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.

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.

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.

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.

Lichenoid drug eruption with palmoplantar hyperkeratosis due to imatinib mesylate: a case report and a review of the literature.

Cutaneous adverse effects of imatinib mesylate (Glivec) are common and various types of skin eruptions have been reported. We report here a 57-year-old man who presented with lichen planus-like lesions on his extremities and palmoplantar hyperkeratosis due to the use of imatinib mesylate for chronic myeloid leukaemia. The skin lesions improved after discontinuation of imatinib mesylate but re-administration of the drug at a lower dose provoked a mild recurrence. He could, however, continue to take the drug at the lower dose and his skin lesions were well-controlled by topical corticosteroid treatment. The literature on lichenoid drug eruption due to imatinib mesylate is reviewed.

Fatal interstitial pneumonia in juvenile dermatomyositis.

A 5-year-old girl presented with a two-month-history of skin rash and general fatigue. She had a slight fever, progressive muscle weakness and liver dysfunction. Gottron's papules on her fingers and purple-reddish papules on her elbows were noted. Serum aldolase levels were highly elevated, however, creatine phosphokinase levels were normal. An MRI revealed abnormal high signal changes in her gluteus minimus muscles. Interstitial pneumonia suddenly developed and she died despite aggressive treatment with methylprednisolone pulse therapy followed by intravenous administration of cyclophosphamide, immunoglobulin and cyclosporine A. Interstitial pneumonia is rare in juvenile dermatomyositis; however, as in adult cases, it can be fatal. In order to prevent severe complications and functional disabilities, early aggressive treatments should be considered when muscle inflammation is refractory to ordinary treatment.

Leg ulceration in chronic venous insufficiency caused by an absent inferior vena cava.

We report here the case of a 55-year-old Japanese man with a one-year history of multiple ulcers on the left crural region. He had had pronounced varicose veins on both legs and the abdominal region for 35 years. Computed tomography images of the thoracic and abdominal regions showed the absence of an inferior vena cava, with pronounced dilatation of paravertebral venous plexus, cutaneous and azygous veins. Conservative topical treatments led to complete healing of the ulcers in one month. An absent inferior vena cava is an uncommon abnormality, often complicated by cardiac and other visceral malformations. It is a rare cause of chronic leg ulcers.

Digital gangrene in systemic lupus erythematosus.

Digital ulcers and gangrene are common skin manifestations of connective tissue diseases, especially systemic sclerosis, although they are relatively rare in systemic lupus erythematosus. We describe here three patients with digital gangrene and systemic lupus erythematosus. None of the patients showed high disease activity of systemic lupus erythematosus at the time the digital gangrene developed. Two patients were positive for anti-RNP antibodies; however, no symptoms of other collagen diseases were present. One patient had anti-phosphatidylserine/prothrombin complex antibodies, and the other had anti-cardiolipin beta2 glycoprotein I antibodies and lupus anticoagulant at low titre. All patients showed narrowing or occlusion of radial and/or ulnar arteries in addition to digital arteries. Although a complication of anti-phospholipid syndrome is considered to be a possible cause, there may be unidentified causes other than thrombosis, atherosclerosis, overlap syndrome and vasculitis.

Cutaneous manifestations and histological features of microscopic polyangiitis.

Purpura and livedo are common cutaneous manifestations of microscopic polyangiitis (MPA); however, only a few clinical analyses focusing on the relationship between clinical symptoms and the affected vessels in the skin have been reported. We herein report the cutaneous manifestations and histological features of four patients with MPA. In two patients, a Henoch-Shönlein purpura-like eruption developed with necrotizing vasculitis localized in the upper dermis. The other two patients presented with livedo racemosa; with histological findings of necrotizing vasculitis of the small vessels around the muscle fibers or from the deep dermis to subcutaneous tissue. Two patients' cases were complicated by systemic sclerosis and had poor prognoses. MPA can present with various cutaneous manifestations. Additional research is required to ascertain the relationship between the prognosis and the affected vessels.

Multiple skin cysts in nevoid basal cell carcinoma syndrome: a case report and review of the literature.

We report a 72-year-old man with nevoid basal cell carcinoma syndrome (NBCCS) who presented with 25 skin cysts on his trunk and extremities. Fourteen of 25 skin cysts (56%) were observed on his hands. Histological examination demonstrated that most of the excised cysts had an epithelial wall with typical epidermal keratinization. However, 4 skin cysts (4/16; 25%) showed a corrugated or festooned inner surface of wall without a granular cell layer, which resembled an odontogenic keratocyst, a characteristic feature of skin cysts in NBCCS. A review of the literature suggested that acral localization of skin cysts is a distinctive feature of NBCCS. In addition, the histological findings of skin cysts resembling an odontogenic keratocyst might be a hallmark of NBCCS.

Successful treatment with bosentan for refractory digital ulcers in a patient with systemic lupus erythematosus.

