Cocaine/Levamisole-Induced, Skin-Limited ANCA-Associated Vasculitis with Pyoderma Gangrenosum-like Presentation

The use of levamisole as the most frequent adulterant of cocaine has merged in previously unknown toxicities, notably a disease entity called cocaine/levamisole-associated autoimmune syndrome (CLAAS). Clinically, CLAAS can manifest with diverse cutaneous and extracutaneous features sharing common laboratory findings (neutropenia, autoantibody patterns). We report the case of a cocaine-abusing female patient with relapsing episodes of painful ulcers, worsening and expanding over a three-year period. The case exhibited all features of a drug-induced, skin-limited, ANCA-associated vasculitis, evolving over time to PG-like findings. In both disease stages, the patient responded well to the cessation of cocaine exposure and systemic glucocorticosteroids. This case demonstrates the continuous nature of cutaneous CLAAS manifestations in a single patient. CLAAS has become a major public health issue in the at-risk group of cocaine users, and clinicians should be alert of this condition when treating cocaine users presenting with single or multiple skin ulcerations

Radiotherapy as a Treatment Option for Local Disease Control in Primary Cutaneous Diffuse Large B-Cell Lymphoma, Leg Type

BACKGROUND Primary cutaneous diffuse large B-cell lymphoma, leg type (PCDLBCL, LT) is an aggressive lymphoma variant. Anthracycline-based chemotherapy with rituximab is recommended as first-line treatment. Radiotherapy (RT) has been considered as a therapeutic option for local disease control in patients with solitary or localized lesions. METHODS We report the results of a retrospective analysis of PCDLBC, LT patients treated either with RT alone or with physician's decision as first-line treatment, aiming to assess disease progression and/or first recurrence in these treatment groups. RESULTS We retrospectively analyzed 20 patients treated either with RT alone (n = 8) or with investigator's choice treatment (n = 12), which included chemotherapy alone or combined with local therapy (RT and wide local excision). Complete response (CR) was achieved in 8 patients from the first group and 9 patients from the second group, with 1 treatment failure. Six patients treated with RT alone progressed with a median time to progression (TTP) of 12.5 months. In the second group, 5 patients progressed with a median TTP of 5.2 months. RT showed good local disease control in both groups without any skin relapses during the follow-up period. CONCLUSION RT as first-line monotherapy followed by watchful waiting did not significantly improve the overall risk of disease progression but resulted in good local disease control. After progression, RT could still easily be combined with systemic treatment. The strength of this analysis needs to be evaluated in a larger patient cohort

Cytokine Release Syndrome During Sequential Treatment With Immune Checkpoint Inhibitors and Kinase Inhibitors for Metastatic Melanoma

Switching from immunotherapy to targeted therapy in metastasized melanoma can be complicated by a cytokine release syndrome (CRS). CRS is a serious complication, which is induced by high levels of circulating cytokines, associated with T-cell engagement and proliferation, and results in a constellation of symptoms with variable organ involvement. We report 2 patients with BRAF V600 mutant melanoma who were previously treated with anti-PD-1±anti-LAG-3 antibodies and were switched to BRAF/MEK-inhibitors because of progressive disease. Both cases depict the complexity of interactions occurring during sequential treatment with immune checkpoint inhibitors and kinase inhibitors. Early identification and management of CRS is crucial to decrease its toxicity and improve safety of further drugs to be given in a therapeutic ladder

Epidemiology of cutaneous lymphomas

Primary cutaneous lymphomas represent the second most frequent type of extranodal non-Hodgkin lymphomas (NHLs) after gastrointestinal lymphomas [9]. The worldwide annual incidence of primary cutaneous lymphomas is estimated to be 1:100,000 [1, 6, 9]. Despite the existence of several large, often clinic-based registries dealing with cutaneous lymphomas (Netherlands Lymphoma Registry, Lymphoma Registry Graz/Austria, Lymphoma Registry Stanford/USA, German Registry for Cutaneous Lymphomas, National Cancer Institute’s Surveillance, Epidemiology, and End Results Program (NCI SEER)) there is a paucity of comparable population-based data concerning this group of diseases. Until 2005, one of the main obstacles for the correct reporting was the lack of appropriate classification to cover all clinicopathological entities, when the new WHO-EORTC was introduced [23]. The comparison of age distribution in different lymphoma registries shows that primary cutaneous lymphomas most commonly arise in older adults, with a median onset time after 60 years of age [1]. One exception to this distribution is lymphomatoid papulosis, which often develops in young adults and can even manifest in childhood [2]. Males seem to be more affected than females, with a male/female ratio reaching up to 4:1 [7, 11, 27]

Cutaneous lymphoma, leukemia and related disorders

Mycosis fungoides (MF) is a general indolent peripheral T-cell lymphoma initially and preferentially present in the skin and showing distinct clinical, histological (except in early stages), immunophenotypical, and genotypical features. It is the most common cutaneous lymphoma [75, 100], characterized by the sequential appearance of patches, developing into plaques and finally into tumors, which tend to ulcerate