Ayano Umekoji

A case of mosaic-type bullous congenital ichthyosiform erythroderma successfully treated with topical maxacalcitol, a vitamin D3 analogue

in lesions of eosinophilic pustular folliculitis by culture but have been histologically observed in a few cases. In our patient, we found dermatophytes both by Grocott and PAS staining and by culture of a biopsy specimen. To our knowledge, this is the first report of identification of T. rubrum by culture in eosinophilic pustular folliculitis. It is of interest that biological response to dermatophytosis can cause the histological features of eosinophilic pustular folliculitis. During the physical examination, we could not find any fungus in scrapings of the lesion despite three examinations by three different dermatologists. The failure to identify fungi in scrapings in this case was probably due to the fact that the fungi were histologically distributed mainly in the hair follicles, not in the epidermis (Fig. 2b). Our findings support the speculation of Haupt et al. that some cases of eosinophilic pustular folliculitis are probably related to dermatophyte infection. In addition, a single or a few serial histological sections may not be sufficient to reveal the presence of fungal bodies from a biopsy specimen of eosinophilic pustular folliculitis. Therefore, both histological examination (including PAS and Grocott staining) of serial sections and culture should be carried out to obtain an accurate diagnosis for patients with features of eosinophilic pustular folliculitis. In conclusion, we emphasize that it is important for clinicians to be aware of the possibility of eosinophilic pustular folliculitis in relation to fungal infection.

Bullous pemphigoid as a dermadrome associated with spindle cell carcinoma of the gallbladder.

Skin disorders that appear in association with internal malignancies are called dermadromes. Bullous pemphigoid (BP), which is a major autoimmune disease of the skin, is considered to be a dermadrome, although there have been conflicting reports. We report a case of BP that preceded the diagnosis of an internal malignancy. Although we could not detect any malignancies on chest, abdominal or pelvic computer tomography on the first hospital admission, intensive screening on the third admission revealed a gallbladder malignancy. Laparoscopic cholecystectomy was performed. Histopathology showed a spindle cell carcinoma of the gallbladder. To the best of our knowledge, this is the first report of a spindle cell carcinoma of the gallbladder in a patient with BP.

Allergic contact dermatitis caused by the preservative 4,5-dichloro-2-n-octyl-4-isothiazolin-3-one in black trousers

Ayano Umekoji1, Kazuyoshi Fukai1, Junko Sowa-Osako1, Marina Manabe1, Maki Kikugawa2, Kyoko Ishii3, Kazumi Sasaki3 and Daisuke Tsuruta1 1Department of Dermatology, Osaka City University, Graduate School of Medicine, Osaka, 545-8585, Japan, 2Consumer Product Safety Technology Division, National Institute of Technology and Evaluation, Product Safety Technology Centre, Osaka, 559-0034, Japan, and 3Biosafety Analysis Division, National Institute of Technology and Evaluation, Biological Resource Centre, Tokyo, 151-0066, Japan

