Lymphocutaneous type of nocardiosis caused by Nocardia vinacea in a patient with polymyositis.

We report a lymphocutaneous type of nocardiosis caused by Nocardia vinacea. A 62-year-old woman with polymyositis presented with some erythematous swellings and subcutaneous abscesses on her right middle finger and the dorsum of her hand, which had persisted for 2 weeks. Culturing of the excised nodule and pus revealed orange to orange-tan colonies with scanty whitish aerial mycelia. The isolate was identified as N. vinacea on the basis of its biochemical and chemotaxonomic characteristics and the results of molecular biological analysis. In our case, oral minocycline (MINO) and trimethoprim-sulfamethoxazole (TMP-SMX) for 7 weeks did not improve the clinical manifestation, even though in vitro susceptibility testing of the isolate predicted its susceptibility to MINO and TMP-SMX. Treatment with partial surgical excision followed by TMP-SMX and meropenem administration was effective. This is the first reported case of a lymphocutaneous type of nocardiosis caused by N. vinacea.

Dermoscopy of keratosis follicularis squamosa.

Although the characteristic clinical appearance and the differences in distribution, it is often difficult to differentiate keratosis follicularis squamosa (Dohi) from other keratotic disorders. Here, we describe the case of a 5-year-old boy with Dohi in the first time that by using dermoscopy. Dermoscpoy of the lesion showed typical lotus leaves on the water appearance and follicular plug in the center. We reported the usefulness of dermosocopy in Dohi.

Toxic epidermal necrolysis with severe hyperbilirubinemia: complete re-epithelialization after bilirubin reduction therapies.

Toxic epidermal necrolysis is a life-threatening skin disorder, and its mortality rate is estimated to be approximately 20-30%. It is characterized that more than 30% of the skin surface is eroded, however, skin lesions are usually re-epithelialized within 2-3 weeks. Previously, we reported a fatal case of toxic epidermal necrolysis with hyperbilirubinemia, and more than 60% of body surface areas had been eroded for 9 weeks. For the reason of delayed re-epithelialization, we hypothesized that hyperbilirubinemia was the culprit because bilirubin damaged cultured keratinocytes in vitro. In this case, we had an opportunity to treat another case of toxic epidermal necrolysis with severe hyperbilirubinemia. In order to reduce serum bilirubin levels, we performed bilirubin adsorption therapies, and skin lesions were successfully re-epithelialized within 4 weeks. Though further studies are required, we considered that bilirubin adsorption therapies are worth trying for toxic epidermal necrolysis with hyperbilirubinemia, especially for the cases suffering from delayed re-epithelialization.

Neurofibromatosis type 2 in an infant with multiple plexiform schwannomas as first symptom.

Neurofibromatosis type 2 (NF2) is an autosomal dominant disorder that is caused by inactivating mutations or a loss of both alleles in the NF2 tumor-suppressor gene. Bilateral vestibular schwannomas are considered to be the hallmark of this disease, with hearing loss and tinnitus which are caused by these tumors, usually presenting as the initial symptoms. In addition to other tumors and ocular findings, skin abnormalities also occur in NF2, however, they are not so characteristic as neurofibromatosis type 1 (NF1). We herein report a case of NF2 which occurred in a 5-year-old boy. He had multiple cutaneous tumors but did not have any symptoms related to vestibular schwannomas. He also had multiple depigmented spots. A histopathological examination revealed these tumors to be plexiform schwannomas; we therefore suspected NF2. As a result of magnetic resonance imaging with gadolinium enhancement, bilateral vestibular schwannomas were detected and a final diagnosis of NF2 was thus made. The association between NF2 and multiple depigmented spots is unknown, we therefore consider that multiple cutaneous plexiform schwannomas may strongly suggest an association with NF2.

Chronic pulmonary complications associated with toxic epidermal necrolysis: report of a severe case with anti-Ro/SS-A and a review of the published work.

