Systemic amyloidosis with an exceptional cutaneous presentation.

Alopecia and nail distrophy are rare signs of systemic amyloidosis. We present a case with both manifestations and give a brief review of the cutaneous signs of this disease. A biopsy of affected or unaffected skin may provide the diagnosis.

Paniculitis facticia y síndrome de Löfgren inducidos por silicona: a propósito de un caso clínico / Facticial panniculitis and Löfgren's syndrome: a case

Se presenta el caso clínico de una paciente que ingresó en nuestro servicio por clínica de poliartralgias, fiebre y lesiones cutáneas que afectaban a la región glútea y pretibial. Refería como antecedente la aplicación de inyecciones de silicona líquida en los glúteos con fines estéticos 3 años antes. Se realizó una biopsia cutánea de las lesiones en la región glútea, cuyo estudio anatomopatológico fue compatible con paniculitis facticia por silicona, así como de la región pretibial, que fueron compatibles con eritema nudoso. La radiografía de tórax y la tomografía torácica mostraron adenopatías hiliares bilaterales y en la biopsia transbronquial se evidenció un componente inflamatorio granulomatoso. La evolución fue satisfactoria con reposo y antiinflamatorios no esteroideos, por lo que no fue necesaria la extracción de la silicona (AU)

We herein report a patient who came to the hospital because of a polyarticular joint pain, fever and cutaneous lesions. She had silicone implants in her buttocks, a surgery performed 3 years before. We made a biopsy of the skin of the buttocks (facticial panniculitis due to silicone) and of the pretibial surface of the inferior extremities (erythema nodosum). A chest X- ray and a CT scan revealed bilateral hiliar lymphadenopathy, and a transbronquial biopsy showed granulomatous inflammation. She had a good response to rest and anti-inflammatory drugs, so the removal of the silicone implants has not been necessary yet (AU)

Facticial panniculitis and Löfgren's syndrome: a case.

We herein report a patient who came to the hospital because of a polyarticular joint pain, fever and cutaneous lesions. She had silicone implants in her buttocks, a surgery performed 3 years before. We made a biopsy of the skin of the buttocks (facticial panniculitis due to silicone) and of the pretibial surface of the inferior extremities (erythema nodosum). A chest X- ray and a CT scan revealed bilateral hiliar lymphadenopathy, and a transbronquial biopsy showed granulomatous inflammation. She had a good response to rest and anti-inflammatory drugs, so the removal of the silicone implants has not been necessary yet.

Three cases of cutaneous phaeohyphomycosis by Exophiala jeanselmei.

Cutaneous phaeohyphomycosis is a rare opportunistic fungal disease, knowledge of which is important because of the increase in organ transplantation, aggressive treatments for malignancies, and chronic use of corticosteroids. We report 3 cases of cutaneous phaeohyphomycosis: two patients treated with oral corticosteroids and one elderly woman with multiple hospitalizations. They showed several different clinical appearances. Histopathologically, the fungal infection affected the dermis and subcutis in all cases and in one of them, also the epidermis. Exophiala jeanselmei was isolated from the purulent exudate in the three cases. Good response was obtained with surgical and antifungal treatment.

Concurrent Sweet's syndrome and leukemia cutis in patients with myeloid disorders.

A 74-year-old woman with chronic auricular fibrillation, arterial hypertension, hypercholesterolemia, ischemic cardiopathy, and peripheral arteriopathy presented with purpuric lesions on the lower limbs (Fig. 1) and, to a lesser extent, on the anterior area of the chest. The mucous membranes were not affected. In 1989, she was diagnosed with anemia that evolved until 1998, when a bone marrow biopsy revealed a myelodysplastic syndrome unclassified in French-American-British Group (FAB). The patient has required periodic transfusions since February 1999. A skin biopsy of the purpuric lesions revealed a leukocytoclastic vasculitis; the lesions cleared with topical corticosteroid treatment. In May 1999, the patient presented with inflammatory and painful lesions localized on the vulva (Fig. 2), which had evolved over several days, without fever. No lesions were observed in other locations. A cutaneous biopsy showed an intense dermal edema and a diffuse and polymorphous dermal infiltrate involving the follicular structures. Exocytosis, spongiosis, and mucin deposits, demonstrated by Alcian blue stain, were observed in the follicular epithelium. Mature neutrophils were predominant in the dermal infiltrate, but a small number of eosinophils and immature cells were also present (Fig. 3). The myelogenous origin of the immature lining cells was further confirmed by positive staining of intracytoplasmic granules with naphthol-ASD chloroacetate sterase (Leder's stain). Vasculitis was not observed. Routine laboratory tests revealed 3030 leukocytes/mm(3) (60% neutrophils), a hemoglobin level of 8.4 g/dL, and 92,000 platelets/mm(3). Treatment with 30 mg/day of prednisone was started, and the lesions cleared slowly within 4 weeks. A new bone marrow biopsy in September 1999 showed a similar appearance to that taken in 1998. The patient died in January 2000 as a result of pneumonia with cardiac and respiratory failure. A 66-year-old man presented with a febrile syndrome that had evolved over 5 days, and painful and pruritic cutaneous lesions on the face and posterior neck (Fig. 4). Three months before, the patient was diagnosed with chronic myelogenous leukemia in acceleration phase. Examination revealed an edematous and erythematous face with pustular lesions on the surface, also involving the neck and the upper part of the back. The histopathologic examination revealed an intense edema and abscesses in the dermis. The infiltrate of these lesions was composed of mature neutrophils with the presence of abundant immature cells with a myelogenous aspect (Fig. 5). Analytical studies revealed 26,130 leukocytes/mm(3) (42% blasts). No specific treatment for Sweet's syndrome was administered and the lesions showed an improvement within 5 days. Eight days after admission, the patient died as a result of acute hemorrhage, before treatment for leukemia was initiated.

