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PubMed2003

Position paper: oral features of mucocutaneous disorders.

Part of periodontology involves the diagnosis and treatment of a variety of non-plaque-related diseases of the periodontium. The International Workshop for a Classification of Periodontal Diseases and Conditions noted that the periodontist may be called upon to manage non-plaque-related mucocutaneous disorders either alone, or as part of a treatment team consisting of physicians, dentists or other allied health care professionals. This informational paper will review the etiology, clinical manifestations, diagnosis, and treatment of the most common chronic mucocutaneous diseases, including those that may present as desquamative gingivitis or intraoral vesiculobullous lesions. This paper is intended for the use of periodontists and other members of the dental profession.

Gingivitis↗
PubMed1994

Update on immunofluorescent testing in bullous diseases and lupus erythematosus.

One of the earliest methods for investigating the immunologic basis of disease was the use of immunofluorescence (IF) on skin biopsy specimens and serum. IF can be used to detect immunoglobulins, complement components, and fibrin. In some diseases, the IF findings are disease specific and diagnostic, especially in certain bullous diseases. The use of IF testing has increased with the recent development of the salt split skin technique. The newer IF findings in some well-established immunodermatologic diseases and several recently described diseases are discussed. A brief description of the techniques involved in direct and indirect IF is also presented.

Epidermolysis Bullosa Acquisita↗
PubMed1997

Autoimmune bullous diseases.

Patients with autoimmune bullous diseases are occasionally encountered in primary care practice, usually in middle-aged and older patients. The differential diagnosis includes nonimmune causes, such as contact dermatitis, infections and bullous reactions to drugs or insect bites. An autoimmune blistering disease may be distinguished by the age of the patient when the disease first appears, the morphology and distribution of the lesions and the presence or absence of mucosal lesions and scarring. Because the clinical presentations of blistering disorders are often similar, special immunofluorescence tests are used to confirm the specific diagnosis. Since diagnosis and management of an autoimmune bullous disease may involve systems other than the skin, coordination by the primary care physician is crucial.

Autoimmune Diseases↗
PubMed1951

The topical and systemic use of cortisone in dermatology.

Part I of this report deals with the topical use of cortisone in a variety of skin diseases. Fifteen patients with chronic discoid lupus erythematosus, four patients with necrobiosis lipoidica diabeticorum, four with psoriasis, one with lichen planus and one with granuloma annulare were treated with cortisone ointment. All the patients with chronic discoid lupus erythematosus had some degree of improvement. In two patients with chronic lupus erythematosus, complete clearing of the eruption occurred. In four patients with necrobiosis lipoidica diabeticorum remarkable involution resulted. Patients with psoriasis, lichen planus and granuloma annulare were not benefited. Part II deals with the systemic use of cortisone. Eight patients with severe serum sickness-like penicillin reaction responded dramatically to parenterally administered cortisone. In two cases of pemphigus vulgaris and one case of Sulzberger-Garbe disease, the disease was kept in remission with cortisone administered intramuscularly as well as orally. Partial improvement resulted in a case of localized myxedema associated with malignant exophthalmus. Two patients with exfoliative dermatitis due to therapy with heavy metals responded dramatically to cortisone. No beneficial effects were noted in patients with chronic urticaria and atopic dermatitis.The systemic use of ACTH and cortisone in dermatology at present should be confined to patients with known fatal or hopelessly incapacitating diseases and to patients with extreme hypersensitivity reactions which may be protracted or life-endangering, and which can be controlled or cured with a relatively small total dosage of the agents in a short time.

Administration, Topical↗
PubMed1980

Internal disorders associated with bullous disease of the skin. A critical review.

Acquired bullous dermatoses, including pemphigus, bullous pemphigoid (BP), dermatitis herpetiformis (DH), and porphyria cutanea tarda (PCT), have been reported in association with multiple internal disorders. These associations, as well as those cases of bullous lesions in specific systemic disorders, may prove to be important markers of internal disease. Patients with acquired bullous disorders may require specialized evaluation or follow-up. Pemphigus is associated with thymoma and/or myasthenia gravis; however, the course of disease is rarely affected. Pemphigus, pemphigoid, and DH are associated with other autoimmune disorders. Particularly important are the associations of pemphigoid and rheumatoid arthritis (RA) and DH and thyroid disorders. PCT may occur with cutaneous lupus erythematosus (LE). Malignancy is rarely associated with bullous dermatoses except coincidentally, with the exception of porphyria and hepatic tumors, and DH and lymphoma of the gastrointestinal tract.

Age Factors↗
PubMedDate not supplied

Corticosteroid-induced osteoporosis.

