Yasuyuki Yamaguchi

A case of drug-associated dermatomyositis following ipilimumab therapy

Ipilimumab is a human monoclonal antibody targeted against cytotoxic T-lymphocyte antigen 4 (CTLA-4). Though many adverse immune-related events have been reported to be associated with ipilimumab [1, 2], only one case of ipilimumab-associated dermatomyositis (DM) has been reported [3], and no cases of cytomegalovirus (CMV) infection associated with ipilimumab have ever been reported. Herein, we report a case of drug-associated DM concurrent with CMV infection following ipilimumab therapy.A 70-year-old [...]

Rapid remission of severe pain from livedoid vasculopathy by apixaban

Editor Livedoid vasculopathy (LV) is a skin vascular disease caused by recurrent thrombosis of the dermal microcapillaries. LV manifests as intractable ulcerations with livedo, purpura, hyperpigmentation and atrophie blanche. Moreover, recurrent thrombus formation causes severe ischaemic pain and impairment of quality of life (QOL). Anticoagulants including low-molecular-weight heparin and warfarin have been widely used for the treatment of LV. However, their adverse effects sometimes result in discontinuation of the drugs. Moreover, the use of heparin remains limited because of the need for parenteral administration. Warfarin requires regular coagulation monitoring and dose adjustment. Such limitations have led to the development of direct factor Xa inhibitors, including rivaroxaban, edoxaban and apixaban. Recent randomized control studies have shown these Xa inhibitors to have superior thromboprophylaxis effects for venous thromboembolisms with less side-effects compared with warfarin. Notably, successful use of rivaroxaban has also been reported in five cases with LV. Moreover, apixaban has been shown to possess a lower risk of bleeding than the other two. These findings collectively suggest a potential for apixaban in the treatment of LV. To the best of our knowledge, however, the efficacy of apixaban for LV has never been reported. Herein, we report a case of recalcitrant LV successfully treated with apixaban. In 2009, a 73-year-old Japanese woman presented with a 1-year history of recurrent purpura and skin ulcers on the legs. Severe ischaemic pain in her legs resulted in walking disability. Physical examination revealed several tender punched-out ulcers of up to 3 cm in size, surrounded by telangiectatic purpuric erythema on the legs. Atrophie blanche and hyperpigmentation were also noted. Histology showed fibrin deposition and occlusion of blood vessels in the mid-to-deep dermis without features of vasculitis. Laboratory tests revealed no significant abnormalities. Collectively, the diagnosis of LV was made. The ulcers initially responded well to oral warfarin. However, highly variable prothrombin time international normalized ratios required frequent dose adjustments and resulted in frequent recurrences of painful skin ulcers. Since her ulcers had gradually increased in size and number (Fig. 1a), she was switched from warfarin to 10 mg/day of apixaban in July 2015. Notably, the severe pain was alleviated within a day after the initiation of oral apixaban. The skin ulcers were also almost epithelialized at 8 weeks (Fig. 1b), with no clinical recurrence at 5-month follow-up without any adverse effects. Since factor Xa, which can be activated through either an intrinsic or extrinsic pathway, plays a key role in haemostasis, it is regarded as a desirable intervention point for anticoagulation therapies. This hypothesis has been verified by the superior efficacy and lower toxicity of Xa inhibitors compared with warfarin in the prevention of thromboembolic events in high-risk individuals. This study provides further evidence of the efficacy and safety of Xa inhibitors in the treatment of LV. Persistent sharp pain was the primary problem in the present case. The rapid alleviation of pain was observed within 1 day after the start of apixaban, which may have resulted from the successful prevention of additional microthrombogenesis. Of particular note, all five LV cases that responded well to rivaroxaban reported considerable pain relief; satisfactory reduction in pain was noted within 2 days in one case, within 1 week in three cases and within 4 weeks in one case (Table 1). Because patients with LV report highly impaired QOL due to pain,

A case of erythroplasia of Queyrat successfully treated with combination carbon dioxide laser vaporization and surgery.

matitis after cardiac catheterism and in 1997, Knautz described the first case of radiodermatitis after TIPS. In the literature are reported 12 cases of patients affected by radiodermatits after TIPS: it is a very rare complication, often making for difficult diagnoses. Generally, some weeks or months after the procedure, patients feel a worsening pain and may develop cutaneous manifestations up to 1 year later. The rarity of the complication, as well as the development time of radiodermatitis, makes it difficult to make a solid association with the fluorososcopy procedure. Therefore, incorrect diagnoses such as morfea, fixed drug eruption, lichen scleroatrophicus, panniculitis, are likely to occur. Patients are thus often subjected to unsuitable and ineffective therapies for controlling the throbbing and distressing pain. In the case we were able to observe, which because of the rarity of the onset led to diagnosis problems, there was the peculiarity of showing cutaneous lesions just a few days after the procedure. Lack of diagnosis led to it becoming chronic, thus causing not only diagnostic problems but therapeutic ones as well. As involved radiographic procedures such as TIPS became more prevalent, physicians need to be aware of possible risks such as radiodermatitis to prevent side-effects. It is important to initiate an exact and early diagnosis to ensure operative therapeutic strategies and ensure a long-term follow-up for malignancy screening.

