|Year : 2022 | Volume
| Issue : 3 | Page : 182-183
Papuloerythroderma of Ofuji as a paraneoplastic phenomenon in a patient with lung cancer
Wei-Yao Wang1, Yue-Chiu Su2, Cheng-Che E Lan1, Szu-Hao Chiu3
1 Department of Dermatology, Kaohsiung Medical University Hospital; Department of Dermatology, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
2 Department of Pathology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
3 Department of Dermatology, Kaohsiung Medical University Hospital; Department of Dermatology, College of Medicine, Kaohsiung Medical University; Department of Dermatology, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, Taiwan
|Date of Submission||05-Mar-2022|
|Date of Decision||19-Apr-2022|
|Date of Acceptance||25-Apr-2022|
|Date of Web Publication||29-Sep-2022|
Dr. Szu-Hao Chiu
No. 68, Jhonghua 3rd Rd., Cianjin District, Kaohsiung City 80145
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Wang WY, Su YC, Lan CCE, Chiu SH. Papuloerythroderma of Ofuji as a paraneoplastic phenomenon in a patient with lung cancer. Dermatol Sin 2022;40:182-3
|How to cite this URL:|
Wang WY, Su YC, Lan CCE, Chiu SH. Papuloerythroderma of Ofuji as a paraneoplastic phenomenon in a patient with lung cancer. Dermatol Sin [serial online] 2022 [cited 2022 Nov 29];40:182-3. Available from: https://www.dermsinica.org/text.asp?2022/40/3/182/357353
Papuloerythroderma of Ofuji (PEO) is a rare inflammatory skin disorder and may present as a paraneoplastic phenomenon associated with various malignancies. Herein, we report a patient with PEO preceding the diagnosis of small cell lung cancer (SCLC).
A 77-year-old Taiwanese man with hypertension and diabetes mellitus presented with intensely pruritic flat-topped erythematous papules and confluent plaques on the torso and extensor areas of the limbs for 6 months. Deck-chair sign with sparing of abdominal creases and papules on the back symmetrically distributed along the Langer's lines were noted [Figure 1]a and [Figure 1]b. Scaly erythematous plaques on the scalp and bilateral palmoplantar hyperkeratosis were also seen [Figure 1]c and [Figure 1]d. There was no lymphadenopathy, psoriatic nail changes, or other associated symptoms. He had not taken any new medications before the skin eruption. Skin biopsy showed confluent parakeratosis, epidermal acanthosis, exocytosis of atypical lymphocytes, and superficial perivascular lymphohistiocytic infiltrate with few eosinophils [Supplementary Figure 1]a. Immunohistochemistry showed normal CD4/CD8 ratio without loss of CD3 or CD7 staining [Supplementary Figure 1]b and [Supplementary Figure 1]c. T-cell receptor (TCR) gene rearrangement showed the absence of monoclonality. Laboratory investigation revealed lymphopenia of 10.9% and markedly elevated immunoglobulin E (IgE) level of 2188 IU/mL. PEO was diagnosed based on clinical manifestation, drug history, and histopathological and laboratory findings.
|Figure 1: (a) Confluent erythematous flat-topped papules with cobblestone-like appearance on the trunk with deck-chair sign (b) Erythematous papules on the back symmetrically distributed along Langer's lines. (c) Scalp erythema and scaling. (d) Palmoplantar hyperkeratosis. (e and f) Pronounced improvement of skin lesions with postinflammatory hyperpigmentation after 2 months treatment|
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Prompt cancer workup was performed. Chest X-ray disclosed an ill-defined mass in the left upper lung, and subsequent tissue biopsy and cancer staging demonstrated extensive-stage SCLC with liver and bone metastasis. Chest and abdominal computed tomographic scans did not detect any abnormalities in the gastrointestinal tract. The patient was treated with narrow-band ultraviolet B (NBUVB) twice weekly, starting at a dose of 180 mJ/cm2 with 20 mJ/cm2 increments at each session till 320 mJ/cm2 for 3 months. Oral prednisolone 15 mg/day with a 5-week tapering course and topical desoximetasone were also administered. The skin lesions improved significantly with postinflammatory hyperpigmentation following combination skin-directed therapy at 2 month follow up [Figure 1]e and [Figure 1]f. Chemotherapy with carboplatin and etoposide was initiated for SCLC. However, he died 1 year after presentation following chemotherapy.
PEO, a rare inflammatory disorder first described in 1984 by Ofuji et al., is characterized by confluent polygonal papules sparing abdominal folds (recognized as deck-chair sign) and variable pruritus. Additional findings may include dermatopathic lymphadenopathy, palmoplantar keratoderma, and scalp scaling.,, The incidence of PEO is approximately 1.5 per million annually, and elderly men are affected more commonly. Differential diagnosis of PEO includes psoriasis, cutaneous drug eruption, cutaneous T-cell lymphoma (CTCL), and atopic dermatitis. Laboratory investigations may reveal peripheral eosinophilia, increased serum IgE level, and lymphopenia. Histopathology of PEO is often nonspecific and generally shows epidermal hyperplasia, spongiosis, exocytosis, and dermal lymphohistiocytic infiltration.
Currently, the exact etiology of PEO remains poorly elucidated. PEO may present either as a paraneoplastic phenomenon or prelymphomatous condition. Some studies have shown that PEO is associated with several malignancies, including 51.3% from the gastrointestinal tract, which indicates that PEO may be a paraneoplastic phenomenon. In addition, PEO has been regarded as a variant of early mycosis fungoides. Previous reports have shown that patients diagnosed with PEO may later develop CTCL.,, Therefore, skin biopsy and TCR gene rearrangement are essential to exclude the possibility of CTCL. In this case, the patient presented with papules following Langer's lines on the back and exocytosis of atypical lymphocytes, which may be signs of CTCL., Although skin biopsy indicated that PEO may present as a prelymphomatous disorder without evidence of T-cell monoclonality, there may be a connection between PEO and lung cancer.
The pathogenesis of PEO has been widely discussed. Recent studies have proposed that dysregulation of T-cell function may play an important role. T-cells were shown to be skewed towards the Th2 and Th22 population,, which may weaken cell-mediated immunity, thus resulting in tumorigenesis. This hypothesis may explain the association between PEO and malignancies.
Treatment for PEO is aimed at relieving pruritus and inducing skin clearance. It is crucial for physicians to identify the underlying causes and administer prompt treatment. The most frequently reported treatments include oral corticosteroids, psoralen and ultraviolet A, oral retinoids, and combination therapy. NBUVB may be effective as an alternative or combination therapy by attenuating the activity of skin-infiltrating effector T-cells. Dupilumab, an interleukin-4 receptor α-antagonist, has emerged as a novel option for this Th2/Th22-mediated disease.
To our knowledge, this is the first case of PEO with concomitant SCLC. Clinicians should be aware that PEO may exist as a paraneoplastic phenomenon and prelymphomatous condition. Thus, prompt cancer survey and periodic screening are warranted when PEO is diagnosed.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.
The authors sincerely thank dermatologist Prof. Stephen Chu-Sung Hu from Kaohsiung Medical University Hospital for his professional opinion and assistance.
Financial support and sponsorship
Conflicts of interest
Prof. Cheng-Che E. Lan, an editorial board member at Dermatologica Sinica, had no role in the peer review process of or decision to publish this article. The other authors declared no conflicts of interest in writing this paper.
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