Tomoko Nomiyama

Trigeminal trophic syndrome: Report of a case and review of the published work

Trigeminal trophic syndrome is a rare complication of trigeminal nerve injury that causes facial ulceration, anesthesia and paresthesia in the same trigeminal dermatomes. We present a case of a 65‐year‐old woman with a history of meningioma resection 18 years prior who presented 16 years later with an intractable ulceration around her left nasolabial sulcus. Pain and light‐touch sensations around the ulcer were decreased. She admitted to frequent manipulation due to a crawling sensation. A skin biopsy showed acanthotic changes and a decreased number of peripheral nerve fibers. Trigeminal trophic syndrome was diagnosed. Carbamazepine was not effective, and the ulcer persisted at 7 months after the initial presentation. We reviewed 36 English‐language publications from 2003 to 2012, and analyzed 61 cases of trigeminal trophic syndrome, including this patient. The mean age was 53.3 ± 19.7 years (range, 6–91). The right side of the face was more commonly affected (57%) than the left side. The ala nasi were involved in 48 cases (79%), followed by the cheek in 17 cases (28%). A corneal lesion was observed in 11 cases (18%), suggesting the importance of ophthalmologic consultations. The two major etiologies were trigeminal nerve ablation (18 cases; 30%) and cerebrovascular accidents (18 cases; 30%). The latent period ranged from days to 30 years. Gabapentin and carbamazepine were frequently administrated with variable efficacy. Application of thermoplastic dressings or negative pressure wound therapy demonstrated favorable outcomes. Surgery was an option with a high recurrence rate. Trigeminal trophic syndrome remains a clinical challenge.

Increased plasma LIGHT levels in patients with atopic dermatitis

LIGHT [the name of which is derived from ‘homologous to lymphotoxins, exhibits inducible expression, competes with herpes simplex virus glycoprotein D for herpes simplex virus entry mediator (HVEM), and expressed by T lymphocytes’], is a member of the tumour necrosis factor superfamily that is involved in various inflammatory diseases. We aimed to estimate the relevance of plasma LIGHT levels as a biomarker for atopic dermatitis (AD). In order to understand the putative role of LIGHT in AD pathogenesis, we also investigate the effects of LIGHT on a monocytic cell line, human acute monocytic leukaemia cell line (THP‐1). We examined plasma LIGHT levels, total serum IgE, serum value of CCL17 and peripheral blood eosinophil counts in patients with AD and healthy subjects. The effects of LIGHT on activation and apoptosis in THP‐1 cells were also investigated. The plasma concentrations of LIGHT in AD patients were significantly higher than those in healthy individuals and the concentrations decreased as the symptoms were improved by treatment. The LIGHT plasma concentrations correlated with IgE levels and the Severity Scoring of AD (SCORAD) index. In addition, LIGHT stimulation increased expression of CD86 and induced production of interleukin‐1β in THP‐1 cells. Apoptosis was inhibited, the Bcl‐2 level increased and the caspase‐3 level decreased in THP‐1 cells stimulated with LIGHT, compared to unstimulated control cells. These results suggest that plasma LIGHT levels may be one of the promising biomarkers for AD.

Three hundred and eight nanometer excimer light therapy for alopecia universalis that is resistant to other treatments: A clinical study of 11 patients.

Three hundred and eight nanometer excimer light therapy has recently been reported to be effective against patchy alopecia areata (AA) in several clinical studies. However, these studies only included a few patients with severe forms of AA, and all of them exhibited poor outcomes. We further investigated the use of excimer light as a therapeutic option for cases of alopecia universalis (AU) that are resistant to other treatments. Eleven treatment‐resistant AU patients were treated with a 308‐nm excimer light at 2‐week intervals for more than 16 sessions. Four patients achieved good responses and two patients exhibited poor responses. Three patients had Japanese skin type 1 and all of them achieved good responses. The radiation dose was increased until the patients exhibited marked erythema. The patients with Japanese skin type 3 who achieved good responses exhibited strong pigmentation at the irradiated sites. In conclusion, 308‐nm excimer light therapy has significant effects on some AU patients who are resistant to other treatments and may be an alternative therapeutic option for AU. During the treatment of AU, high doses of radiation should be administrated until a strong inflammatory skin reaction is seen.

Dermoscopic insight into skin microcirculation--Burn depth assessment.

