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J Fraga

Publications and source records attributed to J Fraga.

At least 55 records · Page 3Linked to original sources
PubMed1999

Pleomorphic sclerotic fibroma.

We report the clinical and histologic features of 2 cutaneous tumors demonstrating many of the usual features of sclerotic fibroma, an entity that is well described in the literature. Our cases differed from the usual form of sclerotic fibroma in that they demonstrated marked focal cellular pleomorphism without increased mitotic activity. We suggest the term pleomorphic sclerotic fibroma for these lesions.

Actins↗
PubMed1998

Follicular pustulous granuloma annulare.

We report a 73-year-old woman presenting with recurrent eruptions of generalized follicular pustules. Histological examination revealed several palisading necrobiotic granulomas with mucin deposits, with a perifollicular distribution. A dense neutrophilic infiltrate in the upper portion of affected follicular structures gave rise to pustulous lesions. Scaly papules and pseudovesiculous lesions over the palms with deeper necrobiotic granulomas involving sweat glands and epidermal perforation coexisted in some of the eruptions with generalized pustules. We propose the term follicular pustulous granuloma annulare for this peculiar form of granuloma annulare, which widens the clinicopathological spectrum of presentation of this disease.

Aged↗
PubMed1998

Cutaneous necrosis by cold agglutinins associated with glomeruloid reactive angioendotheliomatosis.

The cold agglutinin syndrome is a haemolytic disorder that can cause skin lesions, mainly on the acral areas, with acrocyanosis being the most frequent manifestation. Cutaneous necrosis due to cold agglutinins is very rare. Reactive angioendotheliomatosis (RAE) is an uncommon condition that exclusively affects the skin, characterized by a hyperplasia of endothelial cells and pericytes that can result in the formation of glomeruloid structures. The association of cold agglutinin syndrome with glomeruloid RAE has not been previously described. We report a 70-year-old man diagnosed as having a B-cell low-grade non-Hodgkin's lymphoma. He had two episodes of cutaneous necrosis in acral areas which were related to exposure to cold and due to IgM anti-I(T) cold agglutinins. Biopsy specimens showed vessel proliferations composed of dilated vascular spaces in the dermis and subcutis. Some vessel lumina were partially occluded by eosinophilic thrombi of fibrin and erythrocytes. Numerous closely packed capillaries were observed within pre-existing dilated vessels. This intravascular proliferation of capillaries displayed a glomeruloid pattern. We emphasize the possible presence of a cold agglutinin syndrome in patients with skin necrosis and findings of RAE with a glomeruloid pattern. Cold agglutinaemia may cause these distinctive histological changes.

Aged↗
PubMed1998

Dermal infiltrate of enlarged macrophages in patients receiving chemotherapy.

A histologically distinct maculopapular eruption has been associated with the use of recombinant forms of granulocyte and granulocyte-macrophage colony-stimulating factors (GCSF and GMCSF). One of the most distinctive features was an increase in the number and the size of dermal macrophages, which was proposed as a clue to diagnosis of these cytokine-induced dermatoses. We describe 8 patients who received chemotherapy and who developed a maculopapular eruption, of which histological examination showed an increase in the number and the size of dermal macrophages. Three patients had also received GM-CSF or G-CSF, before the onset of rash, but 5 patients had not. In conclusion, an increase in the number and the size of dermal macrophages is not specific for the reaction to GM-CSF or G-CSF, and may also be observed in patients receiving chemotherapy.

Adult↗
PubMed1998

HIV-Associated eosinophilic folliculitis and follicular mucinosis.

The term HIV-associated eosinophilic folliculitis (EF) designates an idiopathic dermatitis that appears in HIV-infected patients with different clinical manifestations but with a distinctive histological feature characterized by a predominantly eosinophilic infiltrate in the follicular infundibula. On the other side, follicular mucinosis (FM) is a reaction pattern in the follicular epithelium, characterized by a mucinous degeneration of the outer sheath of follicles and sebaceous glands. It has been described in association with a variety of unrelated conditions. We report 2 HIV-infected patients with a pruritic papular eruption. Histopathological study revealed the coexistence of EF and FM. To our knowledge, this is the first report that describes this association. The possible relationship between these two entities is discussed.

