Idiopathic linear IgA bullous dermatosis: prognostic factors based on a case series of 72 adults.

BACKGROUND: Linear IgA bullous dermatosis (LABD) is a clinically and immunologically heterogeneous, subepidermal, autoimmune bullous disease (AIBD), for which the long-term evolution is poorly described. OBJECTIVES: To investigate the clinical and immunological characteristics, follow-up and prognostic factors of adult idiopathic LABD. METHODS: This retrospective study, conducted in our AIBD referral centre, included adults, diagnosed between 1995 and 2012, with idiopathic LABD, defined as pure or predominant IgA deposits by direct immunofluorescence. Clinical, histological and immunological findings were collected from charts. Standard histology was systematically reviewed, and indirect immunofluorescence (IIF) on salt-split skin (SSS) and immunoblots (IBs) on amniotic membrane extracts using anti-IgA secondary antibodies were performed, when biopsies and sera obtained at diagnosis were available. Prognostic factors for complete remission (CR) were identified using univariate and multivariate analyses. RESULTS: Of the 72 patients included (median age 54 years), 60% had mucous membrane (MM) involvement. IgA IIF on SSS was positive for 21 of 35 patients tested; 15 had epidermal and dermal labellings. Immunoelectron microscopy performed on the biopsies of 31 patients labelled lamina lucida (LL) (26%), lamina densa (23%), anchoring-fibril zone (AFz) (19%) and LL+AFz (23%). Of the 34 IgA IBs, 22 were positive, mostly for LAD-1/LABD97 (44%) and full-length BP180 (33%). The median follow-up was 39 months. Overall, 24 patients (36%) achieved sustained CR, 19 (29%) relapsed and 35% had chronic disease. CR was significantly associated with age > 70 years or no MM involvement. No prognostic immunological factor was identified. CONCLUSIONS: Patients with LABD who are < 70 years old and have MM involvement are at risk for chronic evolution.

[Acquired cutis laxa and myeloma: large vacuolated cells in the dermis]. / Cutis laxa acquise généralisée associée à un myélome: découverte de grandes cellules vacuolisées dermiques.

INTRODUCTION: Cutis laxa is a rare disorder characterized by loss of elastic tissue. Several organs are often involved such as the skin, lungs, heart, digestive system or genitourinary tract. It may be inherited or acquired, generalized or localized. Its pathogenesis is unclear. Association of acquired cutis laxa with myeloma or plasma cell dyscrasia is very rare. We report a case of acquired cutis laxa associated with a myeloma. CASE REPORT: A 59 year-old woman was admitted for skin hyperlaxity present for a number of years. Light microscopic examination of a skin sample revealed fragmented elastic fibers. Electron microscopic examination of the elastic network demonstrated numerous large vacuolated cells with the appearance of macrophages around abnormal elastic and collagen fibers of the reticular dermis. In addition, a stage-1 IgG lambda myeloma was detected. The patient was treated by thalidomide for one year. After this treatment, electron microscopy examination did not reveal any large vacuolated cells in the dermis, and elastic and collagen fibers were not modified and skin laxity seemed to be stabilized. DISCUSSION: Acquired cutis laxa may be associated with many systemic diseases or can appear after inflammatory skin diseases. Seven cases of generalized cutis laxa associated with myeloma and four cases associated with plasma cell dyscrasia have been reported in the literature. In our case, as in 2 previously described cases, large vacuolated cells resembling macrophages were seen in the dermis. They were thought to play a role in cutis laxa.

Glycation stimulates cutaneous monocyte differentiation in reconstructed skin in vitro.

Glycation reaction is a recognized mechanism related to chronological aging. Previous investigations in cutaneous biology have considered the effect of glycation on the dermal matrix molecules, involved in tissue stiffening during skin aging. However, little is known about a possible direct effect of glycation upon cell differentiation. To address such issue, the effect of glycation has been re-investigated in a reconstructed skin model integrating monocytes that are cells capable of differentiating according to different pathways. The results showed that, in the absence of glycation, a small number of these CD45+ cells could differentiate either into dendritic-like cells (DC-SIGN+, BDC1a+, DC-LAMP+) or macrophage- like cells (CD14+, CD68+, CD163+) whereas, with glycation, the number of monocytes, dendritic cells, macrophage-like cells were found surprisingly increased. In-vivo our results showed also that dendritic and macrophage-like cells were increased and suggest a possible link with the age-dependent glycation level in the skin. In addition, we found that, unlike fibroblasts incorporated in the reconstructed skin, these cells expressed specific receptors for AGEs (RAGE and SRA). Taken altogether, our data show that cells of the monocyte lineage, in the presence of AGEs, can differentiate into dendritic or macrophage-like cells and could lead to a micro inflammatory environment.

Ivermectin-responsive crusted scabies in HTLV1 carrier.

We report a case of HTLV1 infection revealed by crusted scabies and widespread dermatophytosis in an African woman. HTLV1 infection was not complicated by adult T cell leukemia or myelopathy. Crusted scabies is a marker of HTLV1 infection. The importance of oral ivermectin therapy in crusted scabies is emphasized.