Dermatofibrosarcoma protuberans congénito: descripción de un caso / Congenital dermatofibrosarcoma protuberans: a case report

El dermatofibrosarcoma protuberans es un tumor fibrohistiocitario de bajo grado poco común y que suele aparecer en adultos entre los 20 y 50 años de edad. Inicialmente se presenta como una mácula o placa violácea de aspecto vascular sobre la que aparecen posteriormente lesiones nodulares en la superficie. El diagnóstico es histológico y se trata de un tumor de células fusiformes que se disponen en fascículos cortos adoptando un patrón arremolinado característico. Es un tumor con una elevada tasa de recurrencias locales, aunque son raras las metástasis. El tratamiento es quirúrgico con amplios márgenes. Este tumor excepcionalmente afecta a niños menores de 16 años y todavía más raros son los casos congénitos, con sólo 27 casos descritos en la literatura. Se describe el caso de un niño de 10 años de edad con un dermatofibrosarcoma protuberans presente desde el nacimiento y sin signos de recurrencia en la actualidad

Dermatofibrosarcoma protuberans is a rare low-grade fibrohistiocytic tumor with onset normally at ages between 20 and 50 years. It presents as a violaceous plaque or macule with an appearance suggestive of vascular lesion, on which nodular lesions appear later. Histological diagnosis is based on the presence of a spindle-cell tumor arranged in small bundles in a characteristic cartwheel pattern. The local recurrence rate is high but metastases are rare. The treatment is surgical resection with wide margins. The tumor rarely affects children under 16 years of age and it is even less common at birth--only 27 congenital cases have been described in the literature. We describe the case of a 10-year-old boy with dermatofibrosarcoma protuberans present since birth and currently without signs of recurrence

[Congenital dermatofibrosarcoma protuberans: a case report]. / Dermatofibrosarcoma protuberans congénito: descripción de un caso.

Dermatofibrosarcoma protuberans is a rare low-grade fibrohistiocytic tumor with onset normally at ages between 20 and 50 years. It presents as a violaceous plaque or macule with an appearance suggestive of vascular lesion, on which nodular lesions appear later. Histological diagnosis is based on the presence of a spindle-cell tumor arranged in small bundles in a characteristic cartwheel pattern. The local recurrence rate is high but metastases are rare. The treatment is surgical resection with wide margins. The tumor rarely affects children under 16 years of age and it is even less common at birth--only 27 congenital cases have been described in the literature. We describe the case of a 10-year-old boy with dermatofibrosarcoma protuberans present since birth and currently without signs of recurrence.

Atrophic dermatofibroma accompanied by aneurysmatic characteristics.

A 40-year-old man presented a painful haemorrhagic plaque on his chest in the same location where a nodular lesion had been presented for many years. After 2 months, the plaque was replaced by a depressed lesion. The lesion diagnosed as an anetoderma was excised and the biopsy showed an atrophic dermatofibroma accompanied by aneurysmatic characteristics.

[Eosinophilic ulcer of the oral mucosa: 11 cases]. / Ulcère éosinophilique de la muqueuse buccale: 11 cas.

BACKGROUND: The eosinophilic ulcer is a lesion of the oral mucosa, that has been infrequently described in the literature. This is a benign and self-limiting lesion of unknown origin. CASE REPORTS: In this article, eleven new cases of eosinophilic ulcer of the oral mucosa are presented. The clinical, histologic and evolutive features are reviewed. DISCUSSION: Recurrent trauma is clearly involved in the pathogenesis of this entity.

Mycobacterium kansasii infection limited to the skin in a patient with AIDS.

We describe a case of cutaneous Mycobacterium kansasii infection in a 56-year-old man with acquired immunodeficiency syndrome, who received treatment with trimethoprim-sulphamethoxazole for Pneumocystis carinii pneumonia. Resolution of the cutaneous lesion was observed without specific treatment.

Subcutaneous sarcoidosis with dactylitis.

Subcutaneous sarcoidosis appears to be rare and is usually associated with hiliar adenopathy. Finger swelling is well recognized in patients with sarcoidosis and usually results from bony involvement or tenosynovitis. We report a patient with subcutaneous sarcoidosis and dactylitis. Biopsy of a finger and of subcutaneous nodules showed similar features with a granulomatous infiltrate. There were no osseus or tendinous lesions.

