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12 March 2024: Articles  USA

A Rare Coexistence: Cutaneous Lobular Carcinoma with Pyoderma Gangrenosum

Unusual clinical course

Sarah Sutton ORCID logo1ABCDEF, Kathryn Haran1ABCDEF, Amanda Mullins2BD, Cindy Lamerson3ABCDEF*

DOI: 10.12659/AJCR.942488

Am J Case Rep 2024; 25:e942488

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Abstract

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BACKGROUND: Pyoderma gangrenosum (PG) is a neutrophilic inflammatory disease associated with inflammatory and autoimmune conditions and malignancies. Previously identified links between PG and cancer have included hematological malignancies, solid-organ tumors such as gastric adenocarcinoma, and breast cancer. While specific histologic subtypes of breast cancer such as ductal carcinoma have been associated with PG of the extremities, the literature is limited on the association between PG and cutaneous metastatic lobular carcinoma.

CASE REPORT: We describe the case of an 84-year-old woman with recurrent cutaneous metastatic lobular carcinoma of the left anterior chest with concurrent pyoderma gangrenosum on her bilateral lower extremities. The patient was initially diagnosed with lobular carcinoma of the breast and underwent a left breast mastectomy and was in remission. One year later, she developed 2 lower-extremity ulcerations, which at the time were attributed to an injury and underlying venous stasis. She was referred to a wound care clinic, where the lesions worsened with surgical debridement. Six years later, she presented to the dermatology clinic with a rash on her chest wall and worsening of the ulcerations on her ankles bilaterally. Biopsies revealed lobular carcinoma metastatic to the skin of her anterior chest wall and histopathology consistent with pyoderma gangrenosum on her ankles.

CONCLUSIONS: This case demonstrates a unique presentation of worsening pyoderma gangrenosum due to metastatic malignancy in conjunction with a cutaneous manifestation of lobular carcinoma.

Keywords: Breast Carcinoma In Situ, Dermatology, Pyoderma Gangrenosum

Background

Pyoderma gangrenosum (PG) is a rare ulcerative condition with a poorly understood pathogenesis. Up to 50% of reported cases of PG are associated with concurrent systemic conditions such as rheumatoid arthritis, ulcerative colitis, Crohn’s disease, and malignancies such as breast cancer [1–5]. Previous case reports have studied the appearance of PG in patients with invasive ductal carcinoma, but there is limited literature on the concurrent presentation of PG in patients with lobular carcinoma [6,7]. When occurring in conjunction with underlying cancers, PG is frequently localized near the site of the cancerous lesion or in the lower extremities [6,8,9]. Breast malignancies are no exception, with approximately 63.2% of breast cancer-associated PG manifesting in the lower extremities [2]. There have also been numerous case reports of the development of PG at mastectomy operation sites [10].

Independent of PG, cutaneous metastasis of lobular carcinoma is rare. Although breast cancer is one of the most common forms of cancer to produce cutaneous metastases, it is relatively uncommon compared to other sites of breast cancer metastases. Previous research indicates that cutaneous metastasis of breast cancer occurs in 2.42% of cases [11]. Moreover, in contrast to other types of breast cancer (eg, ductal carcinoma), lobular carcinoma rarely metastasizes to the skin [12].

We present a case of an 84-year-old woman with recurrent metastatic lobular breast carcinoma to the skin with chronic, non-healing ulcerations on her bilateral lower extremities that worsened with surgical debridement, thereby exhibiting pathergy – an exaggerated immune response by the skin to minimal trauma [10]. This case is unique in that the patient presented with cutaneous metastasis of her lobular carcinoma that appeared to worsen her PG. Given her presentation of worsening ulceration with debridement over a period of 6 years, our case highlights the importance of having a high clinical suspicion for pathergy and knowledge of the pathergy effect in PG.

Case Report

An 84-year-old woman presented to the dermatology clinic with concern of a “rash” on her anterior chest wall and ulcerations on her lower extremities present for the past 6 years. Her past medical history included rheumatoid arthritis, ulcerative colitis, acid reflux, venous insufficiency, hypertension, stage III chronic kidney disease, and lobular breast carcinoma in remission. She received acetaminophen and tramadol for her arthritis, furosemide for her kidney disease, and hydralazine for her hypertension. Her past surgical history included left breast mastectomy, hysterectomy, bilateral hip and shoulder replacements, and a C-spine fusion.

