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BCOR-CCNB3 sarcoma (BCS) is a rare recently defined undifferentiated sarcoma that predominantly affects children and young adults. The diagnosis of this tumor is difficult due to the highly variable morphology and nonspecific immunophenotype. Emerging data suggest that patients with BCS show response to Ewing sarcoma-based treatment regimen, thus correct diagnosis is of clinical relevance. In this study, we report a case of BCS arising from the big toe of a 15-year-old male patient. The tumor had a prominent population of rhabdoid cells with bright eosinophilic cytoplasm mimicking rhabdomyosarcoma. The tumor cells were focally positive for desmin and myogenin, and negative for CD99. Next-generation sequencing showed the presence of BCOR-CCNB3 gene fusion. BCS with prominent rhabdoid cells has not been described before. This study further expands the morphologic spectrum of BCS.

We present a case of an 82-year-old male with known radiocontrast media (RCM) hypersensitivity who was admitted to our hospital with gangrene of his right toe. The plan for revascularization of his lower extremity required an angiogram. This presented a management challenge as the patient had experienced 2 episodes of delayed anaphylaxis to Omnipaque (iohexol) RCM, and based on a literature review, there was no known or established precedent on a safe procedure in these situations. The patient was premedicated and given a graded dose challenge of an alternative RCM (iodixanol) prior to the radiographic study. He was given 1% of the total expected dose 1 hour before to the procedure and an additional 10% for the 30 minutes prior. He was then given the final dose in the operating room. Following angiogram, the patient was monitored for 18 hours in the postanesthesia care unit, with no adverse reactions. He was placed on a prednisone taper for 1 week, with daily diphenhydramine. The patient remained asymptomatic throughout the hospital course. This novel approach to RCM hypersensitivity management lends itself to a hope that graded dose challenges may play a greater role in the management of these patients.

Purple toe syndrome is a rare complication of warfarin therapy. It occurs usually after 3 to 8 weeks of therapy and it is caused by cholesterol emboli from atheromatous plaque. Sudden onset of pain in affected area, typically in toes and feet, is the main characteristic of the syndrome. We describe a case of a 65-year-old female with purple toe syndrome after 6 weeks of warfarin. Indication of warfarin was a proximal deep venous thrombosis, which developed after prolonged immobilization. Factor V (FV) Leiden and persistent high FVIII activity were found as additional eliciting factors for venous thromboembolism. After warfarin withdrawal and enoxaparin treatment, symptoms disappeared promptly but a slight discoloration of the toe persists.

Onychomatricoma (OM) is a fibroepithelial tumor of nail matrix that occurs in the digits of both the hands and feet. This was first reported by Baran and Kint. They initially described 3 cases, all of which demonstrated a filamentous tumor of matrix tissue that resulted in a thickened funnel-shaped nail. Although apparently benign, it is subject to recurrence, and long-term follow-up is recommended because it is not known whether there is a conversion to malignancy. Even though this neoplasm was first described more than 18 years ago, there remains a dearth of case reports (currently fewer than 50) in the literature. This is a single case report and literature review. Levels of Evidence : Therapeutic, Level IV.

Background: Uterine leiomyomas are commonly reported to cause deep venous thrombosis and rarely arterial compression., Case: A 48-year-old woman was transferred to our institution with acute right lower limb ischemia and tissue loss. She underwent urgent iliac thrombectomy and was subsequently found to have right common iliac artery compression by a large uterine leiomyoma. She underwent successful resection of the tumor followed by endovascular iliac stent placement., Conclusion: This case emphasizes the importance of preoperative imaging when possible in the setting of acute arterial ischemia to evaluate for sources of extrinsic compression. Management requires correction of the etiology of extrinsic compression.

Bizarre parosteal osteochondromatous proliferation (BPOP) of bone is a rare benign neoplasm. This report presents the first case of BPOP associated with gangrenous changes and ulceration after remaining symptomless for 20 years. The authors hypothesize that the cause of gangrene may be attributed either to vascular occlusion due to pressure, trauma, or malignant transformation of the lesion. Although local excision of the tumor is the treatment of choice, the authors resorted to ray excision, as gangrenous changes and ulceration were present and there was the possibility of malignant change.

