|Year : 2021 | Volume
| Issue : 4 | Page : 214-215
Carcinoma erysipeloides deriving from a metastatic eccrine porocarcinoma: A rare case report and brief review of the literature
Flandiana Yogianti, Yohanes Ridora, Sunardi Radiono
Department of Dermatology and Venereology, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada; Department of Dermatology and Venereology, Dr. Sardjito General Hospital, Yogyakarta, Indonesia
|Date of Submission||24-Aug-2021|
|Date of Decision||20-Sep-2021|
|Date of Acceptance||30-Sep-2021|
|Date of Web Publication||14-Dec-2021|
Dr. Flandiana Yogianti
Department of Dermatology and Venereology, Faculty of Medicine, Public Health and Nursing, Universitas Gadjah Mada, Jl. Farmako Sekip Utara, Yogyakarta 55281
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Yogianti F, Ridora Y, Radiono S. Carcinoma erysipeloides deriving from a metastatic eccrine porocarcinoma: A rare case report and brief review of the literature. Dermatol Sin 2021;39:214-5
|How to cite this URL:|
Yogianti F, Ridora Y, Radiono S. Carcinoma erysipeloides deriving from a metastatic eccrine porocarcinoma: A rare case report and brief review of the literature. Dermatol Sin [serial online] 2021 [cited 2022 Dec 7];39:214-5. Available from: https://www.dermsinica.org/text.asp?2021/39/4/214/332515
Eccrine porocarcinoma (EPC) is a malignant neoplasm with a high rate of metastatic potential. This tumor develops from the epithelial cells of the intra-epidermal part of the eccrine duct or acrosyringium. Although EPC is the most common adenocarcinoma of the sweat glands, its prevalence is extremely rare and accounts for only 0.005%–0.01% of all cutaneous tumors. Distant cutaneous metastases from EPC have been reported with variable clinical presentations. Here, we report a case of metastatic EPC presenting as carcinoma erysipeloides.
A 52-year-old woman presented with multiple erythematous plaques and patches extending from the left lower limb to the buttocks, abdomen, and left breast. Eight months earlier, she had been admitted to her local hospital with a complaint of a painful lump on the posterior surface of the left thigh. At that time, an excisional biopsy was performed and showed an EPC with the tumor margin status already negative. The patient then underwent adjuvant radiotherapy. Unfortunately, 6 months later, painful erythematous plaques developed on her left thigh and progressively spread to the trunk.
On physical examination, the entire left leg was edematous and multiple ulcerations were noted on the left thigh and suprapubic region. A relatively sharp demarcated bright red series of patches and plaques also extended from the lower abdomen to the buttock, waist, and left breast, which was slightly warm and painful on palpation [Figure 1]. Hematological investigations were within the normal range. Even though none of the lymph nodes were palpable, magnetic resonance imaging with contrast showed lymphadenopathy in the left inguinal region. Skin biopsy was performed from the left breast and thigh [Figure 2] and showed the presence of a metastatic EPC. Unfortunately, the patient died less than a month after the final diagnosis was made.
|Figure 1: The entire left leg was edematous (a) a relatively sharp demarcated bright red series of patches and plaques extended from the lower abdomen to the buttock, waist, and left breast (b).|
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|Figure 2: Several tumor nests extending from the subepidermal into the dermis (a, H and E stain, ×10); clear tumor cells were evident along with the nuclear atypia, hyperchromatic nuclei, and increased mitotic figures (b, H and E stain, ×40); dermal lymphovascular invasions were seen (c, H and E stain, ×40). The immunohistochemical analysis showed positivity for EMA (d), CK5/6 (e), and PAS (f) on the tumor cells.|
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EPC is an extremely rare tumor that generally affects individuals aged 50–80 years. Given its rarity, no standard protocols currently exist for EPC diagnosis and treatment. Primary EPC may appear as solitary plaque or nodule, sometimes ulcerated, and more commonly seen in the lower extremities (33%) and the head/neck region (32%). This tumor has a high rate of regional lymph node involvement (20%), which markedly increases its mortality rate up to 67%. Histological examination was necessary in almost all cases. Classic histological features of EPC included epithelial proliferation, which may be localized in the epidermis or may extend into the dermis with evident clear cells, nuclear atypia, hyperchromatic nuclei, and increased mitotic activity. Special immunohistochemical staining was also almost always necessary to confirm the acrosyringeal differentiation of the tumor. EMA, CK5/6, and PAS were the most useful immunohistochemical staining reported, with 100% of all confirmed EPC cases positively stained with these staining.
Erysipelas-like lesions also presented in our patient. However, normal hematologic evaluations and the presence of acrosyringeal tumor cells without heavy infiltration of neutrophils from skin biopsy exclude the diagnosis of true erysipelas. However, instead, these findings supported the erysipelas-like cutaneous metastasis or more recognizable as carcinoma erysipeloides., Carcinoma erysipeloides was first described by Rasch in 1931 as erysipelas-like sharply marginated borders of red indurated skin in association with skin metastases., It was more commonly developed from breast cancers, but other carcinomas with a potential lymphatic spreading were also described, including from the lung, ovary, stomach, pancreas, and squamous cell carcinoma.,, A case of carcinoma erysipeloides in a metastatic EPC was reported once. Histologically, dermal lymphatics are usually filled with malignant cells which subsequently lead to lymphatic blockage and induce edema and erythema of the skin, as seen in our patient.
Wide local excision or Mohs micrographic surgery is the mainstay treatment of choice for primary EPC. When presented with metastatic and recurrence EPC, chemoradiotherapy is still the mainstay treatment even though the agent of choice for chemotherapy is still unclear. Some agents that have been used included cyclophosphamide, bleomycin, cisplatin, 5-fluorouracil, and docetaxel, but none of these agents showed consistent clinical benefit in metastatic EPC.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient's daughter has given the consent for images and other clinical information to be reported in the journal. The patient's daughter understands that name and initial will not be published and due efforts will be made to conceal patient identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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[Figure 1], [Figure 2]