Summary
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.
What's already known about this topic?
Linear IgA bullous dermatosis (LABD) is characterized by pure or predominant IgA deposits at the dermoepidermal junction.
In adults, the clinical presentation is heterogeneous and antigen targets vary widely.
Although LABD has a reputation for being a benign disease, its long‐term evolution remains unknown, especially in adults.
What does this study add?
Based on one of the largest analysed series of adult patients with idiopathic LABD, immunoelectron microscopy and immunological findings confirmed the wide diversity of LABD, overlapping with IgA‐related bullous pemphigoid, cicatricial pemphigoid and epidermolysis bullosa acquisita.
One‐third of the patients achieved sustained complete remissions and two‐thirds had chronic or relapsing disease. Age > 70 years and absence of mucous membrane involvement were significantly associated with complete remission.
What are the clinical implications of the work?
Autoimmune bullous disease in patients < 70 years old, with mucous membrane involvement and pure or predominant linear IgA deposits at the dermoepidermal or chorioepithelial junction, supporting a diagnosis of LABD, are significantly more likely to have a chronic evolution.
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Linked Comment: Ishii. Br J Dermatol 2017; 177:16–17
Summary
Background
Anti‐p200 pemphigoid is a rare autoimmune blistering disease (AIBD) of the dermoepidermal junction, characterized by autoantibodies to laminin γ1. The clinical course of anti‐p200 ...pemphigoid in patients remains poorly investigated.
Objectives
We aimed to describe the clinical and immunological features and the course of a series of patients with anti‐p200 pemphigoid.
Methods
We conducted a retrospective study by immunoblotting detection of sera on 200‐kDa dermal protein extracts from the register of the French reference centre for AIBD. We recorded the clinical and immunological features and the course of patients.
Results
A total of 14 patients with a mean age 81·6 ± 6·5 years were included. Only one patient had an associated neurological condition and one had psoriasis. Twelve patients had atypical clinical presentation, including eczematous, urticarial, prurigo‐like, dyshydrosis‐like and rosette‐like skin lesions. Eight patients (57%) had mucosal involvement. Immunoblot analysis of sera on dermal and epidermal extracts showed a 200‐kDa band in 14 and 10 cases, respectively. All eight of the sera tested by enzyme‐linked immunosorbent assay detected recombinant human laminin γ1. Disease control was obtained in six of nine patients treated with topical corticosteroids, and four of five patients who received systemic treatment. Seven patients relapsed (50%) and five patients (36%) died during the median follow‐up time of 12·6 months. At the end of the study, only one of the nine living patients was in complete remission off therapy.
Conclusions
Many patients with anti‐p200 pemphigoid had heterogeneous clinical presentation and a more severe prognosis than previously suspected.
What's already known about this topic?
Anti‐p200 pemphigoid is a recently described rare autoimmune subepidermal blistering disease.
The diagnosis of anti‐p200 pemphigoid requires immunoblotting detection of serum antibodies directed against a 200‐kDa dermal protein, corresponding to laminin γ1.
Anti‐p200 pemphigoid has been associated with psoriasis and patients are thought to have a favourable prognosis.
What does this study add?
Patients with anti‐p200 pemphigoid had heterogeneous clinical presentation, which did not allow clear differentiation between anti‐p200 pemphigoid and other autoimmune bullous diseases of the dermoepidermal junction.
Relapses were frequently observed despite the use of systemic treatments in most patients.
We observed a high mortality rate, which was close to that of bullous pemphigoid.
Linked Comment: Geller and Sprecher. Br J Dermatol 2016; 175:676–677.
ObjectiveTo assess the prevalence of skeletal dysplasias (SDs) in patients with idiopathic short stature (ISS) or small for gestational age (SGA) status.SettingRare Endocrine/Growth Diseases Center ...in Paris, France.DesignA prospective study on consecutive patients with ISS and SGA enrolled from 2004 to 2009.MethodWe used a standardized workup to classify patients into well-established diagnostic categories. Of 713 patients with ISS (n=417) or SGA status (n=296), 50.9% underwent a skeletal survey. We chose patients labeled normal or with a prepubertal slowdown of growth as a comparison group.ResultsDiagnoses were ISS (16.9%), SGA (13.5%), normal growth (24.5%), transient growth rate slowing (17.3%), endocrine dysfunction (12%), genetic syndrome (8.9%), chronic disease (5.1%), and known SD (1.8%). SD was found in 20.9% of SGA and 21.8% ISS patients and in only 13.2% in our comparison group. SD prevalence was significantly higher in the ISS group than in the comparison group, especially (50%) for patients having at least one parent whose height was <−2 SDS. Dyschondrosteosis and hypochondroplasia were the most frequently identified SD, and genetic anomaly was found in 61.5 and 30% respectively. Subtle SD was found equally in the three groups and require long-term growth follow-up to evaluate the impact on final height.ConclusionSD may explain more than 20% of cases of growth retardation ascribed to ISS or SGA, and this proportion is higher when parental height is <−2 SDS. A skeletal survey should be obtained in patients with delayed growth in a context of ISS or SGA.
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