Vascular network tightly enclosing lesions of molluscum contagiosum: Basket-like capillaries of molluscum.

Immunohistochemical studies of one typical and two atypical cases of molluscum contagiosum with anti-CD34 monoclonal antibodies showed a tightly enclosing fine vasculature around the lesional masses of the disease. The thin interstitial septa between the lobules of the molluscum lesions also contained abundant endothelium. An electron microscopic study of a pinched-off lesion of common molluscum contagiosum demonstrated that the tightly enclosing blood vessels lacked muscle layers, suggesting that they were capillaries, being a distance of several hundred nanometers from the basal cells of the molluscum mass. A 3D constructed image of the vasculature confirmed a network of the vessels. These tightly enclosing vascular networks around the lesions of molluscum contagiosum support the rapid growth of this disease.

Case of bilateral acroangiodermatitis due to symmetrical arteriovenous fistulas of the soles.

A 58-year-old woman presented painful chronic inflammation of the outside skin of the bilateral soles. A skin biopsy revealed chronic inflammation of the dermis and subcutaneous tissue, as well as the marked dilatation and proliferation of capillaries and veins, and the thick deposition of elastic fibers around the thick-walled veins. 3-D contrasted computed tomography of the lower extremities demonstrated early bilateral imaging of the veins, and fistulas of the peroneal as well as posterior tibial artery and vein of the bilateral soles. On the basis of a diagnosis of bilateral acroangiodermatitis due to symmetrical arteriovenous fistulas, the patient underwent compression therapy using elastic bandages, and the symptoms reduced in several days.

Perineural xanthoma associated with type 2 diabetes mellitus and hyperlipidemia.

It is well known that patients with diabetes mellitus (DM) may demonstrate skin manifestations, or dermadromes, due to disease-related metabolic, vascular, neurologic, and/or immunologic disturbances; however, the pathogenesis of some of these manifestations remains unknown. Xanthomas often are associated with increased levels of serum cholesterol and/or triglycerides and therefore can present as a dermadrome in patients with a history of uncontrolled DM and hyperlipidemia. The presence of tender lesions in this patient population can indicate a diagnosis of perineural xanthoma. We report a case of perineural xanthoma arising in a patient with type 2 DM and hyperlipidemia.

Heterogeneity of Brunsting-Perry type pemphigoid: a case showing blister formation at the lamina lucida, immune deposition beneath the lamina densa and autoantibodies against the 290-kD polypeptide along the lamina densa.

An otherwise healthy 31-year-old man presented with multiple, vesicular, subepidermal blistering on the head, face, chest and oral cavity, leaving shallow scar formation, typical of Brunsting-Perry type pemphigoid. Direct immunofluorescence showed linear deposition of immunoglobulin (Ig)G and C3 along the basement membrane zone (BMZ), and indirect showed anti-BMZ autoantibodies (IgG, >40×) reacting with the dermal side under the salt-split study. Immunofluorescence staining for type IV collagen and laminins, as well as routine electron microscopy, demonstrated that the cleavage level of the blister was intra-lamina lucida. The immunoperoxidase method applied to lesional skin demonstrated IgG deposits along the lamina densa. The post-embedding immunogold method demonstrated that the autoantibodies against BMZ reacted with the lamina densa and the dermis just beneath it. Immunoblot studies demonstrated that the autoantibodies reacted with the 290-kD polypeptide (suggesting type VII collagen) when dermal extract was used as the substrate. The patient was treated with combination therapy consisting of 30 mg prednisolone, 900 mg nicotinamide and 750 mg tetracycline, and the number of newly forming blisters decreased. We concluded that Brunsting-Perry type pemphigoid, a rare autoimmune blistering disease, includes cases showing characteristics of epidermolysis bullosa acquisita as well as bullous pemphigoid. This case showed discrepancy between the blistering level (intra-lamina lucida) and location of antigen (lamina densa and sub-lamina densa areas).

A randomized double-blind trial of intravenous immunoglobulin for pemphigus.

BACKGROUND: Pemphigus is a rare life-threatening intractable autoimmune blistering disease caused by IgG autoantibodies to desmogleins. It has been difficult to conduct a double-blind clinical study for pemphigus partly because, in a placebo group, appropriate treatment often must be provided when the disease flares. OBJECTIVE: A multicenter, randomized, placebo-controlled, double-blind trial was conducted to investigate the therapeutic effect of a single cycle of high-dose intravenous immunoglobulin (400, 200, or 0 mg/kg/d) administered over 5 consecutive days in patients relatively resistant to systemic steroids. METHODS: We evaluated efficacy with time to escape from the protocol as a novel primary end point, and pemphigus activity score, antidesmoglein enzyme-linked immunosorbent assay scores, and safety as secondary end points. RESULTS: We enrolled 61 patients with pemphigus vulgaris or pemphigus foliaceus who did not respond to prednisolone (> or =20 mg/d). Time to escape from the protocol was significantly prolonged in the 400-mg group compared with the placebo group (P < .001), and a dose-response relationship among the 3 treatment groups was observed (P < .001). Disease activity and enzyme-linked immunosorbent assay scores were significantly lower in the 400-mg group than in the other groups (P < .05 on day 43, P < .01 on day 85). There was no significant difference in the safety end point among the 3 treatment groups. LIMITATION: Prednisolone at 20 mg/d or more may not be high enough to define steroid resistance. CONCLUSION: Intravenous immunoglobulin (400 mg/kg/d for 5 d) in a single cycle is an effective and safe treatment for patients with pemphigus who are relatively resistant to systemic steroids. Time to escape from the protocol is a useful indicator for evaluation in randomized, placebo-controlled, double-blind studies of rare and serious diseases.

