Pompholyx of the hands and feet

The term pompholyx, both because of historical priority and because it does not convey misleading implications about etiology, is the preferable designation for this condition. Irrespective of its localization, the primary lesion is the same: a deep-seated, sago-grain-like, pinpoint to pinhead size blister or pustule, embedded in the normal skin, primarily without inflammatory reaction, and appearing suddenly. Pompholyx, as microbiologic, histopathologic and immunologic investigations have proved, is an endoparasitic-hematogenous eruption, having a characteristic localization. It is caused by the circulating endoparasite,B. endoparasiticus Benedek, 1927. Due to an universal infection immunity it is not infectious and not transmissible. Other microorganisms, like hyphomycetes, yeastlike organisms, common bacteria, etc. play no causative role in any phase of the condition. Dermatophytes, molds as well as yeastlike organisms which may be found in the roof of the blisters and other scrapings of diseased tissues are incidental nosoparasites. Extensive statistical analysis is presented about the occurrence of pompholyx on the hands and feet in military personnel and in civilian patients. Pompholyx has only one etiologically directed therapy: the specific vaccine ofB. endoparasiticus. The vaccine is prepared from the S-type of the bacilli, cultured from the blood.     

Motor cortex representation of the upper-limb in individuals born without a hand

The body schema is an action-related representation of the body that arises from activity in a network of multiple brain areas. While it was initially thought that the body schema developed with experience, the existence of phantom limbs in individuals born without a limb (amelics) led to the suggestion that it was innate. The problem with this idea, however, is that the vast majority of amelics do not report the presence of a phantom limb. Transcranial magnetic stimulation (TMS) applied over the primary motor cortex (M1) of traumatic amputees can evoke movement sensations in the phantom, suggesting that traumatic amputation does not delete movement representations of the missing hand. Given this, we asked whether the absence of a phantom limb in the majority of amelics means that the motor cortex does not contain a cortical representation of the missing limb, or whether it is present but has been deactivated by the lack of sensorimotor experience. In four upper-limb amelic subjects we directly stimulated the arm/hand region of M1 to see 1) whether we could evoke phantom sensations, and 2) whether muscle representations in the two cortices were organised asymmetrically. TMS applied over the motor cortex contralateral to the missing limb evoked contractions in stump muscles but did not evoke phantom movement sensations. The location and extent of muscle maps varied between hemispheres but did not reveal any systematic asymmetries. In contrast, forearm muscle thresholds were always higher for the missing limb side. We suggest that phantom movement sensations reported by some upper limb amelics are mostly driven by vision and not by the persistence of motor commands to the missing limb within the sensorimotor cortex. We propose that prewired movement representations of a limb need the experience of movement to be expressed within the primary motor cortex.     

The hands and feet of Archaeolemur: metrical affinities and their functional significance

Recent expeditions to Madagascar have recovered abundant skeletal remains of Archaeolemur, one of the so-called "monkey lemurs" known from Holocene deposits scattered across the island. These new skeletons are sufficiently complete to permit reassembly of entire hands and feet--postcranial elements crucial to drawing inferences about substrate preferences and positional behavior. Univariate and multivariate analysis of intrinsic hand and foot proportions, phalangeal indices, relative pollex and hallux lengths, phalangeal curvature, and distal phalangeal shape reveal a highly derived and unique morphology for an extinct strepsirrhine that diverges dramatically from that of living lemurs and converges in some respects on that of Old World monkeys (e.g., mandrills, but not baboons or geladas). The hands and feet of Archaeolemur are relatively short (extremely so relative to body size); the carpus and tarsus are both "long" relative to total hand and foot lengths, respectively; phalangeal indices of both the hands and feet are low; both pollex and hallux are reduced; the apical tufts of the distal phalanges are very broad; and the proximal phalanges are slightly curved (but more so than in baboons). Overall grasping capabilities may have been compromised to some extent, and dexterous handling of small objects seems improbable. Deliberate and noncursorial quadrupedalism was most likely practiced on both the ground and in the trees. A flexible locomotor repertoire in conjunction with a eurytopic trophic adaptation allowed Archaeolemur to inhabit much of Madagascar and may explain why it was one of the latest surviving subfossil lemurs.     