The authors describe a case in which well-circumscribed, expansile, and nonencapsulated nodular masses with missing digits were detected on the right and left feet in a 6-year-old female cynomolgus macaque from a routine toxicology study. Grossly, these masses were composed of variably sized and firm nodules containing white, chalklike material in the subcutaneous tissue on cross section. Microscopically, the nodules were composed of irregular lobules containing amorphous to granular, light to dark basophilic material that was surrounded by macrophages and multinucleated giant cells and separated by fibrous connective tissue. The nodule's contents were von Kossa and Alizarin red S positive. Serum calcium and phosphorus levels of this monkey were within normal ranges. Based on the gross pathology, histopathology, serum chemistry, and histochemistry, a diagnosis of dystrophic calcinosis circumscripta was made. Dystrophic calcinosis circumscripta is an uncommon syndrome of mineralization that occurs following tissue damage, without abnormalities in calcium and phosphorus homeostasis, and it is characterized by deposition of calcium salts in soft tissues. To the best of the authors' knowledge, this is the first report of dystrophic calcinosis circumscripta in a cynomolgus macaque.

The aim of the study is to assess the clinical characteristics, risk factors and outcome of patients with systemic lupus erythematosus (SLE) complicated with digital gangrene. In all, 2684 consecutive SLE inpatients admitted to Peking Union Medical College Hospital from December 1997 to August 2007 were studied. Demographic data, clinical features, laboratory findings as well as therapeutic regimens were systematically reviewed and a database was established. Cases with digital gangrene were identified and followed up. 1) Eighteen patients with SLE were complicated with digital gangrene, the average age at event was 33.1 +/- 11.8 years and the average disease duration was 99.1 +/- 60.1 months. 2) Patients with SLE, with long disease duration (> or =4 years), Raynaud's phenomenon and elevated serum C-reactive protein (CRP) were more likely to develop digital gangrene, P = 0.006, 0.001, and 0.031, respectively, OR = 1.03 (95% CI 1.01, 1.04), 35.76 (95% CI 4.67, 273.83) and 9.93 (95% CI 1.23, 80.30), respectively. 3) Fifteen gangrene patients started prednisone > or =1 mg/kg/d, and 18 were treated with cyclophosphamide, although 8 cases failed and ultimately received digital amputation. Prompt corticosteroid treatment (prednisone > or = mg/kg/d started within 3 weeks) decreased the hazard of amputation, P = 0.073, HR = 0.13 (95% CI 0.01, 1.21). Long disease duration, Raynaud's phenomenon and elevated serum CRP were independent predictive factors for SLE to develop digital gangrene. Early and aggressive corticosteroid treatment prevented gangrene from progression and improved prognosis.

The objective of this study is to determine if digital vasculitis (DV), a clinical manifestation with a high systemic lupus erythematosus disease activity index (SLEDAI) score, is associated with lupus severity. DV and other clinical manifestations defined according to the SLEDAI were evaluated in 168 consecutive patients with systemic lupus erythematosus (SLE). Two groups were defined according to presence (DV+, n = 27) or absence of DV (DV-, n = 141) at the time of evaluation. The exclusion criterion was the presence of antiphospholipid syndrome (Sapporo's criteria). The two groups were comparable with regard to age (P = 0.09), gender (P = 1.00), white race (P = 0.81), and disease duration (P = 0.78). Compared to the DV- group, the DV+ group had a significantly higher frequency of mucocutaneous manifestations (66.7 vs. 39.0%, P = 0.01), haematological abnormalities (22.2 vs. 6.4%, P = 0.02) and constitutional symptoms (11.1 vs. 0.7%, P = 0.01). Renal and neurological involvements were similar in both groups (P = 0.57 and P = 1.00, respectively). The evaluation of each SLEDAI parameter confirmed that the DV+ group had higher frequencies of mild manifestations, such as new rash (P = 0.02), alopecia (P = 0.02), oral ulcers (P = 0.045), fever (P = 0.01) and leucopenia (P = 0.005). In contrast, both groups had similarly increased anti-dsDNA (P = 0.78) and decreased complement levels (P = 0.29). In conclusion, DV in patients with SLE identifies a subgroup of a mild disease. The high 'weighted' index attributed to this alteration in the SLEDAI score should therefore be revised.

