Sporotrichosis masquerading as pyoderma gangrenosum: case report and review of 19 cases of sporotrichosis

We present the case of a 59-year-old woman who had large ulcerations on her right leg that were diagnosed initially as pyoderma gangrenosum and treated with three immunosuppressive agents (cyclosporin, prednisone and azathioprine) for 6 months. Results of a biopsy at 6 months showed numerous cigar-shaped bodies consistent with Sporothrix schenckii; identification was confirmed by tissue culture. A retrospective review was performed for all cases diagnosed as sporotrichosis from tissue culture or biopsy specimens at the Mayo Clinic. Nineteen cases were identified. The present case was the only one in which fungal organisms were visible on histological examination. The present case emphasizes the importance of making a definitive histological diagnosis in unusual ulcer cases or in suspected cases of pyoderma gangrenosum before the initiation of immunosuppressive therapy. The large number of cigar-shaped bodies in the tissue is a rare finding in sporothrix infection and has been reported in only two cases previously.     

误诊为坏疽性脓皮病的皮肤播散型孢子丝菌病1例

患者女,63岁,农民。胸腹部结节、斑块、溃疡2年半。曾被误诊为"坏疽性脓皮病",并予糖皮质激素、米诺环素等反复治疗1年余,用药时皮疹有所好转,停药后皮疹加重。皮损组织病理示:呈炎性肉芽肿改变,脓液真菌培养鉴定为孢子丝菌。诊断:皮肤播散型孢子丝菌病。予特比萘芬口服,治疗3个月后皮损明显好转,目前仍在随访治疗中。     

Blastomycosis-like pyoderma.

Seven patients with blastomycosis-like pyoderma had skin lesions of four months' to six years' duration. The criteria for the diagnosis of blastomycosis-like pyoderma include the clinical presentation of large verrucous plaques with multiple pustules and elevated border, pseudoepitheliomatous hyperplasia with abscess histologically, and the growth of at least one pathogenic bacteria from the culture of a tissue-biopsy specimen. The differential diagnosis includes deep fungus infection (especially North American blastomycosis), bromoderma, pyoderma gangrenosum, mycobacterial infections, giant keratoacanthoma, and squamous cell carcinoma. Generally, the patients had one or more conditions that could have affected their systemic or local immunologic competence to infection. We believe that the clinical and histologic features in these cases of blastomycosis-like pyoderma were produced by an unusual, exaggerated, vegetating-tissue reaction to a primary or secondary bacterial infection.     

Herpes misdiagnosed as pyoderma gangrenosum

Retroviral infection is known to cause varied manifestation of any disease. We report a 27-year-old female who presented with non-healing painful ulcer in the left hand of 6 months' duration. The patient was diagnosed as pyoderma gangrenosum and treated with immunosuppressant drugs. One month later, she developed perianal vesicles and Tzanck smear from the vesicles showed multinucleated giant cells. A workup for sexually transmitted disease revealed HIV 1 Elisa to be positive. Diagnosis of herpetic ulcer was made and treated with famciclovir and hand ulcer gradually healed in 25 days. We report this case to highlight the occurrence of herpetic ulcer in an unusual site in a HIV patient and may lead to misdiagnosis of pyoderma gangrenosum.     

Psoriasis vulgaris als Trigger eines superfiziellen multilokulären Pyoderma gangraenosum?

We report on a 60-year-old man with psoriasis vulgaris who had developed disseminated ulcerations of different sizes over the complete integument over a period of 3 months. The initial examination revealed approximately 150 clearly defined ulcerations, which were mostly reported to be painless. The histopathology report of a biopsy taken from the border area of an ulceration showed a nonspecific inflammation with a predominantly neutrophilic infiltrate. In the synopsis of all results, after excluding relevant differential diagnoses, we diagnosed a superficial multilocular pyoderma gangrenosum that had possibly developed in the areas of the preexisting psoriasis vulgaris. Reports of superficial multilocular pyoderma gangrenosum, a variation of pyoderma gangrenosum, are extremely rare. This condition is characterised by multiple superficial, chronically persistent ulcerations. These ulcerations are often described as indolent and usually develop from a pustule in the area of a preexisting condition or trauma. Up to now, psoriasis vulgaris has seldom been described as a trigger for pyoderma gangrenosum. The treatment is very difficult and is based on local and/or systemic immunosuppression. In this patient's case, considerable improvement was attained with a topical polyhexanide gel in combination with moist wound therapy, with no systemic immunosuppression. Our case report underscores that physicians should keep in mind the rare variation of superficial multilocular pyoderma gangrenosum in the differential diagnosis of multiple disseminated, painless ulcerations over the complete integument in patients with psoriasis vulgaris.     