To investigate the effectiveness of dermoscopic observation of skin microcirculation, the dermal capillary integrity of burn wounds was evaluated by dermoscopy according to a proposed algorithm that is designed to distinguish burn wounds between superficial dermal burns: SDB, and deep dermal burns: DDB. As the gold standard for comparison, two widely accepted endpoints of primary healing within 21 days (SDB) or over 21 days after injury (DDB) were used. A number of dermatologists conducted diagnostic imaging by dermoscopy. Comparison among polarized noncontact dermoscopy (PNCD), polarized contact dermoscopy (PCD) and nonpolarized contact dermoscopy (NPD) was also conducted. Images from the three modalities were evaluated for color, pattern and qualitative differences among them. The results of dermoscopy measurements according to the proposed algorithm showed accuracy of 96.7%, sensitivity of 100.0% and specificity of 94.4%. Dermoscopy measurements were significantly more accurate than clinical assessment (p<0.05). The recognition of dots increased for NPD, vessels were most clearly observed under PCD and colours tended to be more distinctly recognized under polarized light. Dermoscopy is a useful and simple tool to evaluate not only epidermal and superficial dermal skin components but also the skin microcirculation.

Myoepithelial carcinoma on the right shoulder: Case report with published work review

Myoepithelial carcinoma is a malignant tumor that can differentiate towards myoepithelial cells and commonly occur in the salivary glands. There have been only a few reports of primary cutaneous myoepithelial carcinoma; however, most cases showed subcutaneous involvement and could also be diagnosed as soft tissue myoepithelial carcinoma arising from the subcutis with dermal involvement. It may thus be impossible to distinguish a primary cutaneous from a soft tissue myoepithelial carcinoma. Herein, we describe a case of myoepithelial carcinoma on the shoulder in an 85‐year‐old Japanese woman. The tumor was located in the whole dermis and subcutis; therefore, it could be diagnosed as either a cutaneous or soft tissue myoepithelial carcinoma. We reviewed previous cases of primary cutaneous and soft tissue myoepithelial carcinomas and compared their clinical and immunohistological features. We found no obvious differences in anatomical distribution or immunohistochemical findings. However, the recurrence rate of cutaneous myoepithelial carcinomas seems to be lower than that of soft tissue carcinomas. Such a difference may be attributable to the adequate surgical margin in cutaneous carcinomas compared with the deep‐seated soft tissue carcinomas. The metastatic frequency did not significantly differ between the two types. Although we could summarize from only a small number of cases, these results indicate the difficulty in distinguishing between cutaneous and soft tissue myoepithelial carcinomas; furthermore, it may not be suitable to distinguish them on the basis of aggressive behavior.

Granuloma annulare-like reaction to the bacillus Calmette-Guerin vaccination

We report the case of a 6‐month‐old girl with a granuloma annulare (GA)‐like reaction to the bacillus Calmette‐Guerin (BCG) vaccination. The eruption developed at the vaccination site 1 month after vaccination and the lesion gradually disseminated over the body within 2 months. A biopsy specimen of the skin lesion showed degenerated collagen bundles surrounded by imperfect palisading histiocytes, lymphocytes and epithelioid cells in the dermis, which led to a diagnosis of GA‐like reaction as a secondary reaction to BCG inoculation. The eruption at the vaccination site and the scattered GA reaction resolved after 1 month of treatment with prednisolone valerate acetate ointment, leaving only pigmentation.

Unusual presentation of relapsing neutrophilic dermatosis limited to the face

1 Kere J, Srivastava AK, Montonen O et al. X-linked anhidrotic (hypohidrotic) ectodermal dysplasia is caused by mutation in a novel transmembrane protein. Nat Genet 1996; 13: 409–416. 2 Monreal AW, Zonana J, Ferguson B. Identification of a new splice form of the eda1 gene permits detection of nearly all x-linked hypohidrotic ectodermal dysplasia mutations. Am J Hum Genet 1998; 63: 380–389. 3 Monreal AW, Ferguson BM, Headon DJ, Street SL, Overbeek PA, Zonana J. Mutations in the human homologue of mouse dl cause autosomal recessive and dominant hypohidrotic ectodermal dysplasia. Nat Genet 1999; 22: 366–369. 4 Kervestin S, Jacobson A. Nmd: a multifaceted response to premature translational termination. Nat Rev Mol Cell Biol 2012; 13: 700–712. 5 Fredrickson EK, Gardner RG. Selective destruction of abnormal proteins by ubiquitin-mediated protein quality control degradation. Semin Cell Dev Biol 2012; 23: 530–537.

