bluuebunny/crossref_metadata_2025 huggingface dataset:
CD36 (OKM5) Antigen Expression on Human Mucosal Epithelia Is Associated with Keratinization Type
Authors: Gomez Renate Schlegel , Pelka Michael , Johannessen Anne C. , Hornstein Otto P. , von den Driesch Peter
Published: April 2015

The glycoprotein CD36 functions as a thrombospondin receptor and is expressed on a variety of cell types, including platelets, monocyte/macrophages, and endothelial cells. In human skin, the presence of CD36 on keratinocytes was initially found in lesional areas of T‐cell mediated inflammatory dermatoses. Controversy still exists on the interpretation of this expression as an inflammatory or differentiation‐associated marker. So far, only limited data are available on CD36 expression in oral epithelia. The present immunohistochemical study was therefore performed to determine the presence of CD36 on keratinocytes of healthy and disease‐affected epithelia of the oral cavity in 80 biopsy specimens. As results, we found an inflammation‐independent strong expression of CD36 in oral epithelia with ortho‐ and parakeratinization, such as the oral side of the gingiva, the hard palate, and the back of the tongue. Non‐keratinized epithelia such as the gingival pocket epithelium, the soft palate, the lip, the buccal mucosa, and the side of the tongue were CD36 negative. We therefore suggest that CD36 expression in keratinocytes from oral epithelia is an epithelial differentiation marker.
Folliculosebaceous Cystic Hamartoma with a Neural Component: An Immunohistochemical Study
Authors: Toyoda Masahiko , Morohashi Masaaki
Published: April 2015

We present a case of follicular cystic hamartoma, a distinctive cutaneous malformation characterized by marked overgrowth of folliculosebaceous units accompanied by appreciable mesenchymal alterations, including fibroplasia, increased vascular components, and numerous adipocytes. A conspicuous feature of our case is an aggregation of thick trespassing nerve bundles in the deep portion of the neoplasm. An immunohistochemical study revealed the nerve bundles were immunoreactive for the general neuronal marker protein gene product 9.5. The nerves, however, stained negatively with antibodies against neuropeptides such as calcitonin gene‐related peptide, substance P, vasoactive intestinal polypeptide, and neuropeptide Y, all of which are known to be contained in normal cutaneous nerves. The presence of these aberrant nerve bundles devoid of the normal expression of neuropeptides supports the concept that folliculosebaceous cystic hamartoma is a true, tumor‐like malformation characterized by abnormal overgrowth of normal components of the skin.
A Case of Paget's Disease of the Vulva Recurring in a Musculocutaneous Flap
Authors: Yoshitatsu Sumiko , Hosokawa Ko , Nishimoto So , Yoshikawa Kunihiko
Published: April 2015

A case of Paget's disease of the vulva which recurred in a reconstructed gracilis musculocutaneous flap without invasing adenocarcinoma is presented. The recurrent lesion was seen in the basal layer of the epidermis of the flap without invasion into the dermis. The possibility of an intraepidermal lateral invasion of Paget's cells into the epidermis of the flap is suggested.
Pigmented Epidermal Cyst
Authors: Akasaka Toshihide , Imamura Yuko , Kon Saiichi
Published: April 2015

A 58‐year‐old Japanese woman with a pigmented epidermal cyst on the upper arch of the right external ear is described.

The blue appearance of the cyst was striking and uncommon in Japanese. Histologically, abundant melanin pigment was observed in the keratinous mass of the cavity, and epithelial cells showed epidermoid keratinization. Increased numbers of melanocytes in the basal layer, large globular cells of melanocytic origin in the spinous layer, and hair‐germ‐like aggregates of basaloid cells and melanocytes were also observed.

The blue clinical appearance is probably due to the rich melanin granules in the cyst, keratinous mass, keratinocytes, and hair‐germ‐like structures.
Bilateral Accessory Tragi on the Suprasternal Region
Authors: Kim Seok Woo , Moon Sang Eun , Kim Jeong Aee
Published: April 2015

We report a 25‐year‐old man who had two protruding firm masses on his suprasternal region. These lesions were present at birth without any family history. He had no other subjective symptoms. In a histological specimen, there were numerous unevenly distributed telogen follicles that contained vellus hairs. Beneath a relatively narrow zone of fibrovascular tissue, lobules of fat with a central core of cartilage were seen. The mass was totally excised; during the follow‐up period, there was no evidence of recurrence.

The tragus derives from the first branchial arch. The accessory tragus can be found along the entire course of embryonic migration. It is usually located unilaterally in the preauricular region. To our knowledge, bilateral development on the suprasternal region has not been previously reported.
Elastic Fibers in Dermis of Juvenile Elastoma
Authors: Kobayasi Takasi , Bartosik Jacek , Ullman Susanne
Published: April 2015

To explore the ultrastructure of elastic fibers in juvenile elastoma, three patients (two without osteopoikilosis and one under examination of bones and joints) were studied by routine electron microscopy. In addition to normal elastic fibers, all the patients also exhibited alterations in elastic fibers. The altered ultrastructures showed lucent, homogenous matrix without peripheral microfibrils. The homogenous matrix were seen in various extensions from the small protrusions of the normal elastic fibers to the complete replacement of the entire fibers. Collagen fibrils occasionally showed twisted figures. Normal shapes of dermal glycosaminoglycans were increased in number. It seems likely that the lucent, homogenous matrix without peripheral microfibrils are the characteristic changes of elastic fibers in juvenile elastoma. The alteration could be nevoid in nature.
Epidermolysis Bullosa Acquisita Localized to the Face
Authors: Choi Gwang Seong , Lee Eun‐So , Kim Soo‐Chan , Lee Sungnack
Published: April 2015

A 39‐year‐old woman had a three‐year history of recurrent bullous eruption localized to her left cheeck. The diagnosis of epidermolysis bullosa acquisita was confirmed by means of direct immunofluorescence and direct immunoelectron microscopic studies performed on the perilesional salt‐split skin. Topical corticosteroid treatment reduced pruritus and bullae formation. This case of localized epidermolysis bullosa acquisita on the face is reminiscent of Brunsting‐Perry cicatricial pemphigoid.

