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Biomedical subjects

R M Pujol

Publications and source records attributed to R M Pujol.

At least 19 recordsLinked to original sources
PubMed2001

Postirradiation multiple minute digitate porokeratosis.

BACKGROUND: Development of multiple minute digitate hyperkeratoses (MMDH) after irradiation has been reported previously. The keratotic lesions in these cases were confined within the irradiation field, and histopathological examination disclosed a focal column of parakeratosis (cornoid lamella) arising from an epidermis devoid of granular layer. OBJECTIVE: We describe a 78-year-old woman who developed multiple, discrete, tiny, filiform, keratotic papules on the anterior aspect of the right chest wall, 13 months after postmastectomy cobalt irradiation therapy for mammary infiltrating ductal carcinoma. CONCLUSION: Postirradiation MMDH represents a peculiar radiation-induced disorder that we believe should be distinguished from other cases of MMDH and included within the spectrum of porokeratosis.

Aged↗
PubMed2001

Hypertrichosis lanuginosa acquisita preceding extraskeletal Ewing's sarcoma.

A 62-year-old woman with acquired hypertrichosis lanuginosa as a paraneoplastic presenting sign of an extraskeletal Ewing's sarcoma is described. Despite initial comprehensive screening to rule out an associated malignancy, a definitive diagnosis of sarcoma was established only 1 year after the onset of the cutaneous symptoms. To the best of our knowledge, this is the first report of hypertrichosis lanuginosa acquisita associated with a soft tissue sarcoma. Our observation expands the spectrum of malignancies associated with this uncommon paraneoplastic disorder.

Female↗
PubMed2001

Anetoderma associated with antiphospholipid antibodies: case report and review of the literature.

The association of primary anetoderma (PA) with antiphospholipid antibodies (APAs; with or without criteria of primary antiphospholipid syndrome) has been observed repeatedly and a possible pathogenic significance of this asssociation has been hypothesized. We report the case of a 21-year-old-woman who developed anetodermic lesions on her upper trunk and arms. The presence of APAs was demonstrated, but no diagnostic criteria of systemic lupus erythematosus were present. A review of the literature disclosed 20 previously reported patients presenting this peculiar association. Thrombotic phenomena, represented by spontaneous abortions or venous thrombosis, are a frequent complication in this setting. Taking these observations into account a systematic search for APAs in patients with PA seems advisable.

Adult↗
PubMed2000

Erythema nodosum: etiologic and predictive factors in a defined population.

OBJECTIVE: To examine the frequency and features of erythema nodosum (EN), establish disease associations, and identify the optimal set of predictors for the occurrence of secondary EN. METHODS: We performed a retrospective study of an unselected population of patients 14 years and older with biopsy-proven EN diagnosed at a referral hospital between 1988 and 1997. Patients were classified as having either idiopathic EN or EN secondary to other diseases if the skin nodules occurred in the context of a well-defined disease, or if there was a precipitating event in close temporal proximity to the onset of EN. RESULTS: One hundred six patients (82 women) were diagnosed as having biopsy-proven EN. At the time of diagnosis, no precipitating events or underlying diseases were identified in 36.8% of patients. Sarcoidosis and nonstreptococcal upper respiratory tract infections (URI) were the most common conditions associated with secondary EN. Only 1 of 35 patients with an initial diagnosis of idiopathic EN and a followup of at least 1 year was finally diagnosed as having secondary EN. The best predictive model of secondary EN included an abnormal results on a chest radiograph, a previous history of nonstreptococcal URI, and a significant change in antistreptolysin O (ASO) titer in 2 consecutive determinations performed in a 2-4-week interval. Also, the presence of peripheral synovitis, a positive tuberculin skin test, and a history of diarrhea suggested the presence of secondary EN. This model showed high sensitivity and specificity. CONCLUSION: Idiopathic EN is common. A basic procedure including careful medical history-taking, a physical examination for peripheral synovitis, 2 consecutive ASO determinations, a tuberculin skin test, and chest radiography may be sufficient to diagnose EN.