The Unique Dermoscopic Structure 'Large black web' in basal cell carcinoma on the areola.

References 1 Gualdi G, Pavoni L, Monari P, Calzavara-Pinton P, Manganoni MA. Dermoscopy of drug-induced aquagenic wrinkling phenomenon. J Eur Acad Dermatol Venereol 2015; [Epub ahead of print] 2 Luo DQ, Li Y, Huang YB, Wu LC, He DY. Aquagenic syringeal acrokeratoderma in an adult man: case report and review of the literature. Clin Exp Dermatol 2009; 34: e907–e909. 3 Uyar B. Aquagenic syringeal acrokeratoderma. Indian J Dermatol 2014; 59: 632. 4 Vildosola S, Ugalde A. Celecoxib-induced aquagenic keratoderma. Actas Dermosifiliogr 2005; 96: 537–539. 5 Khuu PT, Duncan KO, Kwan A, Hoyme HE, Bruckner AL. Unilateral aquagenic wrinkling of the palms associated with aspirin intake. Arch Dermatol 2006; 142: 1661–1662. 6 Tolland JP, Boyle J, Hall V, McKenna KE, Elborn JS. Aquagenic wrinkling of the palms in an adult cystic fibrosis population. Dermatology 2010; 221: 326–330. 7 G€ und€ uz O, Ozsarac KC , Ercin ME. Aquagenic palmar wrinkling induced by combined use of salazopyrin and indomethacin. Case Rep Dermatol 2013; 5: 21–26. 8 Glatz M, Muellegger RR. Drug-associated aquagenic wrinkling of the palms in an atopic male patient. BMJ Case Rep 2014; 2014. doi:10.1136/ bcr-2014-203929 9 Orzan OA, Popa LG, Voiculescu V, Manta R, Giurc aneanu C. Non-steroidal anti-inflammatory drug induced transient reactive papulotranslucent acrokeratoderma. J Med Life 2014; 7: 75–77. 10 Ert€ urk-€ Ozdemir E, € Ozcan D, Sec kin D. Acquired aquagenic syringeal acrokeratoderma: a case series of 10 patients. Australas J Dermatol 2015; 56: e43–e45.

Portable negative-pressure wound therapy for pyoderma gangrenosum: Report of two cases

Pyoderma gangrenosum is a chronic non‐infectious neutrophilic dermatosis that causes undermining ulcers. Topical therapies for the deep ulcers of pyoderma gangrenosum have not been established. To investigate whether negative‐pressure wound therapy is effective for a pyoderma gangrenosum ulcer, we used the PICO single use negative‐pressure wound therapy system (Smith & Nephew, London, UK) for two pyoderma gangrenosum patients. In these cases, the ulcers decreased in size and necrolytic tissue was removed notably. Moreover, there were no secondary infections nor was there Koebner phenomena. Our cases suggest that portable negative‐pressure wound therapy can be a treatment option for deep, intractable ulcers caused by pyoderma gangrenosum. Because portable negative‐pressure wound therapy devices afford increased mobility to patients, they can give the patient a better quality of life than standard negative‐pressure wound therapy systems do.

A case of linear basal cell carcinoma: evaluation of proliferative activity by immunohistochemical staining of PCTAIRE1 and p27