We also review the previously reported cases of localized epidermolysis bullosa acquisita.
An Unusual Female Melanoma Patient with Late Metastases to Both Skin and Ovaries
Authors: Nakano Junji , Shimizu Takahiro , Hirota Toru , Muto Masahiko
Published: April 2015

We presented an unusual case of cutaneous and bilateral ovarian metastases of malignant melanoma. There was no previously identifiable cutaneous or mucous lesion or teratoid element. Past history revealed that the patient had undergone removal of a blackish, elevated, irregular shaped tumor from the right arm eleven years previously. This lesion was considered to be the primary melanoma; therefore, it was thought to be very unusual as metastasis occurred in both the skin and ovaries after an eleven year disease‐free interval.
A Nevocellular Nevus Consisting mostly of Nevic Corpuscles
Authors: Tomita Kouichi , Furukawa Fukumi , Tokura Yoshiki
Published: April 2015

A scalp tumor from a 24‐year‐old male presenting unusual histological finding is described. The tumor was mostly composed of corpuscles resembling Meissner tactile bodies and contained almost no other components.

Immunohistochemical studies revealed the tumor cells to be positive for S‐100 protein and neuron‐specific enolase, so we considered the tumor to be an unusual nevocellular nevus mostly comprised of nevic corpuscles.
Pruritic Skin Diseases in the Elderly
Authors: Thaipisuttikul Yupin
Published: April 2015

A total of 149 elderly men and women with pruritic skin problems were selected for study at the dermatological clinic in the Department of Medicine, Rajavithi General Hospital, Bangkok, Thailand, from 26 November 1996 to 10 January 1997. There were 62 men (41.6%) and 87 women (58.4%). The average age was seventy years. Among these elderly patients, pruritic skin disease was the most common problem, found in about 41%. Xerosis (senile pruritus) was the most common problem at 38.9%. Other pruritic skin diseases found were inflammatory eczema (22.8%), lichen simplex chronicus (12.1%), skin infections (11.4%), psoriasis vulgaris (6.7%), urticaria (4.7%), drug rash (2%), insect bite (0.7%), and anogenital pruritus (0.7%). Xerosis usually occurred with increased bathing frequency and use of strong soaps and detergents. The causes of inflammatory eczema were seborrheic dermatitis, allergic contact dermatitis, dyshidrosis, and stasis dermatitis. Statistical analysis of xerosis and inflammatory eczema by gender showed no difference, but there was more inflammatory eczema among females.
Ofuji Papuloerythroderma Associated with Follicular Mucinosis in Mycosis Fungoides
Authors: Suh Kee‐Suck , Kim Hyun‐Cheol , Chae Young‐Soo , Kim Sang‐Tae
Published: April 2015

Ofuji papuloerythroderma is a distinctive clinical entity of unknown etiology, which may occasionally be associated with B cell and T cell lymphoma or visceral malignancy. We report a case of papuloerythroderma associated with follicular mucinosis in mycosis fungoides (MF) that raises the possibility of papuloerythroderma as a form of prelymphomatous skin eruption. This specific papuloerythroderma responded well to the Re‐PUVA treatment, which is a combination of etretinate and PUVA photochemotherapy.
Pemphigus Vegetans Involving the Esophagus
Authors: Ichimiya Makoto , Nakano Junji , Muto Masahiko
Published: April 2015

A 44‐year‐old man with pemphigus vegetans had severe odynophagia. He received an endoscopic examination for esophageal involvement. Many white plaque‐like lesions with an erythematous base were seen on the esophageal mucosa. Biopsy from the mucosal epithelial layer showed rounded epidermal cells with large nuclei and numerous inflammatory cells including eosinophils.
Neutrophil Eccrine Hidradenitis in a Patient with AIDS
Authors: Krischer Joachim , Rutschmann Olivier , Roten Sonia Vollenweider , Harms Monica , Saurat Jean‐Hilaire , Pechère Marc
Published: April 2015

Neutrophilic eccrine hidradenitis (NEH), first described as a rare, transient, and benign complication of various chemotherapy regimens for acute leukemia, has also been observed in other conditions, including three HIV‐positive patients and even in otherwise healthy individuals (1–3). A similar histological pattern was described after intradermal bleomycin injections into normal human skin (4). We report the first case of NEH in a hemophilic HIV infected patient treated with stavudine, a new reverse transcriptase inhibitor.
Four Cases of Pustulotic Arthro‐Osteitis
Authors: Jang Kyoung Ae , Sung Kyung Jeh , Moon Kee Chan , Koh Jai Kyoung , Choi Jee Ho
Published: April 2015

We describe four cases of pustulotic arthro‐osteitis. All of them suffered from anterior chest wall symptoms, such as swelling and pain on the sternoclavicular joint, sternal angle area, sternocostal joint, and costochondral junction. Although chest X‐ray findings were all normal, 90mTc‐scintigraphy revealed increased uptake in the affected areas in all cases. Laboratory examinations including rheumatoid factor were negative or normal except for elevation of the erythrocyte sedimentation rate and C‐reactive protein in 2 cases. After treatment with non‐steroidal anti‐inflammatory drugs and etretinate or topical PUVA, the skin lesions on the palms and soles and the arthro‐osteitis were moderately improved. Pustulotic arthro‐osteitis should be considered when a patient with pustulosis palmoplantaris complains of anterior chest wall pain and other joint pain.
The Proliferation and Differentiation of Neonatal Epidermal Melanocytes in F1 Hairless Mice of HR‐1 × HR/De in Serum‐Free Culture
Authors: Furuya Rikako , Akiu Satoru , Naganuma Masako , Fukuda Minoru , Hirobe Tomohisa
Published: April 2015

