Case Report Volume 15 Issue 1
Department of Otolaryngology, Kingston Hospital Foundation Trust, England
Correspondence: Mohammed Al-Tayyar, Department of Otolaryngology, Kingston Hospital Foundation Trust, Kingston Upon, Thames, Surrey, London, KT2 7QB, England, Tel +44 07985292563
Received: March 13, 2023 | Published: March 30, 2023
Citation: Al-Tayyar M, Toma A. Case report: a rare presentation of nasal septal perforation due to pyoderma gangrenosum. J Otolaryngol ENT Res. 2023;15(1):44-46 DOI: 10.15406/joentr.2023.15.00526
Background: Pyoderma gangrenosum (PG) is a rare dermatologic non-infectious neutrophilic disease that classically affects the lower extremities and is associated with inflammatory bowel disease. Rarely, it could affect the nasal septum, causing nasal septal perforation.
Methods: We reviewed the case of a 52-year-old male patient known to have PG with ongoing nasal septal perforation and reviewed his blood tests, computed tomography scan findings and histologic results.
Results: A diagnosis of nasal septal perforation due to PG was confirmed after exclusion of other common aetiologies. This was further supported by the presence of extensive ulceration of the nasal squamous mucosa with inflamed granulation tissue and abscess-like areas. These findings were consistent with the diagnosis of PG based on the histology report.
Conclusion: Although nasal septal perforation secondary to PG is considered rare, this presentation should still be kept in mind, especially when other possible causes of nasal septal perforation have been excluded and PG has been already well established.
Keywords: nasal septal perforation, neutrophilic dermatosis, pyoderma gangrenosum
Pyoderma gangrenosum (PG) is a reactive non-infectious inflammatory dermatosis falling under the neutrophilic dermatosis spectrum, which includes Sweet’s syndrome and Behcet’s syndrome.1,2 It was first described in 1930 by Drs. Brunsting, Goeckerman, and O’Leary, who described the typical lesions of PG as enlarging necrotic ulcers with erythematous to bluish undermined borders surrounded by spreading erythema.3,4
Six major variants of the skin condition have since been outlined, including (1) ulcerative or classic, (2) pustular, (3) bullous or atypical, (4) vegetative, (5) peristomal, and (6) post-surgical PG (Table 1).3
Pyoderma gangrenosum |
Common location |
Presentation |
Associated disease |
Ulcerative (classic) |
Lower extremities |
Rapid progression Violaceous |
Inflammatory bowel disease (IBD) |
Bullous (atypical) |
Face |
Superficial bulla Blue-grey border |
Myeloproliferative disease (i.e., acute |
Pustular |
Legs Upper trunk |
Painful pustules Red halo |
IBD |
Vegetative |
Trunk |
Superficial ulcer No violaceous |
None |
Peristomal |
Near stoma site |
Painful ulcer Violaceous |
IBD Enteric malignancies |
Post-surgical (procedural) |
Surgery site (breast, |
Rapid progression Active and undermined |
Fewer cases of underlying systemic |
Table 1 Different clinical presentations of pyoderma gangrenosum and their associated systemic diseases
A 52-year-old male patient was recently diagnosed with the vegetative type of PG as he only had skin lesions in the back without any other systemic manifestations. After the diagnosis, he developed several non-specific nasal issues that were progressive in nature. These included nasal stiffness, nasal obstruction, crustation, and a dull ache.
Previously, he had tried different remedies, either over-the-counter medications or those prescribed by his primary health care providers. These drugs included saline nasal rinses, local steroid sprays5, and local antibiotics; however, despite the use of these medications, the patient’s nasal symptoms continued to deteriorate. Therefore, he was referred to the otolaryngology specialized clinic.
On examination at the clinic, nasal bridge broadening was noted with extreme tenderness at the alar cartilage area. Nasal endoscopy was performed that revealed nearly total nasal septal perforation as well as erosion of the anterior sphenoidal wall. Moreover, there was widespread granulation tissue inside the nasal cavity.
A thorough and detailed approach was used to determine the cause of the septal perforation. There was no history of nasal trauma or surgery, cocaine abuse, or any granulomatous6 diseases apart from PG. Various lab and imaging tests such as cytoplasmic and peripheral antineutrophil cytoplasmic antibodies (c-ANCA and p-ANCA), erythrocyte sedimentation rate (ESR), full blood count (FBC), and chest X-ray (CXR), among others, were ordered in order to arrive at a definitive diagnosis. The results of all these tests were inconclusive. In addition, a computed tomography (CT) scan was ordered, and the results were consistent with the clinical findings (Figure 1).
At that stage, it was prudent to exclude malignancy; therefore, multiple biopsies were taken under general anaesthesia (Figure 2).
The cytology assessment detected no malignant cells; however, there was noted extensive nasal squamous mucosa ulceration with inflamed granulation tissue and abscess-like areas consistent with PG.
Nasal septal perforation is considered reasonably common in otolaryngology consultations. The aetiologies vary greatly from one another, and there is a myriad of possibilities that could cause them. These can be summarized into the categories listed in Table 2.
Traumatic causes |
Surface irritants |
Infections |
Neoplastic |
Inflammatory |
Nasal surgery |
Cocaine insufflation |
Syphilis |
Melanoma |
Granulomatosis with |
Nose picking |
Fumes (chromic/sulphuric acid) |
Tuberculosis |
Squamous cell carcinoma |
polyangiitis |
Bilateral septal cauterization |
Lepromatous leprosy |
Adenocarcinoma |
Sarcoidosis |
|
Foreign bodies |
|
Rhinoscleroderma Mucor |
Lymphoma |
Systemic lupus erythematosus |
Table 2 Aetiologies of the nasal septal perforation
Because of the wide range of aetiologies for septal perforation, history-taking is crucial. Nasal perforations may be the first sign of various granulomatous disorders, including granulomatosis with polyangiitis (GPA) and systemic lupus erythematosus (SLE). Baseline work-up should include an FBC, ESR, serum urea, serum electrolytes,7–9 urinalysis, C-ANCA, treponemal tests, ACE titres, a CXR, and a nasal swab. Routine biopsy of septal perforations to exclude vasculitis has been suggested10–12 but biopsy rarely reveals anything other than chronic inflammation and is usually insufficient for a specific diagnosis.
Nasal septal perforation due to PG is considered rare; therefore, this diagnosis remains a diagnosis of exclusion.3 Head and neck manifestations of PG are known to have poor response to standard treatment.13 The patient mentioned in this study had already received extensive steroid therapy to treat his skin lesions, and yet his lesions still progressed until finally his nasal structures were destroyed.
Although nasal septal perforation secondary to PG is considered rare, this presentation should still be kept in mind especially when other possible causes of nasal septal perforation have been excluded and PG is already well established.
None.
Authors declare no conflict of interests.
This work was supported by the Kingston hospital foundation trust.
©2023 Al-Tayyar, et al. This is an open access article distributed under the terms of the, which permits unrestricted use, distribution, and build upon your work non-commercially.