Radical surgery for anal canal neuroendocrine carcinoma with pagetoid spread: a case report.

Anal canal neuroendocrine carcinoma (NEC) with pagetoid spread (PS) is a rare disease, and its treatment strategy remains unclear. The prognosis of anal canal NEC with PS is poor. Resection margin status is very important for anorectal carcinoma because it affects survival. When accompanied by PS, the defect of the resulting perineal wound following radical surgical intervention may be necessarily enlarged to ensure the appropriate margin status. This case report discusses the treatment of a patient with advanced anal canal NEC with PS, inguinal lymph node metastasis and sphincter infiltration in which total pelvic exenteration with plastic surgery was successfully performed. The plastic surgery incorporated a gracilis muscle flap that was useful for the reconstruction of the enlarged perineal defect.

[Two Cases of Stevens-Johnson Syndrome after Nivolumab Therapy for Gastric Cancer].

Case 1: A 51-year-old man with advanced gastric cancer and peritoneal metastasis was referred to our hospital. He received fourth-line chemotherapy with nivolumab, but it became PD. Next, he received S-1 plus docetaxel therapy as fifth- line therapy. After 2 courses of S-1 plus docetaxel, erythema and blisters appeared on his limbs, with erosions of the oral mucosa and penis. We diagnosed Stevens-Johnson syndrome(SJS)based on the clinical and pathological findings. He received steroid treatment, but the cutaneous symptoms persisted; therefore, it was impossible to continue the chemotherapy because of the SJS. Case 2: A 75-year-old woman with recurrence of peritoneally disseminated gastric cancer received third-line chemotherapy with nivolumab. After 1 course of nivolumab, erythema appeared on her body and limbs, with erosion of the lips and oral mucosa. We diagnosed SJS based on the clinical findings. She received steroid treatment, but the cutaneous symptoms persisted; therefore, it was impossible to continue chemotherapy because of the SJS. It should be noted that the onset of serious irAEs, such as SJS, might make continuous chemotherapy difficult.

Biallelic Mutations in KDSR Disrupt Ceramide Synthesis and Result in a Spectrum of Keratinization Disorders Associated with Thrombocytopenia.

Mutations in ceramide biosynthesis pathways have been implicated in a few Mendelian disorders of keratinization, although ceramides are known to have key roles in several biological processes in skin and other tissues. Using whole-exome sequencing in four probands with undiagnosed skin hyperkeratosis/ichthyosis, we identified compound heterozygosity for mutations in KDSR, encoding an enzyme in the de novo synthesis pathway of ceramides. Two individuals had hyperkeratosis confined to palms, soles, and anogenital skin, whereas the other two had more severe, generalized harlequin ichthyosis-like skin. Thrombocytopenia was present in all patients. The mutations in KDSR were associated with reduced ceramide levels in skin and impaired platelet function. KDSR enzymatic activity was variably reduced in all patients, resulting in defective acylceramide synthesis. Mutations in KDSR have recently been reported in inherited recessive forms of progressive symmetric erythrokeratoderma, but our study shows that biallelic mutations in KDSR are implicated in an extended spectrum of disorders of keratinization in which thrombocytopenia is also part of the phenotype. Mutations in KDSR cause defective ceramide biosynthesis, underscoring the importance of ceramide and sphingosine synthesis pathways in skin and platelet biology.

[Superselective feeder embolization for hemorrhage from cutaneous angiosarcoma in scalp].

A 76-year-old male developed a hemorrhagic nodule and a surrounding purple-red, irregularly shaped macule on his scalp after head trauma. The diagnosis of angiosarcoma was confirmed histologically. A transarterial feeder embolization was performed followed by a surgical excision. It was effective for bleeding from a tumor, and superselective catheterization was important for this specific tumor. The early diagnosis by neurosurgeon may lead to improved survival.

Comparison of the severity of atopic dermatitis lesions and the density of Staphylococcus aureus on the lesions after antistaphylococcal treatment.

We examined the relationship between atopic dermatitis (AD) and Staphylococcus aureus by comparing changes in AD lesions and the bacterial density on the lesions after antimicrobial treatment with cefdinir. We found that there was a greater density of S. aureus on red erythemas and exudative lesions than in light/dark red erythemas and non-exudative lesions of AD. Forty-one of 59 cases (69%) showed a decrease in colony count following antimicrobial treatment. In 28 of 39 cases (72%) there was a decrease of erythema, and in 18 of 22 cases (82%) there was a decrease in the amount of exudate both associated with a decrease in colony density following antimicrobial treatment. Because acute phases of atopic dermatitis, such as red erythemas and exudative lesions, were closely related to the colonization of S. aureus, dense colonization with S. aureus may be an important factor in the exacerbation of AD. We believe that staphylococcal products such as α-toxin, various enzymes, coagulase, and superantigenic exotoxins affect some aspect of the inflammatory process, resulting in exacerbation of AD. J Infect Chemother 1996;2:70-74.