Are You Confident of the Diagnosis?
What you should be alert for in the history
Rhinophyma is a progressive disfiguring condition of the nose associated with rosacea, and is considered to be one of the four rosacea subtypes. There is hypertrophy of sebaceous glands and connective tissue, and it is thought to represent the end stage of severe rosacea.
Four variants of rhinophyma have been described: glandular, fibrous, fibroangiomatous, and actinic. These all have distinct clinical and histologic features. When these hypertrophic changes occur in other locations, they are termed otophyma (ear), gnatophyma (chin), metophyma (forehead), and blepharophyma (eyelid).
Characteristic findings on physical examination
On physical examination, patients with rhinophyma have loss of normal shape of the nose along with overgrowth of nodules, and the presence of telangiectasias and pustules. Patients often have sebaceous discharge from the dilated pilosebaceous units on the nose (Figure 1).
The color of the nose may range from erythematous to purple. Alar creases may become ill-defined and the texture of the nose is firm and thickened. Lobular overgrowth of tissue may produce airflow obstruction by direct compression of the nasal sidewall, or physical occlusion of the nares (Figure 2). The nose may become markedly distorted with loss of the aesthetic subunits. The extreme enlargement is usually limited to the lower two-thirds of the nose.
Patients often suffer significant social embarrassment from the disfiguring appearance and it often may result in social reclusion. Additionally, the conflicting reports that rhinophyma is linked to alcoholism can cause increased embarrassment for patients with this condition. There is little or no statistical evidence to support the association with alcohol consumption.
Expected results of diagnostic studies
The diagnosis of rhinophyma is usually made clinically. The characteristic skin changes and deformities, often in the setting of a patient with a history of rosacea, are usually adequate to make the diagnosis.
When the clinical picture is not clear, a biopsy can be performed. Histologically there are two main variants.
The early common form of rhinophyma will demonstrate features of rossacea in addition to prominent sebaceous hypertrophy within an abundant fibrovascular myxoid stroma.
Fibrosis, acanthosis, and a lymphocytic infiltrate can be seen, and dilated sebaceous ducts filled with sebum are present.
The more severe fibrotic forms of rhinophyma will show marked dermal thickness, absence of sebaceous structures, sclerotic collagen and diffuse dermal telangiectasias. Sarcoidosis, angiosarcoma, granuloma faciale, B-cell lymphoma, and sebaceous carcinoma have been reported to mimic rhinophyma clinically, but can be distinguished histologically.
Who is at Risk for Developing this Disease?
Rhinophyma almost exclusively affects men, with a ratio of men to women ranging from 12:1 to 30:1. Most commonly it is seen in the fifth to seventh decades of life. There have been several patients reported to have developed rhinophyma prior to the age of 30, with the youngest patient having onset at age 17. Rhinophyma is primarily seen in Caucasians with few reported cases in Asian and African populations.
Patients with glandular rosacea, a form of rosacea characterized by thick sebaceous skin with edematous papules and pustules, may be predisposed to the development of rhinophyma.
What is the Cause of the Disease?
The etiology of rhinophyma is unclear, as is the etiology of rosacea. The progression of fibrotic changes seen histologically as the condition becomes more severe suggests that pathogenesis may be related to chronic inflammation and lymphedema.
Systemic Implications and Complications
The degree of distortion may be so severe that the nostrils may become obstructed. Patients may be depressed and suffer from social isolation because of their appearance.
Whereas retinoids, antibiotics, and other topical medicines can be quite effective in the treatment of rosacea, there have been no regimens successful in preventing development of rhinophyma or satisfactory treatment of rhinophyma.
Isotretinoin has been shown to be effective in decreasing nasal volume, when used early in the course of the disease, and in younger patients. This is likely due to isotretinoin’s effect on the reduction in size and number of the sebaceous glands.
Surgery is the only effective treatment for extensive rhinophyma to date. Surgical options include laser therapy, cold steel surgery, cryosurgery, electrosurgery, dermabrasion, excision with grafting, wire loop excision, and Shaw scalpel resection.
The techniques vary in success, but all aim to debulk the overgrowth of tissue and produce normal nose contour with minimal scarring, distortion, and pigmentary change. A combination of surgical approaches may be used for the best aesthetic result.
Research on Tamoxifen
A possible cellular/molecular approach to treating rhinophyma was studied. Due to the prominent fibrosis that occurs, and the up-regulation of growth and fibroblast markers seen in fibrosis, it was postulated that the use of tamoxifen could be of benefit.
Tamoxifen can downregulate TGF-beta, which can be associated with increased fibroblast function in rhinophyma. In one study, tamoxifen was shown to decrease the production and secretion of TGF-Beta 2 in rhinophyma fibroblasts. Further research on tamoxifen or other compounds that can down regulate TGF-beta involved in fibrosis may provide other nonsurgical options in the future.
Optimal Therapeutic Approach for this Disease
When patients with rhinophyma present for treatment, it is important that the rosacea be adequately controlled with topical and/or oral medications. If the rhinophyma is mild, consideration could be given for a trial of isotretinoin. Various dosing regimens have been proposed including low dose (10mg daily) for 6-12 months. If isotretinoin is used, but surgery is to also be performed, a common convention is to wait 6 months prior to surgery but there is little data to support this.
If the patient is a candidate for surgical resection, a variety of modalities are available, as described above. It may be helpful to have the patient bring in a picture demonstrating the natural contour of the nose prior to the development of rhinophyma. This can be helpful in outlining the amount of tissue to be removed to produce the most acceptable result.
