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Pyoderma gangrenosum first presenting as a recalcitrant ulcer of the ear lobe

European Journal of Dermatology. Volume 13, Number 6, 606-9, November - December 2003, Clinical report

Summary

Author(s) : Shigeruko IIJIMA, Toru OGAWA, Yoshikazu NANNO, Takahiko TSUNODA, Kazuhiro KUDOH , Department of Dermatology, Mito Saiseikai General Hospital, 3‐3‐10 Futabadai, Mito, Ibaraki 311‐4198, Japan Department of Dermatology, Yamagata City Hospital Saiseikan, Yamagata Department of Dermatology, Katta General Hospital, Shiraishi, Miyagi, Japan .

Summary : A 59‐year‐old Japanese man with pyoderma gangrenosum occurring at the unusual location of the ear lobe is herein reported. The patient was not associated with any other systemic diseases and had suffered from chilblains at the same site for ten years before the ulcer appeared. The ulcer followed the development of a purpuric exudative lesion and had neither an undermined nor a surpiginous border in the early lesion. It gradually increased in size after various conservative treatments, recurred within a month after being excised and became aggravated after the administration of potassium iodide. Repeated histopathology of the ulcer revealed a mixed inflammatory cell infiltrate with abscesses and an extravasation of red blood cells in the whole dermis, without showing leukocytoclastic vasculitis. A culture of the excised tissue yielded no growth. Laboratory tests were not specific and c‐ANCA was also negative. The ulcer of the ear did dramatically respond to systemic predonisolone of 40 mg\\day. The auricular and periauricular area are quite rare anatomical sites of this disease and the difference between pyoderma gangrenosum and cutaneous Wegener‘s granulomatosis is also discussed.

Keywords : ear lobe, pyoderma gangrenosum, skin ulcer, systemic disease

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ARTICLE

Auteur(s) : Shigeruko IIJIMA¹, Toru OGAWA¹, Yoshikazu NANNO¹, Takahiko TSUNODA², Kazuhiro KUDOH³

¹ Department of Dermatology, Mito Saiseikai General Hospital, 3-3-10 Futabadai, Mito, Ibaraki 311-4198, Japan
² Department of Dermatology, Yamagata City Hospital Saiseikan, Yamagata
³ Department of Dermatology, Katta General Hospital, Shiraishi, Miyagi, Japan

Article accepted on 16/09/2003

Pyoderma gangrenosum (PG), first described by Brunsting et al. [1] in 1930, is a destructive, inflammatory skin disease in which a painful nodule or pustule breaks down to form a progressively enlarging ulcer with a raised, tender, undermined border. Cribriform scarring often follows healing. Any area of the body may be involved in PG, however, the most common sites tend to be the lower extremities. Characteristically, the majority of cases, approximately 80%, had PG lesions associated with such systemic diseases as ulcerative colitis, Crohn's disease, polyarthritis, monoclonal gammopathy or other conditions [2]. Since the underlying systemic diseases usually precede the appearance of PG, the diagnosis of PG is normally not so difficult. However, it is not easy for us to diagnose the patients who may have lesions of the head and neck region as a primary site, have no lesions on the lower limbs, and have no systemic diseases. It is also quite difficult to diagnose such patients correctly because the histopathology of PG is not diagnostic but only suggestive and therefore tends to reveal mixed inflammatory reaction granuloma, abscesses and necrosis, and because there are no specific laboratory tests.

In this report, we present a patient with a highly unusual appearance of pyoderma gangrenosum of the ear lobe without any other associated complications.

