[Rheumatic expression of secondary syphilis]. / Manifestações reumáticas da Sífilis Secundária.

Syphilis is a disease caused by Treponema pallidum infection with protean clinical manifestations. Musculoskeletal complaints are however uncommon and most of the time mild. Occasionally they can dominate the clinical picture and simulate a variety of rheumatic diseases. The authors present the clinical case of a 33-year-old woman who developed a lupus-like syndrome in the postpartum, characterized by polyarthritis, elevated acute phase reactants and positive antinuclear antibodies (ANA). Physical examination revealed a macular non-pruriginous skin rash involving the trunk, upper limbs and palms. The Rapid Plasma Reagin (RPR) and Treponema Pallidum Hemaglutination (TPHA) tests gave a positive result and the patient was diagnosed as secondary syphilis and medicated with 2.4 MU of benzathine penicillin intramuscular weekly for 3 weeks, with complete resolution of clinical signs and ANA negativation. The association of rash and arthritis may occur in several rheumatic diseases but in the presence of palmoplantar involvement, the possibility of syphilis infection should not be overlooked.

Amiloidose sistémica associada a mieloma múltiplo / Systemic amyloidosis multiple myeloma-associated

A amiloidose AL é uma doença que resulta da deposição tecidual de cadeias leves de imunoglobulinas, ocorrendo em 15% dos doentes com mielomamúltiplo. As manifestações mucocutâneas frequentemente antecedem o aparecimento de outros sintomas de gamapatia monoclonal, possibilitando um diagnóstico mais precoce. Descrevemos o caso de um doente de 71 anos que recorreu à consulta por lesões cutâneas nas pálpebras, sulcos retroauriculares e dorso, cuja biópsiarevelou depósitos amilóides. A investigação subsequente identificou a existência de mieloma múltiplo (AU)

AL amyloidosis results from tissue deposition of immunoglobulin light chains, occurring in 15% of patients with multiple myeloma. Mucocutaneouslesions are often the first manifestations, helping in earlier recognition of monoclonal gamapathy.We present the case of a 71-year-old man who presented with cutaneous lesions on the eyelids, retroauricular folds and upper back, which proved to be due to amyloid deposition. Further investigation revealed multiple myeloma (AU)

Amiloidose sistémica associada a mielomamúltiplo / Systemic amyloidosis multiple myeloma-associated

A amiloidose AL é uma doença que resulta da deposição tecidual de cadeias leves de imunoglobulinas, ocorrendo em 15% dos doentes com mielomamúltiplo. As manifestações mucocutâneas frequentemente antecedem o aparecimento de outros sintomas de gamapatia monoclonal, possibilitandoum diagnóstico mais precoce.Descrevemos o caso de um doente de 71 anos que recorreu à consulta por lesões cutâneas nas pálpebras, sulcos retroauriculares e dorso, cuja biópsiarevelou depósitos amilóides. A investigação subsequente identificou a existência de mieloma múltiplo (AU)

AL amyloidosis results from tissue deposition of immunoglobulin light chains, occurring in 15% of patients with multiple myeloma. Mucocutaneouslesions are often the first manifestations, helping in earlier recognition of monoclonal gamapathy.A 71-year-old man who with cutaneous lesions on the eyelids, retroauricular folds and upper back, which proved to be due to amyloid deposition isreported. Further investigation revealed multiple myeloma (AU)

Choroidal neovascular membranes after photodynamic therapy: ultrastructural analysis of two surgically excised membranes.

PURPOSE: To report on the ultrastructural electron microscopic findings of two surgically excised subfoveal choroidal neovascular membranes (CNV) that had undergone photodynamic therapy (PDT). METHODS: Two patients underwent PDT because of subfoveal neovascular membranes (CNV). Due to enlargement of the CNV seen on fluorescein angiography three months after PDT, one patient underwent surgical excision of the membrane; the other patient underwent both surgical membrane excision combined with macular translocation one month after PDT. The membranes were examined under the transmission electron microscope (TEM). RESULTS: The membranes were composed of a core and a rim, the latter being mainly composed of fibrin and collagen fibrils. The core was preeminently composed of endothelium-lined vascular channels associated with retinal epithelium cells. The endothelial cells of blood vessels appeared well-preserved. CONCLUSIONS: The lack of histological signs of recanalization and vascular thrombosis may indicate that in our cases the enlargement of the CNVs seen on fluorescein angiography three months and one month respectively after PDT may originate mainly from reproliferation of choroidal vessels rather than recanalization of previously occluded vessels.

Brooke-Spiegler syndrome: treatment of cylindromas with CO2 laser.

Brooke-Spiegler syndrome is an autosomal dominant inherited disease characterized by the development of multiple trichoepitheliomas and cylindromas. We report two patients with Brooke-Spiegler syndrome whose scalp cylindromas were treated with a high-energy continous wave CO2 laser after debulking with bipolar scissors in one of the patients. The special feature in our report is the coexistence of three adnexal skin neoplasms in one case, and the close association of cylindroma and eccrine spiradenoma in the same lesion. We also support that debulking with bipolar scissors prior to laser therapy can be a technique of great benefit in large tumors.

Reliability of standardized echography before vitreoretinal surgery for proliferative diabetic retinopathy.