Secretan's disease is a rare disorder that has, to date, only been reported in the hand. After minor trauma, edema and hyperplasia of the hand may result, and pathological evaluation demonstrates thick fibrous tissue. We present the first report of Secretan's disease involving the foot in a 15-year-old athletic teenage girl with a remote history of blunt trauma, and review the relevant literature.

Background: Wide variations in definitions of lesser toe deformities exist. In addition, a general consensus regarding treatment of lesser toe deformities is lacking. The objective of this study was to evaluate of the definitions, current concepts, and treatment protocols for lesser toe deformities among orthopaedic departments in The Netherlands., Methods: A questionnaire with statements regarding lesser toe deformities was sent to all 101 Dutch departments of orthopaedic surgery., Results: In total 76 (75%) completed forms were analyzed. A wide variation regarding definitions, concepts, and treatment strategies of lesser toe deformities was reported among the Dutch orthopaedic departments. Only half of all responding departments had a protocol or consensus in the treatment., Conclusions: The definitions of lesser toe deformities used in Dutch orthopaedic departments do not coincide. This might explain the variations in indications and the various performed interventions for different deformities. The differences of opinion among the Dutch orthopaedic departments may have important clinical consequences because an indication for surgery depends on the correct diagnosis. To correctly interpret and improve treatment results, a consensus on this topic should be introduced.

Background: The purpose of this study was to determine the demographics, etiology, and radiographic findings associated with a crossover second toe deformity., Methods: Patients treated operatively for a crossover second toe deformity between 2001 and 2006 were identified. Charts were reviewed for clinical information, and radiographs were examined for pertinent angular measurements., Results: Of 169 patients in the study, 146 (86%) were women. The mean age at surgery was 59 (range 33 to 87) years. The most common complaints of preoperative pain were at the second (156 patients) and first (35 patients) metatarsophalangeal joints (several patients had more than one area of pain). A positive drawer sign was noted in 112 patients. The mean second and third metatarsophalangeal joint angles were -3 degrees and 6 degrees, respectively. There was a significant association of hallux valgus with first metatarsophalangeal joint arthritis (p < 0.01). The relative length of the second metatarsal averaged 0.2 mm less than the first metatarsal., Conclusions: Crossover second toe deformity had a peak incidence in women over the age of 50 years. There was an increased incidence of both hallux valgus and first metatarsophalangeal joint degenerative arthritis in the patient cohort. A positive drawer sign was a reliable and consistent physical examination finding. The most reliable radiographic indicator of a second crossover toe was medial angular deviation of the second metatarsophalangeal joint in relationship to the third metatarsophalangeal joint angle, although the angle was not necessarily a negative value. There was no correlation between a crossover second toe deformity and second metatarsal length, medial cortex thickness or shaft thickness, the 1-2 intermetatarsal angle, metatarsus adductus, metatarsus primus elevatus, or pes planus.

Rheumatoid arthritis (RA) is currently believed to have originated in America, and after the discovery of this continent in 1492, to have been exported to the Old World. We evaluated the genetic predisposition to RA in the "Braids Lady" from Arezzo (Italy), a partially mummified woman's body dating back to the end of 1500 AD which presents the anatomical and pathological features of this disease. The study of the polymorphic HLA-DRB1 locus, which includes alleles strongly associated with RA onset, has received much attention over recent years, especially the loci codifying for the DR1 and DR4 antigens, widely represented in the Mediterranean population, and for DR14, widespread among Native Americans. Molecular analysis was performed on extracts of DNA from the mummy, firstly from histological bone sections and then from the whole bone. Two different HLA typing techniques, PCR-sequence-specific oligonucleotides (PCR-SSO) and PCR-sequence-specific primers (PCR-SSP), were employed to identify HLA-DRB alleles. Both genotyping methods showed that the "Braids Lady" carried the DRB1*0101 allele, the serological equivalent of the DR1 antigen. Although the possession of RA risk factor genes cannot be considered a diagnostic marker, the positive result of the Italian mummy for DRB1*0101 and the RA features present, support the idea that this pathology was present in the Old World from at least the mid-16th century. A pathogenetic hypothesis of RA which might well explain its worldwide diffusion is the "molecular mimicry", resulting from a cross-reactive antibody response between certain microbial antigens and shared epitopes of specific HLA-DR1, DR4 and DR14 susceptibility alleles, the frequency of which varies among different ethnic groups.

