Marjolin’s Ulcer in an Old Burn Scar in an Elderly Female

Introduction

Marjolin’s ulcer (MU) was first described in 1828 by Dr. Jean Nicholas Marjolin, but it was not until 1923 when Dr. John Chalmers Da Costa coined the last name “Marjolin” with the word “ulcer” to describe those malignant tumors formed in burn injuries after having evidenced the same phenomenon in a group of patients over the years [1, 2]. MU is a pathological entity arising from chronic wounds and cutaneous scars. While historically associated primarily with burn injuries, MU is now recognized as a clinical variant of squamous cell carcinoma, a malignant neoplasm originating from epidermal keratinocytes [3]. The described etiologies include non-healing wounds, burn scars, bedsores, pressure ulcers, venous stasis ulcers, traumatic wounds, osteomyelitis, and fistulas [4]. The average age at presentation is 59 years, with a latency period from wound occurrence to malignant transformation of approximately 30 to 35 years. The most commonly affected sex is male [5]. In the upper limbs, MU has an incidence rate of about 18.7% and occurs in approximately 1.7% of all chronic wounds, primarily resulting from burn injuries, which account for approximately 76.5% of cases [4, 6].
MU can be classified as either acute or chronic. It is considered acute if it appears within the first 12 months following the primary injury, and chronic if it develops after 12 months, the latter representing the majority of cases [3].
Herein, we present the case of a 74-year-old female with MU arising in an old burn scar.

Case report

A 74-year-old female patient with a history of a third-degree burn on the right hand at the age of 15 was admitted due to the development of an ulcer on the dorsum of the right hand, which had been present for 4 years and had progressively increased in size. On physical examination, the right hand exhibited a flexion deformity at the level of the dorsum and presented with an ulcerated tumor lesion involving the entire dorsum of the hand, with the presence of fibrinous tissue. Initially, the patient was assessed by the plastic surgery team, who noted a joint deformity associated with the presence of blood clots and crusts, as well as a crusted area approximately 3 cm in diameter covered with fibrinoid tissue, without purulent discharge or necrotic tissue. They concluded that the patient did not require surgical intervention at that time and recommended intermediate wound healing.
During her hospital stay, the internal medicine team detected a hepatic abscess and initiated treatment with piperacillin-tazobactam and metronidazole. Eleven days after hospital admission, the general practitioner documented worsening of the wound. Due to these findings, a dermatology consultation was requested. The dermatology team reported a flexion deformity of the right hand, with complete involvement of the dorsum by an ulcerated tumoral lesion covered with fibrinous tissue, without areas of necrosis, findings compatible with MU (fig. 1). They recommended wound healing with fusidic acid cream and requested a biopsy with histopathological analysis to confirm the diagnosis and assess the need for surgical intervention by the plastic surgery team. The patient was subsequently transferred to a high-complexity institution for continued management of the hepatic abscess.

Discussion

The natural history of Marjolin’s ulcer (MU) involves an initial skin injury that leads to a non-healing or recurrent ulcer [7]. It is characterized by increased epidermal thickness, proliferation of irregular squamous cell tongues with minimal cytologic atypia, and an associated mononuclear inflammatory infiltrate [8]. According to Xu et al. (2020), the most common histological finding in MU is squamous cell carcinoma, which is present in 71% of cases, followed by basal cell carcinoma in 12%, and malignant melanoma in 6% of cases [9].
The characteristic symptomatology of MU includes the presence of a non-healing ulcer that is persistent and associated with itching, hyperesthesia, tingling, and a burning sensation [6]. There are specific features of the wounds that facilitate the diagnosis of MU without the need for a definitive test, such as a biopsy, which is typically accurate with a false-negative rate of less than 2% [9]. Common symptoms include the appearance of crusts or ulcers on the scar, a sudden increase in local pain and ulcer size, the presence of bleeding, an unexpected delay in the healing of small wounds, and increased exudate and secretion [10]. The classic clinical triad consists of nodularity, induration, and ulcers that persist for more than 3 months [4]. Some studies have shown that the lower limb is more commonly affected by MU than the upper one [9–11].
Our patient had a history of long-term ulcer evolution and partial crusted healing in an upper limb consistent with MU, but it was not until the dermatology consultation that the characteristic findings were easily identified. She was hospitalized for over 10 days, evaluated by internal medicine, general surgery, plastic surgery, general practitioners, and even a wound care clinic, yet none of them identified the classic lesions of MU. This highlights the clinical challenges of diagnosing MU, as it is a rare condition, and the lack of awareness and exposure to it complicates the development of a successful diagnostic learning curve.
Regarding treatment interventions, surgical management is the first option, including Mohs surgery [10]. Certain conditions may influence the definitive treatment approach, including the presence of abnormal lymph nodes. Regional lymph node dissection is indicated when a palpable lymph node is present, an enlarged lymph node is detected through imaging, or when metastasis is confirmed following a lymph node biopsy. Another indication for regional lymph node dissection is the presence of a moderately-, poorly-differentiated, or undifferentiated tumor measuring greater than 10 cm in diameter. In this latter scenario, a sentinel lymph node biopsy may also be considered and is the recommended approach for patients with well-differentiated tumors smaller than 10 cm [10, 12, 13]. Additionally, the presence of bone invasion — detected in nearly 30% of MU patients in a retrospective review of 126 cases by Luo et al. — often requires extending the resection area to achieve a 2–4 cm margin, followed by free-flap reconstruction. However, when this is not feasible, amputation of the affected limb may be necessary [12–14]. No established consensus exists regarding adjuvant or neoadjuvant management for MU. Nevertheless, chemoradiation therapy with a palliative aim is useful for patients with widespread metastatic disease. Chemotherapy can also be beneficial for patients who are not candidates for surgery, with treatment regimens involving topical or systemic administration of 5-fluorouracil, cisplatin, bleomycin, or methotrexate. Future treatment options, such as hyperthermic intra-arterial limb perfusion, have been suggested [14].
Prevention of ulceration, achieved through proper wound care and early reconstruction during the acute stage — particularly over joints and bony prominences — is essential to avoid ulcer development [8, 14]. Once MU is established, prompt initiation of treatment is crucial to improve outcomes [15].
Regarding prognosis, the overall mortality rate is approximately 21%. However, factors such as metastasis (reported in 27.5–40% of patients), lymph node involvement, and poor histologic grading have been identified as the main adverse prognostic factors that worsen survival. The overall 3-year survival rate following MU diagnosis is 65–75%, but it may decrease to 35–50% in the presence of metastatic disease [14].

Conclusions

MU develops following skin injury into a non-healing or recurring ulcer and is not exclusively related to burn injuries. Males are more commonly affected, and the lower extremities are the most frequent site of presentation. The classic clinical triad includes nodularity, induration, and ulceration lasting for more than 3 months. Prevention of ulceration remains the best treatment strategy. Mohs surgery is the standard surgical treatment; however, the presence of lymph node abnormalities or metastasis influences both management decisions and prognosis.

Funding

No external funding.

Ethical approval

Not applicable.

Conflict of interest

The authors declare no conflict of interest.

References