Biopsy↗
PubMed2000

Invisible mycosis fungoides: A diagnostic challenge.

We describe a 76-year-old woman who presented persistent generalized pruritus as the only cutaneous manifestation of a cutaneous T-cell lymphoma (mycosis fungoides). No cutaneous lesions were observed throughout the patient's course. Skin biopsies obtained from normal-looking pruritic skin revealed a discrete perivascular lymphocytic infiltrate in the upper dermis and focal intraepidermal clusters of atypical lymphoid cells (Pautrier's microabscesses). PCR analysis of TCR-gamma gene disclosed a monoclonal T-cell rearrangement. Sequencing of the PCR monoclonal product identified the J(8)V(2)C(2) TCR gene rearrangement. This observation illustrates the existence of a peculiar and exceedingly rare form of mycosis fungoides characterized only by persistent pruritus unresponsive to several therapeutic approaches. The diagnostic difficulties of this rare variant are stressed.

Aged↗
PubMed2000

HIV-1 protease inhibitor-associated partial lipodystrophy: clinicopathologic review of 14 cases.

BACKGROUND: A novel type of acquired partial lipodystrophy resulting from chronic treatment with HIV-1 protease inhibitor drugs has recently been described. OBJECTIVE: We studied the clinical and histopathologic features of a series of patients with HIV-1 protease inhibitor-associated lipodystrophy to evaluate the frequency of associated abnormalities. METHODS: The study group consisted of 14 consecutive HIV-infected patients receiving treatment with HIV-1 protease inhibitors, who experienced partial lipodystrophy. Clinical (including anthropometric data) and histopathologic findings, as well as biochemical and virologic data, were evaluated. RESULTS: A significant loss of fat in the face and extremities was associated with fat deposition on the abdomen, breast, and dorsocervical fat pad. Central obesity was frequently present. Histopathologic features disclosed a peculiar type of involutional lipodystrophy. Hypertriglyceridemia was detected in 78.5% of patients. Low serum levels of cholesterol-high-density lipoprotein and high cholesterol-very-low-density lipoprotein were noted. Hyperglycemia, hypercholesterolemia, or hyperinsulinemia were occasionally detected. CONCLUSION: HIV-1 protease inhibitor-associated lipodystrophy represents a new entity with peculiar clinical and histopathologic features. Metabolic associated abnormalities may imply a risk of future atherogenic complications.

Adult↗
PubMed2000

Generalized pruritic eruption with suprabasal acantholysis preceeding the development of bullous pemphigoid.

We report a patient who presented with a papular pruritic eruption of a 3-month duration that histologically showed suprabasal acantholysis accompanied of an eosinophilic inflammatory infiltrate that was consistent with the diagnosis of Grover's disease. Later, erythematous plaques and vesicles appeared which showed a histopathological pattern of eosinophilic spongiosis. The direct immunofluorescence (DIF) study showed lineal IgG and C'3 at the dermal epidermal junction which was consistent with the diagnosis of bullous pemphigoid. No anti-intercellular deposits of immunoglobulin G (IgG) or C'3 were observed. We consider that suprabasal acantholysis may represent the early phase of bullous pemphigoid.

Acantholysis↗
PubMed2000

Cutaneous infections due to nontuberculous mycobacteria: histopathological review of 28 cases. Comparative study between lesions observed in immunosuppressed patients and normal hosts.

