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Factitious illness.

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Attitude of Health Personnel↗

[Cheilitis granulomatosa in a child].

UNLABELLED: Granulomatous cheilitis is a rare disorder characterized by intermittent swelling of one or both lips, which may become persistent. We report a case occurring in a child, which improved with minocycline treatment. CASE REPORT: An eight year-old girl was seen for the swelling of her upper lip, which persisted for more than one year. There was no history of applied irritants, local trauma or atopy. On examination, the upper lip was swollen, indurated, erythematous and fissured with a bilateral perleche. The tongue and gums were normal. There was no facial palsy. The girl was in good health and did not take any drugs. Blood investigations were normal. Patch tests were negative. Antibiotic treatment or local steroids provided only temporary improvement. A biopsy showed extravascular epithelioid and gigantocellular non-caseating granulomas with lymphoid infiltrates. There was no sign of sarcoidosis or Crohn's disease. Treatment with hydroxychloroquine for three months, then with oral metronidazole, did not improve the symptoms. Minocycline (100 mg/d) was effective. CONCLUSION: Granulomatous cheilitis should be considered in children as well and differentiated from infectious, allergic or factitious dermatoses. Granulomatous cheilitis can be associated with Crohn's disease or sarcoidosis, and could precede these disorders from several months or years, thus requiring a long-term follow-up.

Anti-Bacterial Agents↗

UVB treatment of factitious urticaria.

Forty-three patients with factitious urticaria were treated with sub-erythematous doses of UVB 5 times weekly for 2-3 weeks. After an average of 13 treatments, 25 were free from symptoms, 14 improved and 4 were unchanged. The dermographism test was negative or considerably reduced after treatment. Most of the patients had permanent effects from the treatment, but 13 relapsed. As the effect of other treatment modalities in this disorder is unsatisfactory, the encouraging results documented in this clinical trial suggest that UVB therapy should be considered for patients with factitious urticaria.

Adolescent↗

Factitious sickle cell acute painful episodes: a secondary type of Munchausen syndrome.

Three adult patients with factitious acute sickle cell painful episodes are presented and the literature is reviewed. The prevalence of this disorder among patients with sickle cell disease in our program was found to be about 0.9%. The patients described to date were all young adults who demonstrated pathological lying (pseudologico fantastico) and most of them had an underlying authentic medical illness to which the feigned signs and symptoms of sickle cell disease were added, thus making the diagnosis more plausible. It is recommended that all patients who present themselves with the signs and symptoms of sickle cell painful episodes be carefully studied in order to confirm the diagnosis.

Acute Disease↗

Exogenous Cushing's syndrome and glucocorticoid withdrawal.

Glucocorticoid therapy in various forms is extremely common for a wide range of inflammatory, autoimmune, and neoplastic disorders. It is therefore important for the physician to be aware of the possibility of both iatrogenic and factitious Cushing's syndrome. Although most common with oral therapy, it is also important to be alert to the fact that all forms of glucocorticoid delivery have the potential to cause Cushing's syndrome. Withdrawal from chronic glucocorticoid therapy presents significant challenges. These include the possibility of adrenal insufficiency after discontinuation of steroid therapy, recurrence of underlying disease as the glucocorticoid is being withdrawn, and the possibility of steroid withdrawal symptoms. Nonetheless, with patience and persistence, a reasonable approach to withdrawal of glucocorticoid therapy can be achieved.

Cushing Syndrome↗

Neurofibromatosis and Albright's syndrome.

For both NF and Albright's syndrome, the pathogenetic relationships between the various elements of the respective syndromes remain a mystery, and the importance of nontumorous endocrine abnormalities in neurofibromatosis has, in my opinion, been overstated. Nonetheless, the parallel between the two disorders is striking, and the consistent occurrence of skin and skeletal dyplasias in both suggests that a search for the fundamental defect in either can be advanced by focusing on what is common to these two types of tissue. For example, how does one reconcile a disturbance of the melanosome with skeletal aberrations? An answer to that question will put us on the right track. One final question must be asked. Are neurofibromatosis and Albright's syndrome alternate manifestations of the same disorder? In a trivial sense, the answer may be yes. That is, in at least some instances, one condition may have been mistaken for the other, and thus a factitious overlap or "sameness" misconstrued. Upon closer scrutiny, however, there are two important points that would cast doubt on a positive response to this question: Neurofibromatosis, or at least neurofibromatosis I, is heritable as an autosomal dominant trait, whereas heritability has not been documented for Albright's syndrome; and I am unaware of reported cases that describe both disorders, diagnosed by criteria beyond café au lait spots and bone dysplasia, in one and the same individual, although such a case has been shared with me by S. A. Sorensen, M.D., of the Genetics Institute of Copenhagen, Denmark. The full reporting of such a case would be of great interest.

Fibrous Dysplasia of Bone↗