A 25‐year‐old Taiwanese male came to our dermatologic clinic due to muscle weakness, facial erythema, and peri‐orbital swelling for 3 weeks (Figure 1A). He was diagnosed with testicular embryonal carcinoma with lung metastasis (Figure 1E,F) and had just received orchiectomy 2 weeks prior to cutaneous manifestations. Upon physical examination, Gottron's papules (Figure 1C) were seen along with trunk erythema. Skin biopsy in right dorsal hand showed hyperkeratosis, acanthosis, and papillary dermal edema with mucin deposition in the dermis (Figure 1G, H). Direct immunofluorescence revealed scattered ovoid bodies over the dermal‐epidermal junction. Laboratory exam showed increased levels of CPK (2663 IU/L), LDH (293 IU/L), and alpha‐fetoprotein (34.83 ng/mL). Dermatomyositis (DM) was impressed.
FIGURE 1.
A, Facial erythema with marked peri‐orbital swelling. B, Improved peri‐orbital swelling after chemotherapy. C, Gottron's papules on the left dorsal hand. D, Improved erythema and Gottron's papules on the left dorsal hand. E, Gross picture of testicular tumor, size:3.5 × 3.5 × 3.5 cm. F, Embryonal carcinoma shows large, anaplastic cells with nuclear pleomorphism and frequent mitoses, which is arranged in solid pattern. Extensive necrosis is also present. (Hematoxylin–eosin stain, F, ×100 magnification). G, Marked papillary dermal edema and mucin deposition in the dermis. (Hematoxylin–eosin stain, G, ×40 magnification). H, Several dyskeratotic cells within the epidermis and marked papillary dermal edema. (Hematoxylin–eosin stain, H, ×100 magnification)
Prednisolone with 30 mg per day was given initially, but the symptoms persisted. After administered first chemotherapy with bleomycin, etoposide, and cisplatin (BEP) for testicular cancer, his symptoms and skin erythema significantly improved (Figure 1B,D) along with declined level of CPK and tumor markers. Auto‐antibody of anti‐transcription intermediary factor 1‐gamma (TIF1‐γ) showed positive result (1+), and therefore, paraneoplastic DM related to testicular carcinoma was diagnosed. The patient had received a total of six courses of chemotherapy (BEP), and his condition remained stable to date.
DM is an autoimmune disorder that is characterized with cutaneous violaceous erythema and muscle weakness. It is sometimes associated with internal malignancy, mainly adenocarcinomas of origin. 1 Cutaneous manifestations and muscle weakness may be present before any diagnosed malignancy and lasting even after the tumor was removed. 1 Although the exact mechanism of paraneoplastic DM remains unknown, it is hypothesized that auto‐antibodies, anti‐TIF1‐γ, and anti‐nuclear matrix protein 2 (NXP2), generated by the tumor may be related to DM with cancers. 2 In our case, we had observed that these auto‐antibodies may be correlated with tumor occurrence. It is proposed that auto‐antibodies were induced by antigens of tumor cells, which cross‐reacted among skin and muscle tissue, causing erythema and muscle weakness.
Our patient presented with persisted muscle weakness and skin erythema without much improvement despite primary tumor removal and systemic steroid use. His symptoms showed significant improvement after initiating chemotherapy for treating metastatic lesions and relapsed after ceasing chemotherapy. These findings were also identical to those mentioned in previous studies. 3 To sum up, successful treatment for paraneoplastic DM should target both primary and metastatic lesions, reducing tumor burden and thus lowering circulating auto‐antibodies. Systemic treatment with chemotherapy for cancer is the key to improvement of both cutaneous and myositic symptoms.
There are only few reported cases of paraneoplastic DM related to testicular cancer. 4 A search over the Kaohsiung Medical University Hospital database over the past 10 years showed only this one case. In conclusion, although rare, DM may be associated with malignancy which required a complete systemic survey including laboratory examination and imaging study. Physicians should be especially aware of possible visceral malignancy in DM patients with auto‐antibodies of anti‐ TIF1‐γ or anti‐NXP2.
CONFLICT OF INTEREST
All authors declare no conflict of interest.
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