 |
|
|
| Picture courtesy of look4.free website |
Many synonyms exist for this strange affection : Pierre Marie-Strumpell-Bamberger 's disease, Bamberger-Marie's disease, pseudo-acromegaly.
Clinical symptoms are an hypertrophy of soft tissue of distal limb extremities giving the aspect of club-bed fingers or drumstick fingers or hippocratic fingers, with distal neurovascular manifestations like cyanosis, hyperesthesia or sudation.
Radiography (if ever done) shows typicial periosteal appositions.
A characteristic hyperfixation is observed on bone scintigraphy.
The precise physiopathology of this symptom is not very well known: an ectopic secretion of a substance acting like growth hormone has been proposed.
Outside lung cancer or lung chronic disease, this clinical symptom is observed in a few hemopthies, larynx carcinoma and cyanogen cardiac insuffisiency
Idiopathic forms exist without any underlying known pathology.