Acrodysplasia (hands and feet) with scoliosis, by autosomal recessive transmission

Brachydactyly is reported in two brothers born to consanguineous parents (1/8). Additional abnormal features were scoliosis, spina bifida occulta, and carpal synostosis.     

Epidermal patterns of the hands and feet of the pygmy chimpanzee (Pan paniscus)

The characteristics of the epidermal ridge system were studied in a series of eighteen lesser or pygmy chimpanzees (Pan paniscus). The general ridge alignments are very similar to those of the chimpanzee (Pan troglodytes); Biegert ('61). On the average the pattern intensity (P.I.) of the palm configurations is considerably higher in the pygmy chimpanzee than in the chimpanzee, thus representing the highest total palm pattern intensity of all species of the Hominoidea. The sole configurations show parallel main results to those of the palm; however, the decreased sole pattern frequency of the pygmy chimpanzee is of a smaller predominance only as compared to the values of the other species of this superfamily. The preliminary data on the finger tip patterns, translated into P.I. values, are much higher than in chimpanzees and within the range of the mean values of gorillas (Brehme, '73), while those of the toes of pygmy chimpanzees seem to possess the lowest P.I. values of the African apes.     

Treatment of the hands and feet in Apert syndrome: an evolution in management

Apert syndrome is a relatively uncommon condition that is instantly recognizable on the basis of the pan-syndactylies involving both the hands and feet. For more than 10 years, the treatment of Apert syndrome hand and foot anomalies was approached in a comprehensive manner, with attempts to maximize the final results and minimize the total number of operations. Numerous conventions were abandoned in the development of this approach, with the introduction of some new methodologies, including (1) release of all 10 fingers, and toes, in only two operations, (2) elimination of routine digital amputations, (3) abandonment of the zigzag incision in favor of straight-line release, (4) substitution of equal-length anterior and posterior flaps for the long dorsal web space lining flap, (5) leaving of small areas of exposed bone without vascularized tissue coverage, and (6) performance of midphalangeal osteotomies, among older children, to improve hand function. Fifty-seven children with Apert syndrome have been treated at the author's center since 1990, and 43 underwent surgical treatment of their hands and feet by a single surgeon. Treatment can be separated into two phases, early (syndactyly releases) and late (functional osteotomies). Seventeen of those 43 patients were treated at the author's center from birth (type I, 11 patients; type II, two patients; type III, four patients), and 10 fingers and 10 toes were achieved for all patients in two operations. No digital amputations were performed for any of the 43 patients. However, four of 26 patients (15 percent) not treated at the author's center from birth had undergone at least one digital amputation before coming to the center. Twenty-two of those 26 patients required a two-stage syndactyly release to accomplish the separation of all 10 fingers and toes. Aside from the patients who had previously undergone amputations, all other patients successfully achieved 10 fingers and toes, except for one patient (38 of 39 patients, 97 percent). The average operative time for the first-stage syndactyly release of the hands and feet was 4 hours 11 minutes (range, 185 to 300 minutes), and that for the second stage was 3 hours 49 minutes (range, 160 to 300 minutes). Twenty-eight hands were treated with functional osteotomies, which involved permanent angulation of the fused phalanges at the proximal interphalangeal level, to create a "position of function" and provide pulp-to-pulp pinch. In addition, targeted osteotomies of the feet were performed for many of these patients, to decrease pain with walking. There were no major complications. Minor complications included one reexploration because of bleeding, early in the series. There were 13 incomplete secondary syndactylies that required a subsequent operative release (10 spaces treated at the author's center and three treated elsewhere), of a total of 342 spaces (3 percent author incidence). There were no scar contractures or functional limitations that required release. This distinctive protocol seemed to result in improved functional outcomes, with a reduction in the total number of operative procedures.     