To investigate the characteristics of the proliferation and differentiation of epidermal melanocytes in F1 hairless mice of HR‐1 × HR/De parents in vitro, cell suspensions of the neonatal epidermis were cultured in a serum‐free medium supplemented with dibutyryl adenosine 3‘,5‘‐cyclic monophosphate (DBcAMP) and/or basic fibroblast growth factor (bFGF). The differentiation of melanocytes was induced by treatment with DBcAMP. In contrast, the sustained proliferation of melanoblasts was induced by combined treatment with DBcAMP and bFGF. The melanoblasts could be subcultured in serum‐free medium supplemented with the two factors in the presence of keratinocytes, but not in their absence. This is the first report of successful culture of melanoblasts and melanocytes from hairless mice; it is expected to be useful in understanding the mechanism of the development of pigmented spots in the epidermis of (HR‐1 × HR/De) F1 mice, which are reported to be induced by repeated exposure to ultraviolet light B.
Treatment of Cutaneous Leishmaniasis by Direct Current Electrotherapy: The Baghdadin Device
Authors: Sharquie Khalifa E. , Al‐Hamamy Hayder , El‐Yassin Dhafir
Published: April 2015

Electrical stimulation has been used as a mode of therapy for a number of clinical conditions. However, it has not been used for the treatment of cutaneous leishmaniasis (CL). For this purpose, we designed the “Baghdadin device”. A total of 146 lesions of acute CL in 54 patients were treated by this device. Twenty‐one lesions in the same patients were left untreated as controls. In addition, 36 lesions in 15 patients were treated with intralesional sodium stibogluconate. Treatment by the Baghdadin device consisted of weekly sessions of 10 minutes of direct current electrical stimulation. The intensity of the direct current ranged between 5 and 15 milliamperes, and the voltage was kept below 40 volts. Of the 146 lesions, 135 (92.5%) showed total clearance or marked improvement in 4–6 weeks time. Approximately 67% of the lesions needed only one or two sessions. Scarring was not observed after resolution. None of the untreated lesions showed any signs of improvement in 6 weeks. Of the lesions treated with sodium stibogluconate, 32 lesions (88.9%) showed total clearance or marked improvement, which was not significantly different from the results with the Baghdadin device.
High Incidence of IgG Antibodies to Phenolic Glycolipid in Non‐Leprosy Patients in India
Authors: Kumar Bhushan , Sinha Raghu , Sehgal Shobha
Published: April 2015

Purified phenolic glycolipid (PGL‐1) from Mycobacterium leprae was used to detect IgG antibodies against PGL‐1 in leprosy patients in an enzyme‐linked immunosorbent assay (ELISA). A total of 698 sera were screened; they came from patients suffering from leprosy, autoimmune disease, myeloma, tuberculosis and sexually transmitted diseases (STDs). Cases with miscellaneous diseases and persons undergoing AIDS screening were also included. Sera from lepromatous and tuberculoid leprosy patients gave positivity rates of 60.5% and 41.7%, respectively. In non‐leprosy cases, the PGL‐1 ELISA showed an overall positivity rate of 6.9%; this was greatest in patients with tuberculosis (43.8%) followed by autoimmune diseases (40.9%) and miscellaneous cases including liver diseases (37.9%). This study emphasizes that PGL‐1 ELISA has a low predictive value for diagnosis of active infection by Mycobacterium leprae. Positive reactions in a significant percentage of patients with autoimmune disease are intriguing and need indepth study.
Metastasis of Esophageal Carcinoma Manifesting as Whitlow‐like Lesions
Authors: Umebayashi Yoshihiro
Published: April 2015

A rare case of phalangeal metastasis of esophageal carcinoma in an 81‐year‐old man is described. The patient developed a tender, red, and swollen right index finger. From the clinical features, he was erroneously diagnosed with whitlow at first and treated with antibiotics with no success. A bone X‐ray showed that the distal phalanx of the index finger was completely dissolved. Histopathological examination revealed proliferation of squamous cell carcinoma into the deep dermis. It is important to understand that the phalangeal metastases commonly display inflammatory symptoms that mimic acute infection.
Membranous and Soluble Forms of Fas Antigen in Cutaneous Lupus Erythematosus
Authors: Fushimi Misao , Furukawa Fukumi , Tokura Yoshiki , Itoh Taisuke , Shirahama Shigeho , Wakita Hisashi , Takigawa Masahiro
Published: April 2015

The role of Fas‐mediated apoptosis in cutaneous lupus erythematosus (LE) is still unclear, although the Fas/FasL system has been investigated in autoimmune diseases in relation to impaired apoptosis. In order to elucidate the connections between acute cutaneous LE (ACLE) and chronic cutaneous LE (CCLE), we determined the expression of membranous Fas antigen (mFas) on peripheral blood mononuclear cells (PBMC) by flow cytometry and the levels of the soluble form of the Fas antigen (sFas) in sera. The ratio and the mean fluorescence intensity of mFas were much higher in ACLE patients than in others, including patients with CCLE and atopic dermatitis and normal healthy controls. The levels of sFas in ACLE and CCLE patients were also elevated, and there was a significant increase in sFas levels in ACLE patients over that in CCLE patients. Immunohistochemical studies revealed that Fas antigen was predominantly expressed on infiltrating cells around blood vessels and appendages in ACLE and CCLE patients. Based on these findings, it is suggested that the expression of Fas antigen is closely associated with the activation of circulating lymphocytes, especially in ACLE patients, but is not directly associated with keratinocyte damage.
Sarcomatoid Carcinoma of the Skin: Report of a Case
Authors: Ríos‐Martín Juan J. , Parra‐Martín Jose A. , Gómez‐Pascual Amparo , Hevia‐Vázquez Alberto , González‐Cámpora Ricardo
Published: April 2015

