Y. Yoshida

Clinicopathological study of invasive extramammary Paget’s disease: subgroup comparison according to invasion depth

Background  Extramammary Paget’s disease (EMPD) is a distinct form of malignant skin neoplasm. Invasive EMPD is relatively rare and its detailed histopathological features have not been investigated to date.

A morphological study of granulomas induced by subcutaneous injection of leuprorelin acetate

Leuprorelin acetate‐induced granulomas in subcutaneous injection sites have been occasionally reported, but there have been no detailed morphological examinations. Histopathology of leuprorelin acetate‐induced granulomas in six patients was studied, and one of them was evaluated by electron microscopy. Study using haematoxylin and eosin staining revealed that there were many intracytoplasmic vacuoles in the granulomas and degeneration of fat tissue. Ultrastructurally, electron‐lucent spherical bodies (microcapsules of leuprorelin acetate products) were observed in the granuloma cells. Needle‐shaped crystalloid structures in lipid droplets (degenerated lipid droplets) were observed in the granuloma cells and fat tissue. Degeneration of fat tissues caused by leuprorelin acetate is one of main factors responsible for granuloma formation.

Abnormal cell attachment in circumscribed palmar hypokeratosis: ultrastructural observations

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Multiorgan involvement in Sweet's syndrome.

A 59-year-old man who had been hospitalized for endoscopic excision of untreated early gastric cancer (stage 1A) presented with a 1-week history of high fever and indurated painful erythemas on the nape of the neck and the right ear. The gastric cancer had been diagnosed 4 months before, and the patient was hospitalized for endoscopic excision. At the time the eruption first appeared, a diagnosis of myelodysplastic syndrome had been made by a haematologist. The erythemas had gradually appeared on the patient s face, the dorsa of both hands and the left ear during the course of 1 week. Physical examination revealed indurated erythemas with superficial pustules on the patient s face (Fig. 1) and his dorsal hand and finger appeared gangrenous. There were a few reddish nodules on both conjunctiva and swelling on the lower jaw. Laboratory investigations showed pancytopenia, with a red blood cell count of 2.26 · 10 ⁄ L (normal range 1.5– 6.5 · 10 ⁄ L), haemoglobin 79 g ⁄ L (13.0–18.0), white blood cell count of 2.8 · 10 ⁄ L with 66% polymorphonuclear leucocytes (4–11 · 10 ⁄ L and 40–60%) and platelet count of 105 · 10 ⁄ L (150–400 · 10 ⁄ L). Serum granulocyte colony-stimulating factor (G-CSF) was raised at 122 pg ⁄ mL (normal, < 10 pg ⁄ mL). Skin biopsies taken from the lesions on the patient s face and hands revealed a high level of neutrophil infiltration with marked dermal oedema but without any signs of vasculitis (Fig. 2). No microorganisms grew on cultures. These findings led to the diagnosis of Sweet s syndrome (SS). Chest X-ray and computed tomography scan revealed right lower pulmonary infiltrates. Sonography of the lower jaw revealed swelling of the submandibular gland. As there were no signs of another disease (e.g. sarcoidosis, tuberculosis), the pulmonary infiltrates and swelling of the submandibular gland were likely to be related to SS. The patient was started on oral prednisolone 40 mg ⁄ day and sulfamethoxazole–trimethoprim 1 g ⁄ day. The skin lesions gradually improved, but the treatment had no effect on the pulmonary infiltrates or the swelling of the lower jaw. The patient was then given pulse treatment with methylprednisolone (1000 mg ⁄ day), and the symptoms were dramatically improved. After the pulse treatment, the dosage of oral prednisolone was gradually tapered from 60 mg ⁄ day, but when it reached 35 mg ⁄ day, the patient developed a high fever and reduced consciousness. Although the symptoms indicated involvement of the meninges, infectious brain meningitis (due to infection with viruses, tuberculosis or other bacteria) was excluded by the results of cerebrospinal fluid investigations and

A case of symmetrical drug-related intertriginous and flexural exanthema induced by loflazepate ethyl

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Multiple subcutaneous hard nodules.

A 36-year-old-woman presented with multiple hard subcutaneous nodules, 5–10 mm in size, on the trunk and limbs without any other symptoms. The lesions had been present since infancy. The patient also had brachymetacarpia of both fourth fingers (Fig. 1a). Results of laboratory examinations, including levels of serum calcium, phosphate, thyroid hormones, and intact parathyroid hormone (PTH) were within normal limits. The patient’s 1-year-old daughter had similar multiple hard cutaneous nodules on the trunk and limbs. She also had a round face, flattened nasal bridge, obesity and hyperthyroidism, and had been diagnosed as having pseudohypoparathyriodism (PHP) Ia.

A novel PAX3 mutation in a Japanese boy with Waardenburg syndrome type 1

Waardenburg syndrome type 1 (WS1) is a rare autosomal dominant disorder characterized by hair hypopigmentation, abnormal iris pigmentation, and congenital hearing loss. WS1 is caused by mutations in paired box gene 3 (PAX3). We identified a novel PAX3 mutation (c.1107 C>G, p.Ser369Arg) in a Japanese WS1 patient showing abnormal right iris pigmentation, right-sided congenital hearing loss, synophrys, incomplete left cleft lip, and cryptorchidism.

A morphological comparison of a mixed tumour of the skin on the nasal tip: magnetic resonance imaging and histopathological features

A mixed tumour of the skin appears clinically as a slowgrowing, solitary, asymptomatic nodule within the dermis or subcutis, most commonly occurring on the head and face. Correct diagnosis of this tumour is often difficult. We report a case of mixed tumour of the skin on the nasal tip and discuss the features seen on magnetic resonance imaging (MRI) features, and the histopathological correlation. A 30-year-old man was referred to our department for evaluation of a tumour on the nasal tip, which had been enlarging for 2 years before presentation. On physical examination, a dome-shaped, well-demarcated, reddish subcutaneous tumour, 20 mm in diameter, was seen on the nasal tip (Fig. 1a). The surface of the tumour was smooth, and some dilated vessels were visible beneath the skin. The clinical differential diagnosis included lymphocytoma cutis, epidermal cyst and mixed tumour of the skin. MRI was performed preoperatively, and both T1weighted and T2-weighted images revealed a wellcircumscribed round mass, measuring 15 · 11 mm, on the nasal tip. T1-weighted MRI displayed isoechoic signal intensity, whereas T2-weighted MRI showed heterogeneously hyperechoic, isoechoic and hypoechoic signals within the tumour (Fig. 1b). Histopathological examination of a skin biopsy taken from the tumour found it to be composed of numerous tubular structures and solid nests embedded in dense connective tissue stroma with partial myxoid change (Fig. 1c). The tubular lumina were lined by two layers of cells. These histopathological features were typical of mixed tumour of the skin. The tumour was excised under general anaesthesia. We compared the histopathological findings with the T2-weighted images of MRI. The myxoid component corresponded to a high-intensity area on the MRI scan. The tubular structures corresponded to the isoechoic and