A 43-year-old woman ingested soup made from miso (fermented soy paste) and female snow crab. Two hours later, the woman bathed and went to sleep. The patient then presented with systemic urticaria seven hours following ingestion of the soup. The urticaria temporarily subsided after the patient took chlorpheniramine maleate. However, an itching sensation awoke the patient nine hours later; she briefly lost consciousness, then developed emesis and diarrhea. It is thought that she developed late-onset anaphylaxis, which did not involve immediate symptoms. After the patient recovered, prick test (PT) and scratch test (ST) were performed using snow crab tissue as an antigen. Tissue from the outer roe (zygotes) elicited positive responses, but inner roe (ovary) and tissue from the crab butter (liver and pancreas) elicited negative results. Furthermore, when the same tests were performed using muscle tissues taken from a male snow crab, a king crab, and a horsehair crab as antigens, only tissues from the male snow crab and horsehair crab elicited positive responses. The reason for the differential response is thought to be associated with the lesser degree of chromosomal crossover between the king crab species, which belongs to the infraorder Anomura along with the hermit crab, and other species of crabs, which belong to the infraorder Brachyura.

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A 60-year-old woman was referred to our hospital for oral and perianal ulcers. Lichen planus was diagnosed based on biopsy findings and the patient showed a good clinical course in response to topical steroid administration. However, 3 years and 8 months after the initial examination, her general condition deteriorated, showing severe multiple oral ulcers, fever, diarrhea and severe anemia and she became unable to eat due to painful ulcers. Candida was detected in the buccal mucosa; an antifungal drug was started, leading to a partial response, but her fever, diarrhea and anemia persisted. Underlying hematological disease was suspected based on monocytosis; we performed a bone marrow examination and diagnosed myelodysplastic syndrome (MDS) with trisomy 8. Accordingly, MDS seemed to be responsible for her Behçet's disease-like symptoms including buccal and genital ulcers. Oral corticosteroid was effective for both anemia and Behçet's disease-like symptoms.

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Sweet syndrome, which was reported by Sweet in 1964, is characterized by a combination of fever, neutrophilia, tender erythematous nodules or plaques, and histopathologic findings showing a dense infiltrate of mature neutrophils in the upper dermis. Systemic administration of corticosteroids is the “gold standard” for the treatment of Sweet syndrome, and the dramatic response is well known. However, considering the underlying disease and various side effects, administration of corticosteroids may be harmful in some patients. We encountered a patient with Sweet syndrome who showed dramatic and rapid response to systemic administration of potassium iodide. The patient became afebrile and free from symptoms within 48 hours after starting potassium iodide, and there have been no recurrences after cessation of the therapy. Although the mechanism of action is not clear, potassium iodide can be a first line drug, because of its effectiveness, safety, and low cost.

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A 37-year-old man with a past history of drug eruption due to a Kampo (herbal) medicine took an over-the-counter drug including pseudoephedrine for nasal inflammation (Zikina^®). The next day, erythematous macules and pustules developed over his whole body except his face. Laboratory data on the first examination showed leukocytosis. Histopathologically, a subcorneal pustule was noted in the biopsied specimen. Those eruptions were successfully treated with oral prednisolone (initial dose: 30mg/day) for 12 days. The drug lymphocyte stimulation test for Zikina^® was negative. Patch testing with Zikina^® and Maoto, a Kampo medicine including the herb ephedra, showed positive reactions at 48 and 72 hours. There were negative findings on patch tests with ingredients of Zikina^® other than pseudoephedrine. Based on these findings, we diagnosed this patient as having acute generalized exanthematous pustulosis (AGEP) caused by pseudoephedrine in Zikina^®. We advised the patient to avoid drugs containing pseudoephedrine and/or ephedra.

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A 76-year-old female presented with a 3-year history of erythematous macules and vesicles on her extremities. Since her skin eruptions were not improved by oral administration of prednisolone, she consulted our hospital. She had pigmented macules on her lower extremities, some of which had vesicles around the margins forming a sunflower-like configuration. The mucous membranes were not affected. Histopathological examination of a pustule showed neutrophilic infiltration in the epidermis as well as in the upper dermis. Direct immunofluorescence microscopy of a skin biopsy specimen demonstrated IgA deposition on cell surfaces throughout the epidermis. We diagnosed the patient as having the intraepidermal neutrophilic dermatosis type of IgA pemphigus. A combination of DDS (diaminodiphenylsulfone) 50mg/day and prednisolone 7.5mg/day improved her eruptions. The individual lesions disappeared in a few days leaving hyperpigmentation.

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Three elderly male patients aged 55, 76 and 91 years presented with widespread eruptions of erythematous, flat-topped confluent red to reddish-brown papules that spared the skin folds. They had each undergone various unsuccessful treatments (oral antihistamines, topical and oral corticosteroids). We diagnosed these patients as having Ofuji's papuloerythroderma. These eruptions were not associated with malignant tumors. Their eruptions and subjective symptoms started to reduce after approximately 4-7 narrow-band UVB treatments, and the eruptions virtually disappeared after approximately 10 treatments. They had slight exacerbation but the eruptions subsided. We conclude that narrow-band UVB is more useful for papuloerythroderma than other treatments because of its convenience and limited side effects.