We report the histological, immunohistochemical and ultrastructural findings of an exophytic cutaneous tumor composed of a mixture of typical basal cell carcinoma (BCC) and malignant fibrous histiocytoma. Nine previously reported carcinosarcomas of the skin are reviewed. We prefer the term “sarcomatoid carcinoma” for this rare neoplasm. Only the BCC showed a positive immunoreaction to cytokeratin; the sarcomatous component was negative, but it did express vimentin, and, focally, smooth‐muscle‐specific actin and KP1 (CD68). Both components showed p53 immunostaining.
A Case of Rudimentary Meningocele
Authors: Yanagi Toshiaki , Sato Toshihiro , Fuijwara Sakuhei , Takayasu Susumu , Terashi Hiroto , Yokoyama Shigeo
Published: April 2015

A 2‐year‐old girl had an alopecic, atrophied macule on the midline of the occipital area. X‐ray and CT scan of the scalp revealed no underlying bone defect. Histologically, the whole dermis and subcutis showed dense, hyalinized collagen bundles intermingled with cuboidal, epithelioid cells that surrounded pseudovascular spaces or gathered in clusters. These flattened or cuboidal cells were positive for anti‐vimentin antibody; some of them were also positive for an anti‐epithelial membrane antigen antibody, but negative for anti‐factor VIII‐related antigen and Ulex europaeus I lectin.
Leopard Syndrome: A Tropical Rarity
Authors: Gupta Piyush , Loiwal Vikas , Rai Rajesh , Baruah M.C. , Mishra Kiran , Krishna Anurag
Published: April 2015

This is a case report of a classical presentation of the Leopard syndrome from the Indian subcontinent. The male patient had progressively increasing generalised multiple lentiginosis, ocular hypertelorism, pulmonary stenosis, short stature, cryptorchidism, and pectus excavatum. The case is documented for its rarity in this part of the world and typical clinical presentation.
Gigantic Pyoderma Gangrenosum
Authors: Ikeda Isamu , Johno Masayoshi , Higashi Shuichi , Ono Tomomichi
Published: April 2015

A 71‐year‐old Japanese female with gigantic pyoderma gangrenosum is reported. The pyoderma lesions had been treated as an infectious condition for seventeen months and had extended to enormously large areas. The nature of the chronic type of pyoderma gangrenosum may need to be stressed, even for dermatologists.
Neurotropic Melanoma Invading the Median Nerve
Authors: Iyadomi Minako , Ohtsubo Haruhiko , Gotoh Yumiko , Kohda Hiromu , Narisawa Yutaka
Published: April 2015

We report a case of acral lentiginous melanoma of the right thumbnail bed which demonstrated characteristic intraneural invasion and extension along the median nerve. Four years after amputation of the involved thumb, a melanotic tumor recurred on the right thenar. Radiation therapy was given. The tumor invaded the median nerve, however, causing progressive pain and paralysis of the right hand. Despite right arm amputation, the tumor extensively metastasized, and the patient died three years later.

Histopathologically, the tumors were characterized by extensive proliferation of spindle‐shaped cells forming neuroid fascicles especially prominent in the metastatic region. Tumor cells were positive immunohistochemically with S‐100 protein antisera.
Coexistence of Generalized Morphea with Histological Changes in Lichen Sclerosus et Atrophicus and Lichen Planus
Authors: Sawamura Daisuke , Yaguchi Tadashi , Hashimoto Isao , Nomura Kazuo , Konta Ritsuko , Umeki Kaoru
Published: April 2015

We reported a 44‐year‐old Japanese woman with generalized multiple sclerotic plaques, which showed hisological findings of morphea. This patient also had an erosive lesion on her mouth; its histological findings were consistent with lichen planus. A sclerotic lesion on her thigh showed the histological findings of lichen sclerosus et atrophicus (LSA). These data suggest that similar etiologic events or closely related pathologic processes are involved in morphea, lichen planus, and LSA.
Contact Dermatitis Due to Sodium Colistimethate
Authors: Sasaki Satsuki , Mitsuhashi Yoshihiko , Kondo Shigeo
Published: April 2015

Colistin methanesulfonate sodium (CLMS) is a widely‐used antibiotic. To our best knowledge, only two cases of contact allergy to CLMS have been reported. We described a 4‐year‐old girl with contact dermatitis evoked by CLMS that had been applied as an ophthalmic solution. The dermatitis started after administration for 21 days. The diagnosis was confirmed by as is and constituent patch tests. We reviewed the literature in which contact allergy due to CLMS and colistin sulfate is described.
Management of Female Genital Warts with an Analog of Imiquimod 2% in Cream: A Randomized, Double‐Blind, Placebo‐Controlled Study
Authors: Syed Tanweer A. , Ahmadpour Oliver Abbas , Ahmad Seyed Ali , Ahmad Seyed Hamzeh
Published: April 2015

