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Biomedical subjects

François Skowron

Publications and source records attributed to François Skowron.

6 recordsLinked to original sources
PubMed2006

Multiple adult xanthogranuloma: case report and literature review.

BACKGROUND: Non-Langerhans cell histiocytoses form a heterogeneous group defined by the proliferation of cells with macrophage characteristics. Diagnosis is easy in typical cases but becomes more complex in unusual forms. CASE REPORT: We report the case of a 53-year-old patient who presented multiple brown-to-yellowish papules and nodules of the trunk, neck, and head evolving for 6 months. No visceral involvement was found. Histopathological examination revealed histiocytic proliferation with features of secondary xanthomization with the presence of giant foamy multinucleated Touton cells. One year later, all lesions cleared spontaneously. Based on the clinical presentation and evolution and on the immunohistologic data, we retain the diagnosis of adult xanthogranuloma (AXG) in a diffuse shape. DISCUSSION: Multiple AXG is a rare entity (15 cases reported since 1963) with a stereotypic presentation. It is important to recognize because of its good prognosis and the absence of visceral involvement therefore requiring no investigations or aggressive treatments. This case is interesting because of the complete and spontaneous healing of all the lesions within 20 months.

Antigens, CD↗
PubMed2005

Elastosis perforans serpiginosa associated with pseudo-pseudoxanthoma elasticum during treatment of Wilson's disease with penicillamine.

BACKGROUND: Elastosis perforans serpiginosa (EPS) is a reactive perforating dermatosis characterized by the elimination of abnormal elastic fibers from the upper dermis through the epidermis. In a few cases, it occurs as a side effect of treatment by D-penicillamine (DPA). The first case of EPS induced by DPA was described in 1972 in a patient treated for Wilson's disease. Subsequently, cutaneous changes resembling pseudoxanthoma elasticum (PXE) were observed in patients treated with DPA and were reported as pseudo-PXE. CASE REPORT: We report herein the clinical, pathological and ultrastructural study of 2 new cases of DPA-induced EPS and pseudo-PXE. These patients had been treated for Wilson's disease since 14 and 16 years, respectively. Characteristic abnormal elastic fibers were found on histopathological examination of both EPS and pseudo-PXE skin and confirmed by an ultrastructural study. There was no ABCC6 mutation. DISCUSSION: Penicillamine is able to induce widespread, cutaneous and systemic, elastic fiber damage. Our patients present typical features of DPA-induced elastosis, presenting as EPS and pseudo-PXE. ABCC6 mutation is associated with PXE and, as expected, it was absent in our cases of pseudo-PXE. This elastopathy has been related to morphologic changes in elastic fibers secondary to prolonged therapy in most cases. DPA may interfere with elastin cross-linking through inhibition of the enzyme lysyl oxidase, or by formation of complexes with the cross-linked precursors, impairing a normal maturation of elastic fibers. However, no fatal complication of DPA-induced elastopathy has been reported so far. An improvement of the cutaneous lesions is expected after the drug discontinuation.

Adult↗
PubMed2004

Pseudosclerodermatous panniculitis after irradiation and bronchiolitis obliterans organizing pneumonia: simultaneous onset suggesting a common origin.

Due to the technical evolution of radiation therapy of breast carcinoma, new manifestations occur as side effects. Newly described unexpected cutaneous events are morphea of the breast and pseudosclerodermatous panniculitis after irradiation (PPAI). They appear as specific consequences of megavoltage radiation. Radiotherapy-induced bronchiolitis obliterans organizing pneumonia (R-BOOP) is one of the pulmonary manifestations due to recent advances in radiotherapy of breast carcinoma. We report a case of PPAI and R-BOOP of simultaneous onset in a 60-year-old woman 6 months after megavoltage radiation therapy for breast carcinoma. We also noted an improvement of both unexpected reactions with systemic corticosteroid therapy and a common relapse when tapering the treatment. Only eight cases of PPAI have previously been reported. We believe that this entity is often underdiagnosed. The symmetrical onset and parallel evolution suggest a common origin for PPAI and BOOP in our case: the megavoltage radiation therapy. The pathogeny of R-BOOP is not direct toxicity of irradiation because untreated areas can also be affected. Pathogenesis of PPAI remains unknown, but a simultaneous onset with R-BOOP suggests a similar mechanism. While a cumulative dose of irradiation does not seem to be determinant an immune reaction to neoantigens induced by radiation could be an hypothesis.

Biopsy, Needle↗
PubMed2003

Subungual blue nevus.

Junctional or compound melanocytic nevus and melanoma may involve the nail apparatus and are usually discussed in the differential diagnosis of nail pigmentation. Blue nevus has been very rarely reported in this location. We report the second clinicopathologic study of a subungual blue nevus.

Adult↗
PubMedDate not supplied

Apocrine carcinoma developed in nevus sebaceus of Jadassohn.

Apocrine skin carcinoma (ASC) is a rare aggressive cutaneous tumor. We describe a case of ASC occurring on a nevus sebaceus of Jadassohn (NSJ) in a 66-year-old man. Malignant degeneration of NSJ is a rare complication usually found in elderly patients. Most of these tumors are basal cell carcinomas or squamous cell carcinomas. Only five cases of ASC on NSJ have been previously reported. This report recalls the potential of NSJ for degeneration.

Aged↗