The Rudolph sign of nasal vestibular furunculosis: Questions raised by this common but under-recognized nasal mucocutaneous disorder
Published Web Locationhttps://doi.org/10.5070/D30bm7d4x0
The Rudolph sign of nasal vestibular furunculosis: Questions raised by this common but under-recognized nasal mucocutaneous
disorderDepartment of Dermatology, University of Utah School of Medicine, Salt Lake City, Utah
Kevin W Dahle, Richard D Sontheimer
Dermatology Online Journal 18 (3): 6
Nasal vestibular furunculosis is a mucocutaneous disorder commonly seen in the general population. Despite its prevalence in clinical practice, it has been inconsistently described and labeled in the medical literature. We present a case of nasal vestibular furunculosis presenting as recurrent exquisitely tender unilateral erythema and edema of the nasal tip (i.e., the Rudolph sign – as in Rudolph The Red Nosed Reindeer). This symptom complex responded rapidly to topical intranasal mupirocin ointment treatment after having previously failed other treatments including a topical intranasal triple antibiotic ointment and oral doxycycline. This case is instructive as it describes a heretofore under-recognized, but not uncommon, mucocutaneous clinical entity that has been linked to more serious head and neck infections and likely has relevance to the intranasal carriage of Staphylococcus aureus. We review the limited published literature on this mucocutaneous disorder including its nosology and propose future lines of investigation for better defining its clinical significance and pathogenesis.
The personal experience of the authors suggests that the clinical entity described in this report as “nasal vestibular furunculosis” is not uncommon in the general population. As an example, three immediate family members of the senior author (RDS) have experienced recurrence of this symptom complex over a number of years. Yet it has been inconsistently described and labeled in the medical literature. We present a man with recurrent nasal vestibular furunculosis accompanied by unilateral tender erythema of the nasal tip (the Rudolph sign) that responded to topical intranasal mupirocin antibiotic treatment after having failed an over-the-counter topical intranasal triple antibiotic ointment and oral doxycycline. The diagnosis was made based on clinical findings and rapid resolution of symptoms following intranasal mupirocin ointment treatment. This case is instructive as it describes a heretofore under-recognized, not-uncommon clinical entity that has been linked to more serious head and neck infections and could possibly have relevance to the intranasal carriage of Staphylococcus aureus.
|Figure 1. Clinical photo of patient demonstrating the Rudolph sign of nasal vestibular furunculosis.|
A 30-year-old white male presented with a 2-3 week history of a focal area of red, swollen, tender skin on the tip of his nose. He indicated that the right side of the tip of his nose initially became painful and tender to the touch. Shortly thereafter, the skin in this area became red, puffy, and exquisitely tender to the touch. When questioned, he indicated that the inside of the right nasal vestibule underlying the nose tip had also been painful at the outset of his symptoms. He denied associated fever, chills, and malaise. His only other skin concerns were cherry angiomas on his upper trunk for which he sought cosmetic treatment. He was otherwise healthy. He had no history of psoriasis or previously having boils elsewhere on his body. He was not aware of previously having impetigo or other known cutaneous or systemic infections involving S. aureus. He reported that his mother has similar nasal symptoms.
The patient indicated that this same nose problem had been occurring every several months over the previous 2-3 years, with a predominance of symptoms during the winter months. The patient denied any history of nose picking. However, he noticed that his nasal symptoms would often appear after plucking his nose hair with tweezers, trimming his nose hair with a small trimmer, or picking off a scab inside his nose.
The patient had previously self-treated his nose and nasal vestibule with a generic non-prescription topical triple antibiotic ointment with little benefit. He had been treated by other physicians for the same clinical problem with oral doxycycline without improvement. Another physician thought the patient’s problem related to rosacea and treated him with several topical products including metronidazole without benefit.
He was given a prescription for mupirocin ointment to be applied to the entire inner surfaces of both nasal vestibules by sequential use of cotton-tipped applicators twice daily for three consecutive days. The patient returned in one week with complete resolution of nasal pain, skin redness, and swelling.
A. EARLY DESCRIPTIONS
First, a comprehensive literature search was employed to identify previous descriptions and clinical significance of the symptom complex we are referring to here as nasal vestibular furunculosis. Veach described a patient with recurrent “folliculitis” that was resistant to treatment with antiseptics and ultimately responded to topical aluminum chloride in 1940 . Later in 1996, Conners described a patient with uncomplicated “nasal furunculosis” originally treated with a warm compress and oral cephalexin that had a recurrence of symptoms .
The lack of a consensus designation for the common symptom complex that we describe here as nasal vestibular furunculosis was surprising to us. We would like to share our rationale for choosing this particular terminology for this report.
