Oral manifestations of sickle cell disease.

Sickle cell disease is one of the most common autosomal recessive genetic diseases. It gives rise to abnormally shaped red blood cells with altered function, the primary clinical features being haemolytic anaemia and vascular occlusion. Acute complications are frequent and variable and include chest syndrome, stroke, infection mainly due to asplenia, bone pain and priapism. Other chronic complications which can occur are bone necrosis, nephropathy and heart, lung and skin disorders. Oral lesions are also very common and include aseptic pulp necrosis, mucosal damage due to anaemia, fungal infections due to numerous antibiotic therapies, dental eruption delays, bone pain and osteomyelitis of the maxilla, and oral neuropathies, including of the mental nerve of the chin. The oral care of sickle cell patients requires specific precautions such as good management of local anaesthetics, rigorous anti-infective prophylaxis as well as controlled prescription of analgesics. Regular oral follow-up of sickle cell patients is necessary.

[Autoimmune bullous diseases with gingival expression: A proposed non-iatrogenic gingival biopsy technique]. / Maladies bulleuses auto-immunes à expression gingivale : proposition d'une technique de biopsie gingivale non iatrogène.

BACKGROUND: Gingival expression of autoimmune bullous diseases (AIBD) may be inaugural, exclusive or dominant (mucous membrane pemphigoid, pemphigus vulgaris). Histology and direct immunofluorescence are essential to diagnosis. The location of the biopsy and the surgical technique determine the histological quality of the tissue sample. However, gingival tissue is often considered fragile and easily impaired during biopsy. We suggest an original biopsy protocol for the gingival papillae that is simple to perform, non-iatrogenic, and readily accessible to all practitioners who usually treat AIBD patients presenting isolated gingival expression (dermatologists, stomatologists, odontology specialists, ENT specialists). PATIENTS AND METHODS: We conducted a retrospective study from 2012 to 2017 identifying all patients presenting AIBD with gingival expression for whom we performed papillary gingival biopsy for diagnostic ends. Our main objective was to determine the diagnostic efficacy and safety of this surgical technique. RESULTS: Over the study period, 34 papillary gingival biopsies were taken from 19 patients : 15 for histopathological examination and 19 for direct immunofluorescence. Of the 34 biopsies, only one could not be properly analyzed due to lack of epithelium and a second tissue sample was therefore necessary. No short- or long-term complications occurred during post-operative follow-up. CONCLUSION: Gingival papilla biopsy is perfectly suited to the histopathological and immunohistochemical examinations needed for diagnosis of AIBD with isolated gingival expression. This surgical technique shows great efficacy and very good safety. However, additional studies are necessary to confirm our preliminary results, in particular the absence of iatrogenic effects.

[Oral care recommendations for patients with oral autoimmune bullous diseases]. / Recommandations pour la prise en charge buccodentaire des patients atteints de maladies bulleuses auto-immunes avec atteinte buccale.

BACKGROUND: Autoimmune bullous diseases (AIBD) may cause chronic oral lesions that progress insidiously. AIMS: To provide recommendations for optimal oral-dental management of patients presenting AIBD with oral involvement. PATIENTS AND METHODS: In the absence of scientific studies with high levels of proof, these recommendations have been drawn up at two meetings by a committee of experts on AIBD comprising 7 dermatologists, 1 stomatologist, 1 maxillofacial surgeon, 2 odontologists and 4 parodontologists. RESULTS: The oral lesions associated with AIBD may be classified into three grades of severity: severe (generalised erosive gingivitis affecting at least 30% of dental sites), moderate (localised erosive gingivitis affecting less than 30% of dental sites) and controlled (no erosive oral lesions). Good oral-dental hygiene suited to the severity of the oral lesions, must be practised continually by these patients so as to avoid the formation of dental plaque, which aggravates symptoms. Dental and parodontal care must be considered in accordance with the severity grade of the oral lesions: in severe cases, the dental plaque must be eliminated manually with a curette, but several types of care (descaling, treatment for tooth decay, non-urgent extractions, etc.) must be suspended until the grade of severity is moderate or until the disease is stabilised.