Pulmonary nodules and nodular scleritis in a teenager with superficial granulomatous pyoderma gangrenosum

Superficial granulomatous pyoderma gangrenosum, a rare variant of pyoderma gangrenosum, has been considered to be the most benign form of the disease. We present the case of a 15‐year‐old boy with pulmonary involvement and nodular scleritis associated with this unusual type of pyoderma gangrenosum and discuss its differential diagnosis.     

Strategies for optimizing treatment with efalizumab in moderate to severe psoriasis

BACKGROUND: Diagnosis of pyoderma gangrenosum can be difficult, leading to overdiagnosis or underdiagnosis. OBJECTIVE: To identify clinical features helpful in establishing a diagnosis of pyoderma gangrenosum and to compare the characteristics of patients with pyoderma gangrenosum with those of patients with chronic venous ulcers. METHOD: A retrospective chart review was performed in 28 patients with typical pyoderma gangrenosum and compared with the clinical features in 28 patients with chronic venous ulcers. RESULTS: (1) Even when other body sites are affected, pyoderma gangrenosum usually affects the upper and lower legs and feet or peristomal sites compared with chronic venous ulcers that are limited to the lower legs and feet. (2) Pyoderma gangrenosum can be associated with systemic diseases, especially inflammatory bowel disease. (3) Pustules and purulent discharge are features of pyoderma gangrenosum but not of chronic venous ulcers. (4) Crater-like holes or cribriform scarring is commonly seen in pyoderma gangrenosum but not in chronic venous ulcers. (5) Pathergy is a specific but not sensitive finding of pyoderma gangrenosum. It does not occur in patients with chronic venous ulcers. CONCLUSIONS: Diagnosis of pyoderma gangrenosum should be considered in patients with purulent ulcers affecting the legs or peristomal sites. To confirm the diagnosis, specific features should be sought, including pathergy, crater-like holes or cribriform scarring, and association with inflammatory bowel disease. Other causes of ulceration should be excluded.     

Rare association of pyoderma gangrenosum and palmoplantar pustulosis: A case report and review of the previous works

Pyoderma gangrenosum is a rare inflammatory, ulcerative skin disease that mainly involves the lower extremities. It frequently occurs in association with systemic diseases such as inflammatory bowel disease, myeloproliferative disorders and rheumatoid arthritis. Palmoplantar pustulosis is also an inflammatory dermatosis characterized by recurrent sterile pustules localized on the palms and soles. These two dermatoses are histologically characterized by neutrophilic infiltration into the lesional skin. Co‐occurrence of pyoderma gangrenosum and palmoplantar pustulosis in a single patient is extremely rare. We report a case of pyoderma gangrenosum occurred in a patient with palmoplantar pustulosis, with a review of the previously reported cases. A 68‐year‐old Japanese woman with a 10‐year history of palmoplantar pustulosis developed a skin ulcer on the left lower leg. The ulcer was diagnosed as pyoderma gangrenosum based on the clinical and histological findings, and rapidly improved in response to oral prednisolone. In addition to our case, five cases with palmoplantar pustulosis who developed pyoderma gangrenosum have been reported. These cases were thought to have some characteristics in common, such as marked female predominance, no association with inflammatory bowel disease and myeloproliferative disorders, and good response to less aggressive therapy. The co‐occurrence of pyoderma gangrenosum and palmoplantar pustulosis in our case may have an etiological link, rather than being a coincidental complication.     

Pyoderma gangrenosum: a review

Pyoderma gangrenosum is a poorly understood disease characterized by exacerbations and remissions of morphologically unique skin ulcers. It frequently is thought to be the cutaneous manifestation of an underlying systemic disease. In this review of pyoderma gangrenosum, the characteristics of the disease are described and a differential diagnosis is formulated. Associations with systemic diseases are made through a review of the literature. The pathophysiology of pyoderma gangrenosum and probable causes are considered, and special consideration is given to the immunologic mechanisms that may be operative in the disease. Finally the currently available therapeutic alternatives are reviewed.     

Pyoderma gangrenosum: a report of 15 cases and review of the literature

BackgroundPyoderma gangrenosum is a condition that is included among the neutrophilic dermatoses. Given its low incidence, few studies have addressed its epidemiology or treatment.ObjectiveTo describe the epidemiological and clinical characteristics of patients with pyoderma gangrenosum along with our experience of treating the condition in a referral hospital in Malaga, Spain.Material and methodsA retrospective, observational study was undertaken in the Department of Dermatology at Hospital Clínico Universitario Virgen de la Victoria in Malaga, Spain between January 2000 and December 2009 and included all patients diagnosed with pyoderma gangrenosum.ResultsThe incidence of pyoderma gangrenosum in our reference population is 3.26 cases per million inhabitants per year. The most frequent concomitant systemic disease was ulcerative colitis (5 cases, 33%). In 4 patients with that disease, pyoderma gangrenosum appeared during a flare-up. In 80% of cases, patients were not referred to a dermatologist during the initial phase of pyoderma gangrenosum, and most referrals were from gastroenterology or general surgery (4 patients each, 52%).ConclusionsPatients with pyoderma gangrenosum are often referred to dermatologists by other specialists after a varying period of time has elapsed without achieving an accurate diagnosis. In these patients, especially those between 20 and 40 years of age, it is essential to rule out concomitant disease. Adalimumab is a good treatment option for pyoderma gangrenosum.     

