Yuka Inamura

Pazopanib does not bring remarkable improvement in patients with angiosarcoma

Pazopanib is a potent and selective multi‐targeted tyrosine kinase inhibitor that has been reported to extend progression‐free survival in cases of metastatic soft‐tissue sarcoma. However, the efficacy of pazopanib for cutaneous angiosarcoma has not been confirmed. We report eight cases of angiosarcoma treated with pazopanib, and review the efficacy and safety of pazopanib therapy. We retrospectively investigated the clinical information, including age, sex, body surface area, location, performance status, lung or pleural metastasis, preceding treatment, oral dose of pazopanib, response rate, progression‐free survival and adverse effects. Five of the eight patients needed to stop the pazopanib treatment due to severe adverse effects, including thrombocytopenia, anemia, drug‐associated pancreatitis, acute fulminant hepatitis and general fatigue. Progression‐free survival ranged 0.5–3.5 months (mean ± standard deviation, 1.81 ± 1.03). Overall survival ranged 3–26 months (14.13 ± 9.47). Six of the eight cases showed progressive disease, and two of the eight cases showed stable disease. To assess overall survival in angiosarcoma treated with pazopanib, we compared the pazopanib‐treated group (n = 8) with the non‐pazopanib‐treated control group (n = 10). There was no significant difference between two groups (P = 0.19, log–rank test). In conclusion, our case series suggests that pazopanib does not bring remarkable improvement in patients with angiosarcoma.

Dermoscopic features of hidroacanthoma simplex: Usefulness in distinguishing it from Bowen's disease and seborrheic keratosis.

Hidroacanthoma simplex (HAS) is a rare benign eccrine adnexal tumor. HAS is sometimes clinically or pathologically misdiagnosed as squamous cell carcinoma in situ (Bowens disease; BD), seborrheic keratosis (SK) or other adnexal tumor. To date, there has never been a report focusing on dermoscopic features to distinguish HAS from BD and SK. We found the following dermoscopic findings to be characteristic of HAS: fine black dots/globules (75% of cases) and fine scales arranged annularly (100% of cases). In contrast, glomerular vessels, which are typically observed in BD, were not seen in any of the four cases. Cerebriform appearance and milia‐like cysts, which are typically observed in SK, were also not seen in any of the four cases. The existence of “scattered fine black dots/globules” and “fine scales arranged annularly”, and the absence of the glomerular vessels, may contribute to precise diagnosis of HAS. Even though HAS resembles BD or SK clinically, it can be distinguished from these by the characteristic dermoscopic features.

Positron emission tomography–computed tomography can be useful in the early detection of metastases in primary mucinous carcinoma of the skin on the head and neck

Primary mucinous carcinoma of the skin (PMCS) is a rare cutaneous malignant neoplasm; its regional node metastasis is also rare. Currently, positron emission tomography–computed tomography (PET‐CT) is known to be a useful tool to search for early metastatic lesions in various carcinomas. However, PET‐CT is not always specific for head and neck lesions because of physiological uptake in the brain, palatine tonsil, salivary gland, thyroid etc. Herein we present two cases of head and neck PMCS in which metastasis was diagnosed accurately by PET‐CT. In these cases, nodal uptake of fluorodeoxy‐d‐glucose (FDG) histopathologically proved PMCS metastasis, verifying the utility of PET‐CT in detail. A surgeon was involved in the verification to compare the histopathological manifestations with the imaging results. Histopathologically, two of 13 nodes were positive in case 1, and one of 41 nodes was positive in case 2. These positive nodes were completely in accordance with the FDG uptake findings with no false negative findings. In treating PMCS on head and neck lesions, PET‐CT may be useful in the preoperative assessment when planning the extent of resection.

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.

Acrokeratoelastoidosis successfully treated with 10% salicylic acid ointment

Dear Editor, Hyperkeratotic papules along the margin of the hands and feet have been called various names, such as acrokeratoelastoidosis, focal acral hyperkeratosis, hereditary papulotranslucent acrokeratoderma, mosaic acral keratosis, degenerative collagenous plaques of the hands, keratoelastoidosis marginalis and digital papular calcinosis. These diseases were distinguished based on hereditary presence and pathological findings; however, they have been confused with each other. Therefore, Rongioletti et al. have proposed the term “marginal popular acrokeratoderma (MPA)” to encompass these. We herein report a case of acrokeratoelastoidosis successfully treated with 10% salicylic acid ointment. A 79-year-old Japanese man referred to us with a 5-year history of multiple small, round, asymptomatic yellowish papules on the margin of the left foot. The lesions had gradually increased in size and number, forming plaques. He had no history of chronic trauma, friction, hyperhidrosis or excessive sun exposure. His medical history and family history were unremarkable. On physical examination, smooth, hyperkeratotic papules approximately 1–2 mm in diameter were locally distributed on the margin of the left foot, and some papules had coalesced into plaques (Fig. 1a). There were no remarkable changes on his right foot. Histological examination of a biopsy specimen from a papule on the left foot showed marked compact orthokeratotic hyperkeratosis, mild acanthosis and scant lymphocytic infiltrates (Fig. 1c). Hematoxylin–eosin-stained sections showed no evident abnormality of collagen fiber; however, Verhoeff’s elastic stain showed the rarefaction and fragmentation of the elastic fibers (Fig. 1d,e). These clinical and histological findings were consistent with acrokeratoelastoidosis. Ten percent salicylic acid ointment was topically applied to the lesions twice a day and the lesion disappeared in 1 month (Fig. 1b). There was no recurrence during 12 months of follow up.

