Epidermal Langerhans Cells in Patients with Primary Sjögren's Syndrome

Abstract

IgG-binding to the surface of Langerhans cells (LC) is a feature of primary Sjögren's syndrome and might be associated with qualitative and quantitative defects in LC. Fifteen patients with primary Sjögren's syndrome and 15 healthy controls had a punch biopsy performed from clinically unaffected skin. LC were enumerated in epidermal sheets by an immunoperoxidase technique, as well as demonstrated by immunofluorescence in vertical sections, which were furthermore examined for in vivo deposits of immunoglobulins, fibrinogen and C3. A significant (P less than 0.05) reduction in number of LC, measured on epidermal sheets, was found in patients as compared with controls. Examination of vertical sections did not reveal abnormalities in morphology or localisation of epidermal LC. Intraepidermal IgG deposits were demonstrated in 7/15 patients. In vivo deposits were not found in any of the controls. The density of epidermal LC was not correlated to the presence of intraepidermal IgG deposits.

IMMUNOHISTOCHEMICAL CHARACTERIZATION OF INTRAEPIDERMAL IN VIVO IgG DEPOSITS IN PATIENTS WITH PRIMARY SJÖGREN'S SYNDROME

“…The double-labelling experiments demonstrated that IgG is bound to OKT6-positive LC, but also to a certain degree-to keratinocytes. This is in agreement with a previous study (8), but in the present investigation we stained LC with TRITClabelled antibodies in order to maintain FITC as marker for the IgG staining. It is well-known that epidermal Langerhans cells have surface-bound Fc y-receptors (1 3), but it has recently been reported that keratinocytes also have Fc y-receptors (1).…”

“…The applied monoclonal antibodies against the IgG 1-4 subclasses were chosen because they have been found in a recent IUIS/WHO collaborative study (3) to be reactive and sufficiently specific for an immunohistologic fluorescence technique. We detected deposits of IgG 1 and IgG3 exclusively in the epidermis, in agreement with previous findings (6,8). This, and the observation that the IgG deposits are unrelated to P-IgG levels ( 1 I), indicate a selective binding of IgG to the epidermis.…”

“…Immunohistological investigations of clinically unaffected skin of patients with primary SS have shown intra-epidermal in vivo deposits of IgG in 68% of the cases (7,8,11). We have furthermore found similar in vivo deposits in oral labial mucosa (9) and uterine cervical mucosa (10) of these patients.…”

Late neonatal lupus erythematosus onset in a child born of a mother with primary Sjogren's syndrome

“…Immunofluorescence showed deposition of IgM and C3, and IgG deposition in the basal and suprabasal layer but not in the dermoepidermal junction zone. 9 The changes seen are thus compatible with NLE and part of the NLS.…”

Dendritic cells in autoimmune diseases and neuroinflammatory disorders

“…Various subtypes of DCs found within the inflammatory lesions observed in SS have been reported (83)(84)(85)(86)(87)(88). According to Oxholm et al (89), a characteristic feature of SS is IgG-binding to the surface of lDCs. They further demonstrated a qualitative and quantitative defect in lDCs as measured on epidermal sheets of SS patients.…”