Primary aldosteronism due to bilateral micronodular hyperplasia and concomitant subclinical Cushing’s syndrome: A case report

BACKGROUNDAdrenal incidentaloma (AI) has been frequently encountered in the clinical setting. It has been shown that primary aldosteronism (PA) or subclinical Cushing’s syndrome (SCS) are the representative causative diseases of AI. However, the coexistence of PA and SCS has been reportedly observed. Recently, we encountered a case of AI, in which PA and SCS coexisted, confirmed by histopathological examinations after a laparoscopic adrenalectomy. We believe that there were some clinical implications in the diagnosis of the present case.CASE SUMMARYA 58-year-old man presented with lower right abdominal pain with a blood pressure of 170/100 mmHg. A subsequent computed tomography scan revealed right ureterolithiasis, which was the cause of right abdominal pain, and right AI measuring 22 mm × 25 mm. After the disappearance of right abdominal pain, subsequent endocrinological examinations were performed. Aldosterone-related evaluations, including adrenal venous sampling, revealed the presence of bilateral PA. In addition, several cortisol-related evaluations showed the presence of SCS on the right adrenal adenoma. A laparoscopic right adrenalectomy was then performed. The histopathological examination of the resected right adrenal revealed the presence of a cortisol-producing adenoma, while CYP11B2 immunoreactivity was absent in this adenoma. However, in the adjacent non-neoplastic adrenal, multiple CYP11B2-positive adrenocortical micronodules were detected, showing the presence of aldosterone-producing adrenocortical micronodules. CONCLUSIONCareful clinical and pathological examination should be performed when a patient harboring AI presents with concomitant SCS and PA.     

Wound,pressure ulcer and burn guidelines – 4: Guidelines for the management of connective tissue disease/vasculitis-associated skin ulcers

The Japanese Dermatological Association prepared guidelines focused on the treatment of skin ulcers associated with connective tissue disease/vasculitis practical in clinical settings of dermatological care. Skin ulcers associated with connective tissue diseases or vasculitis occur on the background of a wide variety of diseases including, typically, systemic sclerosis but also systemic lupus erythematosus (SLE), dermatomyositis, rheumatoid arthritis (RA), various vasculitides and antiphospholipid antibody syndrome (APS). Therefore, in preparing the present guidelines, we considered diagnostic/therapeutic approaches appropriate for each of these disorders to be necessary and developed algorithms and clinical questions for systemic sclerosis, SLE, dermatomyositis, RA, vasculitis and APS.     

Survey of Japanese dermatological vasculitis specialists on cases of cutaneous arteritis (cutaneous polyarteritis nodosa)

We developed a questionnaire to examine the findings of cutaneous arteritis among dermatological specialists experienced in vasculitis as certified by the Committee for guidelines for the management of vasculitis and vascular disorders of the Japanese Dermatological Association. We sent a questionnaire to 12 dermatological facilities identified through the revised Committee for guidelines for the management of vasculitis and vascular disorders of the Japanese Dermatological Association. Retrospective data obtained from 84 patients at the 12 dermatological facilities between 2012 January 2016 December were evaluated. The 84 patients were categorized into two groups, a systemic steroid treatment group (group 1, n = 52) and a no systemic steroid treatment group (group 2, n = 32). C-reactive protein in group 1 patients was significantly higher than that in group 2 patients. Frequency of fever, arthritis, myalgia- and peripheral neuropathy in group 1 was significantly higher than that in group 2. We propose that these symptoms could serve as early markers for the transfer from cutaneous arteritis to systemic polyarteritis nodosa. We further suggest that patients who are subsequently associated with cerebral hemorrhage and infarction, who are originally diagnosed as having cutaneous arteritis, could progress to systemic polyarteritis nodosa. The study demonstrated that it is important for dermatologists to detect these findings early in order to establish an accurate diagnosis and a timely treatment.     

Pharmaceuticals and Medical Device Agency approval summary: Amenamevir for the treatment of herpes zoster

In July 2017, Japan’s Ministry of Health, Labor and Welfare issued a marketing authorization valid throughout Japan for N-(2,6-dimethylphenyl)-N-(2-{[4-(1,2,4-oxadiazol-3-yl)phenyl]amino}-2-oxoethyl)-1,1-dioxothiane-4-carboxamide (amenamevir) for the first time worldwide. The decision was based on the favorable opinion of the Pharmaceuticals and Medical Device Agency (PMDA) recommending a marketing authorization of amenamevir for treatment of herpes zoster (HZ). Amenamevir has a different action mechanism from previously approved synthetic nucleoside compounds for the treatment of HZ including acyclovir, valacyclovir and famciclovir. The usual adult dose is 400 mg amenamevir p.o. once daily for 7 days. The benefit is its ability to cure HZ as well as preventing postherpetic neuralgia. The most common side-effects are increase of urine N-acetyl-β-D-glucosaminidase and α1-microglobulin levels. However, based on the detailed evaluation of the submitted clinical studies, there seems to be no serious safety concerns about amenamevir regarding the kidney of both renally normal and impaired patients. The objective of this article is to summarize the scientific review of the application. The detailed scientific assessment report and product information, including the summary of product characteristics, are available on the PMDA website ( www.pmda.go.jp/PmdaSearch/iyakuSearch/ ).