Dermatology (Basel, Switzerland) · 2003
Poikilodermatous subacute cutaneous lupus erythematosus.
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Marzano Angelo V, Facchetti Maura, Alessi Elvio
Аннотация
BACKGROUND: Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of lupus erythematosus with unique clinical, immunological and genetic features. Among the unusual variants of SCLE, there is a poikilodermic presentation. However, to date, only 1 case of poikilodermatous SCLE has been reported.
OBJECTIVE: Our goal was to summarize the clinical characteristics and course as well as the pathological, laboratory and immunofluorescence findings of 4 patients with poikilodermatous SCLE.
METHODS: A retrospective study was conducted including 54 patients diagnosed as having SCLE between 1980 and 2002.
RESULTS: Four patients (7.4%) had SCLE. All patients were alive, and none developed severe systemic involvement in up to 36 years (median, 24 years) after the onset of disease. The most noteworthy laboratory finding was the cutaneous deposition of amyloid.
CONCLUSION: Poikilodermatous SCLE represents an uncommon variant within the clinicopathological spectrum of SCLE following a favorable course, in spite of extensive cutaneous involvement. Photosensitivity is the pathomechanism explaining, theoretically, the development of both poikiloderma and cutaneous amyloidosis in such cases.
OBJECTIVE: Our goal was to summarize the clinical characteristics and course as well as the pathological, laboratory and immunofluorescence findings of 4 patients with poikilodermatous SCLE.
METHODS: A retrospective study was conducted including 54 patients diagnosed as having SCLE between 1980 and 2002.
RESULTS: Four patients (7.4%) had SCLE. All patients were alive, and none developed severe systemic involvement in up to 36 years (median, 24 years) after the onset of disease. The most noteworthy laboratory finding was the cutaneous deposition of amyloid.
CONCLUSION: Poikilodermatous SCLE represents an uncommon variant within the clinicopathological spectrum of SCLE following a favorable course, in spite of extensive cutaneous involvement. Photosensitivity is the pathomechanism explaining, theoretically, the development of both poikiloderma and cutaneous amyloidosis in such cases.
Предметные рубрики MeSH
AdultAge DistributionAgedAntimalarialsBiopsy, NeedleDrug Therapy, CombinationFemaleFluorescent Antibody Technique, IndirectFollow-Up StudiesHumansImmunohistochemistryIncidenceItalyLupus Erythematosus, CutaneousMaleMiddle AgedRetrospective StudiesRisk AssessmentRothmund-Thomson SyndromeSeverity of Illness IndexSex DistributionSteroidsTreatment Outcome