The authors herein report the case of a teenage boy who presented with peripheral arterial occlusion of both upper and lower extremities associated with hypereosinophilia. During a 10-year follow-up, corticosteroid therapy was continued for the treatment of hypereosinophilia. The patient underwent bilateral lumbar sympathectomies because of severe ischemia of the bilateral lower extremities with gangrene of the toes. Based on the progress of his disease over the past 10 years, he was suspected to have idiopathic hypereosinophilic syndrome (HES) accompanied by peripheral arterial obstruction. Idiopathic HES is a disease characterized by unexpected hypereosinophilia, which may lead to organ damage. This is a very rare case of peripheral arterial occlusion associated with idiopathic HIS.

Twenty-seven patients with 30 crossover toe (COT) deformities of the second toe were evaluated. This deformity in the transverse and sagittal planes at the second metatarsophalangeal joint may be caused by hindfoot pronation. Compression of the lateral plantar nerve may produce incompetence of the second dorsal interosseous muscle and an increased extension-adduction pull by the lumbrical and first dorsal interosseous muscles. A surgical procedure for tenodesis is described to correct these deformities and restore the extrinsic-intrinsic musculotendinous balance at the second MTP joint. Our results are encouraging, with 83% good or excellent results. There was one recurrence.

The authors present a case of giant cell tumor of the tendon sheath (GCTTS) imitating macrodactyly macroscopically. They give an overview of the differential diagnosis of macrodactyly, the behavior of GCTTS, its most frequent localizations, the scope of surgical procedures available in cases of the disproportionate enlargement of a digit, and also of earlier GCTTS cases recorded in the files of the Orthopedic Clinic of the Semmelweis University.

Healing of forefoot cleft wounds can be a difficult management problem in patients with peripheral vascular disease, diabetes or both. This is a prospective review of 15 patients with these conditions with nonhealing middle-ray cleft wounds managed with a temporary mini-external fixator to close the cleft wound. Fourteen (93%) of the 15 patients had a successful obliteration of the cleft and skin coverage. Twelve (80%) of 15 were able to resume their previous level of activity with the reconstructed, mechanically sound forefoot. Fourteen (93%) of the 15 patients received hyperbaric oxygen treatments as an adjunct to wound healing. All patients avoided a transmetatarsal or higher amputation.

We describe a rare manifestation fibroma in a tendon-sheath in an 83-year-old man. The patient complained initially of a slowly progressive spreading, apart of the right second and third toes. A mass was found in the plantar aspect of the foot. It was completely excised at surgery. Histological examination revealed sparse spindle or stellate cells with slit-like vessels in the dense collagenous matrix. There had been no recurrence at follow-up 17 months after surgery.

Between 1990 and 1995, 38 patients (42 feet) underwent repair for crossover toe deformity, 31 (35 feet) of whom returned for final examination at an average of 51.6 months (range, 24-81 months). Causes included trauma, iatrogenic, and unknown. Presenting complaints included dorsal pain with either metatarsalgia or joint pain, isolated metatarsophalangeal (MP) joint pain, metatarsalgia, painful plantar callus, metatarsalgia and joint pain, and painful dorsal callus. All patients were treated with one of two operative techniques, either the flexor-to-extensor tendon transfer or the extensor brevis tendon transfer. Choice of procedure depended on the stage of preoperative deformity. Twenty-four patients were completely satisfied with the surgical correction, 6 were satisfied with reservations, and 1 was dissatisfied. The average postoperative AOFAS score for all patients was 85 points (range, 54-100 points), which correlated strongly with patient satisfaction. Twenty-two patients stated that they had no postoperative pain, 8 reported some pain, and 1 had frequent pain at the corrected toe. In 30 feet, there was no recurrence; three patients had mild residual crossover toe deformity, and two patients had recurrent deformity, although all MP joints were stable. Follow-up radiographs demonstrated substantial reduction in MP joint angles in both the AP (from 7 degrees to -1 degree) and lateral (from 45 degrees to 25 degrees) projections. This article reviews the surgical technique of both procedures, proposes specific indications for each, and presents outcomes. Based on our findings, the extensor brevis tendon transfer is appropriate for stage 1, stage 2, and flexible stage 3 deformities. Flexor-to-extensor tendon transfer is appropriate for rigid stage 3 and stage 4 deformities and for all patients with a symptomatic neuroma of the second web space (where the extensor brevis transfer is not possible). Stiffness of the MP joint is a potential problem with the flexor-to-extensor tendon transfer.

