Polypoid granulation tissue in pressure ulcers: Significance of describing individual ulcers.

Granulation tissue formation is required for the healing of deep pressure ulcers. The wound healing process is often delayed at the stage of granulation tissue formation. The pathogenesis of pressure ulcers showing granulation tissue may vary; however, no terminology has been defined to describe existing ulcers. Thus, we previously defined terminology for granulation tissue to describe individual ulcers. Based on these terms, we retrospectively evaluated the findings of deep pressure ulcers. In particular, we focused on polypoid granular tissue, a unique morphological feature. Polypoid granulation tissues were frequently observed in pressure ulcers over the sacrum compared with those over the foot. Chronological observation of a few cases indicated that external forces from specific directions during the healing process caused the development of polypoid granulation tissue. In addition, most pressure ulcers showing polypoid granulation tissue exhibited a trench-like appearance in individual wounds. Based on these observations, polypoid granulation tissue may generate from specific external forces, which lead to wound deformity. Morphological findings in an individual wound may be useful to predict the mechanical factors on existing pressure ulcers.

Wound fixation for pressure ulcers: a new therapeutic concept based on the physical properties of wounds.

A pressure ulcer is defined as damage to skin and other tissues over a bony prominence caused by excess pressure. Deep pressure ulcers that develop over specific bony prominences often exhibit wound deformity, defined as a change in the 3-dimensional shape of the wound. Subsequently, the wound deformity can result in undermining formation, which is a characteristic of deep pressure ulcers. However, to date, a concept with respect to alleviating wound deformity has yet to be defined and described. To clarify the issue, we propose a new concept called "wound fixation" based on the physical properties of deep pressure ulcers with wound deformity. Wound fixation is defined here as the alleviation of wound deformity by exogenous materials. The wound fixation methods are classified as traction, anchor, and insertion based on the relation between the wound and action point by the exogenous materials. A retrospective survey of a case series showed that wound fixation was preferentially used for deep pressure ulcers at specific locations such as the sacrum, coccyx, and greater trochanter. Moreover, the methods of wound fixation were dependent on the pressure ulcer location. In conclusion, our new concept of wound fixation will be useful for the practical treatment and care of pressure ulcers. Further discussion and validation by other experts will be required to establish this concept.

Clinical analysis of leg ulcers and gangrene in rheumatoid arthritis.

Leg ulcers are often complicated in patients with rheumatoid arthritis (RA), however, the etiology is multifactorial. We examined the cases of leg ulceration or gangrene in seven RA patients who were hospitalized over the past 3 years. One patient was diagnosed as having pyoderma gangrenosum. Although vasculitis was suspected in three patients, no histological evidence was obtained from the skin specimens. In these patients, angiography revealed the stenosis or occlusion of digital arteries. In the remaining three patients, leg ulcers were considered to be due to venous insufficiency. Treatment should be chosen depending on the causes of leg ulcers.

A case of rapidly progressive, fatal mycosis fungoides presenting as a haematoma-like lesion.

Mycosis fungoides usually follows an indolent clinical course. We report here a rapidly progressive case of mycosis fungoides with peculiar clinical and histological features, presenting as a haematoma-like mass on the thigh accompanied by multiple reddish brown erythematous lesions on the trunk and extremities. Histopathologically, the erythematous lesions showed epidermotropism of atypical T lymphocytes expressing CD4 and prominent syringotropism without syringometaplasia. The haematoma-like lesion consisted of diffuse and dense infiltrates of medium-to-large-sized pleomorphic lymphocytic cells expressing CD30, suggesting that CD30+ large-cell transformation had occurred. Mycosis fungoides presenting as a haematoma-like lesion is rare and may be a poor prognostic sign.

Autopsy case of delayed anaphylactic shock due to contrast medium.