The purpose of this randomized, double‐blind, placebo‐controlled study was to determine the clinical efficacy and tolerability of an analog of imiquimod (2%) in cream to cure genital warts in women. Sixty preselected women, ranging between 18 and 45 years of age (mean 24.3) and having 411 lesions (mean 6.8) with clinical, histopathological and polymerase chain reaction (PCR) confirmed diagnosis of human papilloma virus (HPV) infection were randomized to two parallel groups. Each patient received a precoded 40‐g tube and instructions on how to apply the trial medication to their lesions at home two times daily for five consecutive days per week. The active treatment period was six weeks. Patients were evaluated on a weekly basis. A clinically and PCR established total clearance of target warts was recorded as a cure. By the end of the treatment, 43.3% of patients and 42.8% of warts were cured. Code disclosure revealed that imiquimod cream had cured 83.3% of the treated patients and 84.3% of the treated warts, while the placebo healed one subject and four warts (p<0.0001). Eight patients (13.3%) in the imiquimod group experienced mild to moderate, non‐objective, drug‐induced symptoms with no dropouts. Among the 26 cured patients, five had a relapse after 11 months. In conclusion, the data presented demonstrate that 2% imiquimod in cream with mild to moderate subjective side effects is significantly more effective than placebo in eliminating genital warts in women.
Solitary Basaloid Follicular Hamartoma
Authors: Toyoda Masahiko , Kagoura Masayori , Morohashi Masaaki
Published: April 2015

We present a case of solitary basaloid follicular hamartoma of 8 years' duration on the nose of a 55‐year‐old Japanese man. Clinical examination revealed a solitary, asymptomatic, smooth‐surfaced, black papule. Histologically, the lesion showed multifocal proliferation of basaloid cells forming islands, branching cords and anastomosing strands that were continuous with the basal layer of the epidermis. Individual hair follicles were replaced by these undifferentiated basaloid proliferating cells. Some abortive hair folliclelike structures composed of follicular bulbs and contiguous rudimentary dermal papillae were observed. This solitary papular type of basaloid follicular hamartoma is quite rare; our case is clearly distinguished from other neoplasms with hair follicle differentiation as well as from infundibulocystic basal cell carcinoma, which have been the most problematic differential diagnoses in the recent dermatopathologic literature.
Sublingual Foregut Cyst
Authors: Kim Yi‐Sun , Ahn Sung Ku , Lee Seung Hun
Published: April 2015

Foregut cysts are usually found in the mediastinum, the lung, or the wall of the upper gastrointestinal tract, but only rarely at the base of tongue. We report a sublingual foregut cyst in a 27‐year‐old man.
Linear IgA Bullous Dermatosis with Circulating IgG Autoantibodies to the 230 kD Epidermal Antigen
Authors: Honoki Kumiko , Muramatsu Tsutomu , Tsubakimoto Akiko , Shirai Toshihiko
Published: April 2015

The patient was a 54‐year‐old woman with wide‐spread bullous lesions on her trunk and oral mucosa. Histologic examination revealed a subepidermal blister with infiltration of neutrophils and eosinophils. Direct immunofluorescence showed an exclusively IgA deposition at the basement membrane zone (BMZ). Indirect immunofluorescence showed that the blister fluid, but not the serum, contained IgG antibodies against the BMZ antigen on the epidermal side of salt‐split skin. Using immunoblot analysis with normal human epidermal extracts, both serum and blister fluid reacted with the 230 kD epidermal antigen. Using colloidal gold and direct immunoelectron microscopy, IgA deposition was detected in the lamina lucida. Clinically, the skin lesions responded well to dapsone.

We diagnosed this case as linear IgA bullous dermatosis (LABD) with IgG class circulating autoantibodies against the epidermal 230 kD antigen. These antibodies were considered to be secondary to the damage to the epidermal basal keratinocyte in this case.
Clinical Phenotype of Bart's Syndrome Seen in a Family with Dominant Dystrophic Epidermolysis Bullosa
Authors: Wakasugi Shoji , Mizutari Kumiko , Ono Tomomichi
Published: April 2015

Background: Bart's syndrome is one type of dominant dystrophic epidermolysis bullosa (EB). It is known that, in some familial cases of dominant dystrophic EB, the symptoms differ depending on the individual. We observed the way Bart's syndrome affected four generations in the same family. The proband was a newborn boy who showed congenital localized absence of skin (CLAS) and bullae on the anterior aspects of both legs. Histologically, the bullae were located subepidermally. The CLAS and bullae disappeared within 4 months after birth, leaving scars. His father retained scarring and scaling from the knees down along the anterior aspect of the legs, and the nails of the toes were either lacking or deformed. His paternal grandmother and great‐grandmother also presented deformed nails of the toes, although they had not had CLAS or bullae on the legs at birth. The individuals in this family thus showed some heterogeneity depending on the sex: blistering and CLAS were seen on the legs soon after birth in the male family members, but the female members did not share this pattern of symptoms, suggesting that the expression of symptoms may differ depending on the sex of the affected individual.
Toxic Epidermal Necrolysis with Severe Gastrointestinal Mucosal Cell Death: A Patient Who Excreted Long Tubes of Dead Intestinal Epithelium
Authors: Sugimoto Yasuko , Mizutani Hitoshi , Sato Takayuki , Kawamura Noriko , Ohkouchi Kumiko , Shimizu Masayuki
Published: April 2015

TEN is a severe inflammatory disease which is characterized by generalized epithelial destruction. The epidermis is the most common target of TEN, however, any epithelium can be involved. We report a toxic epidermal necrolysis (TEN) patient who excreted long tubes of dead intestinal epithelium. Epidermal keratinocytes and intestinal epithelium were found to undergo extensive apoptosis by TUNEL method. Drugs were speculated as the causative agents for this case, the causative drug has not been identified. In contrast to marked improvement of cutaneous manifestation and hepatic function by methyl prednisolone pulse therapy, the gastrointestinal symptoms did not respond to therapies, and the patient died by heart failure. Present case suggested a pathogenetic mechanism targeting antigens commonly expressed on the gastrointestinal epithelium and epidermis.
The Discrepancy in Hardness between Clinical and Histopathological Findings in Localized Scleroderma Treated with PUVA
Authors: Yamaguchi Keiko , Takeuchi Izuho , Yoshii Noriko , Gushi Akira , Kanekura Takuro , Kanzaki Tamotsu
Published: April 2015