Adult↗
PubMed1997

Chemotherapy-induced eccrine squamous syringometaplasia. A distinctive eruption in patients receiving hematopoietic progenitor cells.

BACKGROUND: Eccrine squamous syringometaplasia (ESS) has been associated with characteristic clinical eruption in patients receiving chemotherapy. It has been suggested as a diagnostic clue in the diagnosis of chemotherapy-induced reactions vs acute graft-vs-host disease, as well as other drug reactions. We identified 10 cases of ESS in patients in whom a distinctive clinical eruption developed during or after a pretransplantation conditioning regimen with high-dose chemotherapy. A complete clinical and histologic evaluation was performed in all patients. OBSERVATIONS: All patients developed erythematous and edematous plaques or confluent erythematous macular areas in the axillae and/or groin, with painful areas of well-defined erythema and edema on palms and/or soles in 5 patients. Some discrete papular lesions on the trunk or extremities could also be observed in most patients. The histologic hallmark of the eruption was ESS, with a variable degree of cornification and apoptosis. A vacuolar interface dermatitis and a variable degree of cellular atypica were also consistent findings. CONCLUSIONS: Chemotherapy-induced ESS may be associated with a distinctive clinical eruption and should be considered in the differential diagnosis of erythematous eruptions during or after a pretransplantation conditioning regimen with high-dose chemotherapy.

Adult↗
PubMed1997

Relative impact of HLA phenotype and CD4-CD8 ratios on the clinical expression of hemochromatosis.

Hemochromatosis is a hereditary iron-overload disease linked to HLA. The clinical expression of hemochromatosis is influenced by sex and age. However, other factors must account for the notorious heterogeneity of expression of the disease independent of sex, age, and HLA phenotype. The present study attempts to clarify some of these additional factors based on exhaustive statistical analysis of data collected from 43 selected patients with hemochromatosis. The statistical analysis focused on three groups of variables: the first group included variables reflecting the clinical expression of the disease; the second group represented the biochemical and hematological values at the time of diagnosis; and the third group consisted of the independent variables sex, age, HLA phenotype, and T-cell subset profile, i.e., the percentages and total numbers of CD4+ and CD8+ cells and the CD4-CD8 ratios. The results show that the relative expansion of the two main T-cell subsets, in the context of the HLA phenotype, correlates significantly with the clinical expression of hemochromatosis and the severity of iron overload. The present findings substantiate further the postulate that T cells have a role in the regulation of iron metabolism.

Adult↗
PubMed1997

Perforating granuloma annulare.

BACKGROUND: Perforating granuloma annulare (PGA) is considered an histologic subtype of granuloma annulare (GA) and it is described as a very rare disease, usually of children, affecting the dorsum of the hands. Mechanisms leading to perforation are unknown. Our experience suggested a different clinical presentation, so we decided to review our patients and the cases published. METHODS: We present six cases of PGA and review 52 PGA cases reported in the literature. Data regarding sex, age, time of evolution of disease, clinical features, laboratory findings, treatment, and follow-up were collected. The significance level was determined by the chi 2 or Student t test when appropriate. RESULTS: The prevalence of PGA could be up to 5% of GA. Pustular-like lesions can be found in 26% of cases, and scars in 37%; papular, umbilicated, and crusted lesions being the most common finding. While PGA appears as a single lesion in only 9% of cases, and half of the patients are older than 30 years. In GA 50% of cases present as a single lesion and 80% of patients are younger than 30 years. CONCLUSIONS: PGA is different to GA not only histologically but also clinically. It is a disseminated disease, affecting both children and adults, which is characterized by the presence of multiple papules, most of them umbilicated and/or crusted, and characteristically pustular lesions and scars. Histology suggests that the superficial localization of the necrobiotic granuloma leads to the epidermal perforation. Treatment is disappointing.