Since Harvey Cushing first noted the coexistence of excess cortisol and loss of skeletal mass over 50 years ago, it has been accepted that supraphysiologic doses of corticosteroids cause clinically significant bone loss. Currently, high-dose oral corticosteroids are used to treat people with a variety of medical conditions, including: rheumatic diseases, such as rheumatoid arthritis, polymyalgia rheumatica, systemic lupus erythematosus and vasculitis; inflammatory lung diseases, like asthma; gastrointestinal diseases, such as inflammatory bowel disease and chronic liver disease; skin diseases, in particular pemphigus, and more recently those who have undergone transplantation. Clinically significant bone loss occurs in the vast majority of patients exposed to corticosteroids, and fractures at the spine and hip have been reported with corticosteroid use. Between 30 and 50 percent of patients taking long-term corticosteroids will experience fractures. Today, fractures due to corticosteroid-induced osteoporosis may be prevented. A number of well-designed randomized controlled trials have been conducted that demonstrate preservation and, in some instances, actual increases in bone mass with the use of appropriate drug treatment. Some have even demonstrated reductions in fracture risk. As a result, it is extremely important for clinicians to appreciate the very high risk for vertebral fracture, particularly in postmenopausal women on corticosteroids.

Adrenal Cortex Hormones↗
PubMed2000

Pemphigus vulgaris in association with silicosis.

We report on a sixty-seven year old miner with pemphigus vulgaris characterised clinically by a three month history of relapsing oral lesions and blisters/erosions on the trunk, axillae and extremities, histologically by suprabasal cleavage due to acantholysis, immunologically by the epidermal intercellular net-like pattern due to deposits of IgG- and IgM-antibodies and complement C3 in the direct immunofluorescence as well as by serum antibodies to desmoglein 3 (130 KD) and plakoglobin (85 KD) by immunoblotting analysis. Silicosis has already been known for 6 years. In addition, antinuclear antibodies, anti-ssDNA-antibodies and anti-topoisomerase antibodies were found. Clinical improvement and clearing of skin symptoms could be achieved by systemic steroids in combination with cyclophosphamide. However, the patient died of sepsis deriving from recalcitrant pneumonia. Although the association of silicosis with various autoimmune diseases such as systemic sclerosis, systemic lupus erythematosus, rheumatoid arthritis and dermatomyositis has been reported many times, our patient is, to the best of our knowledge, the second case with features of the two diseases: pemphigus vulgaris and silicosis.

Aged↗
PubMed1994

Clinical toxicity of the interferons.

Since their initial description in 1957, the interferons (IFNs) have been increasingly used to treat a wide array of diseases. Acute adverse effects, i.e. 'flu-like' syndromes, hypo- or hypertension, tachycardia, headache, myalgias and gastrointestinal disorders, occur within the first hour or day after starting treatment. They are seldom treatment-limiting and are easily manageable. Sub-acute and chronic effects develop after several days, usually within 2 and 4 weeks of therapy. The most typical is neurological toxicity, including fatigue/asthenia, and behavioural and cognitive changes. Such symptoms may seriously impair quality of life and result in treatment discontinuation. Seizures have seldom been described. Other infrequent central nervous system adverse effects include vertigo, cramp and oculomotor nerve paralysis. Distal paraesthesias and peripheral neuropathy have been reported. IFN-associated autoimmunity is quite rare but a matter of concern. Biological or clinical manifestations usually require several months to become apparent. Autoantibodies have been shown to develop in most patients but have been inconsistently associated with clinical symptoms of systemic lupus erythematosus, rheumatoid-like arthritis and thyroiditis. Both hypo- and hyperthyroidism have been described but are usually reversible. Other infrequent autoimmune reactions include diabetes, pemphigus and worsening of multiple sclerosis. Although several patients present with a pre-existing autoimmune disorder, no predisposing factor has been clearly established. While hypotension and tachycardia are the most frequent acute cardiovascular complications, a few additional cases of cardiac arrhythmias and myocardial ischaemia have been reported after a short course or several weeks of treatment. These latter complications do not appear to be dose-dependent or age-related. Isolated cases of congestive heart failure have also been described. Mild proteinuria has been observed in 15 to 25% of patients, but acute renal toxicity is uncommon. A transient rise in serum aminotransferase levels is frequently noted during the first stage of therapy, especially in patients receiving the highest dosages. Direct hepatotoxicity is extremely rare. Autoimmune hepatitis, which is ill-diagnosed as chronic viral hepatitis, and de novo induction of autoimmune hepatitis, account for the majority of liver diseases. Haematotoxicity is relatively common but mild to moderate, and develops gradually during the first weeks of treatment. Neutropenia is the most common haematological toxicity, but is usually not dose-limiting and resolves rapidly upon drug discontinuation. Myelosuppression, autoimmune and immune allergic haemolytic anaemias and thrombocytopenias have seldom been described. Cutaneous adverse effects comprised nonspecific erythema and hair loss and, less frequently, vasculitis, local ulcerations at the site of injection and exacerbation of psoriasis.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗
PubMed1990

[Oral manifestations of skin diseases].

There exists a multitude of systemic diseases which exhibit consequential oral manifestations. Dermatologic disorders frequently involve intraoral and perioral tissues which dental practitioners should be familiar with. A compilation of disorders including; bullous diseases (such as Pemphigus Vulgaris, Erythema Multiforme, Pemphigoid, Dermatitis Herpetiformis, Epidermolysis Bullosa, and Darier's disease), as well as non bullous diseases (such as Lichen Planus, Psoriasis, Lupus erythematosus, Scleroderma, and Perioral Dermatitis); and their oral manifestations are presented in order to facilitate their early diagnosis and subsequent treatment.

Darier Disease↗