Extramedullary hand mirror cells in pathologic conditions of lymphoid tissue

The hand mirror (HM) cell phenomenon, which usually affects pathologic cells of lymphoid tissue in bone marrow, especially in acute lymphoblastic leukemia (ALL), was seen in B-cell lymphoma cells in cerebrospinal fluid (CSF). It was subsequently detected not only in the bone marrow, but also in extramedullary sites of the lymphoma. Subsequent phase-contrast microscopic study of 45 consecutive specimens of CSF revealed lymphoid cells with HM features in a case of ALL (in which HM cells were subsequently found in the bone marrow) and in a case of acute viral meningoencephalitis. These observations demonstrate that the HM cell phenomenon, which is considered to be a cellular alteration resulting from incorporation in the cell of antigen-antibody complexes, is not unique to bone marrow. It can be present in extramedullary sites and can be seen in exfoliated cells in CSF, where its detection is facilitated by the use of phase-contrast microscopy.     

The community of human malformation syndromes that shares ectodermal dysplasia and deformities of the hands and feet.

Syndromes of human congenital malformation may be classified be recognizing communities of syndromes that share multiple phenotypic similarities involving their principal diagnostic features. A community of syndromes that shares various expressions of ectodermal dysplasia and various deformities of the hands and feet is proposed; these syndromes are divisible into two classes according to the presence or absence of anomalies in the nasal or labial regions of the face. The dysmorphogenetic validity of the division is supported by the fact that the syndromes without nasal or labial anomalies have a high frequency of sensorineural deafness as one expression of ectodermal dysplasia whereas those without such anomalies do not. The usefulness of such a syndromal community as a base for evolving a taxonomic scheme of dysmorphogenetic relatedness amongst different syndromes is illustrated.     

Molecular mapping of a translocation breakpoint at 14q13 in a patient with mirror-image polydactyly of hands and feet

Mirror hands and feet (MIM, 135750) is a rare congenital anomaly, and mirror-image polydactyly is considered to be a variant of mirror hands and feet. To our knowledge, seven patients with the disorder have been reported in the literature. Parent-to-child transmission was reported in two families, which may indicate a single-gene defect inherited in an autosomal dominant fashion. We had previously encountered a boy with mirror-image polydactyly whose karyotype showed 46,XY,t(2;14) (p23.3;q13) de novo. We hypothesized that at least one of the putative genes responsible for the determination of an anterior-posterior limb pattern is disrupted by a translocation breakpoint. In this study, we identified a yeast artificial chromosome clone spanning a translocation breakpoint at 14q13, and the breakpoint was confirmed to be located between two loci, AFM200ZH4 and D14S306, within a genetic distance of 0.6 cM. Received: 25 September 1996 / Revised: 6 December 1996     

The actions of retinoids on cellular growth correlate with their actions on gap junctional communication

Retinoic acid (a possible morphogen), its biological precursor retinol, and certain synthetic derivatives of retinol profoundly change junctional intercellular communication and growth (saturation density) in 10T 1/2 and 3T3 cells and in their transformed counterparts. The changes correlate: growth decreases as the steady-state junctional permeability rises, and growth increases as that permeability falls. Retinoic acid and retinol exert quite different steady-state actions on communication at noncytotoxic concentrations in the normal cells: retinoic acid inhibits communication at 10(-10)-10(-9) M and enhances at 10(-9)-10(-7) M, whereas retinol only enhances (10(-8)-10(-6) M). In v-mos-transformed cells the enhancement is altogether lacking. But regardless of the retinoid or cell type, all growth responses show essentially the same dependence on junctional permeability. This is the expected behavior if the cell-to-cell channels of gap junctions disseminate growth-regulating signals through cell populations.