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Schnitzler syndrome (SchS) is a rare autoinflammatory disease characterized by bone pain, recurrent fever, leukocytosis, and elevated C-reactive protein, along with an urticaria-like rash and monoclonal immunoglobulin (Ig)M or IgG gammopathy. Notably, the condition is distinguished by a relatively persistent recurrent urticarial-like rash. Histopathological features observed in the skin comprise diffuse neutrophil infiltration into the dermis, absence of dermal edema, and vascular wall degeneration, all of which classify SchS as a neutrophilic urticarial dermatosis (NUD). Accumulated histological data from skin biopsies of patients with NUD have revealed a sensitive histopathological marker for NUD, acknowledged as neutrophilic epitheliotropism, which has been proposed as reflecting an autoinflammatory condition. In this report, we present three SchS patients: two men (ages 55 and 68) and a woman (age 75), all displaying neutrophilic epitheliotropism in their skin biopsy specimens. Additionally, a review of eight previously reported SchS cases in Japan identified neutrophilic epithliotropism in five cases. These findings suggest that the inclination of neutrophils toward the epithelial tissue could aid in confirming diagnoses of NUD in most cases that need to be differentiated from conventional urticaria. Consequently, we emphasize that acknowledging neutrophilic epithelial predilection as a hallmark of NUD is critical for expediting early diagnosis and appropriate treatment for SchS., (© 2023 The Authors. The Journal of Dermatology published by John Wiley & Sons Australia, Ltd on behalf of Japanese Dermatological Association.)

Pigmented cosmetic dermatitis-like (Riehl's melanosis-like) pigmentation was reported in three of 27 patients with primary Sjögren's syndrome. But case reports of such eruptions are rare. We describe three cases of such eruptions associated with primary Sjögren's syndrome or anti-SSA antibody and possible associations with specific types of human leukocyte antigen (HLA) and infiltrating lymphocytes. These middle-aged Japanese women had reticular facial pigmentation and histopathological examination revealed interface dermatitis, melanophages, and dense lymphocytic infiltration around hair follicles and sweat ducts. HLA typing revealed common antigenic equivalents or genetic typing of HLA-A2, DR52, DPA1(02:02) and DPB1(05:01). Immunohistochemical staining revealed major subsets of T cells to be CD8 and CD45RO. Some Foxp3- and few IL17-positive cells were found in strong contrast to the major CD4 subset of infiltrated T cells in annular erythema associated with Sjögren's syndrome. Apparently, our patients' pigmentation represented a specific etiology associated with primary Sjögren's syndrome or anti-SSA antibody., (© 2016 Japanese Dermatological Association.)

We performed skin cancer screenings for 2 or 3 days annually from 2006 through 2013 in Oita Prefecture, Japan. Screening of approximately 3000 people in total allowed us to identify and treat several skin cancers, including five cases of malignant melanoma, four of squamous cell carcinoma, 16 of basal cell carcinoma, 11 of Bowen's disease, 17 of actinic keratosis, one of extramammary Paget's disease and one of metastatic breast carcinoma. The sensitivity and specificity for the category defined by an identified lesion associated with risk of cancer and requiring further examination (category C) were 92.7% and 95%, respectively. We cannot estimate the outcome of our skin cancer screenings in terms of cancer mortality because of the small number of subjects examined and the brief follow-up period. However, we did estimate the effectiveness of these screenings in terms of stages or sizes of cancerous lesions. The relative numbers of subjects with malignant melanoma at various clinical stages, identified during skin cancer screenings and during a routine visit to our hospital, were significantly different. We also compared, statistically, the sizes of lesions in Bowen's disease that were found during cancer screenings and during a direct visit to our hospital. The former lesions were smaller than the latter. Our data suggest the benefits of our skin cancer screenings and the importance of campaigns and education to encourage people to visit dermatologists for the detection of skin cancers at an early stage., (© 2015 Japanese Dermatological Association.)

Basal cell carcinoma (BCC) is a common skin cancer that arises from the cells of the basal layer of the epithelium or from the external root sheath of the hair follicle. In the present report, 256 cases treated surgically between 1999 and 2008 in our department were retrospectively analyzed. The most frequent BCC locations included the face (77.8%), especially the nose (26.9%) and eyelids (21.5%). Incomplete excisions occurred in 21 cases. Two patients experienced local recurrence; one of these patients exhibited a bone metastasis while the other had a metastasis of the parotid gland without the local recurrence. The rate of local BCC recurrence was 0.78%, which is lower than that described in previous reports. We categorized BCC into four histological types: superficial, solid, adenoid and infiltrative. The solid type was the most frequent histological type (62.1%). For preventive recurrence, we treated BCC patients with two-step surgery when the tumor was large or histologically invasive. At the first step, we excised the tumor with a sufficient safety margin, and at the second step, we performed reconstruction after the histological confirmation that no remnant malignant cells were in the tumor margins. In the present report, no local recurrence occurred in patients following the two-step surgery. Therefore, two-step surgery is recommended for tumors at locations and with histological types related to frequent recurrence., (© 2011 Japanese Dermatological Association.)

