Yuichi Teraki

Stevens–Johnson syndrome and toxic epidermal necrolysis due to anticonvulsants share certain clinical and laboratory features with drug‐induced hypersensitivity syndrome, despite differences in cutaneous presentations

Background.  Drug‐induced hypersensitivity syndrome (DIHS)/drug rash with eosinophilia and systemic symptoms (DRESS) syndrome is characterized by late disease onset, fever, rash, hepatic dysfunction, haematological abnormalities, lymphadenopathy and often, human herpesvirus (HHV) reactivation. The diagnosis of DIHS is based on the combined presence of these findings. Anticonvulsants are a major cause of DIHS and may also cause Stevens–Johnson syndrome (SJS) and toxic epidermal necrolysis (TEN). We examined whether SJS/TEN due to anticonvulsants display similar clinical and laboratory features seen in DIHS.

A case of generalized psoriasiform and pustular eruption induced by infliximab: evidence for skin-homing Th17 in the pathogenesis

cerned that other TNF antagonists might favour the same hepatotoxicity as infliximab. Ustekinumab treatment proved to be rapid and very effective, achieving an almost complete clearance of the lesions after only two injections. The appearance of some new lesions inside the plaques on remission at week 11 was considered a recurrence of the disease rather than a psoriasiform eruption with a change of morphology as described associated with TNF antagonists. A weight of 92 kg, or the fact that it was a very severe and highly resistant psoriasis, might explain why this reaction was easily controlled with topical therapy, and did not require an increase in the ustekinumab dose nor a reduction of the administration interval. To date, the drug has been demonstrated to be safe. To our knowledge, there are no reports in the literature regarding the use of ustekinumab in patients with pustular psoriasis. Our case demonstrates that ustekinumab can be very effective in the treatment of pustular psoriasis unresponsive to other conventional and biologic therapies. Further evaluation of the drug is warranted in this variant of psoriasis to define its proper role, especially for severe cases.

Annular elastolytic giant cell granuloma associated with diabetes mellitus: a case report and review of the Japanese literature.

its natural course unknown. Hypertrophic scars are benign fibroproliferative disorders of the wound-healing process, thought to be caused by altered regulation of extracellular matrix production by fibroblasts in predisposed individuals, arguably representing a process occurring in the dermal layer of the skin. However, a clear pathogenic mechanism remains to be elucidated. To our knowledge, this is the first case reported of widespread hypertrophic scarring in infancy following chickenpox. Our patient showed a partial spontaneous improvement 6 months after her first assessment. This case highlights an unusual dermatological complication of chickenpox, which can potentially lead to significant functional or aesthetic disfigurement.

Tacrolimus-induced rosacea-like dermatitis: a clinical analysis of 16 cases associated with tacrolimus ointment application.

Background: Recently, reports have indicated that the continuous use of topical calcineurin inhibitors such as tacrolimus may induce rosacea-like dermatitis (RD). Objective and Methods: To assess clinical features of RD associated with tacrolimus, 44 cases of patients diagnosed with RD between 2005 and 2010 at our hospital were retrospectively reviewed. Results: In total, 22 cases were caused by topical steroid use, 8 by topical tacrolimus use, and 8 by consecutive treatment with topical steroids and tacrolimus. Clinical presentation was basically similar among the 3 groups, although the nose was less frequently affected and pustules were rarely observed in the latter 2 sets of cases. Demodex mites were often found in smears of skin lesions from patients with RD caused by steroids and tacrolimus. Treatment with topical metronidazole was effective in most RD patients. Conclusion: Topical tacrolimus is becoming an important cause of RD along with topical steroids.

High Incidence of Internal Malignancy in Papuloerythroderma of Ofuji: A Case Series from Japan

Background: Although it has been reported that papuloerythroderma of Ofuji (PEO) often occurs in association with internal malignancy, the true incidence of malignancy in patients with PEO is unknown. Objective and Methods: To ascertain the incidence of and relationship with internal malignancy in patients with PEO, 11 patients with PEO diagnosed at our dermatology clinic between September 2005 and June 2011 were retrospectively reviewed. Results: Internal malignancy was found in 6 (54.5%) of the 11 PEO patients, and 5 cases were idiopathic PEO. In the 6 cases with associated malignancy, PEO preceded the malignancies, and the diagnosis of malignancy was made just before or shortly after the diagnosis of PEO, but the malignant process and PEO did not always run a parallel course. Conclusions: Although the limitations of this study included a relatively small sample size, the present findings show a high incidence of internal malignancy in patients with PEO.