A 45-year-old woman was referred to the dermatology clinic for assessment of "refractory onychogryphosis." She had a 3-year history of lesions involving distal phalanges of the first and third of her left foot. Initially she described periungual erythema and swelling. Three weeks later she noted a whitish growth and thickening of her third toenail. X-ray films of the digit were reported as normal. Several months later the same changes occurred in her great toe. These lesions were asymptomatic. There was no history of trauma. Numerous fungal cultures were negative. No light microscopic examinations were undertaken. She had a trial of both topical and systemic terbinafine of 3-months duration with no clinical improvement. Several clinical opinions were obtained from two dermatologists, a surgeon, and a chiropodist. Past medical history of note was significant for tubal ligation, cervical cancer, and chronic sinusitis. The latter condition in retrospect was thought to be secondary to sarcoidosis. Physical examination revealed periungual violaceous discolouration of the first and third toes of the left foot. There was evidence of significant nail changes including dystrophy, onycholysis, and hyperkeratosis (Fig. 1). The fingernails were normal. There were no other skin abnormalities. A punch biopsy of the tip of the third toe showed granulomatous inflammation. There was evidence of hyperkeratosis, exocytosis, and a dense infiltrate composed of collections of histiocytes and a few giant cells forming granulomas (Fig. 2). Repeat x-ray films of the foot showed soft tissue swelling of the first and third digits. There was bony resorption in the distal phalanges with a lacey trabecular pattern compatible with sarcoidosis (Fig. 3). Chest x-ray films revealed marked hilar adenopathy. The patient was sent to a respirologist who concurred with the diagnosis of sarcoidosis. Further investigations included a low serum calcium of 2.07 mmol/L, serum ACE of 70 U/L (upper limit of normal is 75), Wintrobe erythrocyte sedimentation rate (ESR) of 10 mm per hour, thyroid stimulating hormone concentration of 0.65 mU/L, and a urinary calcium excretion rate that was elevated at 7.3 mmol/day. Pulmonary function tests were unremarkable. The patient was initially treated with clobetasol under occlusion and intralesional triamcinolone with minimal improvement. She was subsequently started on prednisone, 15 mg per os daily because of the lung and bone involvement with significant improvement noted in the toe lesions with diminution of both the swelling and violaceous discolouration.

After a review of the literature, two cases of osteoid osteoma involving phalanges of the toes are reported. Physical findings of osteoid osteoma vary with the site of the tumor; in the foot, particularly the toes, it presents various atypical and characteristic features. Its more frequent cancellous and subperiosteal localization in the small bones of hand and foot, showing atypical roentgenographic findings, poses particular problems in diagnosis. In the osteoid osteoma of the phalanges, characteristic enlargement of the toe or finger seems to be frequent and with a typical clinical history may be considered a reliable sign of suspected osteoid osteoma. Specialized imaging techniques may hasten diagnosis, but only an accurate clinical history and the characteristic findings with a high index of suspicion can allow for a proper diagnosis.

Clinicians are increasingly aware that mechanical aspects of foot deformities, such as Charcot changes, clawtoes, bunion deformities, or cavus or planus foot deformities, might have an impact on the occurrence, potential healing, and recurrence of foot ulcers. We report the prevalence of plain radiographic changes and attempt to rate the severity of those deformities in the feet of 456 diabetic veteran medicine clinic enrollees. All 456 radiographs were reviewed by orthopaedic surgeons to specifically identify Charcot changes, presence of arterial calcification, dislocation of the lesser toe metatarsophalangeal joints, hallux interphalangeal joint dislocation, and radiographic evidence of previous surgery. Radiographs of 428 patients were taken while weight-bearing, and these were reviewed to quantify hallux valgus angles, intermetatarsal 1-2 angles, fifth metatarsal-proximal phalangeal angles, second metatarsal lengths, lateral talocalcaneal and talar-first metatarsal angles, and claw toe deformities. The prevalence of Charcot changes was 1.4% (six subjects), and all had radiographic evidence of midfoot Charcot changes. Other deformities, such as clawtoes, hallux valgus, lesser toe joint dislocations, and alterations in arch height, are more common in veterans with diabetes.