Patients with toxic epidermal necrolysis (TEN) have been known to have various complications. Though pulmonary complications are often observed, they usually show an acute form; however, chronic complications are quite rare and little is known about either their incidences or clinical manifestations. We herein report a 33-year-old man who presented with chronic pulmonary complications after a recovery from TEN. At the onset of TEN, he had severe respiratory failure and artificial ventilation was instituted. Despite being extubated successfully, respiratory failure reappeared 1 month later. A diagnosis of chronic bronchitis with severe obstructive ventilatory impairment and bronchiectasis was made and he was treated with steroids, bronchodilators and antibiotics, however, he died 1.5 years after the onset of TEN. There have been 13 reported cases of chronic pulmonary complications with TEN or Stevens-Johnson syndrome (SJS) in the English published work. Such cases are usually classified into chronic bronchitis/bronchiolitis with obstructive change (including bronchiolitis obliterans and bronchiolitis obliterans organizing pneumonia), respiratory tract obstruction and bronchiectasis. Approximately 40% of all such patients die while the surviving continue to suffer from these complications because no curative therapy yet exists. As a result, the prognosis seems to be poor. The relationship between TEN and these chronic pulmonary complications remains to be elucidated. Interestingly, our patient was asymptomatically anti-Ro/SS-A positive at the onset of TEN. In addition, eccrine gland involvement and an extremely high level of serum salivary amylase were observed at the onset of TEN, furthermore, Sjögren-like symptoms occurred after recovery from TEN. These findings suggested that the Sjögren-like autoimmune abnormalities induced by anti-Ro/SS-A correlated with the development of chronic pulmonary complications in our patient.

Malignant melanoma and acquired dermal melanocytosis on congenital nevus spilus.

We reported a case of malignant melanoma and acquired dermal melanocytosis that appeared on congenital nevus spilus; this is the first report from Japan. An 85-year-old woman had had a nevus spilus on the right lower leg since birth. A black-brown nodule developed on the nevus three years before treatment. Blue-gray patches were found within the nevus on inspection. Histopathological analysis of these lesions revealed superficial spreading melanoma and acquired dermal melanocytosis, respectively. There have been 19 previous case reports of malignant melanoma on nevus spilus, and there have only been 4 cases of dermal melanocytosis (plaque-type blue nevus) on nevus spilus. We reviewed the reported cases in the literature and discussed the risk factors of nevus spilus.

Preserved IFN-alpha production of circulating Valpha24 NKT cells in primary lung cancer patients.

Human Valpha24 NKT cells bearing an invariant Valpha24JalphaQ antigen receptor, the counterpart of murine Valpha14 NKT cells, are activated by a specific ligand, alpha-GalCer, in a CD1d-dependent manner. Here, we demonstrate decreased numbers of circulating Valpha24 NKT cells in patients with primary lung cancer compared to healthy volunteers. However, Valpha24 NKT cells and DCs from lung cancer patients were functionally normal, even in the presence of tumor. Furthermore, levels of Valpha24 NKT cells in surgically resected lung tissue appeared to be equivalent to those of Valpha14 NKT cells in the mouse lung. Levels of Valpha24 NKT cells in the tumor tissue itself were increased about 2.5 times. Administration of alpha-GalCer-pulsed DCs expanded Valpha14 NKT cells in the lung more than 10 times, and the increased levels were sustained for 1 week. This may explain the previous finding that alpha-GalCer-pulsed DCs exerted strong antitumor activity in mouse lung tumor metastatic models. The potential use of alpha-GalCer-pulsed DCs for immunotherapy aimed at activating endogenous Valpha24 NKT cells in the lung of cancer patients is discussed.

Expansion of lung V alpha 14 NKT cells by administration of alpha-galactosylceramide-pulsed dendritic cells.

NKT cells, a novel murine lymphoid lineage bearing an invariant T cell receptor encoded by V alpha 14 and J alpha 281 gene segments, recognize a specific ligand glycolipid, alpha-galactosylceramide (alpha-GalCer) in a CD1d-dependent manner. Recent research has revealed that activated V alpha 14 NKT cells have dramatic antitumor effects against a wide variety of tumor cell lines in vivo and in vitro. Here, we demonstrate strong in vivo antitumor effects brought about by treatment with alpha-GalCer-pulsed dendritic cells in comparison with in vitro-activated V alpha 14 NKT cells. Furthermore, we show a significant expansion of endogenous V alpha 14 NKT cells in the lung following the administration of alpha-GalCer-pulsed dendritic cells. The feasibility of immunotherapy with alpha-GalCer-pulsed dendritic cells is discussed.