Scleredema adultorum of Buschke presenting as periorbital edema: a diagnostic challenge.
Scleredema adultorum is a rare sclerotic disorder characterized by diffuse swelling and nonpitting induration of the skin. Its occurrence has been documented in association with infections, diabetes mellitus, paraproteinemia, multiple myeloma, and monoclonal gammopathy. We report an unusual case of a 48-year-old man with an asymptomatic bilateral eyelid edema of sudden onset. During a period of 6 months, the condition slowly progressed to extensive nonpitting edematous swelling restricted to the periorbital sites. The presumptive diagnosis of scleredema adultorum was confirmed by the presence of typical histologic findings. This case is unique in that the periorbital swelling remained as the sole clinical manifestation of scleredema during the 5-year follow-up and was complicated with partial vision blockage.
Ioannidou
Despina I
DI
Department of Dermatology, University General Hospital of Heraklion, Crete, Greece. derma@med.uoc.gr
Krasagakis
Konstantin
K
Stefanidou
Maria P
MP
Karampekios
Spyros
S
Panayiotidis
John
J
Tosca
Androniki D
AD
D003094:Collagen; D020405:Dermis / Q000473:pathology; D004487:Edema / Q000209:etiology* / Q000473:pathology; D005141:Eyelid Diseases / Q000209:etiology* / Q000473:pathology; D006801:Humans; D008297:Male; D008875:Middle Aged; D012592:Scleredema Adultorum / Q000150:complications* / Q000473:pathology
D003094:Collagen