Eleven subungual exostoses of the foot (10 on the hallux, 1 on the third toe) were studied. The initial symptom was subungual pain. When a subungual mass of fibrous tissue appeared, the nail was pushed up and in one case the mass became infected. X-rays exhibited a bone mass protruding from the terminal phalanx on the dorsomedial aspect of the toe in all cases. All patients underwent surgical excision of the lesions with partial onychectomy. Three layers were identified in five cases: a cap of fibrous tissue, a middle zone of hyaline cartilage with enchondral ossification, and a deep zone of cancellous bone. In three other cases, the histological pattern was pleomorphic and poorly characterized. The study shows that most subungual bone masses exhibited the pathological features of conventional osteochondromas. Nonetheless, a small number of lesions were pleomorphic and differed from osteochondromas, with abundant fibrous tissue merging irregularly into scattered islets of cartilage that was not organized in columns. Radical excision of the mass achieved complete relief of symptoms and recovery without recurrences in all cases.

One hundred twenty-four children with intoeing and 80 age-matched controls were investigated with the Kistler force plate. Tr2, reflecting the maximum external rotational ground reaction torque, is reduced by 30% to 40% in all intoeing children. There also is an appreciably shorter pathway of the point of application of the ground reaction force in the Y-direction (from 20% to 25%) and an obvious medial shift of the point of application of the ground reaction force in the X-direction (from 105% to 290%), from heel strike to toe-off as compared with controls. In spontaneous correction of intoeing, point of application of the ground reaction force in the X-direction becomes more close to normal. The "foot progression angle," which can easily be calculated from the force plate measurements to judge the degree of intoeing, is different from the values in the literature.

Eighty-six lesser toe basal hemiphalangectomies were performed in 52 patients. The surgical technique included an oblique dorsal incision, resection of 8 mm of bone, and an extensor tenotomy. Minimum follow-up was 2 years (range 2-6/1/2 years). Sixty percent of the patients had total relief of pain. Twenty-nine percent stated that they would not have the surgery again, and we categorized these patients as dissatisfied. An extensor tenotomy increased the satisfaction rate and was found to decrease the radiographic sagittal angulation of the toe. The preoperative diagnosis was significant to the outcome of the surgery. Patients with metatarsophalangeal joint synovitis and rheumatoid toe deformities had high rates of satisfaction; those with transverse deviation, metatarsalgia, and hammertoes with metatarsophalangeal joint subluxation/dislocation had lower rates of satisfaction. Seventy percent of the dissatisfied patients were dissatisfied because of persistent flexion deformity of the PIP joint or pain under the metatarsal head. We now add a PIP fusion if any flexion deformity, even a mild deformity, is present at the PIP joint and a plantar metatarsal condylectomy for metatarsalgia.

Adult hallux varus is an uncommon clinical entity usually caused by an inflammatory arthropathy or overcorrection during bunion reconstruction. We present five cases of unexplained spontaneous hallux varus. Clinically, all patients initially were found to have flexible deformities, and no evidence of underlying inflammatory disease or history of trauma. Symptoms were easily relieved with shoe wear modifications. Two of the patients developed more rigid deformities and subsequent medial callusing of the first toe and varus deformities of the lesser toes. These two patients eventually required surgical intervention. A Keller arthroplasty was performed on the first metatarsophalangeal joint and valgus proximal phalangeal osteotomies were done on the lateral toes when indicated. The outcome for these patients was good. Possible etiologic mechanisms are discussed. It is postulated that in these cases, the abductor hallucis tendon inserts more medially on the phalanx and overpowers the adductor. This produces a medially directed moment and, with time, a varus deformity.

