Epidemiological changes in cutaneous lymphomas: an analysis of 8593 patients from the French Cutaneous Lymphoma Registry.

BACKGROUND: Primary cutaneous lymphomas (PCLs) are a heterogeneous group of T-cell (CTCL) and B-cell (CBCL) malignancies. Little is known about their epidemiology at initial presentation in Europe and about potential changes over time. OBJECTIVES: The aim of this retrospective study was to analyse the frequency of PCLs in the French Cutaneous Lymphoma Registry (GFELC) and to describe the demography of patients. METHODS: Patients with a centrally validated diagnosis of primary PCL, diagnosed between 2005 and 2019, were included. RESULTS: The calculated incidence was unprecedently high at 1·06 per 100 000 person-years. The number of included patients increased yearly. Most PCL subtypes were more frequent in male patients, diagnosed at a median age of 60 years. The relative frequency of rare CTCL remained stable, the proportion of classical mycosis fungoides (MF) decreased, and the frequency of its variants (e.g. folliculotropic MF) increased. Similar patterns were observed for CBCL; for example, the proportion of marginal-zone CBCL increased over time. CONCLUSIONS: Changes in PCL frequencies may be explained by the emergence of new diagnostic criteria and better description of the entities in the most recent PCL classification. Moreover, we propose that an algorithm should be developed to confirm the diagnosis of PCL by central validation of the cases.

[Mycosis fungoides bullosa: A rare clinical presentation]. / Mycosis fongoïde bulleux : une présentation clinique exceptionnelle.

INTRODUCTION: Mycosis fungoides (MF) is the most common form of cutaneous lymphoma and usually manifests as erythematous and scaly patches or plaques. Its phenotypic or histologic presentation can be heterogeneous. Herein we report a very rare form of MF bullosa. PATIENTS AND METHODS: A 73-year-old man presented with a 4-month history of erythematous, scaly and itchy plaques on the trunk, as well as blistering lesions present for 2 months and which appeared on the trunk and lower limbs, both on patches of MF and on apparently healthy skin. Histopathology confirmed the diagnosis of bullous mycosis fungoides. Gene rearrangement of TCR showed a monoclonal profile in the skin. The hypothesis of bullous pemphigoid was ruled out by additional exams. Our patient was successively treated with combined interferon, bexarotene and methotrexate, followed by vorinostat, resulting in partial remission. DISCUSSION: Cases of bullous MF are very rare. In the literature, the clinical presentation is heterogeneous, with tense or flaccid bullae that can occur on unaffected skin or on erythematous plaques. The bullae generally appear after the plaques. The histologic blister site may be subepidermal or, more rarely, intra-epidermal. The exact mechanism of blister formation is not clear. Its treatment is poorly codified but follows the usual treatment of MF in its classical form. CONCLUSION: Bullous MF is a very rare entity that can mimic autoimmune blistering disease, and this diagnosis must therefore be ruled out.

[Hypercalcaemia and malignant lymphoma. One case report]. / Hypercalcémie et lymphome. A propos d'un cas.

Hypercalcaemia occurs in less than 15% of lymphomas but is associated with a poor prognosis. A patient known to have a testicular lymphoma was admitted because of asthenia and weight loss. Hypercalcemia and high serum level of calcitriol were found. A high-grade lymphoma attesting a Richter's syndrome was diagnosed. Palliative treatment consisting in corticosteroids and pamidronate was instituted. Hypercalcaemia in lymphoma has a poor prognosis and is often attributed to an acquired uncontrolled vitamin D 1-alpha-hydroxylase activity by the macrophages close to the lymphomatous cells. Influences of TNFalpha, interleukine 6 and PTHrp are also reported.

Bone microarchitecture in males with corticosteroid-induced osteoporosis.

SUMMARY: Microarchitectural changes in trabecular bone were analyzed by microcomputed tomography (microCT) and histomorphometry in 24 patients with corticosteroid-induced osteoporosis. The microCT images revealed a reduction in trabecular thickness only on frequency distribution curves, with no increase in trabecular separation. Trabecular plate thinning and perforations were easily identified. INTRODUCTION: Corticosteroid-induced osteoporosis (CSIOP) is mediated by direct actions of the drug on bone cells. The result is a decrease in trabecular bone mass and a reduction in trabecular thickness, but connectivity is believed to remain rather well preserved. METHODS: Twenty-four transiliac bone biopsies from patients with CSIOP were studied conjointly by histomorphometry [with two-dimensional (2D) architectural descriptors] and microCT (with 3D analysis of trabecular characteristics, including trabecular thickness and separation). The frequency distribution of thickness and separation were compared with data obtained in nine control subjects. RESULTS: 2D histomorphometry revealed a decrease in bone volume and trabecular thickness in the bone biopsies of the CSIOP patients when compared to those of the controls. MicroCT appeared to be able to identify the reduction in thickness only when the frequency distribution of trabecular thickness was computed. No difference for the curves of the frequency distribution of trabecular separation was evidenced between patients and controls. MicroCT and 2D histomorphometric results were correlated, but 2D analysis appeared to be more sensitive. However, microCT identified a very specific thinning of the trabecular plates in their center that corresponds to the earlier stages of perforations. CONCLUSION: Trabecular plate thinning can be observed and perforations occur on very thin plates in CSIOP patients.

[Malignant non-Hodgkin lymphoma presenting with Garcin's syndrome]. / Syndrome de Garcin révélant un lymphome malin non hodgkinien.

INTRODUCTION: R Garcin described progressive unilateral cranial nerve palsy in 1926. Garcin syndrome is characterized by progressive involvement of the cranial nerves culminating in total unilateral paralysis of all cranial nerves. Carcinoma of the skull base or ENT regions is the most common etiology. CASE REPORT: A 74-year-old man developed signs involving the left Vth (V2 and V3) cranial nerve then the VIth, VIIth and VIIIth cranial nerves and finally the IXth and Xth. MRI showed involvement of these cranial nerves with gadolinium uptake and involvement of the pons at the terminal phase. Careful ENT explorations failed to reveal a cause. The lymphocyte count was elevated in the cerebrospinal fluid. The patient died one year after diagnosis and the general autopsy was normal. The neuropathological studies led to the post-mortem diagnosis of type B non-Hodgkin lymphoma. CONCLUSION: In patients with Garcin syndrome, lymphoma is a possible diagnosis when carcinoma of the ENT regions or of the skull bases are not present.