Evaluation of Associated Lymphomas and Their Risk Factors in Patients with Lymphomatoid Papulosis: A Retrospective Single-Center Study from Turkey

Objective: Lymphomatoid papulosis (LyP) is an indolent skin disease with variable clinical features classified among the primary cutaneous CD30+ T-cell lymphoproliferative disorders. It may show association with cutaneous and systemic lymphomas. We aimed to identify the frequency and characteristics of associated lymphomas among Turkish patients with LyP and to determine the risk factors for secondary lymphomas. Materials and Methods: The files of patients diagnosed with LyP between 1998 and 2018 in a tertiary dermatology clinic were retrospectively analyzed. Univariate and multivariate models were used to assess the possible risk factors for secondary lymphomas, such as demographic and clinical characteristics of the patients. Results: Among 61 patients (47 adults, 14 children) with LyP, a total of 22 secondary lymphomas were observed in 20 patients. Nineteen of them were adults. Mycosis fungoides (MF) was the major associated lymphoma (n=19) followed by systemic anaplastic large cell lymphoma (ALCL) (n=2) and primary cutaneous ALCL (n=1). The most common stage in patients with accompanying MF was stage IB (n=11). While 18 patients showed the classical type of MF, one patient had folliculotropic MF. When the risk factors for association between LyP and other lymphomas were evaluated, only older age was found to be a significant risk factor and existence of ulcerated lesions was found to be a negative indicator. Conclusion: LyP is not rare in the pediatric population. MF is the most common associated lymphoma in patients with LyP. Adult LyP patients are more commonly associated with secondary lymphomas than pediatric patients. Older age at the time of diagnosis of LyP is a significant risk factor for associated lymphomas.

Large Congenital Melanocytic Nevus on the Breast Sparing the Nipple and Areola.

BACKGROUND: Divided nevus is a rare entity that has been described for a special appearance of congenital melanocytic nevi (CMNs) occurring on the eyelid and penis. It is presumed that the formation of divided nevi is related with the embryologic development of the eyelid and penis, thus giving a hint about the occurrence time of CMN in utero. This article focuses on a formerly undescribed observation of another special clinical appearance of CMN discussing possible relation with embryogenesis. METHODS: The data including photographic documentations of a total number of 86 patients with large CMN seen in a single center were reviewed according to the involvement of the nipple and areola by the main mass of the nevus and associated satellite lesions. RESULTS: Eight patients presented with CMNs involving one (n=6) or both (n=2) breast region. In all of them, CMN surrounded the nipple-areola complex without involving these structures. Furthermore, satellite nevi have not been observed on the nipple and areola in any of the 86 patients. CONCLUSION: We presume that different developmental time periods of the breast and the nipple-areola complex and the occurrence of the melanocytic lesion before the embryologic development of nipple-areola complex could explain our observation of CMN sparing the nipple-areola complex. The term "nipple-sparing nevus of the breast" is suggested for this special clinical appearance of CMN.

Vascular involvement in Behçet's disease: a retrospective analysis of 2319 cases.

BACKGROUND: Vascular lesions can be the presenting sign of Behçet's disease (BD) preceding classical symptoms. The aim of this study was to evaluate the prevalence and types of vascular involvement in BD. METHODS: Among 2319 patients diagnosed with BD according to the criteria of the International Study Group for BD, 332 patients (279 male, 53 female) with vascular involvement were included in this study. RESULTS: Prevalence of vascular involvement was 14.3%. Vascular involvement was found to be more common in males (P<0.001, male:female ratio 5.26:1). Mean age at onset of vascular involvement was 30.58+/-7.88. Vascular lesion was the presenting sign of BD in 50 patients (2.1%), whereas in 109 patients (4.7%) it started together with other clinical symptoms at the age of diagnosis. Superficial vein thrombophlebitis (SVT) was the most common vascular symptom (53.3%) followed by deep vein thrombosis (DVT) (29.8%). Arterial lesions were rare (3.6%). Of the patients 13.3% had more than one type of vascular involvement. Lower extremities were the most frequent localization in patients with SVT, whereas the femoral vein was mainly involved in patients with DVT and the pulmonary artery was mainly involved in the patients with arterial lesions. CONCLUSION: In countries like Turkey where BD is more frequent, young male patients presenting with vascular involvement should be evaluated for BD.