We report an autopsy case of delayed anaphylactic shock due to contrast medium. A 17-year-old Japanese man underwent angiography using non-ionic contrast medium under the suspected diagnosis of Buerger's disease. Initial symptoms appeared 6 h after the administration of the contrast medium, and death was confirmed 11 h later. Considering the clinical course and the results of the autopsy, we concluded that the direct cause of the patient's death was severe acute circulatory failure due to a delayed allergic reaction to the contrast medium. The reported incidence of serious delayed reactions or biphasic reactions to non-ionic contrast medium is extremely low; however, we should be aware of such rare adverse reactions.

Isolated cutaneous manifestation of IgG4-related disease.

AIMS: The cutaneous manifestation of IgG(4)-related disease has rarely been reported. The aim of this study is to identify and describe the cutaneous manifestations associated with IgG(4)-positive plasma cell infiltration in the skin. METHODS: The authors investigated two cases of IgG(4)-related disease with solitary skin lesions and compared the immunohistochemical characteristics of infiltrating cells among IgG(4)-related disease, Kimura's disease and cutaneous pseudolymphoma. RESULTS: IgG(4)-related disease manifested as an indurated plaque on the anterior chest in one case and a nodule on the toe in the other case. Histopathologically, skin lesions of IgG(4)-related disease showed a dense, mixed-cell infiltrate containing lymphocytes, plasma cells and eosinophils along with fibrosis. Plasma cells stained positively with anti-IgG and anti-IgG(4) antibodies, and the ratio of IgG(4)+/IgG+ cells was more than 50%. Serum levels of IgG and IgG(4) were not elevated and no lesions were found in other organs. Skin samples taken from Kimura's disease showed histopathological features similar to those of IgG(4)-related disease. The proportion of IgG(4)+/IgG+ was high in Kimura's disease, but not in cutaneous pseudolymphoma. CONCLUSIONS: The solitary skin lesions of IgG(4)-related disease were similar histologically and immunohistochemically to the skin lesions of Kimura's disease. The concept of IgG(4)-related disease may help clarify the pathomechanism of diseases of unknown aetiology that possess features of IgG(4)-related disease.

Clinical follow-up study of adult-onset Still's disease.

Eighteen patients with adult-onset Still's disease have been followed up for 3-22 years in our department. Initial manifestations were fever with skin rash in 14 patients, fever, skin rash and sore throat in two, skin rash in one and arthralgia in one. During the follow-up period, typical skin rash was seen in all patients, of them five patients (29%) revealed atypical skin rash simultaneously. Atypical rash included persistent erythema with pigmentation in two, persistent plaques and papules with linear erythema in two and edema of the eyelids mimicking dermatomyositis in one. Persistent papules and plaques revealed histologically characteristic features, such as dyskeratotic keratinocyte and liquefaction degeneration as well as a sparse superficial dermal infiltrate containing scattered neutrophils. In patients of chronic articular type and polycyclic systemic type, atypical skin rash, lymphadenopathy and hyperferritinemia were noted to be significantly higher than those of monocyclic type. These factors might be prognostic factors of adult-onset Still's disease in our study.

Xeroderma pigmentosum complementation group G patient with a novel homozygous missense mutation and no neurological abnormalities.

We describe an unusual xeroderma pigmentosum (XP) patient with a mutation in XP complementation group G, representing only the third reported Japanese XP-G patient. A 40-year-old men (XP3HM), born from consanguineous parents experienced sun sensitivity and pigmentary changes of sun-exposed skin since childhood. He developed a squamous cell carcinoma on his lower lip at the age of 40. He has neither neurological abnormalities nor Cockayne syndrome. The primary fibroblasts of the patient were hypersensitive to killing by UV (D(0) = 0.6 J/m(2)) and the post-UV unscheduled DNA synthesis was 8% of normal. Host cell reactivation complementation analysis implicated XP complementation group G. We identified a novel homozygous mutation (c.194T>C) in a conserved portion of the XPG(ERCC5) gene, resulting in a predicted amino acid change; p.L65P. We confirmed that this genetic change reduced DNA repair thus linking this mutation to increased skin cancer.