Baseline CRP and D-dimer (1815 ng/mL) were elevated. A dramatic improvement occurred already 3 days after the first omalizumab 300 mg administration, and urticaria became easily controlled by cetirizine 10 mg/day. The improvement persisted throughout the following 5 months under omalizumab; 3 weeks after the third administration, CRP was normal and D-dimer level was 657 ng/mL. Three months after omalizumab withdrawal, urticaria relapsed, D-dimer level rose to 796 ng/mL and CRP was elevated; thus, the drug was resumed. The first two shots were clinically successful although D-dimer remained elevated (763 ng/mL 3 weeks after the first dose), but 3 weeks after the second administration, another exacerbation occurred, and D-dimer levels rose to 2160 and then to 3992 ng/mL. The patient had been taking both perindopril and indapamide for hypertension for more than 3 years. Perindopril withdrawal was followed by a dramatic reduction of urticaria severity within 1 week; after 2 weeks, D-dimer levels were 548 ng/mL and the disease was easily controlled by cetirizine (Fig. 1b). Notably, perindopril had been stopped for >3 weeks also one year before without any benefit. These cases confirm that effective omalizumab treatment is associated with a drop in D-dimer levels, but it is the first time that ACE inhibitors are shown to sustain CSU exacerbations and to annul the effects of omalizumab. These drugs may cause bradykinin-mediated angioedema but are not included among those possibly involved in urticaria exacerbations in the EAACI guidelines. Nonetheless, intradermal bradykinin induces weal and flare reactions by a mechanism that is independent on histamine release. Thus, it cannot be excluded that in these patients, bradykinin generated by ACE inhibitors enhanced the severity of CSU and made it poorly responsive to omalizumab therapy at a certain point. ACE inhibitors were probably not primarily responsible for CSU as the two patients went on having slight urticaria after ACE inhibitors withdrawal, and withdrawal was formerly unsuccessful in one patient. Resistance to omalizumab may appear in patients previously responding to the drug; the possibility that ACE inhibitors may play a role in such events should be kept in mind.

Modified Dufourmentel flap, easy to design and tailor to the defect

How to close skin defects is one of the most important considerations for skin surgeons. Rhomboid flaps are used for many types of skin defect. Such flaps, represented by the Limberg and Dufourmentel flaps, are widely used at any region of the body because they are composed of straight lines and are easy to draw. However, these valuable techniques have the disadvantage of being prone to dog‐ears at a particular area of the flap. Therefore, our talented predecessors have modified these flaps in order to resolve the problems. The modified flaps they designed were difficult to draw because the modified designs were curvy and along esthetic lines. It is difficult for trainees to draw these flaps. To address this issue, we have developed newly modified rhomboid flaps that are based on the Dufourmentel flap. This flap is different from the Dufourmentel flap in omitting the top half of the diamond above the circle. Thus, the tension on the skin decreases and dog‐ears are less likely to occur. In addition, our flaps are geometric and easily drawn even by novices. We believe our flap will be a promising option for closing skin defects at any site.

Plasma cell cheilitis successfully treated with topical calcineurin inhibitors.

Plasma cell cheilitis (PCC) is an uncommon disorder that clinically manifests as erosive or erythematous eruptions on the labial mucosa [1]. Histopathologically, dense plasma cell infiltrates in the upper layer of the lamina propria of the mucosa characterise the disorder. PCC is usually refractory to various topical treatments, including corticosteroids [2], antibiotics [3], fusidic acid [3], and antifungal agents [4]. Although cases of PCC successfully treated with topical calcineurin inhibitors [...]

A solitary reddish nodule on the lower leg

A 25-year-old man was referred to us with a 6-month history of a nodule on the right lower leg that had been slowly enlarging. On physical examination, he was found to have an asymptomatic, solitary reddish nodule of 20 mm in diameter on the right lower leg (Fig. 1). The patient was otherwise healthy, and there was no history of trauma or insect bite at the site of the lesion. Laboratory testing did not find human immunodeficiency virus or human herpesvirus 8 (HHV-8) infections. An excisional therapy specimen of the nodule demonstrated slit-like irregular branched venules with thin walls surrounded by sclerotic stroma

Appearance of antidesmocollin 1 autoantibodies leading to a vegetative lesion in a patient with pemphigus vulgaris

DEAR EDITOR, Pemphigus describes a group of autoimmune bullous diseases, mainly classified into pemphigus vulgaris and pemphigus foliaceus. Pemphigus vulgaris is further subcategorized into a mucocutaneous type [with autoantibodies against desmoglein (Dsg)3 and Dsg1] and a mucosal type (with autoantibodies against Dsg3 but not Dsg1) based on autoantibody profile and clinical features. Pemphigus vegetans (PVeg), a rare variant of pemphigus vulgaris, is characterized by vegetating lesions. However, the mechanism behind the occurrence of these elevated lesions remains unclear. Recently, it was reported that antidesmocollin (Dsc)1 and Dsc3 autoantibodies were frequently detected and potentially pathogenic in PVeg. Here we report a case of pemphigus vulgaris in which a vegetative plaque, accompanied by the elevation of anti-Dsc1 autoantibodies, occurred during treatment. A 39-year-old man was referred to our hospital with blisters and erosions on the whole body and oral mucosal erosions without vegetative lesions. A biopsy specimen obtained from a vesicle on his abdomen showed intraepidermal bullae in suprabasal and prickle cell layers containing many