Atsuko Ohashi

A Case of Epidermolysis Bullosa Acquisita Associated with Laryngeal Stenosis

© 2012 The Authors. doi: 10.2340/00015555-1163 Journal Compilation

A case of Kindler syndrome with severe esophageal stenosis

hyperbaric oxygen therapy prevent extremity necrosis in Nicolau syndrome? Pediatr Int 2012; 54: e15–e18. 6 Yildiz C, Ozkan H, Hakan AY, et al. A case of nicolau syndrome treated with hyperbaric oxygen. Cent Eur J Med 2009; 4: 262–264. 7 Karimi M, Owlia MB. Nicolau syndrome following intramuscular penicillin injection. J Coll Physicians Surg Pak 2012; 22: 41–42. 8 Marangi GF, Gigliofiorito P, Toto V, et al. Three cases of embolia cutis medicamentosa. J Dermatol 2010; 37: 488– 492. 9 Silva AM, Ton A, Loureiro TF, et al. Late development of Nicolau syndrome – case report. An Bras Dermatol 2011; 86: 157–159. 10 Mathieu D. Handbook on Hyperbaric Medicine. Netherlands: Springer, 2006: 135–145.

Fibrosarcomatous variant of dermatofibrosarcoma protuberans with pancreatic metastasis

was applied for palliation and covered the lumbar vertebrae only. Therefore, it is conceivable that the main contributor to the improvement in our patient was docetaxel. Taxanes are known to have both antitumor and antiangiogenic effects. Given that this condition cannot be managed with therapy that targets the DIC, we should consider the use of treatment with taxanes that focuses on both tumor control and DIC inhibition.

Successful treatment of rheumatoid vasculitis-associated skin ulcer with a TNF-α antagonist

to 10 cm. Cutaneous MAs are mainly found on the head and trunk, although they have also been found in peculiar areas like the penile shaft or eyelid. The average time between diagnosis and removal is 8–10 years. Cutaneous MA is a usually painless, slow-growing, benign, mixed neoplasm composed of dendritic melanocytes scattered throughout the keratinocytes. Some authors divide MA into two types: a diffuse type with melanocytes scattered unevenly throughout the epidermis and a clone form with melanocytes and keratinocytes clustered in a small nest, as seen in the clonal type of SK. MA and SK have many histological and clinical similarities. Despite this, we believe there is evidence to justify MA as a different entity. Schlappnner et al. studied the differences between MA and SK with electron microscopy. They found that in MA, the melanocytes were large, richly dendritic, and present in the junctional, central, and upper layers of the epidermis, unlike those in SK. Melanosomes of all stages were present, especially in dendritic cells. Isolated melanin granules were present within the cytoplasm of some keratinocytes but were sparse. In comparison, the melanocytes in SK were small, and abundant melanin granules were seen in the cytoplasm of the basaloid keratinocytes. These findings are consistent with histological reports described by other authors confirming that transference of melanosomes to keratinocytes is decreased or blocked. There is some controversy over whether or not MA could be pigmented or irritated SK. However, a study in which SK were irritated by cotton oil or surgical trauma failed to demonstrate any histological characteristic of MA. As reported above, it appears that the MA can be considered a separate entity, and it should be considered in the differential diagnosis of other pigmented lesions. No malignant cases have been reported, so once the diagnosis is confirmed, excision or follow-up seems unnecessary. To the best of our knowledge, only two cases of multi ple MAs have been described. The present report con cerns a patient with multiple clustered MAs. We believe that this special presentation of MAs can be designated as agminated MAs.

Histopathological improvement of scleroderma induced by paclitaxel in a patient with breast cancer

1 Vogt T, Brockmeyer N, Kutzner H et al. Brief S1 guidelines–cutaneous angiosarcoma and kaposi sarcoma. J Dtsch Dermatol Ges 2013; 11(Suppl 3): 2–10. 2 Linch M, Miah AB, Thway K et al. Systemic treatment of soft-tissue sarcoma-gold standard and novel therapies. Nat Rev Clin Oncol 2014; 11: 187–202. 3 Stacchiotti S, Palassini E, Sanfilippo R et al. Gemcitabine in advanced angiosarcoma: a retrospective case series analysis from the Italian rare cancer network. Ann Oncol 2012; 23: 501– 508. 4 Kajihara I, Kanemaru H, Miyake T et al. Combination chemotherapy with s-1 and docetaxel for cutaneous angiosarcoma resistant to paclitaxel. Drug Discov Ther 2015; 9: 75–77.

