Rieko Isogai

Successful Treatment of Pulmonary Metastasis and Local Recurrence of Angiosarcoma with Docetaxel

Angiosarcoma of the face and scalp of the elderly frequently recurs locally, metastasizes early despite various treatments, and has a poor prognosis. We describe a patient who had angiosarcoma of the scalp with pulmonary metastasis. Local recurrence occurred after excision and local and arterial administration of IL‐2. A weekly administration method of docetaxel was therefore selected, resulting in complete remission of the pulmonary metastasis and a partial response of the local recurrence. This favorable clinical outcome in our case suggests that docetaxel therapy may be an option for the treatment of angiosarcoma of the scalp with pulmonary metastasis.

Myoepithelioma possibly originating from the accessory parotid gland.

Myoepithelioma originates almost exclusively from myoepithelial cells of the salivary, prostate and mammary glands. The skin is a very rare site where myoepithelioma occurs. We describe a patient with a myoepithelioma on the right cheek seen as a subcutaneous nodule that was separated from the parotid gland at surgical resection. Histopathological findings were consistent with those of a myoepithelioma that had originated from the parotid gland, suggesting that this tumor may have developed from the accessory parotid gland.

Disseminated scleroderma of a Japanese patient successfully treated with bath PUVA photochemotherapy.

BackgroundLocalized scleroderma is known to be resistant to therapies. Recently, it has been reported that bath PUVA photochemotherapy is effective for treating this dermatosis.ObjectivesAlthough according in earlier reports mainly white populations have been treated successfully with bath PUVA therapy, there is little knowledge of whether it is effective in treating colored populations. We treated a 64-year-old Japanese woman suffering from disseminated scleroderma with bath PUVA photochemotherapy to see its effects.ConclusionAlthough rather high cumulative UVA doses were required for this patient compared with those needed in earlier reports, no adverse effects were observed. The lesions were markedly improved, suggesting that this therapeutic modality is well-tolerated and useful for colored patients such as the Japanese. Furthermore, it turns out that the thermographical assessment is useful to estimate clinical improvement of this sclerosing skin disorder.SommaireAntécédentsLa sclérodermie localisée s’est avérée résistante aux divers traitements. On a rapporté récemment que la PUVAthérapie est efficace dans le traitement de cette dermatose.ObjectifBien que, selon certains rapports antérieurs, la PUVAthérapie ait donné d’excellents résultats surtout chez les blancs, il existe peu de données indiquant si elle est efficace chez les gens de couleur. Toutefois, une Japonaise de 64 ans souffrant de sclérodermie disséminée a été traitée par PUVAthérapie.ConclusionBien que d’assez fortes doses cumulatives d’UV-A aient été nécessaires dans le cas de cette patiente comparativement aux sujets qui ont fait l’objet de rapports antérieurs, aucun effet indésirable n’a été observé. Les lésions étaient nettement atténuées, ce qui fait croire que ce mode de traitement est bien toléré et qu’il est également efficace chez les patients de couleur comme cette Japonaise. En outre, il s’est avéré que l’examen thermographique est utile pour évaluer l’amélioration clinique de cette affection sclérosante de la peau.

A Case of Herpetiform Pemphigus with Anti-Desmoglein 3 IgG Autoantibodies

Herpetiform pemphigus (HP) is a rare variant of pemphigus characterized by a unique clinical phenotype of erythematous or urticarial plaques and vesicles that present in a herpetiform arrangement. Most HP cases have circulating anti‐desmoglein 1 (Dsg1) IgG autoantibodies, but some HP cases have anti‐desmoglein 3 (Dsg3) IgG. A 92‐year‐old Japanese woman presented with severely pruritic annular erythema and vesicles in a herpetiform arrangement on the trunk. No oral mucosal lesions were present. Histopathologically, these vesicles showed eosinophilic spongiosis as well as suprabasilar acantholysis. Direct immunofluorescence showed in vivo IgG deposition on keratinocyte cell surfaces, and indirect immunofluorescence showed circulating IgG autoantibodies against keratinocyte cell surfaces at a titer of 1:30. Enzyme‐linked immunosorbent assay using recombinant Dsg1 and Dsg3 revealed the presence of anti‐Dsg3 IgG but no anti‐Dsg1 IgG autoantibodies. The lack of oral mucosal involvement and the unique clinical features favored the diagnosis of HP. It remains to be clarified why the anti‐Dsg3 IgG autoantibodies in this patient induced this unique features of HP, rather than the mucosal dominant type of pemphigus vulgaris.