Genotype–phenotype correlations in six Japanese patients with recessive dystrophic epidermolysis bullosa with the recurrent p.Glu2857X mutation

BACKGROUND General genotype-phenotype correlations have been delineated in recessive dystrophic epidermolysis bullosa (RDEB), but these remain complicated and it is still difficult to assess the clinical consequences of individual COL7A1 mutations. OBJECTIVE To characterize recurrent p.Glu2857X mutations and show how other COL7A1 mutations influence the phenotype in RDEB patients harboring p.Glu2857X. METHODS Genotype-phenotype correlations were studied in six Japanese RDEB patients with the p.Glu2857X mutation. RESULTS Besides the common p.Glu2857X mutation, premature termination codon (PTC) mutations were found in three patients, glycine substitution missense mutations in two patients, and a non-glycine substitution missense mutation in one patient. PTC mutations in both alleles generally cause the most severe, mutilating Hallopeau-Siemens (HS) variant of RDEB, whereas none of the PTC mutations resulted in severe phenotypes consistent with the HS subtype when coupled with p.Glu2857X. Missense glycine and non-glycine mutations caused phenotypes of differing severity, suggesting that the extent of destabilization of anchoring fibrils depends on the type of mutation. CONCLUSION A p.Glu2857X mutation exhibits mild pathogenic effects compared to other PTC mutations in COL7A1, and its uniqueness enables detailed analysis and comparison of the destabilizing effects of missense mutations in RDEB patients.

Successful treatment of stucco keratosis with maxacalcitol.

was scraped off the slides, suspended in 50–100 lL of a solution containing 0Æ1 mol L Tris–HCl (pH 8Æ0) and 1 lg lL proteinase K, incubated at 55 C overnight, boiled for 10 min, and finally stored at )20 C until analysed. To amplify HSV DNA using PCR, a pair of primers (Research Genetics, Huntsville, AL, U.S.A.) defining a 92-bp segment of the DNA polymerase gene was used, as described by Cao et al. PCR reactions were performed with AmpliTaq DNA polymerase (Applied Biosystems, Foster City, CA, U.S.A.) under the following conditions: denaturation at 94 C for 45 s, annealing at 63 C for 30 s, and elongation at 72 C for 30 s. After 40 cycles of amplification, the reaction mixture was electrophoresed on a 2Æ5% agarose gel (FMC, Rockland, ME, U.S.A.) and stained with ethidium bromide to visualize the DNA. Southern blot hybridization was performed with an internal P-labelled HSVspecific oligonucleotide probe and was evaluated on X-ray film. All 24 PLE specimens that we examined were negative for HSV DNA by PCR analysis and subsequent Southern blot hybridization (Table 1, Fig. 1). In contrast, 10 of 31 EM specimens (32%) were positive for HSV DNA. This rate of HSV positivity in EM cases was comparable with 35–72% positivity rates seen in previous studies. Interestingly, Yokoi et al. detected HSV DNA in four of seven specimens from postherpetic EM but in none of three specimens from photoinduced EM. However, our inability to find HSV DNA presenting in any PLE skin samples contradicts the hypothesis that a direct immune response to HSV in the skin is involved in the pathogenesis of PLE.

Pemphigoid nodularis associated with psoriatic erythroderma : successful treatment with suplatast tosilate

is difficult to attribute any significance to this change due to the different conditions under which the two figures were derived. It is also true that sensitivity and specificity can trade one against the other. It is for that reason that we used the receiver operator characteristic (ROC) curve as our measure of performance. Using this measure we found no significant difference between the investigator with and without the SIAscope. It is incorrect to claim that the SIAscope performs as well as a dermatologist with 20 years’ experience. The expert dermatologist disagreed with the investigator using the SIAscope in 45% of cases where a biopsy was requested. The expert dermatologist together with biopsy data was used as a gold standard to measure the investigator’s performance with and without the SIAscope. The ROC curve shows that the investigator performed well with and without the SIAscope.

Unilateral prurigo pigmentosa: A report of two cases

of Helicobacter cinaedi infection. Acta Derm Venereol 2013; 93: 165–167. 3 Minauchi K, Takahashi S, Sakai T et al. The nosocomial transmission of Helicobacter cinaedi infections in immunocompromised patients. Intern Med 2010; 49: 1733–1739. 4 Kawamura Y, Tomida J, Morita Y, Fujii S, Okamoto T, Akaike T. Clinical and bacteriological characteristics of Helicobacter cinaedi infection. J Infect Chemother 2014; 20: 517–526. 5 Ishibashi R, Nakamura S, Takemoto M, Mukai C, Yokote K. Helicobacter cinaedi infection in patients with diabetes: a case report. Springerplus 2015; 4: 72.

Eosinophilic pustular folliculitis associated with hematological disorders: A report of two cases and review of Japanese literature

Eosinophilic pustular folliculitis (EPF) occurs in patients with hematological disorders. However, clinical information about hematological disorder‐associated EPF is scarce. We report two cases of EPF associated with mantle cell lymphoma and reviewed the available published work on Japanese cases. We identified a total of 23 Japanese cases, including the two cases reported here, who had hematological disorder‐associated EPF. Fourteen cases were associated with treatment for hematological malignancies (transplantation‐related EPF) and nine cases were associated with hematological malignancies themselves (hematological malignancy‐related EPF). Although the skin eruption was clinically indistinguishable between the two subtypes, transplantation‐related EPF occurred on the face and trunk of young and middle‐aged men and women, whereas hematological malignancy‐related EPF occurred mostly on the face of older men. Peripheral blood eosinophilia was more frequently observed in transplantation‐related EPF. These observations suggest variations among patients with EPF associated with hematological disorders.