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We report a nine-year-old girl who presented with a 3-month history of flat papules and erythemas on the inner side of the left lower limb. Physical examinations demonstrated flat papules, small erythemas, and irregularly shaped erythematous patches as a result of coalescence of the erythemas, forming a broad band along the Blaschko line. Histological examinations demonstrated moderate perivascular infiltration of lymphocytes and mild spongiosis and exocytosis in the epidermis. The lesions spontaneously healed one year after onset, leaving faint brown pigmentation. We diagnosed this patient as having pediatric onset blaschkitis. However, this disease entity has been controversial since its first report in 1990. The most serious problem is that blaschkitis cannot be distinguished from adult onset lichen striatus, a distinct subtype of classical lichen striatus. We consider that blaschkitis includes adult onset lichen striatus and would be differentiated from classic lichen striatus.

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Bowen's disease is a fairly common disease. However, it is seldom seen in the perianal region. We report a case of perianal Bowen's disease in a 50-year-old male. He presented with a whitish nodule on the anus. Biopsy findings demonstrated Bowen's disease. Simple excision with a 1cm margin was performed under spinal anesthesia. We detected HPV16 (human papilloma virus 16) DNA by PCR amplification of the tissue.Skin Research, 9: 151-153, 2010

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We report a statistical survey of extramammary Paget's disease that was pathologically diagnosed at the Department of Dermatology of Kyoto Prefectural University of Medicine between 1992 and 2006. The number of patients increased steadily. The average patient age was 72.3 years old, and the male-to-female ratio was 1.7:1. Sixty-three patients had lesions on the genital area, 3 on the perianal region, 2 on the axilla, 2 on the hypogastric region, 1 in both the genital and axilla, 1in both the hypogastric region and axilla. The 5-year survival rate was 100% in stage IA, IB, 88.9% in stage II, 48% in stage III and 0% in stage IV. The depth of invasion, high CEA level, and lymph node metastases are significant risk factors for a poor prognosis.Skin Research, 9: 154-157, 2010

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We reported epidermal malignant skin tumors that were pathologically diagnosed at the Department of Dermatology at Kyoto Prefectural University of Medicine between 1992 and 2006. A total of 680 cases including 264 with basal cell carcinoma, 163 with Bowen's disease, 135 with actinic keratosis, and 118 with squamous cell carcinoma (SCC). We surveyed not only the incidence every 5 years, but also the age distribution and sites of primary lesion for these tumors.

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A 68-year-old woman presented with pruritic erythematous papules and nodules on her forehead. Histopathology of the lesion showed dense dermal infiltration of lymphoid cells with follicular growth pattern. The follicles were composed of centrocytes, centroblasts, lymphoid cells and tingible body macrophages. UCHL-1 was expressed in lymphoid cells around and in the follicles, and L-26 was expressed in lymphoid cells in the follicles. Based on these observations, we diagnosed the patient as having cutaneous B-cell pseudolymphoma. One year and 5 months later, erythema and nodules appeared on both cheeks. On histological examination biopsy from the cheek, cellular infiltration was identical to that observed in the specimen from the patient's forehead. Southern blot analysis of immunoglobulin heavy chain regions and T-cell receptors (TCR) did not detect any evidence of gene rearrangement. Careful examination and follow up are required for such patients because of the possibility of malignant transformation in the future. Skin Research, 9: 163-167, 2010

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A 76-year-old man had been treated with docetaxel and nedaplatin chemotherapy for advanced lung adenocarcinoma (T2N2M1, stage IV). Since this chemotherapy was not effective, erlotinib monotherapy (150mg daily) was initiated. Eight days later, acneiform eruptions began to appear and were initially treated with nadifloxacin 1% cream. However, these eruptions worsened, and treatment with topical steroids was started. Strong topical steroids were used on the right side of the face, while the left side was treated with a milder version of this medication. After 2 weeks, the right face showed an improvement compared to the left face. The stronger class of steroids was then used to treat his entire face. Treatment with topical steroids caused the eruptions to disappear within 5 weeks without interrupting the erlotinib regimen. We think that it is better to treat adverse cutaneous effects associated with EGFR inhibitor without disrupting the treatment regimen.Skin Research, 9: 168-172, 2010

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A 72-year-old man with a history of resected carcinoma who had been administered 5-fluorouracil (100mg/d) orally, was being treated with corticosteroid (PSL 18mg/d) and cyclosporine A (50mg/d) for recalcitrant bullous pemphigoid, when he became aware of swelling on his left hand. At a consultation in January 2009, he was found to have a slightly raised brown-red swelling extending from the dorsal aspect of the left hand to the 3rd and 4th fingers, with soft nodules. Some of the nodules were asymptomatic, soft, edematous, and partially encrusted, and discharged purulent exudates upon application of pressure. There was no local warmth. On histopathological sections, dense neutrophilic and histiocytic infiltration was observed in the middle dermis, and chains of spore-like fungal elements were observed scattered throughout the lesion on PAS and Grocott staining. Fungal cultivation from the crust of one nodule and discharge from a biopsy specimen demonstrated the organism to be a black mold. Both of these isolates were compatible with Exophiala jeanselmei on morphological findings and DNA sequence of the internal transcribed spacer regions of the ribosomal DNA. The patient was successfully treated with oral terbinafine (125mg/d) combined with local hyperthermia over the course of 1 month.

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