Proliferierende Trichilemmalzyste an der weiblichen Brust

Die proliferierende Trichilemmalzyste (PTC) ist ein seltener benigner Tumor, der vorwiegend Frauen betrifft und am häufi gsten auf der Kopfhaut (78 %) und am Rumpf (13 %) auftritt [ 1 ] . Einige PTC-Läsionen zeichnen sich durch schnelles Wachstum aus, was die Sorge über einen bösartigen Tumor hervorruft. Bisher gab es keine Publikation über die Entwicklung einer PTC auf der Haut der weiblichen Brust. Hier berichten wir über den ersten Fall einer PTC, die in dieser speziellen anatomischen Region auftritt und klinisch ein brustkrebsartiges Erscheinungsbild zeigt. Eine 72-jährige Japanerin wurde mit einer einjährigen Anamnese eines schmerzhaften, schnell wachsenden Tumors an der linken Brust in unser Krankenhaus überwiesen. Die körperliche Untersuchung ergab eine 3,5 cm × 3,1 cm × 2,4 cm große, eitrige, erythematöse Läsion (Abbildung 1 a). Der Tumor war hart, elastisch, unbeweglich und übelriechend. Wie die verstärkte Computertomographie zeigte, umfasste die Raumforderung die Seite unterhalb der Brustdrüse; jedoch gab es keine evidente Durchdringung der Drüse (Abbildung 1 b). Unter Lokalanästhesie unterzog sich die Patientin einer vollständigen Exzision mit Erhaltung der Brustwarze. Das Operationspräparat zeigte eine klar abgegrenzte lobuläre Proliferation squamöser zystischer Inseln in der Dermis (Abbildung 2 a, b). Die aufl iegende Epidermis Clinical Letter war vereitert. Die einzelnen zystischen Inseln ähnelten regelmäßigen Trichilemmalzysten, und die Epithelverkleidung bestand aus geschichtetem Plattenepithel, mit trichilemmaler Keratinisierung (Abbildung 2 c, d). Fremdkörperriesenzellen und Kalkeinlagerungen lagen auch vor (Abbildung 2 e). Innerhalb des Tumors gab es außerdem eine Anhäufung von roten Blutzellen (Abbildung 2 f). Es wurden keine aberranten Mitosen beobachtet. Auf Grundlage der oben erwähnten klinischen und histologischen Befunde wurde die Diagnose der PTC erstellt. Sechs Monate nach der Operation zeigte die Patientin keine Anzeichen eines Rezidivs oder anderer Komplikationen. Proliferierende Trichilemmalzysten sind im Allgemeinen ziemlich groß. Die Läsionen an der Kopfhaut erreichen einen Durchmesser bis zu 6 cm [ 2 ] . Einige PTCs zeigen mehrere zystische Tumoren innerhalb derselben Läsion, was somit zu einem malignen Erscheinungsbild führt. Die Differenzialdiagnosen der PTC umfassen die Trichilemmalzyste, die Epidermoidzyste und das Plattenepithelkarzinom [ 3 ] . Außerdem nimmt man an, dass die Trichilemmalzyste, die PTC und der maligne proliferierende Trichilemmaltumor (MPTT) ein kontinuierliches Spektrum darstellen [ 4 ] . Im vorliegenden Fall entwickelte sich der Tumor auf der Brusthaut und zeichnete sich durch Blutungen und anschließendes schnelles Wachstum aus. Dies wies auf einen malignen Tumor wie Brustkrebs hin. Die klinische und histopathologische Bewertung war daher entscheidend, um die korrekte Diagnose zu erhalten. Klinisch zeigte unser Fall keinen aggressiven Verlauf wie die Bildung von Metastasen oder eines Lokalrezidivs. Dies stimmte mit fehlenden histologischen Merkmalen eines Malignoms (zum Beispiel aberrante Mitosen) überein. Außerdem ergab die Computertomographie keinen Hinweis auf eine Durchdringung des Tumors in die Brustdrüse. Diese Beobachtungen legen nahe, dass die Läsion eine PTC und kein MPTT oder Brustkrebs war. Obwohl es einige Berichte über PTC am Rumpf gibt, liegt bisher kein Fall einer PTC vor, die sich an der Brusthaut entwickelte [ 1–6 ] . Nach unserem Wissen ist unserer der erste derartige Bericht.

