Buschke–Ollendorf Syndrome

(Figure 2.31A, B)
  • AD, LEMD3 (MAN1) gene mutation, encodes inner nuclear membrane protein
  • Elastomas (dermatofibrosis lenticularis disseminata) presenting as yellow papules involving trunk, buttocks, arms, and osteopoikilosis (ectopic calcifications in bone), not prone to fracture
   
 
BUSHke – think of small bush-like opaque areas within the bone (osteopoikilosis)
 
   

Figure 2.31 A: Elastomas (Reprint from Burgdorf WH, Plewig G, Wolff HH, Landthaler M, eds. Braun-Falco’s Dermatology. 3rd ed. Heidelberg: Springer; 2009) B: Osteopoikilosis (Reprint from Dheedene A. The heterozygous Lemd3+/GT mouse is not murine model for osteopoikilosis. Calcified Tissue Int. 2009; 85 (6): 546–51) C: Cutis verticis gyrata (Courtesy of Dr. Michelle B. Bain)
Figure 2.31
A: Elastomas
(Reprint from Burgdorf WH,
Plewig G, Wolff HH, Landthaler
M, eds. Braun-Falco’s
Dermatology. 3rd ed.
Heidelberg: Springer; 2009
)
B: Osteopoikilosis
(Reprint from Dheedene A.
The heterozygous Lemd3+/GT
mouse is not murine model for
osteopoikilosis. Calcified Tissue
Int. 2009; 85 (6): 546–51
)
C: Cutis verticis gyrata
(Courtesy of Dr. Michelle
B. Bain
)