Ehlers-Danlos syndrome type IV with gastric adenocarcinoma.

BACKGROUND: Ehlers-Danlos syndrome has many subtypes. The vascular type (type IV) is characterized by thin, translucent skin, easy bruising, characteristic facial appearance, and arterial, intestinal, and/or uterine fragility. OBJECTIVES: To encourage a better understanding of vascular EDS as a basis for early diagnosis, prevention, and management of complications. A first case of EDS type IV with adeno-carcinoma of the stomach in Thailand was reported and literature was reviewed. RESULT: A 62-year-old Thai priest was admitted in Priest Hospital because of progressive muscle weakness of both legs with neurogenic claudication from compression fracture of L1-2. Abdominal aortic aneurysm were detected with upper gastrointestinal hemorrhage, esophagogastroduodenoscopy showed diffuse gastric body swelling and erythema resulting in chronic gastritis. Gastric biopsy was indicative of adenocarcinoma of the stomach and gastrectomy was done. Dermatologists were consulted due to generalized cutaneous pain, easy bruising following venepuncture, and EKG padding. A vascular EDS type IV was diagnosed. CONCLUSION: After gastrectomy, the patient became drowsy and unconscious from profuse recurrent cerebral hemorrhage and expired.

Rat-bite fever presenting with rash and septic arthritis.

Rat-bite fever is an uncommon disease known for its endemicity to occur worldwide. Although most patients tend to develop mild symptoms with improvement from conventional antibiotics, it can progress with severe complications with a mortality rate as high as 13% without proper treatment. The authors report a complicated case of rat bite-fever involving a 61-year old woman who presented with fever petechial rash, and septic arthritis following a rat bite. Initially, multiple antibiotics were administered but were not effective. As a consequence, invasive procedures such as arthrotomy and joint debridement were done and prolonged antibiotic was administered until clinical resolution. Since many cases do not have a history of rat bite and may present with fever, rashes, and arthritis it is essential to distinguish it from other diseases. Here, the authors will provide details on the etiology, clinical manifestations, diagnosis, and management to aid prompt detection and treatment of the disease.

The treatment of keloids and hypertrophic scars with intralesional bleomycin in skin of color.

Intralesional injection with corticosteroid remains the mainstay of therapy for hypertrophic scars and keloids, however some lesions are unresponsive or may result in skin atrophy. Intralesional bleomycin injection is an alternative therapy that has been widely reported. In order to compare the effectiveness and safety of bleomycin for the treatment of keloids and hypertrophic scars in skin of color population, Fitzpatrick skin type III to V patients with keloids or hypertrophic scars were randomized into two groups. Group A was treated monthly with intralesional triamcinolone acetonide (10 mg/mL), while group B with intralesional bleomycin (1 mg/mL) for three consecutive months. Evaluation of the treatment was performed using "Patient and Observer Scar Assessment Scale" (POSAS), self-rated patient satisfaction score, photography, and ultrasonography. Two patients had their bleomycin blood levels monitored. Twenty-six patients with keloids or hypertrophic scars were recruited. The clinical improvement as assessed by the POSAS was not statistically significant. In terms of patients satisfaction score, one half of both groups reported a very good improvement. Photographic as well as ultrasonographic evaluation showed no difference between the two groups. Bleomycin was found to enter the blood circulation in a very small amount. The major side effect was hyperpigmentation. There was no skin atrophy detected in this study. Intralesional bleomycin is a safe and effective treatment for keloids and hypertrophic scars. The treatment is comparable to intralesional triamcinolone. Unfortunately, hyperpigmentation was the major side effect in darker skin type.

Linear atrophoderma of moulin: a case report and review of the literature.

Linear atrophoderma of Moulin (LAM) is a rare dermatosis in childhood and early adolescence. The exact etiology of LAM is still obscure. Several treatment modalities were reported but none was consistently successful. We report a case of LAM in which a favorable outcome was obtained with topical calcipotriol. The relevant literature is also reviewed.