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Poikilodermatous subacute cutaneous lupus erythematosus
Angelo V Marzano1, Maura Facchetti, Elvio Alessi
1Institute of Dermatological Sciences of the University of Milan and IRCCS Ospedale Maggiore of Milan, Milan, Italy. avmarzano@everyday.com
Dermatology (Basel, Switzerland)
|October 23, 2003
Summary
Poikilodermatous Subacute Cutaneous Lupus Erythematosus (SCLE) is a rare variant with a good prognosis. This study highlights its clinical features and favorable long-term outcomes in four patients.
Area of Science:
- Dermatology
- Immunology
- Pathology
Background:
- Subacute cutaneous lupus erythematosus (SCLE) is a distinct subset of lupus erythematosus.
- Poikilodermatous presentation is an unusual variant of SCLE, with limited prior case reports.
- Understanding rare SCLE variants is crucial for comprehensive patient care.
Purpose of the Study:
- To summarize the clinical characteristics and course of poikilodermatous SCLE.
- To detail the pathological, laboratory, and immunofluorescence findings in these patients.
- To contribute to the understanding of this rare SCLE variant.
Main Methods:
- Retrospective study of 54 SCLE patients diagnosed between 1980 and 2002.
- Inclusion of four patients with poikilodermatous SCLE.
- Analysis of clinical, pathological, laboratory, and immunofluorescence data.
Main Results:
- Four patients (7.4%) presented with poikilodermatous SCLE.
- All patients remained alive with no severe systemic involvement up to 36 years post-onset.
- Cutaneous amyloid deposition was a notable laboratory finding.
Conclusions:
- Poikilodermatous SCLE is an uncommon variant with a favorable clinical course despite extensive skin involvement.
- Photosensitivity is the proposed pathomechanism for poikiloderma and cutaneous amyloidosis in these cases.
- This variant follows a generally good prognosis within the SCLE spectrum.