Successful treatment for recurrence of primary cutaneous anaplastic large-cell lymphoma in elderly patient with etoposide, mitoxantrone, cyclophosphamide, vincristine, prednisolone and bleomycin (VNCOP-B) therapy.

Primary cutaneous anaplastic large cell lymphoma (C‐ALCL) is a malignant lymphoma with a relatively good prognosis, consisting of CD30‐positive, undifferentiated, large cells. We report an elderly patient with C‐ALCL which recurred after cyclophosphamide, doxorubicin, vincristine and prednisone (CHOP) therapy, but was effectively treated with the third‐generation etoposide, mitoxantrone, cyclophosphamide, vincristine, prednisolone and bleomycin (VNCOP‐B) regimen. It is characterized by the completion of treatment in 8 weeks, its applicability on an outpatient basis, and a low incidence of cardiotoxicity and mucosal symptoms. Although our patient had no side‐effects during chemotherapy, patients should be carefully monitored for side‐effects, especially infection. In conclusion, the VNCOP‐B regimen might be an effective treatment for elderly patients with good performance status, CHOP‐resistant patients or patients with aggressive non‐Hodgkins lymphoma.

The presence of tryptase-positive and bikunin-negative mast cells in psoriatic skin lesions

Human mast cells are well known to produce a serine protease, tryptase, which appears to play a pathogenic role in various skin inflammations. It was previously reported that a rat homologue of bikunin may inhibit tryptase activity. Various type of cells (i.e. keratinocytes) are able to produce this protein inhibitor, it still remains unclear if bikunin is present in dermal inflammatory milieu, in which mast cells, through secretion of tryptase, play an inflammatory role. Therefore, the purpose of the present study was to exploit expression and production of bikunin in dermis and dermal constituents. We first compared the dermal mast cells in psoriatic lesions with those in lesional skin of atopic dermatitis or of chronic eczema by use of immunoelectron microscopy and immunohistochemical analyses using antibodies to bikunin and tryptase. Then, we tested what kinds of cytokines may regulate the de novo synthesis of bikunin. To do so, RNA was extracted from a human mastocytic cell line, HMC-1, reverse-transcribed, and semiquantitative RT-PCR was performed using primers specific for bikunin. With immunoelectron microscopy, bikunin was found to localize on the cell membrane, while tryptase was in the secretary granules of the mast cells. In psoriatic lesions, around 70% of dermal mast cells were positive for both tryptase and bikunin, and the remaining was mostly positive for tryptase, but the expression of bikunin was under the detection limit of the experimental setting. This observation was seen in only psoriatic lesions, even in almost cured lesions, while in atopic dermatitis or chronic eczema only mast cells doubly positive for bikuin and tryptase were seen. In HMC-1, bikunin was constitutively expressed at an mRNA level, which was upregulated by stimulation with interleukine-4, but was suppressed by interferon-γ. Bearing in mind the concept that in psoriasis local cytokine milieu is shifted toward a Th1 pattern (predominant secretion of interferon-γ), tryptase-positive, bikunin-negative mast cells may be induced.