Case report

A 59-year-old Japanese man visited our dermatology clinic in Mito Saiseikai General Hospital on November 6 1999, for an evaluation of a purpuric exudative lesion of his left ear lobe. One month before the first visit, the patient noticed a small crust on the frontal surface of the lobe, following a 10-year history of chilblains every winter. The left lobe used to heal with scarring in spring. At the end of October, the patient also had an acne-like papule on his forehead. The crust of the left ear lobe developed into a purpuric, tender and exudative lesion by the first visit, and soon ulcerated and perforated to the back of the lobe by the middle of November.
Laboratory examinations did not show any abnormal findings, including the blood cell count, blood chemistry, electrolytes, coagulation study, blood glucose, thyroid function and urinalysis. Further investigations for collagen diseases and cryogloburinemia, specific antibodies for phospholipid, p-ANCA and c-ANCA were all negative. Immunological tests were within the normal range, and the tuberculin skin test was moderately positive showing 10 × 12/30 × 25 mm. The skin test for Sporotrix schenckii was negative. A swab of the exudate yielded no growth.
Despite negative bacterial cultures, broad-spectrum oral antibiotics were given without any improvement. The lesion also failed to respond to either oral anti-platelet drugs or various topical ointments. The ear lobe gradually became deformed and more exudative while developing into a painful ulcer with a purpuric to dark erythematous infiltration without showing an undermined serpiginous border (Fig. 1). The patient also developed a violaceous plaque with crust on his forehead measuring 7 × 5 mm in size. In March 2000, the two lesions were totally excised for a precise histological examination.
The specimen from the ear lobe showed a dense inflammatory infiltrate in the whole dermis with an abscess in the center, surrounded by histiocytes, foreign body giant cells and lymphocytes, which were consistent with necrotizing granuloma. A large number of red blood cells were extravasated and endothelial cells of small vessels were swollen, however, there was no evidence of vasculitis (Fig. 2a, b). PAS, Grocott and Ziehl-Neelsen stainings revealed no specific microorganisms. Cultures from the excised specimen yielded neither bacteria, mycobacteria nor fungi. A histological analysis of the forehead was fundamentally the same as that of the ear lobe.
A month after the excision, purpuric skin changes appeared at the site of the suture of the left ear lobe and soon developed into an ulcer again. Further surgical biopsies were performed two times for repeated histological examination and tissue culture without any other informative data. However, a therapeutic trial with potassium iodide between 0.8 and 1.2 g/day was applied from June 2000, because a deep mycotic infection could not be clinically ruled out. At six weeks after the treatment, while taking 1.2 grams of the medicine daily, the patient suddenly experienced an intolerable pain of the left ear lobe at night. The ulcer of the lobe rapidly increased in size surrounded by a raised violaceous and serpiginous border (Fig. 3). We finally diagnosed the patient as having pyoderma gangrenosum arising at a very unique site. However, a meticulous search failed to reveal any associated systemic diseases.
Treatment with prednisolone 40 mg/day was started on September 4. A rapid response was thereafter achieved on the third day, and prednisolone was tapered on the 15th day. After four weeks of the treatment, the ulcer healed completely, leaving an extremely deformed ear lobe. When the medicine was tapered to 12.5 mg/day in the following March, several folliculitis-like papules appeared on his back in April. They slowly progressed and developed into ulcers in August, soon after prednisolone was further tapered to 10 mg/day. The ulcer borders were purple-to-red, surrounded by a zone of erythema and healing ulcers showed a cribriform appearance. A dose up to 40 mg/day of prednisolone was effective for the patient's recurring lesions.

Discussion

Our patient was difficult to diagnose, because of the atypicality of the location, poor characteristics of the early lesion and no association of systemic diseases. The diagnosis was also delayed since PG has neither specific laboratory tests nor diagnostic histopathology.
According to an investigation of 86 patients with PG in the Mayo Clinic treated from 1970 to 1983, 67 patients (77.9%) had lesions in the lower limbs, 10 (11.6%) in the trunk and 3 each (3.5%) in the upper limbs, groin, and head and neck region [2]. In contrast, among 25 patients with PG at the San Francisco Veterans Administration Medical Center from 1980 to 1985, 6 (24%) presented with lesions on the head and neck region in the course of the disease [3]. Another report also revealed that two of eight children (25%) under the age of 15 years had lesions at this site [4]. However, auricular or periauricular areas are quite rare anatomical sites for PG, since only six cases have been previously reported in the English literature [3, 5-8]. The clinical features of these cases and ours are summarized in Table I. The age at onset ranged from five to 65 years, with all patients developing auricular or periauricular PG as the first site of the disease. Such PG lesions preceded associated systemic diseases by 11 years in one case [6] and 2 cases including ours had no associated diseases [3], while PG followed systemic diseases in only 4 cases (57%) [3, 5, 7, 8].

Table 1. Pyoderma gangrenosum of the auricular of periauricular regions

Pyoderma gangrenosum		Associated disease		Effective therapy for pyoderma gangrenosum
Age at onset, yr	Site of the first involvement	Diagnosis	Age at onset, yr	Effective therapy for pyoderma gangrenosum
1/F [3]	29	periauricle chin, cheek oral mucosa, leg	none		combination of mPSL 100 mg/day i.v., dapsone 100 mg/day, intralesional triamcinolone 20 mg/wk
2/M [3]	64	retroauricle	perirectal fistula	44	PSL 80 mg/day
3/F [5]	58	auricle (concha)	perirectal fistula ulcerative colitis sclerosing cholangitis	53 54	cyclosporine 10 mg/kg/day
4/F [6]	23	auricle	ulcerative colitis	34	hydrocortisone 500 mg/day
5/M [7]	3.5	preauricle	neonatal asphyxia panhypopituitarism acquired immuno-deficiency syndrome	at birth 7 Mo 3 1/2	dapsone 12.5 mg/day
6/M [8]	65	auricle (pinna)	myelofibrosis diabetes mellitus	before 65	PSL 40 mg/day
7/M (our case)	59	auricle (lobe)	none		PSL 40 mg/day