Superficial granulomatous pyoderma

INTRODUCTION: Superficial granulomatous pyoderma is a rare variant of pyoderma gangrenosum, which is often diagnosed at a late stage because of misleading clinical-histopathological features. We report a new case of this rare disease. CASE REPORT: A 67 year-old man presented with inflammatory lesions that had become ulcerated over the past 4 months despite prolonged antibiotic therapy. The histopathological aspect was initially suggestive of palisading granuloma annulare and subsequently a necrotizing granuloma compatible with a mycobacteriosis. The various supplementary examinations eliminated the possibility of an infectious or systemic disease. The lesions became painful and ulcerated. A second biopsy showed a neutrophilic dermatosis with dermal necrosis and a granuloma leading to the diagnosis of superficial granulomatous pyoderma. The lesions healed after 3 months of systemic corticosteroids at the dose of 1 mg/kg/day. DISCUSSION: The clinical features of superficial granulomatous pyoderma are similar to those of classical pyoderma gangrenosum. However, its histological profile is more superficial and is associated with dermal granulomas. The latter are unusual in neutrophilic dermatosis and would suggest an infectious disease. This explains the frequent diagnostic errors, with inappropriate anti-infectious treatments and notably unnecessary surgical exeresis.     

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

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.     

Deep cutaneous ulcers and sinus formation in an immunocompetent adult

This report describes a case of unusual deep skin ulcers with tortuous sinus tract formation in an immunocompetent woman. She was initially diagnosed with a Staphylococcus aureus skin infection and histopathologically diagnosed with pyoderma gangrenosum. However, culture from the deep end of ribbon gauze inserted into the subcutaneous sinus tract revealed shiny, light-yellow mucoid colonies, which were identified as Cryptococcus neoformans var. grubii. She was treated with fluconazole for nine months and completely healed. Cryptococcosis is an opportunistic infection caused by variants of C. neoformans species. Cutaneous manifestations of cryptococcosis are quite divergent, rarely occurring as deep skin ulcers with sinus formation.     

Pyoderma gangrenosum in an infant: Case report

Pyoderma gangrenosum (PG) is a rare neutrophilic dermatosis of unknown etiology, characterized by ulcers, especially on the lower limbs. There have only been 13 cases of PG in infants (<12 months old) reported in the past 25 years. We report the case of a 9-month-old child with ulcers on the right leg diagnosed as pyoderma gangrenosum. Tissue cultures for bacteria, mycobacteria, and fungus were negative. The child was treated with prednisone 1 mg/Kg/day for 30 days, with complete healing of the lesions during this period. No underlying diseases have been identified in the last two years, but the child is still being followed.     

Pyoderma gangrenosum associated with Takayasu's arteritis responding to cyclosporin

A 33-year-old caucasian woman with pyoderma gangrenosum associated with Takayasu's arteritis responded to treatment with cyclosporin. This patient is unusual in that both ulcerative and vesiculopustular forms of pyoderma gangrenosum were present. This has not previously been reported with Takayasu's arteritis.     

Genital pyoderma gangrenosum: Report of two cases and published work review of Japanese cases

Pyoderma gangrenosum is an ulcerative skin disorder showing characteristic non‐infectious ulcers and affects the lower extremities in approximately 70% of cases. Pyoderma gangrenosum is commonly associated with systemic diseases such as inflammatory bowel disease, rheumatoid arthritis and hematological malignancies. Herein, we report two cases of Japanese patients diagnosed with genital pyoderma gangrenosum. Case 1 was a 74‐year‐old woman without associated systemic complications, whose skin lesion resembled a squamous cell carcinoma and was limited to the vulva. Case 2 is an 89‐year‐old man, who suffered from myelodysplastic syndrome and acute myeloid leukemia, and presented with penile and leg ulcers mimicking pressure sores. Both cases responded well to systemic steroids. We review 13 genital pyoderma gangrenosum cases (76.9% male; aged 30–89 years) from 1996 to 2012 in Japan, including 11 previously reported cases and the present study's two cases. Four of the 13 genital pyoderma gangrenosum cases had associated systemic diseases and their skin lesions spread to the extragenital areas. Eight of the remaining nine genitalia‐localized pyoderma gangrenosum cases had no associated systemic diseases. In conclusion, genital pyoderma gangrenosum is rare and may be misdiagnosed. It should therefore be considered in cases of refractory genital ulcers. In addition, genitalia‐localized pyoderma gangrenosum tends to be without systemic complications.     

