Paraneoplastic pemphigus: a review of the literature.

Paraneoplastic pemphigus is a relatively rare but highly significant acquired mucocutaneous disorder. The condition typically presents in patients with previously diagnosed lymphoreticular disease, primarily malignancies (non-Hodgkin's lymphoma; chronic lymphocytic leukemia). Multiple sites of involvement are common, with the skin affected by a polymorphous eruption that includes lichenoid plaques as well as bullae. The oral lesions occur in all cases and present as painful widespread shallow ulcers with hemorrhagic crusting of the lips. Conjunctival involvement is also seen and may result in scarring. Pulmonary lesions are an ominous sign. Histopathologically, intraepithelial and subepithelial clefting associated with interface dermatitis are seen. A variety of autoantibodies directed against desmoplakins and desmogleins can be identified with immunoprecipitation studies. Treatment with immunosuppressive agents may result in some resolution of the disease, but the prognosis for paraneoplastic pemphigus is considered to be poor.

Two considerations for patients with psoriasis and their clinicians: what defines mild, moderate, and severe psoriasis? What constitutes a clinically significant improvement when treating psoriasis?

The definitions of psoriasis severity and clinically significant improvement in psoriasis are used to classify treatments, obtain Food and Drug Administration approval, and determine product labeling and reimbursement. The Medical Advisory Board of the National Psoriasis Foundation has addressed these issues because of their importance in the clinical trials that are conducted to gain FDA approval of indications. Narrow indications, which are without a sound rational basis, will-in this era of constant oversight by third party payers-affect physicians' ability to manage patients with psoriasis. Body surface area (BSA) is usually used to define severity for clinical trials. It is not optimal for defining psoriasis severity because there are some patients with low BSA involvement who have very severe psoriasis and some patients with high BSA involvement who have mild psoriasis. We conclude that a quality of life (QOL) standard is better than BSA measurement for identifying patients with severe psoriasis. The second issue is what defines clinically significant improvement for patients with psoriasis. Setting an arbitrarily high criterion of clinical efficacy for new psoriasis treatments will likely limit the development and approval of useful treatments. To maximize the availability of useful psoriasis treatments, it is our thesis that psoriasis treatments should be approved when they have been shown to produce a statistically significant level of improvement in well-designed clinical trials.

Cicatrizing conjunctivitis associated with paraneoplastic lichen planus.

PURPOSE: To report two cases of cicatrizing conjunctivitis associated with paraneoplastic lichen planus. METHODS: Case reports. RESULTS: Two patients were examined because of redness and discomfort in both eyes. A 63-year-old woman with follicular, small-cleaved cell lymphoma had cicatrizing conjunctivitis, stomatitis, vulvitis, and skin lesions. A 25-year-old man with malignant thymoma had cicatrizing conjunctivitis, erosive stomatitis, and penile papules. Histopathologic studies of conjunctiva and skin biopsy specimens in the first patient and labial biopsy specimens in the second revealed lichen planus. CONCLUSION: Paraneoplastic lichen planus is a possible cause of cicatrizing conjunctivitis associated with inflammatory skin and mucous membrane disease.

Psoriasis: a clinical update on diagnosis and new therapies.

Psoriasis varies widely in its clinical expression, from a single fingernail pit to widespread disfiguring skin lesions and disabling arthritis. Treatments are divided into five levels, providing a framework for approaching this disease according to severity and recalcitrance to previous treatment. Powerful immunosuppressive drugs are showing some success in treating severe cases.

New and emerging therapies for diseases of the oral cavity.

It is obvious from the review of the literature that most treatments for oral diseases such as lichen planus, pemphigoid, and pemphigus are based on case reports, anecdotes, and small uncontrolled studies. Efforts must be made to perform more controlled studies to evaluate the efficacy of new treatments. Small numbers of patients at each site and multiple-drug therapy make this task difficult. Dermatologists should familiarize themselves with the newer immunosuppressive agents available. Use of these drugs requires knowledge of their pharmacokinetics and potential side effects, so that they may be used effectively and safely. Relatively low doses of azathioprine, cyclophosphamide, and cyclosporine should then be added to the dermatologist's armamentarium for the treatment of severe or recalcitrant diseases. Old drugs are resurfacing with new (but often off-label) uses as the underlying mechanisms of disease become understood. Thalidomide and mycophenolate mofetil are two examples of promising drugs for the future of dermatology.

Unresponsive eczematous dermatitis: a case of pancreatic cancer masquerading as cutaneous T cell lymphoma.