The remission of the patient’s lobular carcinoma and subsequent development of ulcers occurred 6 years prior to her presentation at our clinic. She reported the development of ulcerative lesions on her bilateral lower extremities 1 year after remission of her lobular carcinoma. Despite her history of ulcerative colitis and rheumatoid arthritis, her PG lesions did not develop until her metastatic lobular carcinoma recurred. The lower-extremity lesions first appeared on her left leg and were attributed to venous insufficiency. She then developed ulcerative lesions on her right lower leg. Healing of these ulcerations was slowed due to multiple secondary infections caused by Corynebacterium striatum, which required treatment with intravenous vancomycin and ceftriaxone. Following initial surgical debridement and weekly dressing changes, the lesions progressively worsened, with increased pain and edema in and around the ulcerated areas and increased size of the lesions. She was evaluated by vascular surgery for her venous insufficiency due to the slow healing time, but no additional treatment was recommended. The ulcerations then appeared on her right leg. More lesions developed and worsened concurrently with the development of a rash on her anterior chest wall. In our patient’s case, as with many cases of ulcers on the lower extremities with concomitant infections, venous insufficiency was the working diagnosis despite the pathergy and resultant worsening of her ulcerations with surgical debridement for 6 years.

Upon presentation to our dermatology clinic, physical examination revealed a 20×13 cm erythematous, hardened plaque covering most of the patient’s anterior chest wall (Figure 1). In addition, multiple ulcerations were found on the medial and lateral aspects of both ankles (Figure 2A, 2B). The lesions on her extremities presented as erythematous, friable, bleeding ulcers with a well-defined border. A histopathological review of the chest wall biopsy was consistent with the diagnosis of recurrent cutaneous metastatic lobular carcinoma with lymphovascular invasion. The carcinoma was positive for polyclonal keratin and negative for E-cadherin (Figure 3). The histopathological report of the lower extremities showed reactive epidermal changes and undermining of the adjacent epidermis. These findings are nonspecific, but when clinically correlated, are consistent with well-developed lesions of pyoderma gangrenosum (Figure 4).

After a dermatological evaluation, standardized wound care and oral prednisone treatment were initiated for PG. This included gentle wound cleansing with hydrogen peroxide soaks and wrapping of the lesions with mupirocin and DuoDERM twice weekly. This resulted in 50% improvement based on decreased lesion size, depth, and erythema. The patient received prednisone when she presented to our clinic. The possibility of intravenous immunoglobulin treatment was discussed with the patient for her PG. Radiation therapy for her metastatic lobular carcinoma was also discussed. Unfortunately, due to the coronavirus pandemic, no additional treatment was initiated at that time, and the patient died soon after.

Discussion

While the pathogenesis of PG is still not fully understood and is idiopathic in many cases, its connection to systemic diseases, malignancy, and surgical procedures has been well-established [4,13–15]. PG can occur in response to trauma, such as following a breast reduction surgery, or systemically in response to autoimmune conditions and malignancies like breast cancer [8,9,13]. One study found that PG was associated with a systemic condition in over 50% of patients [16].

Breast cancer is another known systemic condition that is associated with PG [6,8]. Specifically, invasive ductal carcinoma is the most common breast cancer that is associated with PG. Duchnowska et al described the case of a 68-year-old woman who developed PG on the left leg and was later diagnosed with estrogen receptor-positive invasive ductal breast carcinoma. After trying multiple topical and systemic therapies to treat PG, her condition only improved when hormonal therapy was introduced to treat the breast cancer [6]. This emphasizes the importance of diagnosing and treating the underlying systemic condition in patients with PG. Future research should attempt to elucidate the impact of ductal and lobular carcinoma on PG. Learning more about this relationship may increase clinician’s awareness of this association and may help direct management.

This case is unique because the patient presented with 2 cutaneous manifestations of her lobular carcinoma – metastasis to the anterior chest wall as well as PG on her distal extremities. The worsening of our patient’s PG lesions occurring in parallel with the development of her cutaneous lobular carcinoma metastasis suggests that her lobular carcinoma contributed to the worsening of her PG lesions. The development of additional lesions on her contralateral extremity and the worsening of them as her cancer metastasized further suggests that her cancer worsened her PG. However, it is important to note that this patient also had many confounding comorbidities that may have impacted the treatment of her lower-extremity PG lesions. The first is her history of ulcerative colitis, which is known to be associated with PG. The second is her history of venous insufficiency, which can contribute to non-healing ulcerations and secondary infections on the lower extremities. As described by Nahm et al, in patients with chronic PG and a concurrent vascular insufficiency of the lower extremities, PG can be improved by targeting blood flow to the region via compression stockings, elevation of the extremities, and medications [17]. However, the timing of this patient’s PG presentation suggests that there is a relationship between it and her lobular carcinoma to some extent.

Regardless of comorbid conditions, our case underscores the importance of having a high clinical suspicion for PG in cases where pathergy is present. Once diagnosed as PG, the ulcerations began to show improvement following appropriate treatment. Furthermore, the consensus for PG management is that a rapid, nontraumatic treatment method produces the greatest likelihood of PG lesion resolution [18]. Previous studies have recommended that surgical debridement of PG be avoided to limit pathergy, which is an excessive immunological response to a minor trauma [14,19]. Therefore, therapy should focus on reducing inflammation and treating the underlying systemic conditions. Therapies can include immunosuppression with steroids as well as antimicrobial agents when a secondary infection is present. Once appropriately diagnosed, this is the regimen that improved our patient’s PG [15].

