Bullous pemphigoid in association with cutaneous lesions specific to a myelodysplastic syndrome.
Specific cutaneous lesions are a rare occurrence in myelodysplastic syndromes (MDS). The concurrent association of blistering skin lesions similar to those in bullous pemphigoid (BP), even though a rare event, suggests that BP may be a paraneoplastic syndrome. We report an 86-year-old man who had a refractory anaemia with excess bone marrow blasts in transformation, who developed a generalized pruritic blistering eruption. Immunohistopathological tests showed subepidermal blisters with linear deposits of IgG and C3 along the basement membrane zone of the epidermis surrounding a tumoral dermal infiltrate of CD13+ and CD15+ cells. Immunoblotting studies using epidermal extracts revealed circulating IgG antibodies against three protein bands: a 210-215 kDa band, a 180 kDa band which co-migrated with the BP 180 antigen, and a 190 kDa band. The tumour infiltrate may have revealed antigenic determinants which led to the onset of BP. The concept of paraneoplastic pemphigoid remains to be either confirmed or invalidated by further epidemiological studies.
Modiano
P
P
Dermatology Department, Hôpital Saint-Philibert, Lomme, France.
Reichert
S
S
Barbaud
A
A
Bernard
P
P
Weber
M
M
Schmutz
J L
JL
D000368:Aged; D000369:Aged, 80 and over; D000754:Anemia, Refractory, with Excess of Blasts / Q000150:complications* / Q000276:immunology / Q000473:pathology; D000906:Antibodies / Q000032:analysis; D001324:Autoantigens / Q000032:analysis; D015153:Blotting, Western; D002352:Carrier Proteins; D003094:Collagen; D003176:Complement C3 / Q000032:analysis; D003598:Cytoskeletal Proteins / Q000276:immunology; D051180:Desmoplakins; D000071821:Dystonin; D006801:Humans; D007074:Immunoglobulin G / Q000032:analysis / Q000276:immunology; D008297:Male; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D010391:Pemphigoid, Bullous / Q000150:complications* / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000276:immunology / Q000473:pathology*; D000097805:Collagen Type XVII
D000906:Antibodies; D001324:Autoantigens; D002352:Carrier Proteins; D003094:Collagen; D003176:Complement C3; D003598:Cytoskeletal Proteins; D051180:Desmoplakins; D000071821:Dystonin; D007074:Immunoglobulin G; D009419:Nerve Tissue Proteins; D024041:Non-Fibrillar Collagens; D000097805:Collagen Type XVII; C486268:DST protein, human