To evaluate the histopathological features observed in patients with cutaneous infections due to nontuberculous mycobacteria (NTM) and to compare the histopathological patterns observed in immunosuppressed patients and normal hosts. Twenty-eight biopsy specimens corresponding to 27 patients with cutaneous infections due to NTM were reviewed. Eighteen biopsies corresponded to normal hosts (14 Mycobacterium marinum, 2 Mycobacterium chelonae, 1 Mycobacterium terrae and 1 Mycobacterium gordonae) and 10 biopsy specimens were obtained from 9 immunosuppressed patients (3 Mycobacterium chelonae, one of which had two biopsies, 1 Mycobacterium abscessus, 2 Mycobacterium kansasii, 1 Mycobacterium marinum, 1 Mycobacterium avium complex and 1 Mycobacterium simiae). A panel of histopathological features was evaluated by two independent observers in each biopsy specimen. Epidermal changes (acanthosis, pseudoepitheliomatous hyperplasia, exocytosis) were mainly observed in M. marinum infections. In immunosuppressed patients the infiltrate tended to be deeper, involving the subcutaneous tissue (100%) with a more diffuse distribution and constant abscess formation. A marked granulomatous inflammatory reaction was observed in 83% of immunocompetent and in 60% of immunosuppressed patients. In immunosuppressed patients a relationship between the chronic evolution of the disease and granuloma formation was demonstrated. A diffuse infiltrate of histiocytes with occasionally foamy appearance was noted in three biopsy specimens from three patients with AIDS. Acute and chronic panniculitis was detected in 8 biopsy specimens. In one biopsy (M. chelonae) an acute suppurative folliculitis was observed. Different histopathological patterns can be noted in biopsy specimens from cutaneous nontuberculous mycobacterial infections. The evolution of the disease and the immunologic status of the host may explain this spectrum of morphological changes. Tuberculoid, palisading and sarcoid-like granulomas, a diffuse infiltrate of histiocytic foamy cells, acute and chronic panniculitis, non-specific chronic inflammation, cutaneous abscesses, suppurative granulomas and necrotizing folliculitis can be detected. Suppurative granulomas are the most characteristic feature in skin biopsy specimens from cutaneous NTM infections. Some histopathological patterns seem more prevalent in immunosuppressed patients.

Acquired Immunodeficiency Syndrome↗
PubMed2000

Graves' disease presenting as localized myxoedematous infiltration in a smallpox vaccination scar.

We describe a 54-year-old woman with diffuse myxoedematous infiltration at the site of a smallpox vaccination scar as the presenting symptom of Graves' disease. Associated features included acute ocular symptoms (vascular congestion of the sclera, epiphora and blurred vision) and transient erythema on both shins. However, there were no signs of pretibial myxoedema. A number of neoplastic, inflammatory and systemic diseases may localize to scar tissue in skin, including at smallpox vaccination sites, but this case demonstrates the unusual occurrence of myxoedematous infiltration at such a site and illustrates a most atypical cutaneous presentation of Graves'disease.

Cicatrix↗
PubMed1999

Subcutaneous adipocyte apoptosis in HIV-1 protease inhibitor-associated lipodystrophy.

BACKGROUND: Inhibitors of HIV-1 protease produce a rapid decrease in plasma HIV-1 RNA, with concomitant immune reconstitution. However, severe metabolic side effects together with a previously unseen form of lipodystrophy have been associated with long-term use of protease-inhibitor therapy. The pathogenic mechanisms underlying HIV-1 protease inhibitor-associated lipodystrophy are still largely unknown. METHODS: Fourteen HIV-infected patients with HIV-1 protease inhibitor-associated lipodystrophy had a biopsy of subcutaneous fat performed in the antero-lateral aspect of the right leg. The samples were submitted for standard pathologic study together with a careful search for adipocyte apoptosis. Apoptosis was assessed by the terminal deoxynucleotidyl transferase dUTP-digoxigenin nick end labelling (TUNEL) method, using the ApopTag kit (Oncor, Gaithersburg, Maryland, USA). The procedure was performed between three and five times for each sample. Appropriate positive and negative controls were used. Controls which were subcutaneous fat biopsies from patients with untreated melanoma were also examined for the presence of apoptosis. RESULTS: Fourteen HIV-infected patients with a mean exposure to HIV-1 protease inhibitors of 12.6 +/- 3.7 months (range: 6-21 months), developed the characteristic features of HIV-1 protease inhibitor-associated lipodystrophy. All but one patient had an abnormal waist:hip ratio, and they all exhibited an abnormal serum lipid profile. Pathologically, subcutaneous fat atrophy was a constant feature, along with focal lipogranuloma formation and vascular proliferation. One of the eleven assessable biopsy samples was negative for the presence of apoptosis, six showed focally positive apoptotic cells, and the remaining four biopsies demonstrated moderate positivity. Apoptotic changes were also detected in endothelial cells. Apoptotic changes were more pronounced in patients with higher increases in CD4 and CD8 counts, and in those with a greater decay in plasma viral load. CONCLUSIONS: Subcutaneous adipocyte apoptosis occurs in lipoatrophic areas of patients with HIV-1 protease inhibitor-associated lipodystrophy.