Koebner Phenomenon in an ANCA-Positive Patient with Pyoderma Gangrenosum

A male with pyoderma gangrenosum is reported. The clinical and histological features were typical. The initial lesions resolved with characteristic cribriform scars. A few days after the complete recovery, he developed several necrotizing focal lesions localized to the scarred areas. A further histological examination revealed a granulation tissue rich in neutrophils and signs of necrotizing vasculitis. We found a high titer of circulating perinuclear antineutrophil antibodies (p-ANCA), which are a serological marker for various systemic diseases. An immunological circulating factor has been repeatedly suggested to be the “primum movens” of pyoderma gangrenosum. We discuss the unusual clinical presentation interpreted as a Koebner phenomenon and the possible role of immune factors in enhancing circulating-endothelial cell interactions in relation to the pathogenesis of pyoderma gangrenosum.     

坏疽性脓皮病36例临床特点分析及PARACELSUS评分与Delphi标准的比较

【摘要】 目的 分析36例坏疽性脓皮病患者的临床特点及治疗转归,比较和评价PARACELSUS评分和Delphi标准的适用性和一致性。方法 收集2000年1月至2022年1月在中国医学科学院皮肤病医院住院确诊为坏疽性脓皮病患者36例的临床资料。将PARACELSUS评分和Delphi标准分别应用于上述患者的诊断,并根据kappa值评估两种诊断方法的一致性。结果 36例患者中,6例（16.67%）发病前有明确的诱发因素,31例（86.11%）自觉皮疹不同程度疼痛。31例（86.11%）皮疹形态以溃疡型为主,主要累及双下肢,16例（44.44%）为全身多部位受累。4例（11.11%）合并炎症性肠病,3例（8.33%）合并炎症性关节炎。系统用药以糖皮质激素、雷公藤和环孢素为主,8例（22.22%）使用了肿瘤坏死因子α拮抗剂。用PARACELSUS评分诊断22例（64.71%）,用Delphi标准诊断17例（50.00%）。kappa一致性检验显示,两种诊断标准的一致性为中等,kappa值为0.47（95% CI：0.19 ~ 0.76,P = 0.004）。结论 坏疽性脓皮病的临床表现以经典溃疡型为主,常伴有炎症性肠病和炎症性关节炎,糖皮质激素和免疫抑制剂为主要治疗药物。PARACELSUS评分和Delphi标准侧重于坏疽性脓皮病的不同方面,在缺乏典型病理结果的情况下,推荐使用PARACELSUS评分诊断坏疽性脓皮病。     

Topical disodium cromoglycate in the management of pyoderma gangrenosum

A 52-year-old woman who had had ulcerative colitis for more than ten years experienced a third relapse of pyoderma gangrenosum of her lower extremities. She was treated with topical disodium cromoglycate. The dosage of salicylazosulfapyridine and steroids she was taking, and by which the ulcerative colitis, but not the pyoderma gangrenosum could be controlled, was not increased. Because pyoderma gangrenosum is often very difficult to treat and may require an aggressive approach using drugs that can cause serious side effects, we believe that a topical therapy is worth trying.     

Autoinflammatory syndromes associated with hidradenitis suppurativa and/or acne

Autoinflammatory syndromes associated with hidradenitis suppurativa (HS) and/or acne are rare but potentially debilitating disorders if not diagnosed and treated correctly. They share a common pathogenesis involving a dysregulated innate immune system with abnormal interleukin (IL)‐1 signaling leading to sterile neutrophilic inflammation. The clinical features are recurrent episodes of fever, painful arthritis, and skin lesions consistent with HS, acne, and pyoderma gangrenosum (PG) accompanied by elevated systemic inflammatory markers in blood. So far, several clinically different syndromes have been reported in the literature including pyoderma gangrenosum, acne, and pyogenic arthritis (PAPA), pyoderma gangrenosum, acne, and hidradenitis suppurativa (PASH), pyoderma gangrenosum, acne, and spondyloarthritis (PASS), pyoderma gangrenosum, acne, pyogenic arthritis, and hidradenitis suppurativa (PAPASH), psoriatic arthritis, pyoderma gangrenosum, acne, and hidradenitis suppurativa (PsAPASH), and pyoderma gangrenosum, acne, and ulcerative colitis (PAC). The rarity of the syndromes complicates the establishment of evidence‐based treatment guidelines. Furthermore, treatment can be challenging due to lack of response to standard treatment modalities. Therefore, it is important to increase the awareness about these diseases in order to optimize disease management and ultimately improve the quality of life of patients.