Topical PUVA therapy was applied to a patient with localized scleroderma. Her localized scleroderma responded very well to the topical PUVA therapy, i.e., her sclerotic skin softened to normal skin texture. However, despite this dramatic clinical change the histopathological findings did not change at all and were still “hard”.
HLA Class I and II Alleles and Susceptibility to Generalized Pustular Psoriasis: Significant Associations with HLA‐Cw1 and HLA‐DQB1*0303
Authors: Ozawa Akira , Miyahara Motomi , Sugai Junichi , Iizuka Mariko , Kawakubo Yo , Matsuo Itsuro , Ohkido Muneo , Naruse Taeko , Ando Hitoshi , Inoko Hidetoshi , Kobayashi Hitoshi , Ohkawara Akira , Takahashi Hidetoshi , Iizuka Hajime , Morita Eishin , Yamamoto Shoso , Hide Michihiro , Taniguchi Yoshiki , Shimizu Masayuki
Published: April 2015

HLA alleles in generalized pustular psoriasis (GPP) were investigated to clarify the etiology and/or pathogenesis of this disease. Not only serological typing of HLA class I and II antigens but also genotyping of HLA class II alleles were carried out in twenty‐six unrelated Japanese patients with GPP. These patients were classified according to their history of psoriasis vulgaris (PV). Serological typing revealed a significantly high incidence of HLA‐Cw1 (Pc=0.04) in the patients as compared with Japanese healthy controls. The frequency of HLA‐B46 was particularly high in the patients with GPP and a previous history of PV. Genotyping of HLA class II alleles showed a highly significant increase in HLA‐DQB1*0303 (Pc=0.01) in the patients vs. the healthy controls. In particular, HLA‐DQB1*0303 was significantly more frequent in the patients with no prior history of PV than in those with a history of PV. Analysis on linkage disequilibrium showed remarkably different patterns for HLA class II haplotypes between the patients and the healthy controls. Based on the comparative analysis among the amino acid sequences of the β1‐domain of the HLA‐DQB1*03 alleles, proline at residue 55 was suggested to be important as a common amino acid for determination of the susceptibility to GPP. These results revealed not only an association between the etiology and/or pathogenesis of GPP and HLA, but also different mechanisms of the immune response between the patients with GPP and PV.
Peripheral T‐Cell Activation in Non‐Segmental Vitiligo
Authors: Mahmoud Fadia , Abul Habib , Al‐Saleh Qasem , Haines Donald , Burleson Joe , Morgan Gareth
Published: April 2015

Vitiligo is a hypopigmentary dermatosis of probable autoimmune origin. Previously reported aberrations in peripheral blood mononuclear cells (PBMC), especially T cells and T cell subsets, have been inconsistent. Lymphocyte subpopulations were examined using flow cytometry and monoclonal antibodies against CD4, CD8, CD20, CD25, CD45RA, and HLA‐DR in 34 patients with non‐segmental vitiligo. Twelve patients had not received any previous treatment and 22 had previously received at least one course of PUVA therapy that was discontinued at least four months prior to our study. Compared to matched controls, we found significant increases in CD25 and HLA‐DR in vitiligo patients (p=0.000). An inverse correlation was observed between HLA‐DR and patient status with regard to treatment (p=0.001). These results suggest a role for T cells in the pathogenesis of vitiligo and imply that previous PUVA therapy may be reflected by an alteration in circulating DR + ve cells.
Stevensjohnson Syndrome Caused by a Health Drink (Eberu®) Containing Ophiopogonis Tuber
Authors: Mochitomi Yuji , Inoue Akiyo , Kawabata Hisashi , Ishida Sanemasa , Kanzaki Tamotsu
Published: April 2015

Stevens‐Johnson syndrome is considered to be a severe type of erythema exsudativum multiforme. It is characterized by erythema with bullous and eroded lesions of skin and mucous membranes. We report a case of Stevenjohnson syndrome following consumption of a health drink containing ophiopogonis tuber. A 66‐year‐old female took an O.T.C. health drink for fever. The next morning, she noted erythema and swelling of her face, neck, and chest. She started to develop bullous and eroded lesions on the skin of her entire body and the mucous membranes of her oral cavity, conjunctiva, and cornea, and she became feverish. She had high degrees of corneal erosion and liver dysfunction. Skin biopsy showed diffuse necrosis of the epidermis. After admission to the hospital, steroid pulse therapy (1000 mg/day of methylprednisolone sodium succinate) was continued for 5 days. The health drink induced a positive drug lymphocyte stimulation test (DLST) and patch test. A challenge test was done with a one hundredth dose, and it was positive. We did patch tests with all components of the drink and found that Mai‐Meu‐Dong‐Tang (ophiopogonis) alone was positive at 72 hours. There is no previous report of Stevens‐Johnson syndrome caused by a health drink or Mai‐Meu‐Dong‐Tang. Even though it is a health drink, we should be aware of the possibility of a severe reaction.
A Case of Nonscarring Inflammatory Epidermolysis Bullosa Acquisita: Characterization of IgG Autoantibodies by Immunofluorescence, Immunoblotting and Immunogold Electron Microscopy
Authors: Honoki Kumiko , Muramatsu Tsutomu , Nakatani Chie , Iida Takashi , Shirai Toshihiko
Published: April 2015