Proliferating trichilemmal cyst of the mamma

A rare benign tumor predominantly affecting women, proliferating trichilemmal cyst (PTC) most commonly occurs on the scalp (78 %), followed by the trunk (13 %) [ 1 ] . Some PTC lesions are marked by rapid growth, which gives rise to concerns about malignancy. To date, there has been no publication of PTC arising on the mammary skin. Herein, we report the fi rst case of PTC occurring in this particular anatomic region, clinically exhibiting a breast cancer-like appearance. A 72-year-old Japanese woman was referred to our hospital with a one-year history of a painful, rapidly growing tumor on the left mamma. Physical examination revealed a purulent, erythematous lesion, measuring 3.5 cm × 3.1 cm × 2.4 cm (Figure 1 a). The tumor was hard, elastic, immobile, and foul-smelling. On enhanced computed tomography, the mass was shown to involve the inferior aspect of the mammary gland; however, there was no evident penetration of the gland (Figure 1 b). The patient underwent total excision under local anesthesia, with preservation of the nipple. The surgical specimen showed a well-defi ned lobular proliferation of squamous cystic islands in the dermis (Figure 2 a, b). The overlying epidermis was ulcerated. The individual cystic islands resembled regular trichilemmal cysts, and the epithelial lining was made up of stratifi ed squamous epithelium exhibiting trichilemmal keratinization (Figure 2 c, d). Foreign-body giant cells and calcifi cation were also present (Figure 2 e). In addition, there was an accumulation of red blood cells inside the tumor (Figure 2 f). Aberrant mitoses were not observed. Based on the aforementioned clinical and histological fi ndings, the diagnosis of PTC was made. Six months post surgery, she is showing no signs of recurrence or other complications. Proliferating trichilemmal cysts are commonly quite large, with scalp lesions growing up to 6 cm in diameter [ 2 ] . Some PTCs show multiple cystic tumors within the same lesion, thus giving rise to a malignant appearance. The differential diagnoses of PTC include trichilemmal cyst, epidermoid cyst, and squamous cell carcinoma [ 3 ] . In addition, trichilemmal cyst, PTC, and malignant proliferating trichilemmal tumor (MPTT) are thought to represent a continuous spectrum [ 4 ] . In the present case, the tumor developed on the mammary skin, and was marked by hemorrhage and subsequent rapid growth, which was suggestive of a malignant tumor such as breast cancer. The clinical and histopathological evaluation was therefore key to arriving at the correct diagnosis. Clinically, our case did not exhibit aggressive behavior such as metastasis or local recurrence. This was consistent with the lack of histological features of malignancy (for example, aberrant mitoses). Furthermore, computed tomography did not show any evidence of tumor penetration into the mammary gland. These observations suggest that the lesion was PTC and not MPTT or breast cancer. Although there are some reports of PTC on the trunk, there has been no previous case of PTC developing on the mammary skin [ 1–6 ] . To our knowledge, ours is the fi rst such report.

Reticulated acanthoma with sebaceous differentiation

Reticulated acanthoma with sebaceous differentiation (RASD) is a rare benign sebaceous tumour that is histopathologically characterized by basaloid cell proliferation in a plate-like configuration, underlain by sebaceous glands [1]. It was first described as “superficial epithelioma with sebaceous differentiation” (SESD) [2]. Since the term “epithelioma” is likely to be confused with malignancy, RASD was proposed as a new designation by Steffen and Ackerman [3]. Based on the lack of apocrine [...]

A case of bullous pemphigoid presenting with severe oedema of the hands during recurrence

Bullous pemphigoid (BP) is a common autoimmune bullous disease which is mainly caused by autoantibodies to the 180-kDa bullous pemphigoid antigen (BP180) [1]. BP is characterized by generalized bullous lesions and itchy oedematous erythemas, although some localized forms have been reported [2]. Herein, we report a case of BP presenting with an unusual feature of severe oedema of the hands during recurrence.A 69-year-old Japanese man presented with itchy erythemas and tense blisters on the trunk, [...]

Vulvar basal cell carcinoma with adhesion of the labia majora and minora.

plucked hair for DIF has been suggested. This is based on the presence of pemphigus antigens throughout the ORS of the hair follicle and dermal bulb matrix. Our first patient had follicular deposition of IgG with only patchy deposits in the epidermis. This may be due to increased expression of antigens on the hair follicles compared with epidermis. The increased volume of antigens offered by the follicular epithelium might have facilitated intercellular acantholysis in the follicular epithelium. In conclusion, our cases highlight an atypical presentation of PV. This report also supplements the existing evidence on Koebner phenomenon in pemphigus and the use of hair follicle as a substrate for DIF studies.