Case of azacitidine‐induced maculopapular erythematous eruption

Dear Editor, Myelodysplastic syndromes (MDS) are a varied group of hematological diseases that involve ineffective production of the myeloid class of blood cells. Recently, azacitidine has been shown to prolong survival and improve quality of life in patients with MDS, while maintaining a favorable adverse effect profile. Skin reaction at the injection site is a common adverse effect. However, other adverse effects in the skin are uncommon. Here, we report a case of azacitidine-induced maculopapular erythematous eruption together with usual injection-site reaction. A 60-year-old Japanese man had a 1-year history of breathlessness and tiredness. As his symptoms progressed, he visited a local hospital and was diagnosed with acute myeloid leukemia transformed from MDS. He was treated with 75 mg/ m per day of azacitidine s.c. for 7 consecutive days every 4 weeks. Local skin reaction occurred at the injection site within a few hours after injection. Burning red eruptions appeared over the trunk several hours after the third injection of the first course, and the erythema changed to pigmentation 1 week later. The erythema on the trunk reappeared after the first injection of the second course, and he was referred to our department. Physical examination revealed small maculopapular erythemas on the trunk and extremities together with several erythematous macules at the injection site (Fig. 1a,b). Skin biopsy from the maculopapular erythema showed partial parakeratosis, mild spongiosis and liquefaction degeneration with infiltration consisting of lymphocytes and some eosinophils around the vessels (Fig. 1c). Laboratory tests were normal except for cytopenia. Although we did not perform challenge test or patch test, a diagnosis of azacitidine-induced allergic drug eruption was made because the eruption reappeared on the trunk immediately after the first injection of the second course. One week later, the maculopapular erythema had changed to pigmentation. Use of azacitidine was stopped, and the erythema did not reappear. The patient was prepared for hematopoietic cell transplantation. Azacitidine inhibits DNA methyltransferase, and has been shown to prolong overall survival in patients with MDS along with subsets of leukemia. Azacitidine followed by hematopoietic cell transplantation is beneficial in patients with high-risk MDS because this combination shows a low recurrence rate as well as reduced toxicity. The adverse reactions to azacitidine include cytopenia, injection-site reactions and gastrointestinal symptoms. Usually, adverse events occur transiently at the beginning of the treatment cycle and are resolved during ongoing therapy. With regard to skin reactions, injection-site reaction occurs in 46–72% of patients as toxic reaction and usually resolves during treatment. Other skin reactions are uncommon. Almeida reported generalized urticarial skin reaction in patients treated with azacitidine, and recommended the use of concomitant low-dose steroids. Azacitidine-associated Sweet’s syndrome was also reported with prompt symptom resolution after discontinuation of azacitidine use and administration of appropriate corticosteroid therapy. Azacitidine is highly effective in patients with MDS and other myeloid neoplasms. Azacitidine caused allergic reactions as well as toxic reaction in our case. While toxic reaction at the injection site is common, allergic reaction is not widely known. We should require attention to systemic skin reactions, because the incidences are likely to increase with the increasing use of azacitidine.

A case of hypocomplementaemic urticarial vasculitis with a high serum level of rheumatoid factor.

We report a case of hypocomplementaemic urticarial vasculitis with an elevated serum rheumatoid factor level. Hypocomplementaemic urticarial vasculitis is an immune complex‐mediated disease characterised by urticarial eruptions. High levels of rheumatoid factor may be associated with hypocomplementaemia due to the consumption of complement, because the rheumatoid factor can form immune complexes with immunoglobulin. It is necessary to pay attention to the amounts of complement in cases of urticarial eruptions with elevated rheumatoid factor level. The eruptions were relieved with a combination of prednisolone and colchicine.

Polyarteritis nodosa in a patient with haemophilia A

Polyarteritis nodosa (PAN) is a systemic vasculitis in which necrotizing arteritis occurs in medium and/or small arteries [1], and is occasionally associated with viral infection. Haemophilia A is a genetic disorder caused by insufficient clotting factor VIII. Patients are at risk of viral infection because repetitive infusions of clotting factor are necessary for the treatment. Here, we report a case of PAN during a course of haemophilia A.A 33-year-old Japanese man was treated for haemophilia A [...]

Usefulness of high‐frequency sonography for the diagnosis of asymptomatic myopathy in Löfgren’s syndrome

trauma to the scalp. Br J Dermatol 1986; 118: 445–447. 5 Stead JW, Henry CMT, Simpson RHW. Rare case of autoinoculation of orf. Br J Gen Pract 1992; 42: 395–396. 6 Rieger H, Wetlerkamp D, Kühn J, et al. Echtyma contagiosum (orf) as an uncommon differential diagnosis of infections of the hand. Unfallchirurg 2003; 106: 204–206. 7 Chan KW, Hsu WL, Wang CY, et al. Differential diagnosis of orf viruses by a single-step PCR. J Virol Methods 2009; 160: 85–89. 8 Hosamani M, Scagliarini A, Bhanuprakash V, et al. Orf: an update on current research and future perspectives. Expert Rev Anti Infect Ther 2009; 7: 879–893. 9 Andrei G, Snoeck R. Cidofovir activity against poxvirus infections. Viruses 2010; 2: 2803–2830.

Cutaneous angiosarcoma of the leg showing radiation sensitivity.

We report a case of cutaneous angiosarcoma occurring on the leg of a 97‐year‐old Japanese woman. Considering the patients age and general condition, she was treated with electron beam irradiation, which led to the almost complete disappearance of the tumour. Because cutaneous angiosarcoma is an aggressive tumour with a high propensity for local recurrence and distant metastases, therapy preferably involves a multimodal approach. However, monotherapy with radiation may be effective in some cases of cutaneous angiosarcoma.