This clinical entity has been described in modern textbooks under a variety of names including “vestibule furunculosis” , “nasal vestibulitis” , and simply “nasal infection” . We found the most precise clinical description of this symptom complex in a current otolaryngology textbook:Nasal furunculosis and vestibulitis are localized infections of the hair-bearing nasal vestibule. A furuncle is a localized painful area of cellulitis surrounding a hair follicle; vestibulitis is a more diffuse process, often with crusting. The causative organism is almost always S. aureus. Therapy includes local heat compresses, elimination of digital manipulation, topical antibiotic ointments and systemic antibiotics directed against S. aureus, such as dicloxacillin, second-generation cephalosporins, or rifampin. Many of these patients are chronic carriers of S. aureus in the nasal vestibule .
We performed a comprehensive literature search using the Medline and Oldmedline databases accessed via PubMed to identify previous clinical names for this symptom complex. Our PubMed search keywords included “nose furunculosis,” “nasal furunculosis,” “nose furuncule,” “nasal furuncule,” “nose vestibule furunculosis,” “nasal vestibule furunculosis,” “nose vestibular furunculosis,” “nasal vestibular furunculosis,” “nose folliculitis,” “nasal folliculitis,” “nose vestibule folliculitis,” “nasal vestibule folliculitis,” “nose vestibular folliculitis,” “nasal vestibular folliculitis,” “nose vestibulitis” and “nasal vestibulitis.” In addition, similar searches were performed using the European Biomedical Institute Literature Database and the Google search engine to identify publications not indexed by PubMed.
When someone refers to “furuncles,” most think of boils on the skin of the trunk and/or extremities. Typically, one does not think of boils on or inside the nose when the term “furuncle” is mentioned. The term “nasal furuncle” is more specific to the nose, however it does not specify the anatomic part of the nose that is affected. In the literature, this term often describes lesions on the exterior surface of the nose [7, 8].
The term “folliculitis” is often used in dermatology to refer to conditions that have multiple follicles targeted by inflammation concurrently that tend to be more pruritic than painful and tender. Common forms of cutaneous folliculitis typically do not cause the acute focal symptoms of tenderness and pain that was evident in our case. The term “nasal folliculitis” is often used to denote focal inflammation around the base of multiple hairs at the orifice of the nasal vestibule, not within the vestibule itself.
While “nasal vestibulitis” is more specific to the nasal vestibule itself, it does not identify the hair follicle as the nidus of symptomatic inflammation. As we will describe below, it is our hypothesis that staphylococcal overgrowth and invasion of a nasal hair follicle is likely to be the primary source of the mucocutaneous symptom complex that we describe here as nasal vestibular furunculosis. We prefer this term because it is specific to the nasal vestibule and the acute focal symptoms that are present. However, the authors look forward to further discussion concerning the most appropriate designation and classification of this symptom complex (this was a major goal for the publication of this case report).
To our knowledge, there have been no published studies attempting to quantify the incidence and prevalence of nasal vestibular furunculosis. Anecdotally, our experience suggests that it is a fairly common condition encountered in clinical practice. There are reports in the literature of rare complications of similar nasal infections leading to cavernous sinus thrombosis and necrotizing pneumonia [9, 10]. Further epidemiologic studies are needed to clarify the epidemiology of nasal vestibular furunculosis and the true rates of associated complications. It would be of interest to know whether nasal vestibular furunculosis is seen more commonly in individuals who are intranasal carriers of S. aureus, as was stated in the otolaryngology textbook passage cited above .
D. CLINICAL OBSERVATIONS
One of the authors (RDS) has personal experience with nasal vestibular furunculosis. At least three members of his immediate family have intermittently experienced this symptom complex over a period of three decades. None of those individuals has ever had other clinical patterns of cutaneous or systemic staphylococcal infections. In addition, he has seen a number of such patients over his three-decade career of dermatology practice. Management of such patients by previous other physicians involved diverse treatment modalities including oral antibiotics, reflecting confusion about this clinical entity.
Typically, the initial symptom is focal pain in the tissue overlying one of the two nasal vestibules (simultaneous bilateral involvement with nasal vestibular furunculosis symptoms appears to be very rare). When the skin overlying the area of pain is inspected, there is no perceptible surface change. However, when the skin overlying the area of pain is palpated, it is often found to be tender. Later, the painful focus of skin overlying the lateral tip of the nose can become reddened. At that point there is exquisite tenderness on palpation.
However, pustules rarely develop within the area of painful, tender erythema at the surface of the skin. In addition, frank abscess formation at the surface of the skin with fluctuance is rarely ever seen. Systemic symptoms including fever and chills do not accompany this localized form of presumed bacterial nasal hair follicular inflammation. Over a period of time if left untreated the intranasal focus of pain and surface tenderness +/- skin surface erythema will spontaneously resolve. However, this can be hastened considerably by treatment with an intranasal topical product. It is not uncommon for an individual with this symptom complex to experience multiple similar recurrent episodes over months to years.
