers cellular activation with the generation of the key proinflammatory mediators interleukin (IL)‐1β and tumour necrosis factor (TNF)‐α.

Pseudoxanthoma elasticum-like papillary dermal elastolysis developed in early middle age

Pseudoxanthoma elasticum‐like papillary dermal elastolysis is a rare acquired elastolytic disorder characterized by papules that resemble pseudoxanthoma elasticum, and it typically affects elderly women. Histopathological examination shows atrophic epidermis and band‐like loss of elastic tissue in the papillary dermis. The pathogenesis is assumed to be related to intrinsic aging because it affects elderly people and shows the loss of elastic tissue. We report a case of pseudoxanthoma elasticum‐like papillary dermal elastolysis in early middle age presenting typical clinical and histopathological findings. The patient was a 41‐year‐old woman who had had her lesions for 10 years. We propose that younger patients, hitherto unknown, can be affected by this disorder and suggest that mechanisms other than intrinsic aging are involved in its pathogenesis.

Metal allergy in eyelid dermatitis and the evaluation of metal contents in eye shadows.

Allergic contact dermatitis is a common diagnosis in eyelid dermatitis. Sensitization to metals is prevalent in eyelid dermatitis and colour cosmetic products are frequently suspected as the source of metal exposure.

A Case of Sjögren's Syndrome That Presented with Alcohol-induced Purpura

Sjögren syndrome (SS) is a systemic autoimmune disease that mainly affects the salivary and lacrimal glands. It may exist as a primary condition or in association with other systemic autoimmune diseases. Patients with SS usually complain of persistent dryness of the mouth and eyes and other features, including diverse general symptoms and cutaneous symptoms such as purpura. We report here on a case of 34-year-old woman who presented with purple non-blanching palpable purpura on both lower legs, and these lesions had developed soon after drinking alcohol 2 days previously. She had a 2 year history of repeatedly developing rashes in association with drinking alcohol. The physical examination showed dry eyes and dry mouth. The laboratory tests showed positivity for anti-Ro/SS-A antibody and RF and hyperimmunoglobulinemia. She was diagnosed as suffering with primary SS. Herein we report on a patient with primary SS and this patient initially presented with recurrent purpura in association with alcohol ingestion. Drinking alcohol had played a role as a possible aggravating factor for the cutaneous purpura of this patient with SS.

Cystic sebaceous tumour with no evidence of Muir‐Torre syndrome

Editor Cystic sebaceous tumours are distinct sebaceous neoplasm presenting as well-circumscribed dermal sebaceous proliferations with a cystic growth pattern. They have been characterized as marker lesions of Muir-Torre syndrome (MTS). However, several cystic sebaceous tumours which are not associated with MTS have also been described. A 56-year-old Korean woman presented with a brownish nodule on the upper back which lasted for several years. No family history of colon cancer was remarked. Physical examination revealed an asymptomatic well-demarcated nodule on the upper back (Fig. 1). Microscopy showed a cystic tumour filled with homogenous eosinophilic material in the dermis (Fig. 2a). Basaloid and sebaceous cells were lining the cyst and differentiation of basaloid cells into sebaceous cells towards the centre was observed (Fig. 2b). The cystic tumour was filled with homogenous eosinophilic materials with clefts and disintegrated sebaceous cells, most likely the products of holocrine secretion. Focal adenomatous area of basaloid cells was noted along the wall. EMA staining highlighted mature sebaceous cells, but CEA and GCDFP-15 stainings showed no immunoreactivity. The differential diagnosis included steatocystoma but eosinophilic cuticle lining along the surface suggestive of steatocystoma was not observed. There was no architectural or cytologic atypia, so that the tumour was regarded as a benign cystic sebaceous tumour. In the evaluation for visceral malignancies, including colonoscopy, abdominal and gynaecological ultrasonography, the patient had no abnormalities. The immunohistochemistry for DNA mismatch repair gene expression, MSH2 and MLH1, was performed and showed positive nuclear expression of both proteins (Fig. 2c,d). Furthermore, molecular genetic analysis of the tumour exhibited microsatellite stability. Muir-Torre syndrome is an autosomal dominant inherited syndrome which predisposes to the development of internal malignancies and cutaneous neoplasms. The cutaneous neoplasms include a variety of benign and malignant sebaceous tumours and keratoacanthomas. The sebaceous tumours of MTS are typically adenomas, sebaceomas/sebaceous epitheliomas, or carcinoma. However, unusual architectural patterns, such as cystic structures, solid basaloid sheets, mucinous areas and convoluted glands, can be observed. Cystic sebaceous tumours are uncommon and have been claimed to be highly predictive of MTS. However, cystic sebaceous tumours clinically not associated with MTS had also been illustrated. Muir-Torre syndrome is considered a subset of hereditary non-polyposis colorectal cancer and caused by familial germline mutations in the DNA mismatch repair genes, most often MSH2 or MLH1. Accordingly, detection of microsatellite instability in sebaceous tumours by immunohistochemistry of MSH2 and MLH1 has been suggested to be a reliable screening method, manifesting microsatellite instability as an absence of nuclear staining. Cystic sebaceous tumours not only in MTS patients but also in patients with no clinical evidence of internal malignancy demonstrated no expression of DNA mismatch repair genes, one or more of them, in immunohistochemistry. Even germline mutation of MLH1 was found in a patient with cystic sebaceous tumour without any internal tumour. Strong aetiological relationship between MTS and cystic sebaceous tumour was suggested so that screening for internal malignancies was recommended in individuals presenting with cystic sebaceous tumour and showing microsatellite instability in immunohistochemistry or genetic testing. However, this tumour showed positive nuclear staining in both MSH2 and MLH1 immunohistochemistry and no microsatellite instability was found in the genetic study. To our knowledge, this is the first case of cystic sebaceous tumour revealing no microsatellite instability. The present case suggests that a cystic sebaceous tumour may not be a consistent marker for MTS. Nonetheless, the question whether this tumour is an isolated form with no association with MTS or the initial presenting symptom of MTS needs to be determined in the future. Figure 1 A brownish nodule was observed on the upper back.