Eyelash length in HIV-infected patients.

Acquired trichomegaly of the eyelashes in HIV-infected patients usually appears at the late stage of HIV infection. Eyelash length was measured in a series of 204 HIV patients, and no correlation with CD4 cell count, viral load, Centers for Disease Control and Prevention category, and AIDS case criteria was established. Our data support the finding that eyelash trichomegaly is currently uncommon in HIV-infected patients, perhaps because of antiretroviral therapy or an improvement in their immune situation.

Exantema generalizado granulomatoso durante un tratamiento con factor estimulante de colonias de granulocitos / Generalized granulomatous rash during treatment with granulocyte-macrophage colony-stimulating factors

Los factores estimulantes de colonias de granulocitos (G-CSF) y de granulocitos y macrófagos (GM-CSF) son factores de crecimiento hematopoyéticos recombinantes que pertenecen a la familia de los factores estimulantes de colonias. Se han descrito diversas toxicodermias relacionadas con estos dos fármacos, como empeoramiento de dermatosis previas, reacciones cutáneas locales en el punto de inyección y erupciones cutáneas diseminadas. En algunos casos de erupciones cutáneas diseminadas inducidas por G-CSF o GM-CSF se ha descrito la presencia de histiocitos de tamaño mediano o grande con citoplasma espumoso y nucleolo prominente. Se presenta el caso de un paciente que desarrolló un exantema granulomatoso asociado al tratamiento con G-CSF. Aunque esta reacción cutánea no se había descrito previamente, parece posible que la estimulación de la línea de macrófagos y monocitos pudiera producir una reacción granulomatosa (AU)

Venous lakes: a report of 32 cases treated by carbon dioxide laser vaporization.

Venous lakes (VL) are acquired venous ectasies of the superficial dermal venules, usually observed in older people. Thirty-two adult patients with VL in several localizations were treated by carbon dioxide laser vaporization. Two passes were performed to each lesion within the same session. A continuous and defocused mode, with a power density of 5 W/cm2, was used in the first pass, and a continuous focused mode with the same power density was in the second pass. Photographic controls were performed before and after treatment. VLs were removed, and the smooth surface of the treated area was re-established by a single laser session. Only one case recurred after the treatment. No significant secondary effects were observed. Carbon dioxide laser is a good and safe method for treatment of skin VL. With adequate cautions, excellent cosmetic results can be obtained with a single session of treatment.

Indinavir-induced retinoid-like effects: incidence, clinical features and management.

Since 1998, many cases of antiretroviral therapy-related paronychia of the toes or fingers and ingrown toenails have been reported. Most of them were related to indinavir. Other indinavir-induced mucocutaneous disorders resembling the adverse effects of systemic retinoid therapy have also been reported. Although there is some uncertainty in the literature regarding a cause-effect relationship, results of several epidemiological and in vitro studies, together with cumulated clinical experience leave no doubt that indinavir causes a retinoid-like effect and nail alterations. Indeed, indinavir is the only antiretroviral drug that produces these disorders, although ritonavir may enhance indinavir-induced retinoid-like effects through pharmacokinetic interactions leading to increased plasma indinavir concentrations. Approximately 30% of patients receiving indinavir show two or more retinoid-like manifestations and 4-9% develop paronychia. These adverse effects are not related to other epidemiological variables such as the patient's sex, age or other risk factors or immune status. They seem to be exposure dependent and, therefore, largely dose-dependent. Chronic paronychia is considered generally to be caused by contact irritants and candidal infection. Nevertheless, indinavir is currently the most frequent cause of chronic or recurrent paronychia in HIV-infected patients. In addition, retinoid-like manifestations such as cutaneous xerosis and cheilitis are frequent mucocutaneous adverse effects related to indinavir. The exact mechanism of indinavir-induced retinoid-like effects is unclear. Hypotheses for pathogenesis include interference with retinoid metabolism by enhancing the retinoic acid signalling pathway, or by increasing retinoic acid synthesis, or by reducing cytochrome p450-mediated retinoic acid oxidative metabolism. Replacement of therapy by an antiretroviral regimen not containing indinavir, while retaining other protease inhibitors and lamivudine, resolves retinoid-like manifestations without recurrences.

Membranous fat necrosis: a non-specific histological finding.

Two patients presented with nodular lesions on their lower limbs. Histologically, the dermis, in one case, and the panniculus, in the other, displayed pseudocystic lesions delimited by a serpiginous membranous structure showing the staining characteristics of ceroid. One patient had sclerosing panniculitis while the other had a traumatic panniculitis.These cases illustrate that membranous fat necrosis is a non-specific histological finding and that multiple processes are involved in its etiopathogenesis.