The conservative management of foot deformities often requires the use of a modified shoe. A higher toe box is one of the most common variations required when the abnormality is located in region of the forefoot. High toe boxes are available in a variety of footgear, including athletic sneakers, comfort shoes, and prescription footwear. A rational approach to accommodating a deformed forefoot would be to first measure the maximum height of the abnormal toes in a weightbearing position. Then, by referring to a table listing shoes and available toe box space, the physician could match the foot with a shoe. The purpose of this study was to collect and tabulate measurements of toe box height for a variety of shoes. Impressions were taken from various shoes using a moldable plastic material. Toe box height was then measured and recorded. Results were tabulated for various types of men's and women's shoes. The depth of shoes at the point 5 cm from the tip of the toe was about 44 mm for most styles. The sagittal profile (i.e., vertical height available) of extra-depth shoes, comfort shoes, and athletic shoes was similar.

A seventy-six-year-old man with ischemic heart disease, peripheral vascular disease, and chronic renal failure developed bilateral cyanotic toes, which upon muscle biopsy, were shown to be caused by atheromatous emboli. The probable source was atheromatosis of the abdominal aorta. The toes became gangrenous, but surgical therapy was deferred because the patient was considered a high risk. With lovastatin therapy there was complete healing and except for transient cyanosis related to temporary cessation of therapy, there has been no recurrence for the past thirty months. The possible role of lovastatin in the conservative treatment of this disorder is discussed.

Four uremic patients with advanced peripheral arterial occlusive disease (PAOD) of lower limbs causing rest pain and ischemic-necrotic lesions were treated with a four-hour intravenous infusion of iloprost at doses of 0.75-2.5 ng/kg/min for twenty-eight days. After a week of the therapy all patients experienced disappearance of rest pain and prolonged walking distance. At the end of the trial a diabetic patient showed a complete regression of the necrotic areas of two toes while the other patients still showed ischemic-necrotic foot lesions that were well demarcated. Iloprost therapy can be effective in uremic patients with severe PAOD.

In the present study, systolic blood pressure in all toes was measured by using a bladder-free cuff in 20 normal limbs and 106 limbs with arterial occlusive disease, and the clinical significance of blood pressure measurement in all toes was studied. In 97 of 106 limbs with arterial occlusion, all 5 toes showed a significantly decreased blood pressure, indicating that any toe can be used for the purpose of diagnosis of arterial occlusions. However, 41% of cases showed the difference in blood pressure of more than 15 mm Hg between any 2 toes. Great variations of blood pressure among 5 toes were observed especially in limbs with gangrene or with other skin ischemic symptoms such as cold sensitivity, numbness, or history of gangrene in the toes. In these cases, evaluation of hemodynamics in each toe is considered to be of use for prognostic study of gangrene, selection and evaluation of treatments, and follow-up study. In limbs with intermittent claudication, however, blood pressure measurement in all toes may have little value in clinical practice because of small variation among blood pressure values of 5 toes. In comparison of blood pressure values in each toe from limbs with gangrene, toes with gangrene showed a significantly lower blood pressure than toes without gangrene. However, even in these toes without gangrene, a significantly lower blood pressure was observed than in toes from limbs without gangrene. These findings indicate that severe damage to digital circulation due to an occlusive process proximal to the toes is a primarily indispensable condition for development of gangrene in arterial occlusive disease.

The three forms of dystrophic epidermolysis bullosa include the autosomal recessive form and two autosomal dominant variants, the Cockayne-Touraine and Pasini forms. Whereas all three forms are clinically characterized by milia, nail dystrophy, and scarring, the autosomal recessive form tends to be the most mutilating with frequent mucosal lesions. The Pasini variant often has generalized blistering commonly associated with mucosal involvement but to a lesser degree. The Cockayne-Touraine variant is usually localized to the extremities and tends to be the mildest form of dystrophic epidermolysis bullosa. Ultrastructurally, the blister is located below the basal lamina in all three variants. The anchoring fibrils are either absent or hypoplastic in each condition. The autosomal recessive form alone also demonstrates dermal collagenolytic activity. The ultrastructural differentiation between the two forms of the dominant dystrophic epidermolysis bullosa depends on a quantitative rather than qualitative assessment of the anchoring fibrils.