Early blistering, poikiloderma, hypohidrosis, alopecia and exocrine pancreatic hypofunction: a peculiar variant of Rothmund-Thomson syndrome?

A 20-year-old male developed early blistering, poikiloderma, hypohidrosis, alopecia and exocrine pancreatic hypofunction caused by atrophy and fatty replacement of the pancreas. At 5 months of age, he initially presented at the hospital with numerous blisters on his extremities, inguinal and genital area. A biopsy specimen from a vesicular lesion showed a subepidermal bulla. Electron microscopic examinations of a vesicular lesion revealed vacuolar changes of the basal cells without hemidesmosomes. Subsequently, the blisters gradually resolved and healed without scars. At the age of 11, he was admitted for the treatment of cellulitis on his foot and at that time, laboratory examinations detected a decreased level of pancreatic enzymes due to exocrine pancreatic hypofunction. Abdominal ultrasonography and computed tomography (CT) showed the pancreas to be atrophic with fatty replacement. A genetic analysis revealed no mutation in his RECQL4 gene, which is responsible for the pathogenesis of Rothmund-Thomson syndrome (RTS). Although marked blister formation and exocrine pancreatic hypofunction are unusual complications of RTS, this case showed many typical clinical features of RTS. Therefore, this case was considered to be a peculiar variant of RTS.

Tinea corporis with acute inflammation caused by Trichophyton tonsurans.

A 13-year-old Japanese boy presented with acute skin inflammation on the extremities. He belonged to a judo club of a junior high school in which club tinea capitis and tinea corporis seemed to be prevalent. Vesicles and pustules appeared on his right forearm and right leg. They increased in numbers and formed annular lesions. Pruritic erythema appeared surrounding these lesions. Direct microscopic examination of the lesions detected hyphae, and culture for the fungi yielded yellowish colonies. The result of culture from pustules revealed Staphylococcus aureus. At first, a topical antifungal drug and systemic antibiotics seemed to cure annular lesions, but pustules arose again. A large surrounding erythema was cured by topical treatment with a steroid agent. A biopsy specimen from a pustule showed hyphae of fungi within a hair shaft and in the bulb. The restriction fragment length polymorphism in the internal transcribed spacer regions of the ribosomal gene (polymerase chain reaction restriction fragment length polymorphism) revealed a banding pattern compatible with Trichophyton tonsurans. Treatment with systemic itraconazole was begun and lesions disappeared immediately. Systemic antifungal therapy was needed in our case. Tinea corporis with inflammation necessitates systemic antifungal therapy.

Linear IgA dermatosis: report of an infantile case and analysis of 213 cases in Japan.

A 3-year-old boy presented with multiple vesicles, showing a rosette-like arrangement around the crusts. Histopathological and immunohistochemical examinations demonstrated subepidermal blistering with neutrophilic infiltration associated with deposition of IgA, but not IgG, linearly distributed along the basement membrane zone (BMZ) of the epidermis. Indirect immunofluorescence revealed circulating antibodies (IgA class, x160) against the BMZ of guinea pig lip skin. Based on the diagnosis of linear IgA dermatosis (LAD) of childhood, administration of dexamethasone (2 mg/day) was started, and the eruptions diminished immediately. Western blot analysis using extract of the HaCaT cell as a substrate, demonstrated the corresponding antigen at 120-kDa molecular weight. There have been 213 cases of LAD reported in Japan including conference abstracts and these were studied to determine whether infantile cases differed from adult ones, and whether cases associated with IgG as well as IgA (IgA/G type), differed from the cases associated with IgA only (IgA type). IgG contributed less frequently to the infantile type (age of onset, < or =15 years) than to the adult type (age of onset, > or =16 years). Clinical appearance did not show any obvious difference between the IgA/G type and IgA type. However, three-quarters of cases showing localization of antigen to the dermal side were the IgA/G type.

Longitudinal epidemiology of Chlamydia trachomatis serovars in female patients in Japan.

The objective of this study was to clarify the longitudinal epidemiology of Chlamydia trachomatis serovars in Japan. A total of 339 endocervical swab specimens obtained from female patients who attended the Department of Obstetrics and Gynecology, Saitama Medical School, were used. Positive specimens of either transport medium of IDEIA Chlamydia (1st group, from 1999 to 2001), or DNA extract of Cobas Amplicor STD-1 Chlamydia trachomatis (2nd group, from 2003 to 2005) were used for serotyping. Typing of C. trachomatis serovars in DNA extracts was performed by polymerase chain reaction-restriction fragment length polymorphism. Ten serovars, A, B, D, E, F, G, H, I, J and K, were identified in the 1st group, and serovar E was most frequently identified (27.6%). In the 2nd group, nine serovars, B, C, D, E, F, G, H, I and K, were identified, and serovar D was most frequently identified (24.7%). Serovars B and Ba were significantly more common around 2000 and the mid-1990s (from 1993 to 1996), respectively. Numbers of serovar I increased significantly during the research period. In addition, serovar I was more frequent in the 2nd group than in the 1st group in women aged 20-29 years. There were no significant differences of serovar distribution between pregnant and non-pregnant women.