We report a case of nonscarring inflammatory epidermolysis bullosa acquisita in a 59‐year‐old Japanese woman. She developed blisters and erosions on her lip, trunk and extremities. Sodium aurothiomalate was effective for the skin lesions. The patient had been free from bullous skin lesions for the last 13 years and had shown no scarring. Indirect immunofluorescence (IF) study on 1 M NaCl‐split skin revealed IgG autoantibodies against the dermal side of the split skin. Immunoblotting using normal human dermal extracts disclosed IgG autoantibodies reactive with the 290 and 145 kD antigens. Circulating IgG autoantibodies were deposited on the lamina densa by immunoelectron microscopy. IF mapping using several antibodies for the components of the basement membrane zone revealed blister formation at the lamina densa. These results suggest that the cleavage at the lamina lucida does not necessarily exclude the diagnosis of EBA and that the definite diagnosis of EBA should be confirmed by immunoblotting or immunoelectron microscopic study.
Congenital Capillary Hemangioma and Its Therapeutic Approach in Infants: A Case Report
Authors: Hassan Elsheikh , Giannakopoulou Christina , Stefanaki Kalliopi , Mathioudaki‐Koumantaki Elma , Delides George , Koumantakis Eugene
Published: April 2015

Hemangiomas, common congenital lesions in infants and children, are thought to arise when islands of angioblastic tissue fail to connect with the developing vascular system. They are not usually life‐threatening.

A case of congenital capillary hemangioma in an infant, which was surgically excised, is reported, and therapeutic approaches are discussed.
A Case of Calcinosis Universalis Successfully Treated with Low‐Dose Warfarin
Authors: Matsuoka Yukari , Miyajima Susumu , Okada Natsuko
Published: April 2015

A 27‐year‐old male patient with calcinosis universalis resulting from dermatomyositis was successfully treated with low‐dose warfarin. On his trunk and extremities, there were many subcutaneous calcified nodules, and knee flexion was difficult. After oral warfarin therapy for three years, the calcified nodules became smaller, and the knee mobility improved. His serum vitamin K level was abnormally high, decreased just after starting warfarin therapy, and then remained within the normal range. Since vitamin K has been known to play an important role in the Ca2+ binding process in bones or tissues, we suggest that this therapy is effective in reducing subcutaneous calcification through the vitamin K cycle.
Poikiloderma‐like Cutaneous Amyloidosis in an Ethnic Chinese Girl
Authors: Ho Man Hon , Chong L.Y.
Published: April 2015

Primary cutaneous amyloidosis is the deposition of amyloid in the skin without involvement of internal organs. It is easily diagnosed when presented in its typical manifestation. Atypical or rare clinical presentations can pose diagnostic difficulties. Poikiloderma‐like cutaneous amyloidosis (PCA), a rare variant of primary cutaneous amyloidosis, was first reported in the literature in 1936 (1). It is characterised by: 1) poikilodermatous skin lesions; 2) lichenoid papules; 3) cutaneous amyloid deposit in the pigmented and lichenoid lesions; 4) light sensitivity; 5) short stature; and 6) other features such as blister formation or palmoplantar keratosis. Ogino coined the term PCA syndrome when these unusual features present early in life (2). We report a 26‐year‐old Chinese woman who presented with poikilodermatous skin lesions and was misdiagnosed as poikiloderma atrophica vasculare (PAV) on the basis of clinical appearance without any histological proof. The diagnosis of PCA was made after skin biopsy which showed amyloid deposits in the skin. This condition can easily be confused with other true poikiloderma skin diseases. Histology is important in confirming the diagnosis.
A Case of Congenital Infantile Fibrosarcoma of the Right Hand
Authors: Kimura Chu , Kitamura Takashi , Sugihara Tsuneki
Published: April 2015

Congenital infantile fibrosarcoma is a rare soft‐tissue malignant tumor that usually presents as a mass involving the arm or leg. We report a case of congenital fibrosarcoma on the right hand of a newborn infant.

The patient was a 12‐day‐old male. From the time of delivery, he had a red tumor on his dorsal right hand. The tumor was initially associated with bleeding within a hemangioma and compressed. The course was observed. It appeared that the tumor might be malignant because it increased in size to 10 × 8 × 3 cm, and ulceration and bleeding appeared on the surface after 10 days. A biopsy suggested a fibrosarcoma.

The tumor was resected 32 days after birth at the narrow margin in consideration of preservation of function.

However, a recurrence was observed under the graft after 2 weeks and another resection was performed. Because the tumor had passed through the interosseous muscle and invaded the palm of the hand, the middle finger, ring finger, and little finger had to be amputated. One year and 7 months postoperatively, no recurrence or metastasis has been observed.
Eosinophilic Pustular Folliculitis (Ofuji's Disease) in Myelodysplastic Syndrome
Authors: Jang Kyoung‐Ae , Chung Sung‐Tae , Choi Jee‐Ho , Sung Kyung‐Jeh , Moon Kee‐Chan , Koh Jai‐Kyoung
Published: April 2015

We describe a case of eosinophilic pustular folliculitis (EPF, Ofuji's disease) in a 12‐year‐old male who suffered from myelodysplastic syndrome. Bone marrow study revealed an increase in the eosinophil cell line without peripheral blood eosinophilia in our case.

