My second installment of unusual and weird diseases that plastic surgeons occasionally see.
Necrotizing fasciitis, commonly portrayed and spoken of in the media as a flesh-eating disease or flesh-eating bacteria, is actually not that uncommon and is not a medical disease that is new. It is an infection of the deeper layer of the subcutaneous tissues below the skin which easily spreads along the fascial covering of the muscles. While it is more likely to occur in diabetic and immunocompromised patients, I have seen it develop in otherwise healthy patients with no such medical problems. I have seen and taken care of dozens of these cases over the years but my most memorable was the healthy 30 year-old female who developed it after just a skin scratch. Within days, she required extensive debridement to control the infection and ended up losing all her skin and fat from her armpit down to her waist. It is a disease at times that can be hard to understand why in some cases.
Many have probably seen this nasal problem but didn’t know what it was. This nose deformity appears as a large, bulbous, ruddy appearance of the nose. It often progresses to such thickening of the skin that lumps and bumps appear like cauliflowers, particularly in the nasal tip area. I have seen rhinophymas so large that it appears as a second nose on top of the orignal one. The name is derived from the Greek word rhis (‘nose’) and phyma (‘growth’). It is caused by a chronic infection of the oil glands often due to untreated rosacea. It appears nowhere else on the face. Contrary to popular belief, it is not due to alcoholism. Treatment is rather barbaric but very effective. The skin is pared down with a loop cautery, shaving it down like carving out a nose from a bar of soap.
Marble Bone Disease (Osteopetrosis)
In this unusual congenital disease, one’s bones become extremely dense, hard, and brittle. It often affects the bones of the skull and face which is the cases that I have seen. Also known as osteopetrosis, the bones become so dense that marrow cavities no longer exist. The bones continue to thicken and grow so that eventually the cranial nerves become compressed by the narrowing of their passageways as bone is deposited in the skull. This results in loss of vision for example as the optic nerve gets slowly pinched off. The bone is so dense that if it develops a wound, it will not heal as it has no blood supply. Teeth extractions are a common source of chronic non-healing wounds in these patients….and there is no good treatment for them.
Duputryen’s Contracture (Disease)
This hand ailment is unusual as it has no known reason as to why it develops but is largely restricted to men of northern European and Scandinavian descent. This slowly develops into a fixed tendon contracture where the fingers bend towards the palm and cannot be fully extended (straightened). Most commonly the ring and little fingers are most commonly affected but almost never the thumb or index finger. Why these fingers and not the others is unknown. Dupuytren’s contracture progresses slowly and is usually painless as the involved fingers bend backward and become stiff. It is known that the palmar aponeurosis tissues thicken and become a contracting band…but why? While I was trained in hand surgery (but do not practice it), a series of geometric zig zag skin incisions are made and the bands are released. While the operation is eloquent, the recurrence rates are high. It is my understanding that an injectable drug is in clinical trials for treatment. Even if only moderately effective, that is usually preferable to surgery.
This genetic disorder is manifest by skin that can really stretch, like a rubber band. There is a defect in a certain type of collagen (the ‘glue’ of our skin) that makes the skin fragile, the joints loose (they can bend backwards), and easy bruising. As a result, those affected are prone to developing really loose neck and jowl skin as they age. I have treated two such patients who underwent facelift surgery. The skin could really be pulled back up and over the ears before trimming. Unfortunately, as easily as the skin is pulled back, it is prone to early relapse showing little results after only a short period of time.
Dr. Barry Eppley