Bosentan for digital ulcers in patients with systemic sclerosis.

Recurrent digital ulcers are manifestations of vascular disease in patients with systemic sclerosis (SSc). We report six patients with severe digital ulcers who were treated with bosentan administered p.o., 62.5-125 mg daily. The mean duration from the diagnosis of SSc to the initiation of bosentan was 9.5 years, and the observation period after bosentan administration was from 7 months to 4.5 years. In case 1, neither new digital ulcers nor Raynaud's phenomenon developed for 4.5 years. In case 2, digital ulcers recurred after the discontinuation of bosentan; however, re-administration of bosentan lead to the improvement. In cases 3-5 with recurrent digital ulcers, no new lesions have developed. In these five patients, pain evaluated by visual analog scale was significantly reduced. In three patients, bosentan was discontinued because of severe liver dysfunction. These results suggest that bosentan is an effective treatment for refractory digital ulcers associated with SSc; however, liver function should be carefully monitored. Compared to the doses of bosentan used to treat pulmonary hypertension, relatively lower doses may effectively control painful digital ulcer/gangrene in patients with SSc.

Detection of iron deposition in dermal fibrocytes is a useful tool for histologic diagnosis of nephrogenic systemic fibrosis.

Nephrogenic systemic fibrosis (NSF) is a fibrotic disease that presents with a history of renal dysfunction. The differential diagnosis generally includes scleromyxedema, systemic sclerosis, and morphea. Especially, scleromyxedema can be extremely difficult to distinguish microscopically. Although the fibrocytes in NSF are often positive for CD34 and procollagen-I, this is not specific for NSF. We identified positive iron staining in the skin of a patient with NSF and investigated whether this was a specific feature among 9 patients with NSF reported in Japan. We found that 6 of 9 patients showed positive iron staining in the dermal fibrocytes. The amount of iron deposition seemed to have no correlation with the degree of fibrosis or duration of the skin lesions but correlated with apparent history of the use of gadolinium-based contrast agents. As controls, skin biopsies from patients with scleromyxedema, morphea, and systemic sclerosis were evaluated by iron staining. None of these control patients showed iron deposition, indicating that positive iron staining may be specific to NSF and can be a useful tool for NSF diagnosis.

Lupus pernio with multiple bone cysts in the fingers.

A 32-year-old Japanese man presented with a 3-year history of purple reddish, and painful swelling of his fingers along with indurated erythema on his nose and ears. He was diagnosed as having sarcoidosis 8 years prior because of uveitis and bilateral hilar lymphadenopathy. X-rays of the hands revealed multiple cystic lesions in the phalanges. Histological examination of the ear revealed epithelioid cell granulomas in the dermis. Oral prednisolone 20 mg/day improved his finger swelling and pain; however, his finger deformities and erythema remain unchanged. Bone involvement is sometimes seen in sarcoidosis and the hands are the most frequently affected areas. The frequency of bone involvement is higher in lupus pernio in comparison with other types of skin sarcoidosis. Systemic corticosteroids could be the first choice of treatment to relieve the symptoms.

Reactive proliferation of endothelial cells and pericytes associated with arteriovenous malformation.

Arteriovenous malformation (AVM) is a structural vascular abnormality with no proliferation of cellular components. We report on a 53-year-old man who presented with a 15-year history of a progressively enlarging nodule on his lower lip. A dark-reddish, easy-bleeding nodule diagnosed as AVM was resected to reduce the volume and troublesome bleeding. Histologically, the nodule revealed that the proliferating cellular area was composed of endothelial cells and pericytes in addition to the area of dilated vessels. We speculated that the cell proliferation developed secondary to AVM. We also discuss the histological differential diagnosis of spindle cell hemangioma and pseudo-Kaposi's sarcoma.