The early lesion before the operation in our patient had poor clinical characteristics regarding PG. It developed from a chilblain-like purpuric lesion, not from a papule, a pustule or an erythematous nodule. Furthermore, it revealed neither an undermined, serpiginous configuration of the border nor a cribriform scarring. After carefully reviewing the cases in Table I, auricular PGs did not have the latter characteristics, although periauricular PGs did. This is probably due to the morphological and anatominal characteristics of the ear, where cartilage exists, preventing inflammatory cells from infiltrating the dermis and the ulcer from growing downward. One of the characteristic features of auricular PG might thus be to rarely show a typical appearance, especially in early lesions.
It is well known that minimal trauma such as insect-bites as well as surgical operations may lead to new lesions or can aggravate ulcers in about one fourth of PG [2]. Repetitional scarring due to chilblains and the three scalpel procedures may have provoked and led to a recurrence of the lesion in our patient. Furthermore, oral potassium iodide may have been the most precipitating factor. Some authors indicated that the ingestion of potassium iodide could be one of the exacerbating factors [1, 9, 10]. It is noteworthy that such a pathergy to medicine as well as minimal trauma is suggestive in the diagnosis of PG.
Systemic corticosteroids represent the most effective treatment of PG. Sulfa drugs and immunosuppressive agents are its alternatives. In the 7 cases in Table I, corticosteroids were intravenously, orally or intralesionally effective in 5 patients [3, 6, 8]. The other 2 were successfully treated with dapsone [7] or cyclosporine [5], respectively. Dapsone was tried in a pediatric case with acquired immunodeficiency syndrome after some unresponsive local therapy [7]. In contrast, Cyclosporine was administered in a recalcitrant case after successive therapeutic failures with a lot of conventional approaches over an 18-month period [5].
The referring diagnosis of our patient was cutaneous Wegener's granulomatosis (WG). This is a disease with necrotizing granuloma and vasculitis, which is preferentially involved in the upper and lower respiratory tracts and kidney [11], and its cutaneous lesions appear in 14 to 50% cases, showing as an initial presentation in 8.6 to 13% [12,13]. Serologically, the serum IgG antibodies against the cytoplasmic component of neutrophils (c-ANCA) are specific in which sensitivity depends on the extent and activity of the disease [13]. Necrotizing ulcerations resembling PG have also been described on the face, especially in the periauricular region, as the presenting sign of WG [14-16]. We ruled out WG in our case, because the repeated histopathology did not reveal any vasculitis findings and c-ANCA was negative even in the active stage of the disease, and we confirmed the diagnosis of PG by the later occurring typical PG lesions of the back. n

References

1. Brunsting LA, Goeckerman WH, O'Leary PA. Pyoderma (echthyma) gangrenosum: clinical and experimental observation in five cases occurring in adults. Arch Dermatol 1930; 22: 655-80.

2. Powell FC, Schroeter AL, Perry HO. Pyoderma gangrenosum: a review of 86 patients. Q J Med 1985; 55: 173-86.

3. Snyder RA. Pyoderma gangrenosum involving the head and neck. Arch Dermatol 1986; 122: 295-302.

4. Powell FC, Perry HO. Pyoderma gangrenosum in childhood. Arch Dermatol 1984; 120: 757-61.

5. Shelly ED, Shelley WB. Cyclosporine therapy for pyoderma gangrenosum associated with sclerosing cholangitis and ulcerative colitis. J Amer Acad Dermatol 1988; 18: 1084-8.

6. Lysy JL, Zimmerman J, Ackerman Z, Reifen E. Atypical auricular pyoderma gangrenosum simulating fungal infection. J Clin Gastroenterol 1989; 11: 561-4.

7. Paller AS, Sahn EE, Garen PD, Dobson RL, Chadwick EG. Pyoderma gangrenosum in pediatric acquired immunodeficiency syndrome. J Pediatr 1990; 117: 63-6.

8. Oluwole M. Pyoderma gangrenosum: an unusual cause of periaural ulceration. BJCP 1995; 49: 330-1.

9. Perry HO, Brunsting LA. Pyoderma gangrenosum. A clinical study of nineteen cases. Arch Dermatol 1957; 75: 380-6.

10. Ayres S Jr, Ayres S 3. Pyoderma gangrenosum with an unusaul syndrome of ulcers, vesicles, and arthritis. Arch Dermatol 1958; 77: 269-80.

11. Fahey JL, Leonard E, Churg J, Godman GC. Wegener's granulomatosis. Amer J Med 1954; 17: 168-79.

12. Reed WB, Jensen AK, Konwaler BE, Hunter D. The cutaneous manifestation in Wegener's granulomatosis. Acta Dermato-Venereologica 1963; 43: 250-64.

13. Daoud MS, Gibson LE, DeRemee RA, Specks U, el-Azhary RA, Daniel Su WP. Cutaneous Wegener's granulomatosis. clinical, histopathologic, and immunopathologic features of thirty patients. J Am Acad Dermatol 1994; 31: 605-12.

14. Thomas RHM, Payne CMER, Black MM. Wegener's granulomatosis presenting as pyoderma gangrenosum. Clin Experiment Dermatol 1982; 7: 523-8.

15. Chyu JYH, Hagstrom WJ, Soltani K, Faibisoff B, Whitney DH. Wegener's granulomatosis in children: Cutaneous manifestation as the presenting signs. J Am Acad Dermatol 1984; 10: 341-6.

16. Daoud MS, Gibson LE, Dahl PR, Muller SA. Cutaneous Wegener's granulomatosis presenting as pyoderma gangrenosum-like ulceration. Eur J Dermatol 1995; 5: 23-7.

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