Acral Malignant Melanoma and Striated Palmoplantar Keratoderma (Brunauer-Fohs-Siemens Syndrome): A Fortuitous Association?

Background. Striated palmoplantar keratoderma or Brunauer-Fohs-Siemens syndrome is a very rare, focal, nonepidermolytic palmoplantar keratoderma with autosomal inheritance. Unlike other palmoplantar keratodermas, no association with visceral or skin cancer has ever been reported.
Objective. We report a case of malignant melanoma arising in the hyperkeratotic lesions on the right heel of a patient with striated palmoplantar keratoderma. The lesion was completely excised; our patient also underwent sentinel lymph node biopsy and then was treated with high-dose interferon adjuvant therapy.
Methods. Sentinel lymph node biopsy incision was made in elliptical fashion, long enough to harvest a full-thickness skin graft to cover the wide local excision defect. The skin graft was defatted by sharp dissection. Several perforations were made in graft and it was secured in place with sutures and bolster dressing.
Results. At follow-up, the grafted skin showed hyperkeratotic changes but no local or systemic signs of the disease was observed.
Conclusion. The association between striated palmoplantar keratoderma and acral melanoma is discussed.     

Infusion manometry and detection of sphincteric function of crural diaphragm

The principles of infusion manometry in the measurement of lower esophageal sphincter (LES) pressure were laid down in the mid-1960s by L.D. Harris and his coworkers. Dodds and his colleagues were largely responsible for the improvements and advent of the low-compliance manometry. Using side-hole manometry, it is possible to detect accurate LES pressure that correlates with the strength of the antireflux barrier. The LES pressure as measured by the side-hole manometry, shows respiration-induced pressure oscillations. These pressure oscillations were initially thought to be due to the influence of abdominal and thoracic pressures on the LES. However, it was later pointed out that these pressure oscillations were due to the relative motion of the point pressure sensor (side hole of the manometric catheter) and the LES during respiration. Recent studies suggest that active contraction of the crural diaphragm during inspiration is responsible for the pressure oscillations observed in the cat LES pressure tracings. The use of the sleeve device in the measurement of LES pressure during contraction of the diaphragm has been described recently. Using the principles of manometry and sleeve device, it is now possible to identify two lower esophageal sphincters: the smooth muscle LES, traditionally known as the LES, and crural diaphragm, which we have referred to as the external lower esophageal sphincter. The purpose of the following paper is to summarize the general principles of the infusion manometry in the measurement of intraluminal pressure, specifically the LES pressure. The recently developed technique of detection of the sphincteric function of the crural diaphragm by the sleeve device will be discussed. In the last paragraph the limitations of manometry in detection of the muscular contractile activity are described.     

Late-Onset Superficial Lymphatic Malformation: Report of a Case and Review of the Literature

BACKGROUND: Superficial lymphatic malformations are predominantly developmental malformations of infancy, but they may arise at any age. OBJECTIVE: To describe a patient with a late-onset superficial lymphatic malformation. METHODS: Case report and literature review. RESULTS: A 53-year-old woman was evaluated for a colored lesion that developed spontaneously on the anterior abdominal wall. The lesion was treated by surgical excision. Findings on histopathologic examination of the specimen were consistent with superficial lymphatic malformation. There was no recurrence of the lesion at 4 months after the operation. CONCLUSION: Superficial lymphatic malformations can develop in adults spontaneously without the presence of any predisposing condition. Because the majority of such late-onset malformations reported in the literature are localized lesions similar to the one in our patient, surgical removal with inclusion of subcutaneous tissue usually results in cure without recurrence.     

Outbreak of Mycobacterium Abscessus Infection After Soft Tissue Augmentation

BACKGROUND: Illicit soft-tissue augmentation performed in New York City resulted in an outbreak of Mycobacterium abscessus infection in 2002. OBJECTIVE: To report two cases of women who developed tender, subcutaneous nodules of the face and buttocks after illicit soft-tissue augmentation with a hyaluronic acid derivative. METHODS: Two case reports are presented, and the literature is reviewed. RESULTS: Empiric treatment with clarithromycin (for M. abscessus infection) and prednisone (for foreign body reaction) resulted in clearance of lesions. CONCLUSION: Contaminated or impure material used for soft-tissue augmentation can result in a clustered outbreak of infection or foreign body reaction.     

Intravenous Pyogenic Granuloma of the Hand

BACKGROUND: Intravenous pyogenic granuloma is the intravasal counterpart of cutaneous pyogenic granuloma. This rare, benign lesion appears clinically as a subcutaneous nodule usually located in the upper extremity or neck in middle-aged people. It is hard to make an exact diagnosis on clinical basis, and there is a risk of recurrence because of undertreatment. OBJECTIVE: To report a case of an intravenous pyogenic granuloma located in the hand and to describe the clinical and histopathologic features of this rare vascular tumor. METHODS: An operation was performed on a 58-year-old female who presented with a small, painless mass in the right palm, and the pathologic examination revealed an intravenous pyogenic granuloma. CONCLUSION: This rare lesion should be differentiated from a regular pyogenic granuloma and should be excised with the vein segment to avoid recurrence.     

Benign neonatal sleep myoclonus: case report and follow-up of four members of an affected family.

Benign neonatal sleep myoclonus (BNSM), characterized by myoclonic jerks of the extremities only in non-REM sleep, occurs in the first months of life with spontaneous disappearance within 3-4 months. We examined five siblings with typical BNSM, at the 3-10 years follow-up neurological examination. Psychomotor development, cognitive functions and EEG were completely normal. These cases confirm that BNSM is a self limited and nonepileptic disorder.