We report a 32-year-old woman with a 12-year history of systemic lupus erythematosus. Physical examination revealed indurated plaques with small ulcers on her extremities and trunk, which were histologically diagnosed as lupus erythematosus profundus. On her arms and knees, multiple small calcified nodules were noted in the dermis and subcutis. An elevated level of anti-phosphatidylserine/prothrombin antibodies was noted. She had been suffering from digital ulcers on the left fourth finger. Despite conservative therapies, new ulcers appeared on other fingers. After the administration of bosentan, no new lesion has developed. Bosentan, recognized as a choice of treatments for digital ulcers in systemic sclerosis, is worth trying in systemic lupus erythematosus patients with refractory digital ulcers.

Leukemia cutis in a patient with acute monocytic leukemia presenting as unique facial erythema.

A 67-year-old woman was referred to our department with a 1-month history of facial exanthemas. She had been diagnosed as having acute monocytic leukemia (French-American-British classification, M5b) based on the histological findings of bone marrow. Physical examination revealed diffuse edematous erythema on her cheeks, eyelids and glabella with scattered reddish papules. Histological examination demonstrated dense infiltration of atypical mononuclear cells in the dermis. Specific cutaneous lesions could occur in acute monocytic leukemia more frequently than in other types of leukemia, but rarely show symmetrical edematous erythema limited to the face.

Infantile bullous pemphigoid treated with intravenous immunoglobulin therapy.

A 3-month-old boy presented with a 2-week history of rapidly spreading skin rashes. Physical examination revealed generalized urticarial plaques with tense bullae and small vesicles. Histologic examination and immunofluorescence established the diagnosis of bullous pemphigoid. The disease was resistant to conventional therapies with the combination of corticosteroids, dapsone, and erythromycin. Finally, intravenous immunoglobulin therapy brought about a remarkable improvement. We suggest that intravenous immunoglobulin therapy is a valuable treatment option for intractable bullous pemphigoid in infants as well as in adults.

Successful treatment with lymphaticovenular anastomosis for secondary skin lesions of chronic lymphedema.

The treatment of severe lymphedema is a difficult challenge. We performed lymphaticovenular anastomosis on two patients with secondary skin lesions of chronic lymphedema; one patient exhibited acquired lymphangioma circumscriptum of the vulva and the other presented elephantiasis nostras verrucosa of the lower leg. Both patients obtained a remarkable improvement in skin lesions and also in the reduction of lymphedema of the lower extremity. During a 6-month-follow-up period, constant reduction in the circumference of the lower extremities without exacerbation of skin lesions was achieved in both patients. Lymphaticovenular anastomosis is a useful surgical treatment for secondary lymphedema in the lower extremities. In addition, this surgical treatment is effective for secondary lesions of lymphedema, including acquired lymphangioma circumscriptum and elephantiasis nostras verrucosa.

Eosinophilic fasciitis: report of two cases and a systematic review of the literature dealing with clinical variables that predict outcome.

We reported two patients with refractory eosinophilic fasciitis (EF) and provided a systematic review of the literature to determine the clinical variables associated with prognosis of EF. We enrolled 88 cases, whose clinical characteristics were analyzed by separating the patients into two or three groups based on outcome. The incidence of certain clinical and pathological features differed among the groups. In particular, the incidence of morphea-like skin lesions in patients with refractory fibrosis was significantly higher than in patients without refractory fibrosis (p = 0.003). Patients with morphea-like skin lesions were 1.9 times more likely to develop persistent fibrosis than patients without these lesions (95% confidence intervals, 1.5-2.5). A younger age (under 12 years) at onset was associated with a 1.6 times greater risk of residual fibrosis (95% confidence interval, 1.1-2.2). Trunk involvement was associated with a 1.4 times greater risk of residual fibrosis (95% confidence interval, 1.0-2.0). Histopathologically, the presence of dermal fibrosclerosis was associated with a 1.4 times greater risk of refractory fibrosis (95% confidence interval, 1.0-2.1). We consider these clinical characteristics, notably the presence of morphea-like skin lesions may be an important risk factor for developing residual fibrosis in EF patients.

Juvenile systemic sclerosis: report of three cases and review of Japanese published work.

Juvenile or childhood onset systemic sclerosis (SSc) is rare. In our department, we encountered three juvenile SSc patients among the overall 206 SSc patients. All three cases were diffuse cutaneous type SSc (dSSc) with positive antinuclear antibodies. In this report, we report the three patients and analyze clinical and serological features of 58 Japanese patients with juvenile SSc. Forty-eight patients (92.3%) were classified as dSSc and 21 out of 35 patients (60%) had anti-topoisomelase I antibodies, while anticentromere antibodies were not detected in any patients. Prevalence of lung fibrosis and scleroderma renal crisis was low, however, the rate of cardiac involvement was higher than that in adult patients.

Reverse latissimus dorsi musculocutaneous flap for reconstruction of lumbar radiation ulcer.

The surgical treatment of large, deep defects on the midline of the lower back or lumbar area comprises difficult reconstructive challenges. Various flaps have been designed to reconstruct the defect area. We herein report a 70-year-old Japanese woman with a large, deep ulcer in the midline of the lower back, caused by postoperative radiation therapy for eccrine porocarcinoma. The ulcer was successfully treated with surgical debridement followed by reverse latissimus dorsi musculocutaneous flap. This flap is reliable and useful for reconstruction of a large, deep midline defect of the lower back, having large and bulky tissue with a sufficient blood supply.