281/307. psoralens used for cosmetic sun tanning: an unusual cause of extensive burn injury. Psoralen combined with long-wave ultraviolt radiation (UV-A) has become a standard method of psoriasis treatment. A well-known and often appreciated 'side-effect' is the hyperpigmentation caused by this treatment. Three patients demonstrating a novel cause of severe skin loss, 'psoralen burn', are presented. No patient was afflicted by psoriasis and all three had used psoralen and ultraviolet exposure with the intent to enhance sun tanning. In the case histories presented, it is notable that two of the patients share an alarmingly extensive skin injury (90-95 per cent body surface area), while the third had an extensive but rather superficial injury. In addition, a very similar time-table for the development of the injury could be observed, with a maximum distribution of skin loss not reached until 7 or 8 days after exposure. Psorelen-UV-A can cause life-threatening skin losses when used in an erratic manner. Early recognition of the nature and knowledge of the time-course in the development of these lesions is necessary for optimal treatment. Some principles of treatment are discussed. Dermatologists prescribing PUVA treatment should further increase their warnings of uncontrolled use of psoralens in non-psoriatrics. ( info) |
282/307. Pilomatricoma with a bullous appearance. A 15-year-old Japanese girl had an asymptomatic nodule on the right thigh of seven months' duration. The clinical appearance was similar to that of a bulla. There was a history of blunt trauma from dog scratch to the skin over the tumor shortly before tumor growth. Histopathological findings were consistent with pilomatricoma. In the overlying dermis, the collagen bundles were compressed to the tissue surrounding the tumor and the large space was seen. Around the tumor, some dilated endothelium-lined vascular channels were found, which were identified as lymphatic vessels. ( info) |
283/307. hemoptysis from an emphysematous bulla developing after open-heart surgery: report of a case. We present herein the case of a 62-year-old woman with an emphysematous bulla who developed intractable hemoptysis 16 days after undergoing mitral and aortic valve replacement with tricuspid annuloplasty. A bronchoscopic examination with balloon occlusion of the bronchial lumen revealed that the blood source was the right middle lobe bronchus. A computed tomographic (CT) scan of the chest subsequently demonstrated a blood-filled emphysematous bulla in the right middle lobe. A right middle lobectomy was performed and the bulla was observed to be swollen with clotted blood. The respiratory tract bleeding stopped immediately after the lobectomy. Pathohistological examinations suggested that disruption of the pulmonary vessels in the wall of the bulla had caused the respiratory tract bleeding. ( info) |
284/307. Bullous esophagitis due to chronic graft-versus-host disease. Vesiculobullous lesions of esophagus in patients post bone marrow transplantation may occur due to infectious agents or acute graft-versus-host disease (GVHD). We report the case of a patient who developed a large bulla in the esophagus as a result of chronic GVHD. Potential etiologies, diagnosis and management of bullous esophagitis are reviewed. ( info) |
285/307. Homozygous alpha6 integrin mutation in junctional epidermolysis bullosa with congenital duodenal atresia. Junctional epidermolysis bullosa with congenital pyloric or duodenal atresia is a distinct variant within this group of autosomal recessive blistering skin diseases. In this study we demonstrate, for the first time, a homozygous mutation in the alpha6 integrin gene (ITGA6) in a family with three affected individuals. For this purpose, we first determined the genomic organization of ITGA6, and placed the gene on chromosome 2q by high resolution radiation hybrid mapping. heteroduplex analysis of PCR products containing the individual exons of ITGA6, followed by direct nucleotide sequencing, revealed that the proband was homozygous for a G-to-T transversion in the 1 position of intron 12. This mutation, 1856 1G-->T, affects an invariant base of the 5' donor splice site predicting aberrant splicing involving exon 12. The mutation was verified in the proband's dna by restriction enzyme digestion which also confirmed that the parents were heterozygous carriers of this mutation. Altered expression of alpha6 integrin, which forms a heterodimer with the beta4 subunit at the dermal-epidermal junction, would explain fragility and blistering as a result of minor trauma to the skin. ( info) |
286/307. A case of dermatopathia pigmentosa reticularis. Dermatopathia pigmentosa reticularis (DPR) is a very rare disorder with the diagnostic triad of generalized reticulate hyperpigmentation, noncicatricial alopecia, and onychodystrophy. Many other dermatologic findings have been associated with this triad, including adermatoglyphia, hypohidrosis or hyperhidrosis, palmoplantar hyperkeratosis, and nonscarring blisters on the dorsa of the hands and feet. The mode of inheritance is unclear but may be autosomally dominant. To our knowledge, only 11 cases have been reported in the world, and none has previously been described in the Orient. We present a Korean patient with the typical features of the DPR triad, along with adermatoglyphia, hypohidrosis, and nonscarring blisters on the dorsa of the feet. ( info) |
287/307. A new case of extensive congenital erosions and vesicles healing with reticulate scarring. Congenital erosions and vesicles that heal with reticulate scarring comprise a new entity first described in 1985 in 3 girls aged 3, 5 and 6 years. Two years later, an 8-year-old boy in whom the skin lesions were accompanied by neurological disorders was described, as in one of the patients described in the 1985 study. In 1990, similar findings were reported in a neonate, and recently the 7th case has been reported in the American literature. We describe an 8-month-old infant born with cutaneous lesions that subsequently formed reticulate scars peculiar to this clinical entity, which is characterized by variable clinical and histological expression. ( info) |
288/307. Nikolsky's sign: is it 'dry' or is it 'wet'? Nikolsky's signs refers to the ability to induce peripheral extension of a blister as a consequence of applying lateral pressure to the border of an intact blister. Although initially used in reference to the pemphigus group of blistering dermatoses, a positive Nikolsky's sign can be seen in other bullous diseases such as toxic epidermal necrolysis and staphylococcus scalded skin syndrome. Appreciating whether the blister is 'wet' or 'dry' at the site of a positive Nikolsky's signs may have both diagnostic and prognostic significance which I illustrate with several clinical cases. Lastly, I review the significance of a positive Nikolsky's sign. ( info) |
| A patient with acute carbon monoxide poisoning due to leakage of gas from the exhaust system in his automobile noted edema and blister formation over large areas of the skin of one half of his body. rhabdomyolysis, acute but transient renal insufficiency, and hemolytic anemia developed subsequently. ( info) |
290/307. Home-made endoloops for bullous lung disease: a case report. Various surgical techniques for bulla ablation have been used since the introduction of video-assisted thoracoscopic surgery. Endoscopic stapler resecting (Endo-GIA) is now the most common procedure for bulla ablation. Other endoscopic techniques include bulla suturing, clipping, laser ablation and electrocauterization. We present here a select report on using a "home-made" endoloop on a patient with bullous lung disease. No intraoperative morbidity was attributable to the procedure. The operating room time was 110 minutes, and ventilator support was given for 18 hours. The chest tube was removed on day 7 and the patient discharged on the ninth postoperative day uneventfully. The patient exhibited subjective improvement in his symptom preceptions during an one-month follow-up examination. A comparison of pre- and post operative functional evaluation showed increase in FEV1 (from 0.45 L to 1.02 L) during 3-month follow-up. From our experience, thoracoscopic home-made endoloop ligation is a safe and cost effective means of volume reduction surgery for bullous emphysema that interferes with optimum function of the adjacent lung parenchyma. ( info) |