A review of the cutaneous paraneoplastic associations and metastatic presentations of ovarian carcinoma.

Ovarian carcinoma possesses cutaneous and paraneoplastic associations. The aim of this study was to review the paraneoplastic associations and metastatic presentations of ovarian carcinoma. PubMed was searched through December 2006 for references to cutaneous metastatic ovarian carcinoma (CMOC). CMOC occurs in 2-7% of cases, manifests in advanced disease and indicates a poor prognosis. The paraneoplastic associations of ovarian carcinoma include acanthosis nigricans, Raynaud's phenomenon, scleroderma, dermatomyositis and palmar fasciitis with polyarthritis. Dermatomyositis, in particular, can precede the diagnosis of ovarian carcinoma. Ovarian carcinoma has many cutaneous paraneoplastic effects and metastatic presentations, all of which portend a poor prognosis. Dermatomyositis is sometimes the initial manifestation of ovarian cancer, thus women > 40 years of age with dermatomyositis should be checked for ovarian carcinoma. It is possible that paraneoplastic dermtomyosititis can be distinguished from nonparaneoplastic dermatomyostitis by the former's lack of (i) associated Raynaud's phenomenon, (ii) response to treatment, (iii) autoantibodies, (iv) overlap and association with other collagen vascular diseases and (v) the presence of the prodromal symptoms of ovarian carcinoma such as gastrointestinal symptoms, urinary symptoms and/or fatigue or malaise.

Malignant atrophic papulosis.

Malignant atrophic papulosis (MAP; also known as Degos' disease) has a purely cutaneous variant and a systemic variant with cutaneous manifestations. Both have similar cutaneous eruptions. MAP manifests as erythematous, pink or red papules (2-15 mm), which evolve into scars with central, porcelain-white atrophic centres. Purely cutaneous MAP is a benign condition that can be life-long. Systemic MAP has a grim prognosis, but is not uniformly fatal. The cause of death is usually intestinal perforation. Death usually occurs within 2-3 years from the onset of systemic involvement. Systemic MAP can involve the nervous, opthalmological, gastrointestinal, cardiothoracic and hepatorenal systems. No specific laboratory test can be used to aid in diagnosing MAP. Histopathologically, a wedge-shaped degeneration of collagen is present with a prominent interface reaction with squamatization of the dermoepidermal junction, melanin incontinence and epidermal atrophy. No treatment has been shown to be effective in the treatment of MAP.

Is blistering distal dactylitis a variant of bullous impetigo?

Blistering distal dactylitis (BDD) manifests as acral oval bullae 10-30 mm in diameter, and is caused by infection with Gram-positive bacteria. BDD was first linked to infection with group A beta-haemolytic streptococcus in children, but has more recently linked to Staphylococcus aureus and noted in adults. BDD most commonly occurs as bullae on the volar fat pads of the fingers but can occur on the proximal phalangeal and palmar areas of the hands and can manifest as multiple bullae. The bullae can evolve into erosions over the course of several days. BDD can coexist with and may be secondary to clinically imperceptible infections of the nasopharynx, conjunctiva or anus, which underlines the need for systemic antibiotic therapy. Multiple bullae appear to be a predictor that S. aureus is the causative agent of a case of BDD. When BDD is suspected, treatment involves: (i) incision and drainage of bullae, (ii) wet to dry compresses to dry the eroded areas, and (iii) a course of a beta-lactamase-resistant antibiotics, necessary because S. aureus, now found to be a common cause of BDD, is usually resistant to penicillin. No treatment failures have been reported.

A comprehensive review and evaluation of the side effects of the tumor necrosis factor alpha blockers etanercept, infliximab and adalimumab.