Vascular type of Ehlers–Danlos syndrome associated with mild haemophilia A

A 21-year-old man was referred for evaluation of possible vascular-type Ehlers–Danlos syndrome (EDS), formerly known as type IV EDS. His father had died of aortic rupture at the age of 49 years, and his maternal grandmother had also been diagnosed with vascular-type EDS. The patient had experienced repeated episodes of subcutaneous haemorrhage, and severe bleeding had occurred when he underwent neurosurgery for a Rathke s cleft cyst at the age of 18 years. Physical examination revealed thin skin, and the finger joints were more flexible than normal. However, these changes were not as clear as in the classic type of EDS, and the joint hypermobility did not meet Beighton s criteria. Chest X-ray revealed mild pulmonary haemorrhage. The platelet count was normal. The bleeding time was prolonged to 8 min (normal < 5 min). In platelet aggregation tests, the responses to collagen and adenosine diphosphate (ADP) were slightly raised at 86% (normal range 45–75%) and 82% (35–75%), respectively. Coagulation screening tests, including prothrombin time and activated partial thromboplastin time, were normal. Factor IX and von Willebrand factor were normal. Notably, factor VIII activity was reduced to 32% of control levels, indicating mild haemophilia A. This patient had thin skin, translucent veins on the forearms, and hypermobile joints of the fingers, although the possibility that all of these findings were within the range of normal variation could not be fully excluded. We therefore performed Western blotting of the proteins secreted into the media of cultured skin fibroblasts from the patient. The fibroblasts produced a limited amount of type III collagen, approximately one-tenth that of control fibroblasts, although production of type I collagen was preserved. The diagnosis of vascular type EDS was thus confirmed by biochemical analysis. We prepared type III collagen cDNA from cultured fibroblasts to perform a mutation study. We sequenced the cDNA but failed to detect any mutations; the genomic DNA of the patient was not examined. Several months later, the patient died from sudden and severe pulmonary bleeding. Two cases of association of EDS and factor VIII deficiency have been reported previously. However, the type of EDS was not determined in either of those cases. To our knowledge, this is the first case in which confirmed vascular-type EDS was noted to be associated with haemophilia A. Anstey et al. 4 examined platelet and coagulation activities in 51 cases of EDS, seven of which were found to be type IV (vascular type). One of these seven patients exhibited a platelet release defect. Of the other six cases, three had a prolonged bleeding time, and four had reduced platelet aggregation to ristocetin. Results of platelet aggregation tests with collagen, ADP and arachidonate were all normal. As this patient s haemophilia A was mild, the fatal pulmonary haemorrhage was probably due to the vasculartype EDS. Clotting factor deficiencies in EDS may be more common than previously thought. EDS should be considered as a diagnosis in clinical situations such as pulmonary or intestinal bleeding, even if abnormalities are observed on clotting studies or in coagulation factors, which indicate other disorders.

Probable allergic contact dermatitis from hydroquinone presenting as leukomelanoderma: Report of two cases

Dear Editor, Hydroquinone creams are widely used as manually compounded cosmetics in Japan. Here, we report two cases of leukomelanoderma associated with probable allergic contact dermatitis caused by hydroquinone. A 61-year-old woman was referred for evaluation of pigmentary changes on her face. She had used 10% hydroquinone cream on her face and neck for 2 months. Despite the development of a stinging sensation and slight erythema, she continued to use the cream. On examination, well-demarcated

Malignant Skin Tumors in Patients with Oculocutaneous Albinism

Background: Patients with oculocutaneous albinism (OCA) are prone to develop skin cancers. Aim and Objectives: To analyze reports of skin cancers in albinos published in the English and the Japanese literature in order to prepare guidelines for the successful management of skin cancer in albinos. Methods and Materials: We searched on PubMed, Web of Knowledge for English literature, and Ichushi-Web for the Japanese literature. Results: Between 1978 and 2013, 11 malignant melanoma, 4 basal cell carcinoma, and 6 squamous cell carcinoma cases were reported in Japan. The subtypes of the 11 malignant melanomas were nodular in 7 cases, superficial spreading in 3 cases, and metastatic lesions in 1 case. Amelanotic melanomas comprised 7 of the 11 malignant melanoma cases in Japanese patients with OCA. Twenty-five malignant melanoma, 50 basal cell carcinoma, and 151 squamous cell carcinoma cases were reported worldwide in the English literature during the period between 1952 and 2013. Conclusion: Regular skin monitoring by board-certified dermatologists is required for adult OCA patients.

Rapid detection of Bartonella henselae heat shock protein DNA by nested polymerase chain reaction from swollen lymph nodes of a patient with cat-scratch disease

Dear Editor, Diagnosis of cat-scratch disease by dermatologists is not always straightforward. Eruptions of the disease involve small scratched skin lesions, and are easily overlooked. The only useful clue to the diagnosis is the history of exposure to cats or occasionally dogs. The nested polymerase chain reaction (PCR) technique was recently developed to detect Bartonella DNA from the specimens of the patients, and fast and specific diagnosis has been available for cat-scratch disease. While the presence of antibodies to Bartonella henselae supports the diagnosis of cat scratch disease, the specificity of the serological test needs to be improved. Here, we report a case of catscratch disease in which B. henselae DNA was detected from a swollen lymph node by nested PCR. A 75-year-old man visited our clinic in September 2006 because of high fever and swollen right inguinal