Drug-induced hypersensitivity syndrome (DIHS) is characterized by a serious adverse systemic reaction that usually appears after a 3-6-week exposure to certain drugs, for example, anticonvulsants. Many different precipitating factors have been reported, but the pathophysiology of DIHS remains unknown. However, reactivation of members of the human herpesvirus (HHV) family, and of HHV-6 in particular, has been reported in patients with DIHS. We report the case of a 64-year-old man who developed a generalized erythematous rash, fever, hepatic failure, lymphadenopathy and an increased number of atypical lymphocytes. In addition, reactivation of HHV-6 and cytomegalovirus (CMV) was demonstrated by real-time quantitative amplification by polymerase chain reaction. The patient was given a diagnosis of DIHS due to carbapenem antibiotics based on his clinical course, laboratory data, and results of lymphocyte-stimulation tests with various drugs. This is the first report, to our knowledge, of DIHS induced by carbapenem antibiotics.

The factors determining the severity of a Mamushi (Agkistrodon blomhoffii) bite were analyzed based on the findings of our 34 cases and those appearing in the published work. It was not possible to forecast the final severity at the time of the patient's arrival and by the initial blood examination data. The maximal creatinine kinase (CK) values elevated proportionally with the time from the bite, and the relation approximated the equation of y = 300chi, where y represents the maximal CK value and chi indicates the time from the bite to the peak of CK level. In the severe cases which required intensive care, the level of the CK deviated remarkably from this line, and could be grossly distinguished from the non-severe cases. The maximal white blood cell (WBC) count also gradually increased in concordance with the time from the bite, and in addition, the WBC count of most of the severe cases exceeded 20,000/microL. This evidence suggests that the rate of the CK value elevation in relation to the time from the bite can be a useful indicator of the severity of a Mamushi bite, and the WBC count also reflects the severity.

Antihistamines have been used for the treatment of not only allergic diseases such as allergic urticaria and rhinitis, but also of eczematous skin diseases because of their anti-pruritic effects. Moreover, the pruritus associated with eczematous diseases is considered to be induced, in part, by histamine. However, it is unclear whether antihistamines inhibit the itch of eczematous diseases in the absence of topical corticosteroids. In this study, we investigated the anti-pruritic effect of the antihistamine, fexofenadine, on the itch of contact dermatitis that was induced by topical application of diphenylcyclopropenone for the treatment for alopecia areata. Thirteen patients with alopecia areata, who had been treated weekly with topical immunotherapy with diphenylcyclopropenone for 3 months to 2 years, recorded the severity of their itching on a visual analog scale before and 3, 6, 12, 24, 48 and 72 h after application of diphenylcyclopropenone for 4 consecutive weeks. Seven patients took fexofenadine during the first and third weeks, and six patients took fexofenadine during the second and fourth weeks. The severity of itching reached a maximum 6-12 h after the induction of the contact dermatitis in most of the patients. However, fexofenadine partially but rapidly reduced the severity of itching for 72 h during the entire period of treatment in the absence of topical corticosteroids. Our results suggest that fexofenadine can be beneficial in the daily management of patients with itching due to eczematous disease.

We performed skin cancer screening from 2000 to 2004 at two locations in Japan's Oita Prefecture: Himeshima, a small fishing island, and Naoiri, an inland agricultural area. We found 108 and 21 cases of AK in Himeshima and Naoiri, respectively. None of the AKs transformed into SCC, and 21.7% of the AKs underwent spontaneous remission during our observation period. The prevalence and incidence of AK in Himeshima were five times higher than in Naoiri: 1,399 and 826 per 100,000 population, respectively, in the fishing village, vs. 261 and 164 in the agricultural community. Seven and three cases of BCC were observed in Himeshima and Naoiri, respectively. There were two cases of SCC in Himeshima. The highest risk ratio of skin types I to III was 9.2 in Himeshima. Although people engaged in outdoor occupations are thought to be more prone to skin cancer and precancerous skin lesions, our results suggested different potentials for AK in people engaged in different outdoor occupations.