Nail scabies as an initial lesion of ordinary scabies

SIR, A non-immunosuppressed 86-year-old-male inpatient with aspiration pneumonia was seen in November 2000 with a 1-month history of pruritic papules on the trunk and upper extremities. He had no past history of Down’s syndrome, diabetes mellitus, onychomycosis, tinea pedis, or crusted (Norwegian) scabies. Potassium hydroxide (KOH) examination of papules from the hands showed mites and eggs. These skin lesions were cured after 2 weeks’ treatment with 25% benzyl benzoate lotion and crotamiton ointment. Some nurses who cared for this patient simultaneously suffered pruritic papules from which mites were also found. We re-examined the patient and discovered thickened and whitened toenails with subungal horny debris; there was no nail deformity or periungal scales and crusts (Fig. 1). His family had noticed this 5 months previously, but neither he nor his family complained of nail lesions. KOH examination from the toenails showed numerous mites and eggs (Fig. 2), but not hyphae, indicating nail scabies. Topical treatment of 1% lindane ointment with occlusive dressing was started in December 2000; clinical and KOH examination revealed complete cure after 9 weeks of treatment (Fig. 3). Although uncommon, we believe that nail involvement in this patient may have been an initial lesion of ordinary scabies. Subungal areas have been reported to harbour mites, therefore, ordinary scabies could be added to a panel of nail involvement of skin disorders including psoriasis, lichen planus, alopecia areata, autoimmune diseases, and crusted scabies.

The successful treatment of diffuse neonatal hemangiomatosis with flashlamp pulsed dye laser.

Abstract: Diffuse neonatal hemangiomatosis is a rare disease with the distinctive features of multiple hemangiomas of the skin and visceral organs. These lesions have been treated with systemic corticosteroids, interferon‐α, and their combination. We report a patient with diffuse neonatal hemangiomatosis who had multiple cutaneous and hepatic hemangiomas. Single therapy with the flashlamp pulsed dye laser was effective for cutaneous hemangiomas, whereas the hemangiomas of the liver remained stable and no liver dysfunction or hemorrhage has occurred so far, even with no treatment.

A case of acral lentginous melanoma: the correlation between CD95L expression on melanoma cells and apoptosis of tumor infiltrating lymphocytes.

There is an increasing amount of evidence that melanoma cells express the ligand for CD95 (CD95L), a potent inducer of apoptosis, which contributes to creating the immune privileged circumstances of tumor sites. However, it still remains to be demonstrated whether the capacity of melanoma cells to express CD95L is acquired during the progression. We addressed this question with a case of acral lentiginous melanoma by employing immunostaining using an antibody directed against CD95L as well as by in situ TUNEL staining. H&E‐staining of tumor specimens revealed that there were two different growth patterns. The central part of the tumor showed a deeper invasion into the dermis (Breslow thickness>4‐mm). The horizontally growing edge of the tumor proliferated more superficially (Breslow thickness<3‐mm). Relatively fewer lymphocytes were observed around the melanoma nests in central areas, which expressed detectable amounts of CD95L. In contrast, more lymphocytes were observed among the melanoma cells in the peripheral lesion, where CD95L was not detected. To evaluate the relevance of the CD95L expression, in situ TUNEL staining was performed. This indicated a significant correlation of lymphocyte apoptosis with CD95L expression on melanoma cells. Together, the data suggest that expression of CD95L is turned on depending on the level of melanoma, and that it may contribute to creating immune privileged circumstances by initiating apoptosis of tumor infiltrating lymphocytes.

Factors involved in the development of diaper-area granuloma of the aged.

An infant skin disease with clusters of nodules in the diaper contact area was reported as granuloma gluteale infantum. Recently, the number of elderly patients with this condition has increased, and it has been reported as diaper‐area granuloma of the aged. These lesions are presumably caused by irritation from feces and urine. We observed similar cutaneous symptoms in six elderly patients, five of whom revealed clusters of nodules arranged in a circular pattern in the gluteal region presenting a peculiar clinical picture. They were all bedridden and wore cloth diapers. We speculate that wearing a cloth diaper seems to be a major factor behind this condition. Another factor was sitting during the day. A subject had lesions with nodules on the scrotum. The distribution of nodules appeared to be related to the body position and vigorous movement of the patient. In all cases, topical steroid therapies were not effective. They were all positive for bacteriuria. Chronic urinary tract infection further irritates the skin. It is necessary to reduce urine contact and keep the lesion clean.