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Cancer-associated fasciitis panniculitis.

BACKGROUND: Eosinophilic fasciitis and the related fasciitis panniculitis syndrome (FPS) are the clinical and morphologic expression of a variety of disorders, of which chronic inflammation and fibrous thickening of the subcutaneous septa, fascia, and perimysium are common. FPS in patients with cancer has been reported sporadically. METHODS: In the course of our studies of FPS we have encountered three patients who had an associated neoplasia. Nine reports of patients have been taken from the literature. The clinical and histologic data of FPS in the 12 patients were analyzed in the search of distinctive features from FPS in patients with no evidence of malignancy. RESULTS: Among patients with cancer-associated FPS there was a female predominance (8 patients), predilection for hematolymphatic malignancies (9 patients), precedence of the FPS to cancer diagnosis (10 patients) by a median lag time of 1 year, and unresponsiveness to prednisone therapy in most patients (7 of 8 patients). CONCLUSIONS: Cancer-associated FPS has several characteristics of a paraneoplastic syndrome: it occurs at a distance from the tumor, certain types of tumors are overrepresented among patients with FPS, it evolves in concert with the neoplasia, and it sometimes remits after successful cancer surgery. In contrast to idiopathic FPS, cancer-associated FPS shows a female predominance, and it usually fails to respond to corticotherapy.

Adenocarcinoma↗

Acenocoumarol and vasculitis: a case report.

A 62-year-old woman was referred to the dermatology department for a history of fever, asthenia and cutaneous rash, which appeared after a 3-day course of digitalin and acenocoumarol for atrial fibrillation. The physical examination revealed multiple round confluent purpuric lesions over her entire legs with no blistering. Laboratory exams were all negative. Biopsy of the involved skin was compatible with leucocytoclastic vasculitis. The acenocoumarol treatment was withheld and the skin lesions resolved spontaneously over the next 10 days. The cause of this purpura was seemingly acenocoumarol because of the close temporal relationship between exposure to the drug and the onset of the symptoms, and the spontaneous resolution of the lesions after acenocoumarol was discontinued. This observation illustrates a rare association between vasculitis and acenocoumarol. Clinicians should be aware of this potential adverse effect and recommend interrupting the drug intake when temporal relation is evocative.

Acenocoumarol↗

Peripheral skin necrosis in the recipient of monochorionic twins complicated by twin-twin transfusion syndrome.

Twin-twin transfusion syndrome (TTTS) complicates approximately 15% of monochorionic, diamniotic twin pregnancies and if untreated carries a high perinatal loss rate of between 80% and 100%, depending on the stage. The underlying pathology in TTTS appears to be an imbalanced intertwin perfusion difference, secondary to functional unidirectional arteriovenous anastomoses within a monochorionic placenta. We report two cases of monochorionic twin pregnancy complicated by TTTS, in which the pregnancies were treated by serial aggressive amnioreduction. However, the recipient twin in each pregnancy developed 'ischemic damage' to a lower limb.

Adult↗

TSH and TSH receptor antibody-binding sites in fibroblasts of pretibial myxedema are related to the extracellular domain of entire TSH receptor.

The role of TSH receptor antibodies in the pathogenesis of pretibial myxedema is still unclear. This study was designed to determine whether patients with pretibial myxedema had higher serum titers of TSH receptor antibodies, and whether there were TSH and TSH receptor antibody-binding sites on plasma membranes of fibroblasts derived from the skin of pretibial myxedema. If there were, were the binding sites similar to the TSH receptor? The TSH receptor antibodies were determined with radioreceptor assay in 20 normal subjects, 18 hyperthyroid Graves' disease patients without ophthalmopathy, 26 hyperthyroid Graves' disease patients with ophthalmopathy, and 11 patients with pretibial myxedema associated with Graves' ophthalmopathy. TSH and TSH receptor antibody-binding sites were studied on plasma membranes of fibroblasts cultured from the skin of pretibial myxedema with radioreceptor assay. RNA was also extracted from the fibroblasts of pretibial myxedema and reverse transcribed using random primers as the primers for cDNA synthesis. The resulting cDNAs were subjected to amplification by polymerase chain reaction with the use of a set of primers spanning the 5' region (+256/+275 and +616/+635) and the 3' region (+1819/+1838 and +2405/+2424) of the TSH receptor cDNA (+1 transcription start codon). They were further identified by Southern blot hybridization, with the probe spanning the 5' region (+272/+612) and the 3' region (+1908/+2268) of the TSH receptor cDNA (+ 1 transcription start codon), and sequencing. The results showed that patients with pretibial myxedema had higher titers of TSH receptor antibodies in the serum. TSH and TSH receptor antibody-binding sites were present on plasma membranes of fibroblasts derived from the skin of pretibial myxedema patients and related to the extracellular domain of the TSH receptor. These data suggest a common antigenic site in the skin and in the thyroid as a putative target for TSH receptor antibodies or lymphocytes of Graves' disease.

Adult↗

Treatment of pyoderma gangrenosum with disodium cromoglycate.

The case of a 35-year-old black woman with a 10-year history of ulcerative colitis and a 3-year history of pyoderma gangrenosum is presented. The pyoderma gangrenosum was treated successfully with topical application of disodium cromoglycate.

Administration, Topical↗

Hemophilus influenzae biotype III cellulitis in an adult.

The case of a patient with systemic lupus erythematosus presenting with severe leg cellulitis caused by Hemophilus influenzae non-B biotype III is reported. Skin infections caused by H. influenzae in general, and of the extremities in particular, seem to be rare in adults. This is the first reported case of cellulitis caused by H. influenzae biotype III. The infection was treated successfully with antibiotics. This case highlights the importance of blood cultures and prompt antimicrobial treatment in febrile adults with cellulitis, especially immunocompromised patients.

Adult↗

[Development of squamous epithelial carcinoma on the lower leg in long-term verrucous lichen planus].

Lichen ruber planus and squamous cell carcinoma are common diseases of the skin. Several publications have dealt with the association between these two types of dermatosis. Extensive trials have shown the potentially premalignant nature of lichen planus of the oral mucosa. However, no study conducted so far was able to show that lichen planus of the skin increases the risk for squamous cell carcinoma. We report a 65-year-old man who developed a squamous cell carcinoma in a long standing verrucous lichen planus in the lower leg.

Aged↗

Orbital decompression in Graves' ophthalmopathy associated with pretibial myxedema.

Pretibial myxedema (thyroid dermopathy), an uncommon autoimmune manifestation of Graves' disease, is almost always associated with significant ophthalmopathy and may be a marker for an unusually prolonged or different course for the ophthalmopathy. However, it is not known if the response to therapy for eye disease is different in patients with pretibial myxedema. We compared the results of orbital decompression in 385 patients without and 52 patients with pretibial myxedema operated on between 1969 and 1989. Preoperative and postoperative characteristics of severe Graves' ophthalmopathy did not differ in patients with or without pretibial myxedema except for slightly, but not significantly, more proptosis and diplopia in the pretibial myxedema group. In response to a 1989-1990 questionnaire (83% response rate, n = 364), 89.1% without (n = 319) and 88.9% with (n = 45) pretibial myxedema reported satisfactory or acceptable appearance of the eyes. Self-assessment of visual acuity, eye comfort, diplopia, and overall rate of satisfaction with the status of the eyes did not differ between the groups. The presence of pretibial myxedema does not herald an unsatisfactory response to transantral orbital decompression.

Adolescent↗