Conclusions

This case demonstrates a unique presentation of worsening pyoderma gangrenosum due to metastatic malignancy in conjunction with a cutaneous manifestation of lobular carcinoma.

It also highlights the importance of considering the role of malignancy in exacerbating persistent pyoderma gangrenosum. Future research should aim to examine the impact of meta-static breast cancer on PG, specifically looking at the immune response and solutions for earlier diagnosis of PG in this patient population. Additionally, from a more clinical perspective, we feel our case serves as an important reminder to continually reevaluate a diagnosis if standard management – surgical debridement of her ulcerations for 6 years in this case – is not leading to improvement. Continuing education courses on the presentation of PG and its connection to pathergy may also assist in earlier diagnosis and treatment.

References:

1.. George C, Deroide F, Rustin M, Pyoderma gangrenosum – a guide to diagnosis and management: Clin Med (Lond), 2019; 19(3); 224-28

2.. Shah M, Sachdeva M, Gefri A, Jfri A, Paraneoplastic pyoderma gangrenosum in solid organ malignancy: A literature review: Int J Dermatol, 2020; 59(2); 154-58

3.. You HR, Ju JK, Yun SJ, Paraneoplastic pyoderma gangrenosum associated with rectal adenocarcinoma: Ann Dermatol, 2018; 30(1); 79-82

4.. Gallo R, Parodi A, Rebora A, Pyoderma gangrenosum in a patient with gastric carcinoma: Int J Dermatol, 1995; 34(10); 713-14

5.. Ahronowitz I, Harp J, Shinkai K, Etiology and management of pyoderma gangrenosum: Am J Clin Dermatol, 2012; 13(3); 191-211

6.. Duchnowska R, Ziajka E, Góralska A, Grala B, Recurrent pyoderma gangrenosum precipitated by breast cancer: A case report and review of the literature: J Med Case Reports, 2014; 8(1); 226

7.. Vernaci GM, Meroni M, Dieci MV, Postsurgical pyoderma gangrenosum in a breast cancer patient: A case report and literature review: Case Rep Oncol, 2021; 14(1); 160-64

8.. Roga G, Shobha V, Bhat I, Shetty N, Recurrent and treatment-resistant pyoderma gangrenosum, secondary to hidden underlying breast cancer: Internet Journal of Rheumatology and Clinical Immunology, 2015; 3(1); CS6

9.. Sharma A, Horgan K, Holt P, The management of acute pyoderma gangrenosum of the breast with a coincidental invasive breast cancer: J Dermatol Treat, 1994; 5(4); 215-17

10.. Patel DK, Locke M, Jarrett P, Pyoderma gangrenosum with pathergy: A potentially significant complication following breast reconstruction: J Plast Reconstr Aesthetic Surg, 2017; 70(7); 884-92

11.. Hu SCS, Chen GS, Wu CS, Rates of cutaneous metastases from different internal malignancies: Experience from a Taiwanese medical center: J Am Acad Dermatol, 2009; 60(3); 379-87

12.. Lookingbill DP, Spangler N, Helm KF, Cutaneous metastases in patients with metastatic carcinoma: A retrospective study of 4020 patients: J Am Acad Dermatol, 1993; 29(2, Part 1); 228-36

13.. Doren EL, Aya-ay ML, Pyoderma gangrenosum following breast reduction: treatment with topical tacrolimus and steroids: Aesthet Surg J, 2014; 34(3); 394-99

14.. Faghihi G, Abtahi-Naeini B, Nikyar Z, Postoperative pyoderma gangrenosum: A rare complication after appendectomy: J Postgrad Med, 2015; 61(1); 42-43

15.. Teagle A, Hargest R, Management of pyoderma gangrenosum: J R Soc Med, 2014; 107(6); 228-36

16.. Bhat RM, Nandakishore B, Sequeira FF, Pyoderma gangrenosum: An Indian perspective: Clin Exp Dermatol, 2011; 36(3); 242-47

17.. Nahm WJ, Mota JA, Rojas S, Improvement of ulcerations in treatment-resistant chronic scarring in a patient with pyoderma gangrenosum after improving vascular insufficiency, gently removing necrotic debris, and decreasing wound fluid: Am J Case Rep, 2018; 19; 844-48

18.. George C, Deroide F, Rustin M, Pyoderma gangrenosum – a guide to diagnosis and management: Clin Med, 2019; 19(3); 224-28

19.. Leiphart PA, Lam CC, Foulke GT, Suppression of pathergy in pyoderma gangrenosum with infliximab allowing for successful tendon debridement: JAAD Case Rep, 2017; 4(1); 98-100

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American Journal of Case Reports eISSN: 1941-5923
American Journal of Case Reports eISSN: 1941-5923