Plasma cell-directed therapies in monoclonal gammopathy-associated scleromyxedema.

Scleromyxedema is a rare skin and systemic mucinosis that is usually associated with monoclonal gammopathy (MG). In this French multicenter retrospective study of 33 patients, we investigated the clinical and therapeutic features of MG-associated scleromyxedema. Skin molecular signatures were analyzed using a transcriptomic approach. Skin symptoms included papular eruptions (100%), sclerodermoid features (91%), and leonine facies (39%). MG involved an immunoglobulin G isotype in all patients, with a predominant λ light chain (73%). Associated hematologic malignancies were diagnosed in 4 of 33 patients (12%) (smoldering myeloma, n = 2; chronic lymphoid leukemia, n = 1; and refractory cytopenia with multilineage dysplasia, n = 1). Carpal tunnel syndrome (33%), arthralgia (25%), and dermato-neuro syndrome (DNS) (18%) were the most common systemic complications. One patient with mucinous cardiopathy died of acute heart failure. High-dose IV immunoglobulin (HDIVig), alone or in combination with steroids, appeared to be quite effective in nonsevere cases (clinical complete response achieved in 13/31 patients). Plasma cell-directed therapies using lenalidomide and/or bortezomib with dexamethasone and HDIVig led to a significant improvement in severe cases (HDIVig refractory or cases with central nervous system or cardiac involvement). The emergency treatment of DNS with combined plasmapheresis, HDIVig, and high-dose corticosteroids induced the complete remission of neurological symptoms in 4 of 5 patients. Quantitative reverse-transcriptase polymerase chain reaction analysis of 6 scleromyxedema skin samples showed significantly higher profibrotic pathway levels (transforming growth factor ß and collagen-1) than in healthy skin. Prospective studies targeting plasma cell clones and/or fibrotic pathways are warranted for long-term scleromyxedema management.

Cutaneous secondary localization of multiple myeloma at Heparin injection sites.

We report two cases of multiple myeloma skin localizations at Heparin injections sites in patients followed at the University Hospital of Martinique. These skin manifestations on traumatized areas are a marker of aggressiveness in the natural history of multiple myeloma.

[Cutaneous malakoplakia: A rare pseudo-tumor to know in immunocompromised patients]. / Malakoplakie cutanée : une pseudo-tumeur rare à ne pas méconnaître chez l'immunodéprimé.

Cutaneous malakoplakia is a rare pseudo-tumor that occurs in immunocompromised patients. It is a reaction to an infection caused by Gram negative bacteria. The clinical presentation is nonspecific and the diagnosis is histological. The evolution is recurrent and the combination of a surgical treatment, antibiotics and adaptation of immunosuppressive therapy is necessary to cure the disease. The emergence of antibiotic resistance in bacteria responsible for the pathology can complicate the treatment and require additional microbial sampling. We report a case that occurred in a renal transplant patient with a complex diagnostic and therapeutic management.

Acquired Lymphangioma Circumscriptum of the Penis Treated by Electrocoagulation.

Lymphangioma circumscriptum (LC) is a vascular malformation resulting from a developmental anomaly of the superficial lymphatic system of the skin. It is benign albeit uncommon. LC less frequently occurs on the penis. LC may be either congenital or acquired. Acquired cases appear to develop most frequently after interventions in the area or underlying pathologies. It is often mistaken for genital warts or molluscum contagiosum. We report here about a case of LC misdiagnosed with genital warts for 15 years. A biopsy eventually provided histopathological evidence. Various treatments are available for LC including surgical excision (which is the gold standard), CO2 laser ablation, cryotherapy, flash lamp pulsed dye laser, and electrocoagulation therapy. For our patient, one session of electrocoagulation was performed under local anesthesia. This treatment allowed an almost complete regression of the lesion without recurrence after 3 years of follow-up. Electrocoagulation may be an efficient treatment for LC in locations that may be surgically challenging such as penis.

Kite-surfers' sun risk in the tropics.

A preliminary inquiry, conducted on Martinique Island, sought to determine kite-surfers' sun-protection knowledge and behaviours. Results revealed good levels of knowledge and behaviours, but very frequent sunburns (74% had at least one sunburn during the last 6 months), with particularly severe sunburns exclusively among vacationers. These results argue for the double need to focus sunburn-prevention programs on vacationers and technically adapt sun-protection means to a tropical maritime environment.

Human infection with Shewanella putrefaciens and S. algae: report of 16 cases in Martinique and review of the literature.

Shewanella spp. are saprophytic bacteria that are part of the marine microflora in warm climates and are rarely pathogenic. However, Shewanella spp. infections are being increasingly reported, and there has been no comprehensive review of the literature describing these infections. This article reports 16 cases of Shewanella spp. infections in Martinique since 1997 and reviews another 239 cases reported in the literature since 1973. Patients experienced soft tissue infections, ear infection, or abdominal and biliary tract infections. A skin or mucosal portal of entry was found for 53% of the patients and exposure to the marine environment was reported for 44%; 79% of patients had an underlying condition. Bacteriema were frequent (28%). Most (87%) patients recovered, although ear infections can become chronic. Death occurred in 13% of the patients. Most Shewanella spp. isolates are susceptible to cefotaxime (95%), piperacillin and tazobactam (98%), gentamicin (99%), and ciprofloxacin (94%).