Low-dose interferon alpha combined with zidovudine in patients with AIDS-associated Kaposi's sarcoma.

Forty patients with AIDS-associated Kaposi's sarcoma (KS) treated with the combination of interferon alpha-2b (IFN-alpha) 10-20 MU day-1 and zidovudine (ZDV), 500-800 mg day-1, were evaluated for safety and efficacy. Eighteen patients (45%) had an overall response (CR+PR) at 3 months and a response persisting for a median of 14 (3-27) months. Patients with a CD4 count of less than 300 mm-3, prior to opportunistic infections or constitutional symptoms, were less likely to respond. However, between 28.5% and 36% of patients with a low CD4 count did respond to combined therapy. This is higher than would be predicted from single agent IFN-alpha therapy. Twelve of 28 patients (42.8%) receiving 10 MU day-1 of IFN-alpha (low dose) had an overall response. In addition, patients tolerated this dose of IFN-alpha better, presenting fewer flu-like symptoms and displayed a trend toward less anaemia. p24 antigen decreased in six out of nine evaluable cases, four of whom were treated with low-dose IFN-alpha. Low-dose IFN-alpha plus ZDV seems to be a useful and well-tolerated therapy for KS with antitumoral and antiviral activity. Patients without 'bad prognostic markers' are most likely to show improvement.

Quantitative and morphometric analysis of Langerhans cells in non-exposed skin in renal transplant patients.

Renal transplant recipients have a high incidence of cutaneous complications such as neoplasia and viral or fungal infections. Morphologic alterations of epidermal Langerhans cells (LC) have furthermore been described in these patients. Since these changes have been mainly found in sun-exposed skin, a direct effect of immunosuppressive therapy remains a matter of discussion. A quantitative and morphometric study of epidermal LC in non-exposed skin was performed in 28 renal transplant patients (RTP). RTP were divided in two groups according to immunosuppressive treatment: group A; azathioprine + prednisone (14 cases) and group B; cyclosporine + prednisone (14 cases). Twenty sex-age matched non-immunosuppressed patients acted as controls (group C). Epidermal sheets were obtained by incubation in EDTA and stained for ATPase activity and with the monoclonal antibody T6 (CD1) using the avidin-biotin peroxidase method. Langerhans cells were counted using a calibrated graticule (400x) and expressed as the mean number of LC/mm2. The mean area of the LC and the number of primary dendrites (pd) and secondary dendrites (sd) were determined with a morphometer adapted to an Apple II computer. The mean number of positive cells in controls was: ATPase, 677 +/- 157; T6, 695 +/- 164. Patients in group A had the maximum reduction in both ATPase and T6 LC density (ATPase, 339 +/- 142; T6, 402 +/- 194). Patients in group B had an intermediate reduction in the number of LC (ATPase, 494 +/- 121; T6, 529 +/- 112).(ABSTRACT TRUNCATED AT 250 WORDS)

[Hairy leukoplakia: a new disease of the oral mucosa associated with infection by the human immunodeficiency virus]. / Leucoplasia vellosa: una nueva enfermedad de la mucosa oral asociada a infección por el virus de la inmunodeficiencia humana.

In 23 out of 227 patients with positive serological tests for the human immunodeficiency virus (10%), seen between June 1987 and May 1988, lingual lesions of hairy leukoplakia (HL) were found. HL was present in 16/188 drug abusers (9%) and in 7/19 homosexuals (32%). In 3 cases HL was the only clinical manifestation of disease, in 11 it was associated with other symptoms of acquired immunodeficiency syndrome related complex (ARC), and in 9 it was found in patients with a previous or concomitant diagnosis of acquired immunodeficiency syndrome. The mean count of CD4 lymphocytes in the 23 patients was 0.22 X 10(9)/l. The diagnosis was made on the basis of the characteristic clinical features. In 3 cases biopsy was carried out, and parakeratosis and vacuolization of the spinous layer cells were found. Remarkably, particles of the herpesvirus group were also found. The lesions oscillated in size or even spontaneously disappeared, at least transiently; however, in the patients treated with zidovudine the improvement appeared to be more significant. The detection of HL discloses a likely infection by the HIV; it is usually associated with other features of ARC and/or severe immune depression, and it requires the institution of antiretroviral treatment.