Pityriasis rubra pilaris is an inflammatory dermatologic disorder of unknown cause and often confounded with psoriasis. It is characterised by hyperkeratotic follicular papules, scaly erythematous plaques, palmoplantar keratoderma, and a progression to generalised erythroderma. Here, we report the case of a 68-year-old man with pityriasis rubra pilaris, who was successfully treated with ixekizumab, an interleukin-17A inhibitor.
Summary
Mycosis fungoides (MF) is a primary cutaneous T‐cell lymphoma with unfavourable prognosis for patients with advanced stages of the disease. Refractory disease and advanced‐stage disease require systemic therapy. We report on a rare case of an atypical predominantly CD8+ folliculotropic MF, a subtype of MF with poorer prognosis, in a 59‐year‐old woman. She was initially diagnosed with MF restricted to the skin, of T3N0M0B0/stage IIB according to the current World Health Organization–European Organisation for Research and Treatment of Cancer classification. First‐line treatment with local percutaneous radiotherapy in combination with systemic interferon alfa‐2a resulted in complete remission. However, 21 months later the disease progressed to T3N0M1B0/stage IVB with development of cerebral manifestation and thus very poor prognosis. Allogeneic stem cell transplantation (SCT) was not a therapeutic option due to the lack of a suitable donor. We initiated methotrexate and cytarabine chemotherapy, followed by high‐dose chemotherapy with thiotepa and carmustine with autologous SCT. Despite rapid response and complete remission of the cerebral lesions, disease recurrence of the skin occurred soon after. Interestingly, readministration of interferon alfa‐2a as a maintenance treatment after the salvage autologous SCT resulted in a durable complete remission during the follow‐up period of currently 17 months after autologous SCT.
What's already known about this topic?
Mycosis fungoides is a primary cutaneous T‐cell lymphoma with unfavourable prognosis for the advanced stages of the disease.
A refractory course of disease requires systemic therapy.
What does this study add?
We report on an unusual case of a patient with mycosis fungoides with cerebral involvement, in which a durable complete remission was achieved upon autologous stem cell therapy and interferon alfa‐2a maintenance therapy.
Surgical excision followed by histopathological examination is the gold standard for the diagnosis and staging of melanoma. Reoperations and unnecessary removal of healthy tissue could be reduced if non-invasive imaging techniques were available for presurgical tumor delineation. However, no technique has gained widespread clinical use to date due to shallow imaging depth or the absence of functional imaging capability. Photoacoustic (PA) imaging is a novel technology that combines the strengths of optical and ultrasound imaging to reveal the molecular composition of tissue at high resolution. Encouraging results have been obtained from previous animal and human studies on melanoma, but there is still a lack of clinical data. This is the largest study of its kind to date, including 52 melanomas and nevi. 3D multiwavelength PA scanning was performed ex vivo, using 59 excitation wavelengths from 680 nm to 970 nm. Spectral unmixing over this broad wavelength range, accounting for the absorption of several tissue chromophores, provided excellent contrast between healthy tissue and tumor. Combining the results of spectral analysis with spatially resolved information provided a map of the tumor borders in greater detail than previously reported. The tumor dimensions determined with PA imaging were strongly correlated with those determined by histopathological examination for both melanomas and nevi.
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