Standardized A- and B-scan echography was performed in 45 patients (68 eyes) affected by proliferative diabetic retinopathy, before vitreoretinal surgery, to evaluate the percentage of agreement between the ultrasonographic data and the surgical findings. The agreement was quite good for tractional retinal detachment (93%) and for posterior vitreous detachment (91%). The main cause of failure was the presence of vitreoschisis, while preretinal and vitreal membranes can easily be detected.

Echographic study of the vitreoretinal interface in giant retinal tears.

Vitreous and vitreoretinal interface changes were evaluated in giant retinal tears in order to plan surgical approach. Vitreal sineresis was evident in patients affected by primary giant retinal tears; in patients affected by retinal disinsertion the vitreous was adherent to the edge of the posterior flap, and in giant retinal tears secondary to IOL implantation, posterior vitreous detachment but no sineresis was displayed.

Standardized echography, pattern electroretinography and visual-evoked potential and automated perimetry in the early diagnosis of Graves' neuropathy.

Twenty-four patients (47 eyes) affected by Graves' disease were enrolled to evaluate the role of standardized echography, pattern electroretinogram (P-ERG), visual evoked potentials (P-VEPs) and automated perimetry in the early diagnosis of the compressive optic neuropathy (CON). The P-ERG amplitude reduction was the most sensitive parameter to demonstrate an early impairment of the optic nerve (ON) function. We found a significant negative correlation between the ON diameter and the P-ERG amplitude. VEPs responses were also altered, but their ability in detecting an early ON damage was less sensitive and specific than P-ERG. The visual field damage was often aspecific and delayed with respect to electrophysiological alterations.

Idiopathic plantar hidradenitis.

We describe four cases of idiopathic plantar hidradenitis (IPH), a form of neutrophilic eccrine hidradenitis (NEH) localized on the feet of otherwise healthy young persons. Our patients were all males, 8-17 years old, in apparently good health, but presenting erythematous painful papules, plaques and nodules on the soles. They were afebrile and there were no constitutional symptoms. The differential diagnoses included erythema nodosum, vasculitis and traumatic plantar urticaria. The histological features of IPH are similar to those of NEH, except for the absence of syringosquamous metaplasia. The patients were treated with topical steroids and oral NSAID, with total resolution of the lesions in 1 or 2 weeks.

Responses to chromatic and luminance contrast in glaucoma: a psychophysical and electrophysiological study.

Increasing anatomical evidence indicates that large retinal ganglion cells (M-cells) are preferentially damaged in primary open angle glaucoma (OAG), while the smaller ganglion cells (P-cells) are relatively spared. In 13 patients with defined OAG and modest visual field defects, we evaluated the responses to stimuli that are expected to involve primarily the function of the P-pathway and compared them with those of control subjects. The psychophysical contrast sensitivity (CS), the PERG and the VEPs were measured for red-green gratings of pure chromatic contrast, as well as yellow-black gratings of pure luminance contrast. As compared with controls, OAG patients had reduced CS for both luminance and chromatic contrast stimuli by about 6 dB. PERGs and VEPs to luminance stimuli were little affected, whereas those to chromatic stimuli were both reduced in amplitude and delayed. These results indicate that visual dysfunction in glaucoma is not selective for the M-pathway, and that responses to equiluminant colour-contrast stimuli may be of diagnostic value.

[Typing of human papillomavirus (HPV) in the male and female genitalia]. / Estudo do tipo de papilomavírus humano (HPV) no aparelho genital feminino e masculino.

Diagnosis and typing of HPV was started in November 1990 at the Virology department of the National Health Institute, Lisbon. Eighty one samples from 70 patients were studied, 57 from women observed at the cervico-vulvar out patient unit of the Gynecology department of Sta Maria Hospital, Lisbon, and 13 from male contacts of some of these women. Forty nine women (86%) had colposcopic, histological or cytological evidence of HPV infection. Eleven men (85%) have clinical signs of infection and 2 (15%) had had sexual intercourse with infected partners with clinical evidence of infection. HPV DNA was detected in 43 patients (61%). Forty two were infected with HPV 6/11 (98%) and one with HPV 16 (2%). These preliminary results suggest that genital infection by HPV must also be considered a public health problem in the field of sexually transmitted diseases in Portugal.

[Regional mucocutaneous plasmacytosis]. / Plasmocitose muco-cutânea regional.

The case of a 48 year old woman is reported with confluent nodules on the base of her nose and adjacent infraorbital areas, isolated ones on her face, and endotracheal masses with severe lumen reduction. Histologically the dermis and chorion of the nasal and tracheal mucosa were infiltrated by normal looking plasma cells. There was an increased ESR and a polyclonal gammapathy but no radiological evidence of skeletal involvement, bone marrow infiltration by atypical plasma cells or Bence Jones proteinuria. Cobalt-therapy was rapidly effective in reducing the endotracheal masses. The skin lesions were unaffected by roentgen-therapy and by several chemotherapeutic schedules. Laboratory values almost reverted to normal and her general condition has been satisfactory for the last two years. The differential diagnosis with multiple myeloma and extramedullary plasmocytoma was discussed and the condition classified as chronic, probably benign, mucocutaneous plasma cell infiltration.