For more than 5 years, infliximab and etanercept have been utilized to treat rheumatoid arthritis and Crohn's disease. There is therefore much post-approval data on their side effects. A variety of Medline searches were done at the beginning of June 2004 using the terms 'etanercept', 'infliximab' and 'adalimumab' and the words 'lymphoma', 'infection', 'congestive heart failure', 'demyelinating disease', 'lupus', 'antibodies', 'injection site reaction', 'systemic', 'side effects' and 'skin'. Approximately 150 articles were so identified. In addition, FDA and manufacturers' data obtained by internet searches using Google were reviewed. The important side effects that have been most extensively related to TNFalpha blockers include: lymphoma, infections, congestive heart failure, demyelinating disease, a lupus-like syndrome, induction of auto-antibodies, injection site reactions, and systemic side effects. The risk of these side effects is very low. Nevertheless, it is important for clinicians to be aware of these side effects when prescribing therapy.

Adalimumab (Humira): a brief review for dermatologists.

Adalimumab is a new purely human TNF-alpha monoclonal antibody that has been approved for the treatment of rheumatoid arthritis as monotherapy or in combination with methotrexate. It is administered by subcutaneous injection in a 40-mg dose every other week. The one published Phase II trial of adalimumab for psoriasis has provided very encouraging results for its efficacy. Its most important side effects relate to the development of infection while it is being used. It is a promising medication and research regarding its use continues.

Identification of a recurrent mutation in the CYLD gene in Brooke-Spiegler syndrome.

Brooke-Spiegler syndrome is an autosomal dominantly inherited disease with predisposition to neoplasms of the skin appendages. The disease has been mapped to 16q, and mutations in the CYLD gene have been identified in families with this disorder. We describe an individual with BSS exhibiting clinical heterogeneity in which a heterozygous frameshift mutation in CYLD, 2172delA, has been identified. These findings extend the body of evidence that mutations in CYLD are involved in Brooke-Spiegler syndrome and provide additional information for phenotype-genotype correlation.

Crohn's disease and lichen nitidus: a case report and comparison of common histopathologic features.

We describe a 54-year-old black woman with Crohn's disease, who developed lichen nitidus, the third report of a patient with both diseases. The rarity of these diseases individually and the histopathologic features in common imply that the two diseases are linked. Multinucleated giant cells, a common finding in the lesions of Crohn's disease, are less common in the lesions of lichen nitidus. The presence of multinucleated giant cells in lichen nitidus in all three case reports is distinctly unusual. The infiltrates of Crohn's disease and lichen nitidus contain CD-68-positive macrophages. As such, the subset of lichen nitidus with giant cells should be recognized as a cutaneous manifestation of Crohn's disease.

New-onset ichthyosis and diabetes in a 14-year-old.

Diabetes has not been linked to acquired ichthyosis or ichthyosis vulgaris. We report a newly diagnosed diabetic 14-year-old girl with bilateral tibial and sacral ichthyosiform plaques and a hemoglobin A1c of 20.1%. The patient had no personal or family history of atopy or ichthyosis and lacked keratosis pilaris or hyperlinear palms. A biopsy specimen of an ichthyosiform plaque showed compact lamellar orthohyperkeratosis and hypogranulosis, histopathology consistent with either ichthyosis vulgaris or acquired ichthyosis. We speculate that our patient's new-onset diabetes induced acquired ichthyosis.

Warfarin skin necrosis in a postpartum woman with protein S deficiency.

BACKGROUND: Warfarin-induced skin necrosis is a rare and dangerous complication affecting 0.01-0.1% of patients on warfarin. Deficiencies in protein C or protein S in association with other factors have been implicated in its etiology. No report has described this disorder in the immediate postpartum period in patients with protein S deficiency. CASE: A 1-week postpartum woman with known protein S deficiency presented with skin necrosis after a previously uneventful course of warfarin. CONCLUSION: Reduced levels of free protein S during the antepartum and immediate postpartum periods predispose protein S-deficient women to warfarin skin necrosis. Previously uncomplicated courses of warfarin do not obviate the possibility of skin necrosis with future warfarin administrations. Initiation of low-dose warfarin with heparin can reduce the likelihood of this disorder.