Association of both Langerhans cell histiocytosis and Erdheim-Chester disease linked to the BRAFV600E mutation.

Histiocytoses are a group of heterogeneous diseases that mostly comprise Langerhans cell histiocytosis (LCH) and non-LCH. The association of LCH with non-LCH is exceptional. We report 23 patients with biopsy-proven LCH associated with Erdheim-Chester disease (ECD) (mixed histiocytosis) and discuss the significance of this association.

Recalcitrant pseudotumoral anogenital herpes simplex virus type 2 in HIV-infected patients: evidence for predominant B-lymphoplasmocytic infiltration and immunomodulators as effective therapeutic strategy.

Background: In patients with human immunodeficiency virus (HIV) infection, genital herpetic lesions may be extensive and tend to persist for longer periods; in addition, atypical hypertrophic, ulcerative, or pseudotumor forms have been reported, frequently showing resistance to acyclovir (ACV) treatment.

Methods: Between 2003 and 2011, 10 HIV-1-infected patients presenting with chronic pseudotumoral anogenital herpes simplex type 2 (HSV-2) infections were studied.

Results: All patients developed chronic, hypertrophic HSV-2 anogenital lesions with multilesional presentation in 7 cases and involvement of 2 anatomical sites in 6 of them.

Clinical efficacy of intravenous immunoglobulins for the treatment of dermatomyositis skin lesions without muscle disease.

Background: Treating dermatomyositis (DM) with isolated skin involvement is difficult and inconsistently performed. Intravenous immunoglobulins (IVIg) are recommended for corticoresistant or corticodependant DM, but only a few cases of IVIg use in DM with isolated skin involvement have been reported.

Design: We performed a retrospective monocentric study of 27 patients who were treated with IVIg for severe DM skin lesions (no or minor muscle involvement) after failure of photoprotection and at least one line of treatment.

Specific lymph node involvement in scleromyxedema: a new diagnostic entity for hypermetabolic lymphadenopathy.

Scleromyxedema is a generalized skin disease mostly associated with monoclonal gammopathy. In its chronic course, it can lead to systemic disorders related to mucin deposits in organs. We describe here specific lymph node involvement, hitherto not reported in scleromyxedema.

Intravenous immunoglobulins in difficult-to-treat ulcerated livedoid vasculopathy: five cases and a literature review.

Background: Livedoid vasculopathy (LV) is a thrombotic vasculopathy of the skin of unknown origin. No treatment has been validated in this indication, but case reports suggest the successful use of intravenous immunoglobulins (IVIG) in LV.

Methods: Outcomes in five patients treated with IVIG for treatment-resistant ulcerated LV were retrospectively analyzed.