Skin diseases may be the presenting sign of malignancy, but strict criteria are required to make the diagnosis of a paraneoplastic syndrome. Common dermatoses may also herald an underlying malignancy without meeting these criteria. We report the case of an elderly man with an unresponsive eczematous dermatitis whose evaluation eventually revealed a pancreatic carcinoma. This case is presented to alert the clinician that a common dermatosis may be the first sign of an occult malignancy, especially if it behaves in an atypical or aggressive manner or is recalcitrant to standard therapies.

Selected drug complications and treatment conflicts in the presence of coexistent diseases.

The presence of different coexistent systemic diseases often times complicates the selection of the appropriate treatment of an underlying rheumatologic condition. In this article, some controversial treatment conflicts that are frequently encountered in the daily practice of rheumatology are clarified and guidelines for the best available therapeutic options are provided.

Lupus erythematosus associated with erythema multiforme: does Rowell's syndrome exist?

We describe a patient with lupus erythematosus who experienced an unusual erythema multiforme-like eruption suggestive of Rowell's syndrome. We compare our case and 9 other reports of lupus erythematosus associated with erythema multiforme to the 4 cases reported by Rowell. Our findings indicate that Rowell's original criteria are not well preserved. The coexistence of lupus erythematosus with erythema multiforme does not impart any unusual characteristics to either disease, and the immunologic disturbances in such patients are probably coincidental.

Allergic contact dermatitis from transdermal clonidine in a patient with mycosis fungoides.

Although therapy with transdermal clonidine is considered an effective method of hypertension control, this mode of delivery has been associated with localized dermal reactions in numerous patients. We present a patient with coexistent mycosis fungoides and allergic contact dermatitis from transdermal clonidine. The association of these two dermatologic processes has not been previously reported.

Contact hypersensitivity to mercury in amalgam restorations may mimic oral lichen planus.

Oral lichenoid lesions caused by hypersensitivity to mercury in amalgam fillings may mimic oral lichen planus on clinical and histologic examination. A positive patch test reaction to more than one mercurial allergen increases confidence in the diagnosis and justifies the removal and replacement of all amalgam fillings with those made of other materials. A complete remission may be expected about 3 months after the last amalgam filling is removed.

Squamous cell carcinoma of the tongue arising in lichen planus: a case report and review of the literature.

Lichen planus is a relatively common inflammatory disorder of unknown cause that affects the skin and mucosal surfaces. The relationship of oral lichen planus to oral squamous cell carcinoma is a matter of concern and debate. We present a case of squamous cell carcinoma of the dorsal surface of the tongue that arose in a previously biopsy-confirmed area of lichen planus. The literature on the development of oral cancer in patients with lichen planus is reviewed.

Treatment of pemphigus.

A general review of the treatment of autoimmune diseases classified as pemphigus is presented and includes pemphigus vulgaris, pemphigus vegetans, pemphigus foliaceous, pemphigus erythematosus, druginduced pemphigus, IgA pemphigus, and paraneoplastic pemphigus. The nurse plays an essential role in educating patients and their families about local skin and oral care related to these diseases.

Paraneoplastic pemphigus occurring in a patient with B-cell non-Hodgkin's lymphoma.

We present the case of a 71-year-old white male with paraneoplastic pemphigus associated with a B-cell non-Hodgkin's lymphoma. Diagnosis of paraneoplastic pemphigus was made by the characteristic findings on immunoprecipitation performed on a serum specimen. Paraneoplastic pemphigus is a severe autoimmune disease comprised of polymorphous mucocutaneous lesions, characteristic laboratory findings, association with one of several types of neoplasms, and a very poor prognosis.

Acute generalized exanthematous pustulosis secondary to diltiazem therapy.

Acute generalized exanthematous pustulosis in a rare pustular exanthem that is most commonly drug induced. We report a case occurring secondary to diltiazem therapy and briefly review the typical characteristics of this entity.

Methotrexate-induced toxic epidermal necrolysis in a patient with psoriasis.

We describe a fatal case of low-dose methotrexate (MTX) toxicity in a patient with psoriasis, emphasizing the factors that exacerbate MTX toxicity and presenting rescue techniques. The patient had a toxic epidermal necrolysis-like condition. MTX cutaneous reactions ranging from toxic epidermal necrolysis to specific ulcerations have been described. The use of granulocyte colony stimulating factor for leukopenia associated with MTX toxicity is discussed.