Pilomaticoma or Pilomatrixoma also known as cacifying epithelioma of Malherbe. It is a rare calcifying epithelioma of hair follicle matrix cell origin. It happens in the Head and Neck 50% of the time. It is sometimes associated with other genetic disorders such as Turner Syndrome, Myotonic Dystrophy, Garder’s Syndrome and Cranial Dysostosis. It seems to be slightly more prevelent in females and in young people 8-13 years of age.
The term rhinophyma is derived from the Greek rhis (‘nose’) and phyma (‘growth’). Rhinophyma is a large, bulbous, ruddy nose caused by granulomatous infiltration, commonly due to untreated rosacea. It has often been referred to as “potatoe nose”. It is most common in white men with a mean age of 50.
Signs and symptoms
Rhinophyma is characterized by prominent pores and a fibrous thickening of the nose, sometimes with papules. It is caused by chronic overgrowth of the sebaceous glands and overgrowth of the colonizing bacteria Dermodex Folliculorum. It is associated with the common skin condition rosacea and can be thought of as the end stage of acne rosecea. It can carry a strong psychological impact due to its effect on one’s personal appearance.
Alcoholism is mistakenly attributed as a cause of this disease, but heavy alcohol consumption does aggravate the condition due its to chronic vasodilation effects. Rhinophyma may be diagnosed without testing, but a skin biopsy can confirm the diagnosis. Surgical treatment may be beneficial.
Rhinophyma is a slowly progressive condition due to hypertrophy of the sebaceous glands of the tip of the nose often seen in cases of long-standing acne rosacea; it is not a neoplasm. It presents as a pink, lobulated mass over the nose with superficial vascular dilation; it mostly affects men past middle age. Patients seek advice because of the perceived unsightly appearance of the enlargement, or obstruction in breathing and vision.
Treating with topical Metrogel seems like a good idea in theory with its activity against Dermodex folliculorum, however, it really does very little in reality. Treatment consists of paring down the bulk of the tissue with a sharp instrument or carbon dioxide laser and allowing the area to re-epithelialise. Sometimes, the tissue is completely excised and the raw area skin-grafted. I personally have never tried putting a skin graft on this and many of my colleagues think this would be a bad idea. With the deep and ever present epithelial elements I would expect it to re epithelialize completely in less than a month with just conservative cares (H2O2 and bactroban applied TID). The painting on the right is from Domenico Ghirlandaio (1449 – 11 January 1494), he was an Italian Renaissance painter from Florence.
I had not seen a case of Burkitt’s Lymphoma since I was in Africa in 1989. I was amazed at that time the resolution the young patient had with chemotherapy. I recently encountered a 3 year old with a massive right tonsil. It was the most difficult intubation I have encountered and after removing the tonsil, it took nearly an hour to obtain complete hemostasis.
Methylene blue and lacrimal probes assist in the dissection. Over probing can lead to cyst wall penetration and over dissection. Often the surgeon needs to combine direct larygoscopy with the external approach to identify the origin of the fistula. Cauterization of the mucosa at the origin has proven acceptable vs some type of a stitch closure.
Ponaris Nasal Emmolient is also a staple of our practice. Pretz, Blairex, Nose Better, Nasal Moist, and Rhinaris are all moisturizing sprays. Entertainer’s Secret is a special formulation used as a throat spray that some have used as a nasal moisturizer. Some have advocated Conjugated Estrogens dissolved in saline. Mayo clinic has compounded rose geranium in sesame oil they say works well.
Obviously the eyes need to be exposed during the case. Often what is going on intranasally gives little indication of the problem. The eye rapidly grows proptotic and tense. Immediate cantholysis and canthotomy is necessary to allow the globe to release anteriorly. The next move I made was to decompress the orbit a bit intranasaly. I also made an immediate Lynch incision and bipolared the offending artery. Some Arista was also used and the final ethmoid packing was fairly loose.
It is not unusual for recurrent polyps or papillomatous sinus disease to lead to dehissance of the Lamina Papyrecea. The Anterior Ethmoid Artery is in close proximitry to this region and injury is possible. I have probably done as many sinus surgeries as anyone alive and kind of felt that my super human skills left me immune to this complication, until now. The intranasal surgery showed little sign of any problem, but the exposed eye become immediately proptotic and tense.