Adult↗
PubMed1997

Cutaneous metaplastic synovial cyst in an Ehlers-Danlos patient.

Metaplastic synovial cyst is a recently reorganized entity characterized by a cystic space lined by polygonal cells and villous structures resembling synovium pointing toward the lumen. It usually develops following trauma. We report a 15-year-old girl with Ehlers-Danlos syndrome who developed a nodular lesion of her elbow without previous trauma. Histopathological and immunohistochemical studies yielded results similar to those reported in cases of cutaneous metaplastic synovial cyst. This case appears to be the first one reported of cutaneous metaplastic synovial cyst associated with an Ehlers-Danlos syndrome. Cutaneous fragility and anomalous scarring typical of Ehlers-Danlos syndrome may be related to the development of this pseudocyst. If so, other connective-tissue diseases with increased skin fragility could be susceptible to development of such lesions.

Adolescent↗
PubMed1997

Herpetic syringitis associated with eccrine squamous syringometaplasia in HIV-positive patients.

Herpetic syringitis has been described as a rare manifestation of herpes virus infection in patients with an immunodeficiency, usually secondary to human immunodeficiency virus (HIV) infection. Eccrine squamous syringometaplasia (ESS) is an infrequent alteration of the eccrine duct epithelium reported in association with several conditions, including chronic ulcers, inflammatory processes, and patients receiving chemotherapy. The association of herpetic syringitis with ESS has not been reported before. We identified 3 cases of herpetic syringitis associated with ESS in patients with the acquired immunodeficiency syndrome. In 2 of 3 cases the signs of herpetic syringitis were limited to the metaplastic duct epithelium, but in 1 case there were also herpetic alterations without ESS. The histological features of herpetic infection in HIV-positive patients may be atypical and lack the typical epidermal alterations, observing only an extensive epidermal necrosis. In those cases, the alterations of the eccrine ducts may be a diagnostic clue in the diagnosis of herpetic infection. ESS of the ductal epithelium is probably secondary to the herpetic infection, although it might also stimulate the extension of the herpetic infection. Further studies are needed to elucidate the association of ESS and herpes virus infection.

AIDS-Related Opportunistic Infections↗
PubMed1997

Pseudoxanthoma elasticum-like papillary dermal elastolysis. A report of two cases and review of the literature.

Pseudoxanthoma elasticum-like papillary dermal elastolysis is a peculiar idiopathic elastolytic disorder with cutaneous lesions clinically resembling pseudoxanthoma elasticum with partial or total band-like elastolysis of the papillary dermis histopathologically, and without systemic complications. We here report 2 new cases and review the clinicopathological features of patients with this diagnosis in the literature. The possible pathogenesis of this recently described entity is discussed.

Aged↗
PubMed1996

Leishmaniasis presenting as a dermatomyositis-like eruption in AIDS.

Three patients are described with leishmaniasis and AIDS, with cutaneous lesions mimicking dermatomyositis. Leishmania organisms were observed in great numbers in the dermis of lesional skin biopsy specimens. They were also present inside keratinocytes in all layers of the epidermis in one patient. Skin cultures from all patients and bone marrow culture in patients 1 and 3 revealed Leishmania infantum. Leishmania organisms were also found in nonlesional skin. The absence of proximal symmetric muscle weakness, elevated muscle enzymes, myopathic electromyograms, or characteristic histopathologic and immunologic features of dermatomyositis, and the rapid and complete clearance or marked improvement of the cutaneous lesions after treatment for leishmaniasis, make us consider true dermatomyositis unlikely. We suggest that leishmaniasis be included in the list of diseases capable of inducing a dermatomyositis-like eruption.

AIDS-Related Opportunistic Infections↗