We report a case of giant porokeratosis combined with ulcerative squamous cell carcinoma. In our patient, we biopsied the skin, including the edge of the skin lesion, four times until we obtained histologic proof of a cornoid lamella. After we had established the diagnosis, we totally excised the affected skin including a 10-mm safety margin, because such lesions tend to develop into skin cancer. We reconstructed the excised area using a skin graft. After the operation, we took 13 samples of skin (seven from the lower leg and six from the sole of the foot) from the edge of the main lesion, including a putative cornoid lamella. Five of the six samples from the sole and one of the seven from the lower leg were demonstrated histologically to include a cornoid lamella. Our results suggest that skin biopsies should be taken from various sites at the edge of a giant porokeratotic lesion and in particular from the prominent ridge to prove the presence of a cornoid lamella. Shallow keratin-filled invaginations and underlying squamous cells with eosinophilic cytoplasm were observed at the edge of the lesion on the lower thigh, which might suggest a diagnosis of porokeratosis with an incomplete cornoid lamella if porokeratosis was strongly suspected from the patient's clinical features. Better recognition of giant porokeratosis is required, so that an earlier diagnosis can be made and appropriate therapy initiated in a timely manner.

A rare case of dermatomyositis associated with primary intramuscular malignant lymphoma is described. A 40-year-old Japanese woman noticed swelling of the right thigh during the treatment of dermatomyositis with prednisolone, azathioprine and cyclophosphamide. A biopsy specimen taken from the right quadriceps muscle revealed infiltration of lymphoma cells which were positive for CD20. We reviewed 12 cases of dermatomyositis associated with malignant lymphoma reported in Japanese literature between 1984 and 1996.

A case of cutaneous Ki-1 positive anaplastic large cell lymphoma which developed in the plaque stage of mycosis fungoides was described. A 73-year-old woman who had suffered from pruritic scaly eruptions over her entire body for more than two decades was admitted because of an ulcerated tumor measuring 45 x 55 x 15 mm and several satellite tumors on the buttock. All tumorous lesions were resected without recurrence to date. Histochemical study revealed that the tumor consisted of large anaplastic cells which were Ki-1 (CD30)-positive and LCA-negative. Some of the erythematous plaques contained LCA-positive, small-sized atypical lymphocytes. In other plaques which developed two years later, there were large Ki-1-positive atypical cells. In the specimens obtained from the tumor and the plaque, the same pattern of T-cell receptor gene rearrangements was detected. These findings indicate that both Ki-1 positive anaplastic cells in the tumor and atypical lymphoid cells in the plaques were derived from the same T cell clone.

A 62-year-old woman with diabetic triopathy developed widespread erythematous macules, numerous pustules, and a high fever after she underwent electric coagulation for vitreous hemorrhage. She was administered several drugs at that time. After discontinuation of the drugs, the eruption disappeared, and the fever returned to normal within two weeks. A positive patch test with isepamicin sulfate highly suggested that the symptoms described above were due to drug allergy. Cadralazine, which was positive in the drug lymphocyte stimulation test (DLST), could not be excluded from the causative drugs. A false-positive DLST with ofloxacin was confirmed by an accidental challenge test. To our knowledge, this is the first report of acute generalized exanthematous pustulosis due to isepamicin sulfate and/or cadralazine.

A markedly obese, 41-year-old Japanese man who had suffered from psoriasis vulgaris for several years visited us with elephantiasis-like swelling of his lower legs of three months' duration. His right lower leg showed marked papillomatosis with thick scales, and the left lower leg was eroded and papillomatous. Although direct lymphography of his lower extremities showed no abnormality, indirect lymphography revealed local lymphatic damage in the involved skin. Histological examination showed hyperkeratosis, marked papillomatosis, proliferation of capillaries in the upper dermis, and lymphectasia in the lower dermis. The lesions were much improved by washing and topical use of corticosteroids for two months. It was suspected that obesity and the preceding psoriatic lesions caused local lymphatic disturbances, followed by the development of stasis papillomatosis.

Pyomyositis is a bacterial infection with abscess formation affecting large skeletal muscles. It is predominantly caused by Staphylococcus aureus. The disease is common in tropical areas, but rare in temperate climates. We report a patient with multiple myeloma who developed a giant elastic tumor on the right thigh and a hen egg-sized tumor on the right upper arm. MR imaging revealed cystic spaces in the femoral quadriceps and brachial biceps muscles. A large amount of pus with foul smell was removed by incision, drainage and aspiration of the two tumors. The lesions were successfully treated with intravenous administration of antibiotics. Repeated bacterial cultures yielded only Bacteroids fragilis. To our knowledge, this is the first report of pyomyositis due to Bacteroides fragilis.

Thirteen cases among 16,260 patients seen at our department during the nine years from 1981 were diagnosed as pseudoxanthoma elasticum (PXE). The sex and age distributions were similar to those so far reported in the literature. There were two cases of the autosomal dominant type. One patient had skin lesions typical of PXE in association with Ehlers-Danlos syndrome. In two of three patients with cutis laxa-like lesions, eruptions of elastosis perforans serpiginosa were present. In three cases, we failed to detect deposition of calcium in typical skin lesions in either an early or a late stage by histochemical staining, in spite of the presence of slightly degenerated elastic fibers.