Later in 1924, Darier, J and Ferrand,  identified it as a progressive recurrent dermatofibroma. In 1925 it was E Hoffmann who coined the term Dermatofibrosarcoma protuberans. Bednar tumor was first described by Bednar in 1957. ICD Coding
ICD-0: 8832/3 - dermatofibrosarcoma protuberans, NOS
ICD-0: 8833/3 - pigmented dermatofibrosarcoma protuberans
ICD-0: 8834/1 - giant cell fibroblastoma
Fibrosarcomatous dermatofibrosarcoma protuberans: no distinct coding identified

Additional images
See also
List of cutaneous conditions

References
External links
Dermatofibrosarcoma protuberans in NIH Genetic and Rare Diseases Information Center
Most SPS patients with high-titer GAD antibodies also have antibodies that inhibit GABA-receptor-associated protein (GABARAP). Autoantibodies against amphiphysin and gephyrin are also sometimes found in SPS patients. The antibodies appear to interact with antigens in the brain neurons and the spinal cord synapses, causing a functional blockade with gamma-aminobutyric acid. This leads to GABA impairment, which probably causes the stiffness and spasms that characterizes SPS. There are low GABA levels in the motor cortexes of SPS patients.It is not known why GAD autoimmunity occurs in SPS patients, and whether SPS qualifies as a neuro-autoimmune disorder has been questioned. It is also unknown whether these antibodies are pathogenic. The amount of GAD antibody titers found in SPS patients does not correlate with disease severity, indicating that titre levels do not need to be monitored. It has not been proven that GAD antibodies are sole cause of SPS, and the possibility exists that they are a marker or an epiphenomenon of the conditions cause.In SPS patients, motor unit neurons fire involuntarily in a way that resembles a normal contraction. Motor unit potentials fire while the patient is at rest, particularly in the stiff muscles. The excessive firing of motor neurons may be caused by malfunctions in spinal and supra-segmental inhibitory networks that utilize GABA. Involuntary actions show up as voluntary on EMG scans; even when the patient tries to relax, there are agonist and antagonist contractions.In a minority of patients with SPS, breast, ovarian, or lung cancer manifests paraneoplasticly as proximal muscle stiffness.