Adipocytes↗
PubMed1999

Grover's disease in patients with chronic renal failure receiving hemodialysis: clinicopathologic review of 4 cases.

In 4 patients undergoing hemodialysis for chronic renal failure, a transient or persistent, papular and keratotic eruption developed on the trunk and arms. Histologic examination disclosed focal acantholysis with dyskeratosis. The lesions were clinically and histologically indistinguishable from those of Grover's disease. A possible association with Grover's disease and chronic renal failure and/or hemodialysis is postulated. Possible implicated pathogenic mechanisms are discussed. We suggest that Grover's disease should be included in the differential diagnosis of cutaneous eruptions in patients with chronic renal failure.

Adult↗
PubMed1999

Cutaneous granulomatous lesions in common variable immunodeficiency: complete resolution after intravenous immunoglobulins.

A 64-year-old man with common variable immunodeficiency developed a persistent papulonodular ulcerative eruption on the right leg. Histopathological examination disclosed a chronic inflammatory infiltrate with central necrosis and palisading granuloma. Repeated microbiological (bacteriological, mycological and mycobacteriological) studies failed to isolate any microorganism. After treatment with intravenous immunoglobulins, a progressive resolution of the skin lesions was observed with a complete clearing after 10 months. Clinicopathological features and therapeutic approaches of sterile granulomatous lesions associated with primary immunodeficiencies are reviewed.

Common Variable Immunodeficiency↗
PubMed1999

Benign lymphoepithelial tumor of the skin ("cutaneous lymphadenoma").

"Benign lymphoepithelial tumor of the skin" or "cutaneous lymphadenoma" is a distinctive benign adnexal tumor presenting a characteristic combination of lobules of epithelial basaloid cells with a peculiar histopathological pattern and a dense intraepithelial T-cell lymphoid and histiocytic population. We report an additional example of this peculiar neoplasm. In addition to the characteristic histopathological features, focal areas showing unequivocal follicular differentiation were observed at the periphery of the tumor. In agreement with other authors we consider that this neoplasm should be included within the spectrum of neoplasms of pilosebaceous origin. Nevertheless, we consider that the original and simple concept of "benign lymphoepithelial tumor of the skin" seems more suitable and illustrative than the more widely used term of "cutaneous lymphadenoma" to define this rare benign adnexal neoplasm.

Adenolymphoma↗
PubMed1998

Persistent supravenous erythematous eruption: a rare local complication of intravenous 5-fluorouracil therapy.

We describe a 47-year-old man who developed a linear serpentine erythematous eruption, overlying the superficial veins on both arms, after treatment with intravenous 5-fluorouracil. No evidence of superficial phlebitis or extravasation were observed. Histologically, a prominent vacuolar alteration of basal cells, necrotic keratinocytes, pigment incontinence, and a perivascular lymphocytic infiltrate in the upper dermis were observed. In a review of the literature we encountered only 5 reports of a similar clinicopathologic picture. The term "persistent supravenous erythematous eruption" (PSEE) seems suitable to describe this rare and peculiar cutaneous eruption that develops after intravenous cytotoxic drug treatment.

Antimetabolites, Antineoplastic↗