Poikilodermatous subacute cutaneous lupus erythematosus.
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.


CONCLUSIONS
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.
Marzano
Angelo V
AV
Institute of Dermatological Sciences of the University of Milan and IRCCS Ospedale Maggiore of Milan, Milan, Italy. avmarzano@everyday.com
Facchetti
Maura
M
Alessi
Elvio
E
D000328:Adult; D017677:Age Distribution; D000368:Aged; D000962:Antimalarials / Q000627:therapeutic use; D001707:Biopsy, Needle; D004359:Drug Therapy, Combination; D005260:Female; D019084:Fluorescent Antibody Technique, Indirect; D005500:Follow-Up Studies; D006801:Humans; D007150:Immunohistochemistry; D015994:Incidence; D007558:Italy / Q000453:epidemiology; D008178:Lupus Erythematosus, Cutaneous / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D018570:Risk Assessment; D011038:Rothmund-Thomson Syndrome / Q000188:drug therapy / Q000453:epidemiology / Q000473:pathology*; D012720:Severity of Illness Index; D017678:Sex Distribution; D013256:Steroids / Q000627:therapeutic use; D016896:Treatment Outcome
D000962:Antimalarials; D013256:Steroids