Various triggers for nasal vestibular furunculosis have been described in the literature. Nose picking and hair plucking have been implicated . Additionally, Veach reported that there was a predominance of symptoms in the winter months . As stated above, our patient noticed a recurrence of symptoms after plucking his nose hair with tweezers, trimming his nose hair with a small trimmer or picking off a scab inside his nose.
In 1940, Veach treated a patient with antiseptics, tincture of mercury, and eventually aluminum chloride . It is important to note that antibiotics were not readily available at that time. Connors recommended using warm compresses and oral anti-staphylococcal antibiotics followed by drainage of the furuncle. Further recurrences were treated with topical mupirocin ointment . However, it has been our personal experience that drainage is not necessary once topical intranasal mupirocin applications are started. The senior author has observed one patient whose symptoms were controlled initially with topical intranasal application of an over-the-counter topical antibiotic ointment containing neomycin, polymyxin and bacitracin. Over time, recurrences of this symptom complex ceased to respond to this combination topical antibiotic preparation. However, the patient quickly responded to the institution of topical mupirocin, suggesting acquired bacterial resistance to the topical triple antibiotic combination.
It is our experience that topical antibiotic treatments are the most effective treatment. Typically, the pain and erythema start to improve within 12 hours after the initiation of topical therapy with an antibacterial ointment or cream applied twice daily by cotton tip to the entire mucosal surface of the nasal vestibule. This treatment is best continued for 2-3 days consecutively. Initially, over-the-counter triple antibiotic creams or ointments containing neomycin, polymyxin, and bacitracin are effective. However, as noted above some individuals who have experienced nasal vestibular furunculosis intermittently for a longer period of time appear to become resistant to the therapeutic effect of over-the-counter triple antibiotic topical preparations. Starting a prescription-strength topical antibiotic preparation such as mupirocin or retapamulin ointment can provide further relief in such patients.
F. ETIOLOGY OF NASAL VESTIBULAR FURUNCULOSIS
It is the authors’ hypothesis based upon review of the literature and extrapolation from personal observations that the nasal hair follicle is the portal of entry for staphylococcal tissue invasion in nasal vestibular furunculosis. Whether a single or group of hair follicles is involved is unclear. The initial pain and tenderness of nasal vestibular furunculosis is very localized, arguing against a more widespread mucocutaneous surface infection. Inflammation within the follicles of nasal hair could account for such localized, asymmetrical point tenderness and pain. This inflammation must then spread through multiple tissue layers in order for erythema to be evident on the nasal skin. As such, cutaneous erythema of the nasal tip may or may not be present, depending on the level of inflammation. When cutaneous erythema is present, it typically follows the onset of focal, intranasal pain.
Ideally, the authors would have performed a nasal vestibule culture for S. aureus in the patient whose case we are reporting. Based upon the senior author’s past experience of successfully treating nasal vestibular furunculosis empirically with intranasal topical antibiotic ointments, it was felt that the results of performing a nasal vestibule culture for S. aureus would not have had a clinical impact on our treatment decisions in this case. In any further studies of nasal vestibular furunculosis, such cultures should be performed in a standard manner.
In our literature review we have been unable to find reports of the histopathological changes of nasal vestibular furunculosis. In addition, the authors were unable to find a histopathologic description of the cutaneous erythema of the nasal tip that may be seen with nasal vestibular furunculosis. While an intranasal biopsy would be extremely helpful in elucidating the pathophysiology of nasal vestibular furunculosis, it is important to note the impracticality of performing an intranasal biopsy. The instruments required to perform such a biopsy are not readily available in a typical dermatology office setting.
G. CLINICAL CORRELATIONS/IMPLICATIONS
Nasal vestibular furunculosis raises a number of important clinical questions. Do patients with nasal vestibular furunculosis have greater S. aureus colony counts? Do they have higher rates of methicillin resistance? Do they have more virulent strains of S. aureus? If S. aureus does form intranasal biofilms, would such biofilm formation potentiate the development of nasal vestibular furunculosis? The association between the Panton-Valentine Leukocidin (PVL) positive S. aureus and cutaneous furunculosis has been well documented in the literature [11, 12, 13]. Is there a similar correlation between PVL positive S. aureus and nasal vestibular furunculosis? Is there an association between nasal vestibular furunculosis and recurrent herpes simplex virus type 1 (HSV-1) infection? Further studies are needed to clarify these correlations.
Additionally, further work is needed to elucidate the most effective treatment for nasal vestibular furunculosis. It is the authors’ preference to treat initially with topical antibiotics. Does this practice differentially decrease staphylococcal carriage rates in individuals with nasal vestibular furunculosis compared to those who do not have nasal vestibular furunculosis? The authors have observed a number of patients that initially respond to over-the-counter antibiotic treatment but subsequently stop responding. Does topical use of intranasal antibiotics potentiate the development of new staph-resistant strains? Do oral antibiotic regimens designed to rectify MRSA colonization benefit patients with recurrent nasal vestibular furunculosis? All of these questions need to be clarified in order to better treat this common clinical condition.
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