Whatever method is employed, it is often wise to remove slightly less tissue than planned, in order to account for post procedure fibrosis and contraction, and loss of tissue with eschar formation. Surface texture and color are most normal when the level of resection of treatment does not go below the depth of the pilosebaceous apparatus. This allows preservation of the normal pilosebaceous pore pattern and color.
Surgical treatment may be performed under local anesthesia using lidocaine with epinephrine. In some cases, general anesthesia or conscious sedation could be considered.
Use of the Shaw scalpel to resect rhinophyma has been used successfully. The main advantage of this method is the hemostatic properties of the scalpel. Because of the highly vascular nature of a rhinophymatous nose, many other approaches are difficult because of extensive bleeding, making it difficult to visualize the surgical area and perfectly contour the nose.
The Shaw scalpel is a heated scalpel, that can be used at temperatures of 150-200 degrees Celsius or higher. The hemostatic properties allow for adequate visualization of the underlying tissue, and also provide the precision of a scalpel for contouring. A specimen is preserved that may be sent for pathologic examination, which is an advantage over several other techniques (Figure 3, Figure 4).
Carbon dioxide laser has been successfully used with reports in the literature demonstrating long-term efficacy. Initial undertreatment with subsequent correction may reduce risk of scarring noted in early studies. The bloodless field allows excellent visualization, which is an advantage over some other modalities such as cold steel surgery or dermabrasion. There are low complication rates and good cosmetic outcomes.
The Shaw scalpel, carbon dioxide laser, combined erbium-YAG/CO2 laser, electrosurgery, dermabrasion, cold steel surgery are all partial thickness (decortication) excisions and wounds are left to heal by second intention. Wound care consists usually of petrolatum and bandages as the wound re-epithelializes over the following 2-3 weeks. Risk of hypopigmentation, scarring, thermal damage, loss of normal texture and pore pattern on the skin are all risks that need to be discussed with the patient, and can direct selection of appropriate treatment method for each patient.
Full thickness excisions of rhinophyma prevent the theoretical risk of recurrence, but must be repaired with full thickness or split thickness skin grafts, and these results may be cosmetically inferior to partial thickness excisions with any of the above mentioned approaches.
After surgery, patients should continue with their rosacea treatment regimen. This may include oral or topical antibiotics, oral or topical retinoids, or other topical medications. If recurrence of the rhinophyma is noted at follow-up, a trial of oral isotretinoin could be considered if employed early. Surgical intervention is also an option using any of the described modalities. The earlier the intervention is performed, the higher the likelihood of obtaining a more normal nasal contour.
Unusual Clinical Scenarios to Consider in Patient Management
Although unusual, there are case reports of various cutaneous malignancies detected histologically when the excised tissue was submitted for pathologic examination. These malignancies have included basal cell carcinoma, sebaceous carcinoma, squamous cell carcinoma, angiosarcoma, and lymphoma in submitted tissue.
Of note, many patients suffer from emotional distress or depression associated with the disfigurement of rhinophyma. Psychological counseling may be of benefit in these patients. Most patients who are successfully treated for rhinophyma regain a confidence and often no longer avoid social situations. There is often a tremendous impact on the quality of life for these patients.
What is the Evidence?
Sadick, H, Goepel, B, Bersch, C, Goessler, U, Hoermann, K, Riedel, F. “Rhinophyma: diagnosis and treatment options for a disfiguring tumor of the nose”. Ann Plast Surg. vol. 61. 2008. pp. 114-120. (This surgically devoted article describes the surgical approach to nasal tumors. The discussion on rhinophyma is well done.)
Vural, E, Royer, MC, Kokoska, MS. “Sculpting resection of rhinophyma using the Shaw scalpel”. Arch Facial Plast Surg. vol. 11. 2009. pp. 263-266. (Article on the surgical use of the Shaw scalpel to sculpt the nose in patients with rhinophyma. Excellent review.)
Aloi, F, Tomasini, C, Soro, E, Pippione, M. “The clinicopathologic spectrum of rhinophyma”. J Am Acad Dermatol. vol. 42. 2000. pp. 468-472. (The authors present the wide clinical and pathological findings that can be seen in patients with rhinophyma. Photos are used to emphasis the degree of differences.)
Madan, V, Ferguson, JE, August, PJ. “Carbon dioxide laser treatment of rhinophyma: a review of 124 patients”. Br J Dermatol. vol. 161. 2009. pp. 814-818. (Large review of patients treated with the CO2 laser. Retrospective review.)
Crawford, GH, Pelle, MT, James, WD. “Rosacea: I. Etiology, pathogenesis, and subtype classification”. J Am Acad Dermatol. vol. 51. 2004. pp. 327-341. (This reference, along with the one below, is wonderful in-depth reviews of all types of rosacea: epidemiology, therapy, both medical and surgical.)
Pelle, MT, Crawford, GH, James, WD. “Rosacea: II. Therapy”. J Am Acad Dermatol. vol. 51. 2004. pp. 499-512. (See above – Rosacea I.)
Bittencourt, C, Accionirover, P, Filho, AB, Cintra, ML, Ypiranga, S. “Rhinophyma in an adolescent”. J Europ Acad Derm Venereol. vol. 20. 2006. pp. 603-5. (Case report of rhinophyma in an adolescent. This is an unusual occurrence.)
Payne, WG, Ko, F, Anspaugh, S, Wheeler, CK, Wright, TE, Robson, MC. “Down-regulating causes of fibrosis with tamoxifen”. Ann Plast Surg. vol. 56. 2006. pp. 301-305. (Discussion of the use of tamoxifen to down regulate fibrosis formation. Discusses pathomechanism of how this may work.)
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