We suggest that the immunologic abberations ascribed to myelodysplastic syndrome and the increase in the eosinophil cell line in the bone marrow might play roles in the development of EPF in our case.
Fibromyxoma of the Skin
Authors: Watanabe Takahiro , Okochi Hitoshi , Kikuchi Kanako , Furue Masutaka
Published: April 2015

A patient presented with a solitary large subcutaneous tumor homogeneously composed of loose fibroblasts interspersed with abundant mucinous material. The literature was reviewed, and the origin, pathogenesis and clinical course of this rare neoplasm were briefly discussed.
Digital Images in Dermatology and the Dermatology Online Atlas on the World Wide Web
Authors: Diepgen Thomas L. , Eysenbach Gunther
Published: April 2015

The digital revolution opens new ways of storing, retrieving and distributing images. The informativeness of digital images of dermatological conditions as compared to conventional photographs and slides has proven to be statistically similar; in many cases, digital images may even substitute for dermatologic physical examination. Current developments in high‐speed, high‐capacity international networks and the growing popularity of the World Wide Web are converging in ways that have great potential for enhancing access to biomedical information, including medical images. However, lack of metainformation and indexing services specialised in retrieving images makes finding images—as opposed to textual information—on the Web difficult. To provide an image collection and entry point into dermatological resources on the World Wide Web, we have developed an image database (Dermatological Online Atlas—DOIA). The database, which is freely available on the Internet at http://dermis.net, contains about 3,000 clinical images covering more than 600 dermatological diagnoses. It is designed for worldwide use; international submissions are encouraged. It serves as a teaching tool for medical students, doctors and patients, assists professional users by being an easy gateway to other databases such as MEDLINE, PDQ, and OMIM and serves as a platform for conducting research by means of administering Internet surveys to users. We conclude that an online image atlas has multiple educational, clinical, and research applications.
Principles of Hair Cycle Control
Authors: Paus Ralf
Published: April 2015

The hair follicle (HF) undergoes life‐long cyclic transformations between “resting” (telogen), growth (anagen), and apoptosis‐driven regression (catagen). Contrary to conventional wisdom, cyclic remodelling affects even the distal HF epithelium; telogen is not a mere resting period, since it shows substantial metabolic and proliferative activity and may encompass a phase of controlled hair shaft‐extrusion (“exogen”). Even under physiological circumstances, very few (malfunctioning?) HF may leave this cycle over time to be removed by inflammatory cells (“programmed organ deletion”). Although numerous systemic, metabolic, immunological, and nerve‐derived factors (e.g. hormones, cytokines, neuropeptides, neurotransmitters, mast cells) can profoundly alter hair growth in vivo, neither vascular nor neural stimuli nor extrafollicular cells are essential for HF development or cycling. Rather, an intrafollicular “hair cycle clock” of as yet unknown nature drives the HF cycle. This elusive chronobiological timing device likely exploits secondary changes in the intra‐ and perifollicular signalling milieu for guiding the HF through its transformations. However, the supreme generator of cycling activity (“oscillator”) that dictates any of these signalling switches is still as unknown as is its exact location. Since, clinically, the control of catagen is of paramount importance (too early anagen termination: alopecia, effluvium; catagen too late: hirsutism, hypertrichosis), the controls of catagen‐associated keratinocyte apoptosis and of dermal papilla secretory activities are discussed as crucial targets for future therapeutic manipulations.
Dermatology in Germany
Authors: Barth Joachim
Published: April 2015

In Germany, dermatology has a long tradition as a medical specialization. The first dermatological university departments were established about 120 years ago. From the beginning, venerology was integrated in this field. Today it also covers andrology, allergy, medical cosmetology, mycology, dermatosurgery, phlebology and photodermatology. This broad spectrum more and more gives rise to competition with other medical fields. About 77% (n=3281) of all German dermatologists (n=4240 in 1997) work in private practices. The others are employed in clinical departments. The official number of working physicians in Germany in 1997 was 343,556; about 1.5% of them were dermatologists. This means that one dermatologist takes care of 20,000 people. The overwhelming majority of dermatological patients directly contacts the specialist and are not referred by general practitioners (GPs) who comprise about 40% of all German physicians. This is a great advantage over those countries in which patients primarily have to consult the GP. At present, there is a discussion initiated by GPs to change this system in Germany and to reestablish the GP's role as “gatekeeper”. Dermatologists together with other specialists are trying to prevent this an to maintain the traditional broad spectrum of German dermatology.
Urinary Porphyrin Excretion Measurements in Healthy Neonates
Authors: Ozalla M. Dolores , Herrero Carmen , Ventura Pere‐Joan , Lecha Màrius , Alvarez Luisa , Mascaro José M.
Published: April 2015

The objective of this study was to establish normal reference ranges for porphyrins in healthy neonates. There is little information about urinary porphyrin excretion in this age group. This knowledge may provide an early diagnostic tool for detecting subtle alterations or latent forms in disorders of heme biosynthesis. Fifty healthy neonates were selected from the Department of Obstetrics. Total urinary porphyrins in random specimens were analysed by a spectrofluorometry method. The measurement of porphyrin fractions was made by fluorometric high‐performance liquid chromatography (HPLC). The results were adjusted to urinary creatinine excretion to correct any imprecision and interindividual variation in body mass. The urinary total porphyrin had a median value of 331.50 (nmol/L). A statistically significant relationship between total porphyrin (nmol/L) and creatinine (mmol/L) was found (p<0.01) The porphyrin/creatinine ratio showed a median value of 56.30 nmol/mmol creatinine. The study of individual porphyrins revealed that coproporphyrin and uroporphyrin were the major porphyrins excreted in neonates (coproporphyrin represents 81.98% and uroporphyrin 16.64% of total porphyrin); in both cases, isomer I was predominant with median values of 22.36 and 6.25 nmol/mmol creatinine, respectively. No significant relationships were found between porphyrin excretion and sex, gestational age, weight, or height. Our data provide the reference limits for porphyrins in neonates as a diagnostic guideline for evaluation of subtle alterations in heme biosynthesis.