Shiro Iino

CD34-positive infantile myofibromatosis: Case report and review of hemangiopericytoma-like pattern tumors

We describe a case of CD34‐positive infantile myofibromatosis with hemangiopericytoma‐like pattern. A 2‐day‐old Japanese boy presented with multiple hemispherical nodules on the extremities and back. There was a biphasic histological growth in the dermis, accompanied by a hemangiopericytoma‐like pattern with antler‐like branching vessels. Tumor cells were oval to spindle‐shaped myoid cells with bland appearance. Immunohistochemically, vimentin, calponin and CD34 were positive, while α‐smooth muscle actin, h‐caldesmon, HHF35 and desmin were negative. Although CD34 was positive, the present case could be diagnosed as infantile myofibromatosis. Myopericytoma, myofibroma/myofibromatosis, glomus tumor, glomangiopericytoma and angioleiomyoma share a continuous spectrum of benign hemangiopericytoma‐like pattern tumors. Myofibroma/myofibromatosis is nearly included in myopericytoma among pericytic (perivascular) tumors, and could be positive for CD34. Several immunohistochemical panels of smooth muscle markers are needed for the diagnosis of pericytic (perivascular) tumors.

A Case of Erythema Multiforme Major Developed after Sequential Use of Two Immune Checkpoint Inhibitors, Nivolumab and Ipilimumab, for Advanced Melanoma: Possible Implication of Synergistic and/or Complementary Immunomodulatory Effects

Immune checkpoint inhibitors, such as ipilimumab and nivolumab, reverse the imbalance of antitumor self-tolerance and enhance T-cell responses. Currently, ipilimumab and nivolumab have a reported therapeutic impact on unresectable or metastatic melanomas; however, they also induce immune-related adverse events (irAEs). Ipilimumab-induced cutaneous irAEs are mostly low grade and manageable, although all-grade rash may occur in approximately 45% of all patients. We here report the case of a young woman with erythema multiforme major, which developed after sequential use of these 2 immune checkpoint inhibitors for advanced melanoma of the scalp. Initially, she received 12 cycles of nivolumab monotherapy followed by ipilimumab. A week later, multiple erythematous papulo-erythemas appeared on almost her entire body, with high-grade fever, mucosal involvements, and dyspnea. Immunohistochemistry using the lesioned skin revealed lymphocytic infiltration predominantly positive for CD8, contrasting with those for CD4 and Foxp3. Ipilimumab was stopped but she continued to receive empirical antibiotics; additionally, she was treated with intravenous steroid pulse therapy and immunoglobulin, followed by oral prednisolone. Her symptoms subsided rapidly, allowing a restart of nivolumab monotherapy alone. In our case, the long-standing preceding nivolumab monotherapy may synergistically and/or complementary have contributed to – in combination with the later administration of ipilimumab – recover antigen-responsive T-cell immunity, which is similar to the concept of immune reconstitution inflammatory syndrome, resulting in the establishment of an underlying immunopathology of erythema multiforme and life-threatening airway obstruction.

Effectiveness of IVIG and balloon dilation combination therapy for refractory dysphagia in anti-TIF1-γ antibody-positive dermatomyositis

Abstract Dermatomyositis is an autoimmune inflammatory myopathy with characteristic cutaneous features. Dysphagia secondary to oropharyngeal and oesophageal involvement develops in a part of patients with dermatomyositis and may affect prognosis. Systemic immunosuppressive therapy is usually effective for dysphagia as well as cutaneous lesion and myositis. Here, we report a 78-year-old man with anti-TIF-1γ antibody-positive dermatomyositis that presented with an early gastric carcinoma and dysphagia resistant to oral prednisolone and tacrolimus therapy. Endoscopic examination demonstrated saliva retention in the piriform recess and saliva entering the larynx. A videofluoroscopy swallow study revealed a stricture of the oesophagus and accumulation of contrast media in the pharynx. A repetitive combination therapy of intravenous immunoglobulins and balloon dilatation was effective for dysphagia. In dermatomyositis patients with dysphagia, swallowing function should be evaluated at an early stage. Moreover, combination therapy with intravenous immunoglobulins and balloon dilation may be an optional treatment for refractory dysphagia.

A rare case of chronic expanding haematoma in the occipital region mimicking a malignant soft tissue tumour

tion characterised by progressive necrosis of the skin, s.c. tissue and fasciae. In immunocompromised patients, necrotising fasciitis can occur even after minor breaches of the skin. In our patient, her past medical history included diabetes mellitus. High doses of oral prednisolone were administered. The patient developed severe skin lesions which became colonised with MSSA. Although necrotising fasciitis represents an unusual complication of bullous pemphigoid, the insertion and placement of the catheter may have caused the onset of the disease. I.v. immunoglobulin is currently used to treat a wide variety of autoimmune and inflammatory diseases. It provides a beneficial therapeutic outcome for patients with bullous pemphigoid who are resistant to steroid therapy. I.v. immunoglobulin has also been evaluated for the adjunct treatment of refractory and difficult-to-treat chronic infections. Taken together, i.v. immunoglobulin therapy is a good therapeutic option to treat bullous pemphigoid complicated by severe bacterial infections, as in our patient.

Two-phase Surgery Using a Dermal Regeneration Material for Nail Unit Melanoma: Three Case Reports

CASE REPORT Two-phase Surgery Using a Dermal Regeneration Material for Nail Unit Melanoma: Three Case Reports Shiro Iino, Suguru Sato, Natsuki Baba, Naoki Maruta, Wataru Takashima, Noritaka Oyama, Takahiro Kiyohara, Masato Yasuda and Minoru Hasegawa Department of Dermatology, Division of Medicine, Faculty of Medical Sciences, University of Fukui, Fukui, Japan Department of Dermatology, Kansai Medical University, Osaka, Japan Department of Plastic Surgery, University of Saga, Saga, Japan