Papillon-lefevre syndrome: Ultrastructural study and successful treatment with acitretin

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CURRENT LITERATURE Papillon-Lefevre Syndrome: Ultrastructural Study and Suc- cessful Treatment With Acitretin. Nazzaro V, Blanchet- Bardon C, Mimoz MS, et al. Arch Dermatol 124:533, 1988 The authors review this rare hereditary disorder char- acterized by diffuse palmoplantar keratoderma and pre- mature loss of primary and succedaneous teeth. They present cases in four siblings aged 2, 5, 8, and 11 years (from parental consanguinity) allowing an appreciation of the chronological evolution of the above manifesta- tions. The children were examined clinically and micro- scopically. Three-millimeter punch biopsies were taken from typical lesions on the elbows of each child, with normal abdominal skin from two unaffected siblings used as a control. The three older siblings were treated with 0.5 mgkgld systemic acitretin for 16 months. Skin samples were taken again, and all were used for ultrastructural evaluation. Clinically, the skin became lesion-free after 2 months and remained that way after completion of treat- ment. The authors report the teeth that erupted after ini- tiation of therapy were free of periodontal disease; how- ever, they do not attempt to quantify or qualify this state- ment in this report. No laboratory study side effects or radiographic abnormalities as a result of therapy were noted. The authors note the teratogenecity of retinoids but point out the very short half-life of acitretin and there- fore its decreased likelihood of affecting a female achiev- ing pregnancy shortly after discontinuation. The results of this study give hope that the use of acitretin should allow patients with Papillon-Lefevre syndrome (PLS) to have a normal dentition at adulthood by beginning treatment prior to permanent dentition eruption.-T.B. HENNIG Reprint requests to Dr Blanchet-Bardon: Clinique des Maladies Cutanees, H6pital Saint-Louis, 2 Pl du Dr A. Foumier, 74575 Paris Cedex 10, France. Lymphoid Neoplasia Associated with the Acquired Immu- nodeficiency Syndrome (AIDS). Knowles DM, et al. Ann Intern Med 108:744, 1988 A study of the clinical, morphologic, and immunophe- notypic spectrum of AIDS-associated lymphoid neoplasia of 105 patients was undertaken by the authors. The 105 patients were diagnosed with AIDS or AIDS-related com- plex, or an increased risk for AIDS as well as a patho- logically documented lymphoid neoplasm was identified. Of these patients, 85% were diagnosed as having non- Hodgkins lymphoma, 12% were diagnosed with Hodgkins disease, and 3% were diagnosed with chronic lymphocytic leukemia. The non-Hodgkins lymphomas displayed a diffuse architectural growth pattern on histo- pathologic examination. The immunophenotypic and ge- notypic characteristics of the tissues supported the view that most AIDS-associated non-Hodgkins lymphomas are B-cell neoplasms, and the profiles of morphology are similar to B-cell non-Hodgkins lymphomas occurring in the general population. It was found through evaluation of the histopathology and immunophenotypic and geno- typic characteristics of the patients with Hodgkins dis- ease that these tumors lacked clonal IgH or T, gene ar- rangements similar to patients with non-AIDS-associated Abstracts Hodgkins disease. The authors found that two of the three patients with chronic lymphocytic leukemia had a clonal expansion of the mature, peripheral T, + T, - T, + suppressor-cytotoxic T-cell subsets. The study also included the efficacy of traditional therapy on these neoplasms and their behavior and aggressiveness.-S. FREIJE Reprint requests to Dr Knowles: Columbia University, College of Physicians and Surgeons, Department of Pathology, 630 W 168th St, New York, NY 10032. Sagittal Palate Fracture. Thompson R, Myer CM III. Ann Otol Rhino1 Laryngol 97:432, 1988 Sagittal fractures of the palate are unusual, as most maxillary or Le Fort fractures are horizontal. Sagittal pal- atal fractures may be isolated or occur in conjunction with horizontal fractures of the pterygoid plates, and may be associated with mandibular, nasal, or malar (zygo- matic) fractures. Sagittal palate fractures are usually paramedian, with rotation of the palate fragments caus- ing, respectively, a gap and opposite narrowing anteriorly or posteriorly. Inadequate reduction may result in maloc- clusions. Plain radiographs may be diagnostic, but CT scanning is more useful, although open exploration may be needed for comminuted fractures. Reduction requires stabilization in three planes-rotational, horizontal, and vertical, necessitating interosseous fixation. Healing is rapid and infection uncommon. Nonunion is uncommon, but malunion results in open bite.-G.H. SPERBER Reprint requests to Dr Myer: Department of Otolaryngology and Maxillofacial Surgery, Childrens Hospital Medical Center, El- land and Bethesda Aves, Cincinnati, OH 45229-2899. Histopathologic Study of Eustachian Tube in Cleft Palate Patients. Shibahara Y, Sando I. Ann Otol Rhino1 Laryn- go1 97:403, 1988 Eight temporal bones from cleft palate (CP) patients and eight age-matched normal temporal bones were com- pared. Examination of the CP specimens revealed that the angle between axial lines through the tensor veli palatini (TVP) and the superior portion of the eustachian tube (ET) lumen was narrow; the angle between axial lines through the lateral and medial laminae of the carti- lage was wide; the angle between axial lines through TVP and the lateral lamina of the cartilage was narrow; and the angle between axial lines through the superior and infe- rior parts of the ET lumen was wide. From these findings, ET dysfunction occurring with CP is the result of abnor- malities of ET and its cartilage and of abnormal anatomic relationships of these structures to the TVP muscle. CP patients have pathologically narrow or wide angles, caus- ing weaker opening forces on the ET lumen. Otitis media with effusion in CP is due to functional obstruction caused by the smaller, less efficient angle at which TVP pulls the ET lumen.-G.H. SPERBER Reprint requests to Dr Sando: Division of Otopathology, Depart- ment of Otolaryngology, Eye and Ear Hospital, 230 Lothrop St, Pittsburgh, PA 15213-2592. 314

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