Immunohistological analysis of in-transit metastasis in a patient with advanced melanoma treated with combination therapy of cytosine guanine dinucleotide oligodeoxynucleotide, dacarbazine and beta-interferon: a case report.

cal “congenital pattern features’’ could be seen. The nodule at the margin of the plaque was confirmed as MM because of characteristic dermoscopic and histological findings. An epidermal melanoma was supposed to occur at the margin of a medium-sized CMN. Although the pathogenesis of malignant transformation is not clear, two possibilities are supposed. The first is the chance theory in which the risk is thought to be increased by the innumerable melanocytes in CMN. The second is a genetic predisposition. Activating mutations within the kinase domain of the BRAF gene are a common somatic event in MM as well as in melanocytic nevi. Small CMN and acquired nevi are inclined to demonstrate BRAF mutations, while giant CMN possess NRAS mutations. MM occasionally have NRAS mutations in addition to the frequent BRAF mutations. Although the contribution to malignant transformation is not verified, medium-sized CMN sometimes demonstrate NRAS mutations. Giant CMN and associated proliferative nodules are suggested to be derived from a common precursor because there are similar features of frequent NRAS mutations and infrequent BRAF mutations. However, Bastian et al. reported genetic differences between proliferative nodules and MM in giant CMN. These genetic analyses have been mostly reported in giant CMN to our knowledge. In conclusion, we describe an NRAS-mutated MM arising from an NRAS-mutated medium-sized CMN, which was rather small.

Successful treatment of refractory alopecia universalis by persuading a patient not to sleep with her dog

Alopecia areata (AA) is a common disorder that generically involves the sudden loss of circular patches of scalp hair. The mechanism of AA is gradually being clarified, and currently available evidence suggests that AA can be considered as a T-cell-mediated autoimmune disease.1 A history of atopic disposition and autoimmune disease is associated with an increased risk of AA or alopecia universalis (AU), which is a severe form of AA, and the prognosis looks less favorable.2,3 The severer AA is at onset, the worse the prognosis.4 A 57-year-old woman who had mild atopic dermatitis was referred to our hospital for treatment of scalp hair loss that had rapidly expanded to the whole body (Fig. 1a). A skin biopsy taken from her scalp revealed a hair follicle infiltration in the peribulbar area (Fig. 1b), and we diagnosed the patients condition as AU in 2009. Laboratory investigations showed that serum IgE was increased (6908 IU/mL; normal, <380 IU/mL), with no other abnormal findings. She was treated with a topical corticosteroid, an oral anti-histamine, and cepharanthine during one year at the former hospital. Furthermore, we performed semi-pulse corticosteroid therapy (500 mg/daily for three days) and cryotherapy for 14 months in addition to those treatments. Since 2011, we treated the patient with excimer light therapy (4.2 J/cm2 total dose) at two-week intervals for 24 sessions. Although hair growth was partially achieved during the treatment with cryotherapy and excimer light therapy, alopecia reoccurred. Therefore, the patient opted for only conservative medical treatments such as an oral antihistamine. In 2012, we belatedly discovered that she had kept a dog since 2002 and slept with her dog next to her every day. Laboratory tests revealed that serum value of the specific IgE antibody against dogs dander was very high (2460 UA/mL; normal, <0.34 IU/mL). We persuaded the patient not to sleep next to her dog, and she followed the advice from May 2012. After three months of follow-up, the alopecia gradually improved (Fig. 1c, d). The hair growth was slow, but favorable (Fig. 1e, f). Additionally, her atopic dermatitis also got much better as the SCORAD score decreased from 21.0 to 5.3. Finally, two years and seven months later, her alopecia was completely in remission, including body hair (Fig. 1g, h). Laboratory tests showed that the specific IgE antibody of the dogs dander was clearly lower than that at the first time (894 UA/mL in 2014).

Verrucous lesions arising in lymphedema and diabetic neuropathy: Elephantiasis nostras verrucosa or verrucous skin lesions on the feet of patients with diabetic neuropathy?

Verrucous skin lesions on the feet in diabetic neuropathy (VSLDN) develop in areas with sensory loss in diabetic patients. Although various types of chronic stimulation, such as pressure or friction, are considered an important factor in the development of such lesions, the precise pathogenesis of VSLDN remains obscure, and there is currently no established treatment for this disease. Here, we present a case of VSLDN on the dorsum of the right foot. However, because lymphedema was also observed at the same site, this lesion could also be diagnosed as elephantiasis nostras verrucosa arising in diabetic neuropathy. The lesion was successfully treated with a combination of elastic stocking and mixed killed bacterial suspension and hydrocortisone ointment, which suggested that VSLDN might have been exacerbated by the pre‐existing lymphedema. Because various types of chronic stimulation can trigger VSLDN, treatment plans should be devised on a case‐by‐case basis. Therefore, it is important to investigate the presence of factors that can induce or exacerbate chronic inflammatory stimulation, such as lymphedema in our case, in each patient with VSLDN.