Possible involvement of gelatinase A (MMP2) and gelatinase B (MMP9) in toxic epidermal necrolysis or Stevens-Johnson syndrome.

Toxic epidermal necrolysis (TEN) and Stevens-Johnson syndrome (SJS) are considered to be drug-induced diseases, and are characterized by extensive mucocutaneous disorder and epidermal necrosis which result in the detachment of the epidermis. Inactive and active forms of metalloproteinases (MMP2 and MMP9) secreted by skin explants maintained in organ culture for 72 h and in blister fluid from two TEN and three SJS patients were investigated. Interestingly, lesional skin from both the TEN and the SJS patients cultured for 3 days in conditioned medium showed high levels of both 72 kDa progelatinase A and 66 kDa activated gelatinase A, and the 66 kDa activated form was not observed in cultures of skin from control individuals. Furthermore, indirect immunodetection showed the presence of MMP2 and MMP9 in TEN and SJS patients' skin. Increased gelatinase activity in the culture medium of TEN and SJS skin maintained in organ culture and in blister fluid indicates that these gelatinases may be responsible for the detachment of the epidermis in these drug-induced necrolyses.

Protective effect of a vegetable extract from Lupinus albus (LU 105) on human gingival elastic fibers degradation by human leukocyte elastase.

The aim of this study was to determine if a vegetable extract from seeds of Lupinus albus (LU 105) has the capacity to inhibit human leukocyte elastase and/or protect gingival elastic fibers against proteolytic degradation. LU105 was extracted from seeds of L albus and is freely soluble in water. In this study the ex-vivo elastolytic activity of human leukocyte elastase and the potential inhibitory effect of LU 105 were determined using human gingival cryostat tissue sections and computerized morphometric analysis. The gingival tissue sections pre-treated or not with LU 105 were submitted to the action of human leukocyte elastase or submitted to the simultaneous action of human leukocyte elastase and LU 105, and then analyzed using automated image analysis. In such conditions, LU 105 at 0.1%, 0.01%, and 0.001% developed a dose dependent protection of gingival elastic fibers against enzymatic proteolysis due to human leukocyte elastase, and LU 105 at 0.1% or 0.01% was able to inhibit the elastolytic activity of leukocyte elastase itself. It is proposed that LU 105 is an option for the treatment of gingival inflammation in which leukocyte elastase is involved.

Effects of a vegetable extract from Lupinus albus (LU105) on the production of matrix metalloproteinases (MMP1, MMP2, MMP9) and tissue inhibitor of metalloproteinases (TIMP1, TIMP2) by human gingival fibroblasts in culture.

This study examined the effects of a vegetable extract from Lupinus albus (LU105) on MMPs and TIMPs secreted by human gingival fibroblasts in culture. LU105 was extracted from seeds of L. albus and is freely soluble in water. Gelatin zymography showed that control human gingival fibroblasts maintained in culture for 48 h express pro-MMP2 (progelatinase A) in the culture medium while the active form of MMP2 (gelatinase A), the active form of MMP9 (gelatinase B), and pro-MMP9 (progelatinase B) are not detected. Fibroblasts derived from inflamed gingiva expressed in the culture medium increased amounts of pro-MMP2 (progelatinase A) compared with controls and significant amounts of pro-MMP9 (progelatinase B). LU105 diminished the expression by gingival fibroblasts derived from inflamed tissue of both pro-MMP2 and pro-MMP9. Furthermore LU105 did not modify the amount of TIMP2 expressed in culture by controls or by gingival fibroblasts derived from inflamed tissue. TIMP1 and MMP1 significantly decreased when LU105 was added in the culture media of gingival fibroblasts derived from inflamed tissue compared with control fibroblasts. Thus LU105 seems to offer an opportunity to restore a correct balance between MMP2, MMP9, MMP1, and their natural inhibitors, i.e., TIMP1 and TIMP2 in human inflamed gingiva.