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SYNOPSIS The simian crease is a familiar feature of Down's syndrome. Less well known is the extended proximal transverse palmar crease (‘the Sydney line’). A study of the incidence of unusual palmar creases in 105 patients with a clinical diagnosis of Down's syndrome has shown a statistically significant increase in the incidence of both the simian and Sydney lines in these patients. The total incidence of unusual palmar creases in Down's syndrome is thus higher than it was previously thought to be.

The dermatoglyphic patterns of 100 children with congenital rubella were studied and compared with those of 100 matched normal controls. There was a statistically significant increase in the incidence of abnormal palmar creases, digital whorl patterns, patterns in interdigital space III, and clinodactyly. The Sydney line, or extended proximal transverse palmar crease was seen in 27 of the patients and was established as an index of abnormality in children born with congenital rubella in New South Wales since 1955. Seventeen patients had simian lines and a total of 41 patients had one or more abnormal palmar creases. Fifty patients had either abnormal palmar creases or eight or more digital whorl patterns. Several mechanisms may cause dermatoglyphic abnormalities in children with congenital rubella.

Dermatoglyphic abnormalities have been demonstrated in children born in the 1960s with congenital rubella. The dermatoglyphic patterns of adult rubella patients born after the 1940 epidemic have been compared with a hundred normal controls of similar age and racial background. There was a significantly increased incidence of digital whorl patterns in rubella-affected adults. No increase was found in the incidence of simian lines. A previously undescribed, atypical palmar crease (the "Sydney line") was demonstrated more frequently in female patients. Since dermatoglyphic defects occurred in congenital rubella before 1960, it is unlikely that this effect can be used to support the hypothesis of recent viral mutation. Clinically, dermatoglyphics aid the diagnosis of congenital rubella where a typical defect is associated with a high incidence of digital whorl patterns.

The present study was conducted among the two population groups, namely Hindus and Christians, who are living in areas of high natural radiation in the southwest of Kerala. The effect of high natural background radiation on palmar crease variation was studied. Statistically significant differences were observed between the exposed group and a control group with regard to radial base crease variation and simian crease frequency.

An analysis of palmar crease variants was carried out in a group of "at risk" newborns, without any evident congenital anomalies. This group consisted of 108 prematures, 74 infants who were small for gestational age, 62 newborns with history of gestational complications, and 46 newborns with a history of intrauterine methadone exposure. A system of classification was developed based on observations of 500 normal newborns as control subjects, 466 normal mothers, and 200 normal children. The palmar crease variants can be divided into four main groups, schematically presented as normal variants, simian crease and its variants, Sydney line and its variants, and another group of unusual variants which do not fit into the other groups. A study of these groups revealed that familial components, race, sex, and age are factors that can influence the expression of palmar crease patterns. There is an increased frequency of abnormal creases in each of the groups of "at risk" newborns. Moreover, there is an apparent association of interrupted transrerse creases and intrauterine methadone exposure.

The physical and dermatoglyphic features obtained from published reports of 128 patients with the trisomy 9p syndrome and 27 patients with the partial 9p monosomy syndrome are tabulated. This information is also provided on two new individuals with each of these chromosomal disorders. The dermal ridge patterns and palmar creases of trisomy 9p which are most helpful from a diagnostic standpoint are zygodactylous or absent palmar digital triradii, brachymesophalangy, reduced total finger ridge count, complex thenar/ID I patterns, transverse palmar ridge alignment, simian creases, distal axial triradii, and great toe and hallucal arch patterns. The characteristic features in partial 9p monosomy include dolichomesophalangy with accessory finger flexion creases, digital whorl patterns and elevated total finger ridge count, distal axial triradii, simian creases, and palmar dermal ridge dissociation.

In a recent paper Maserati et al. (1986) demonstrated that the XO male reported by Forabosco et al. (1977) has Yp material translocated onto a chromosome 18 and that he is monosomic for a portion of the short arm of chromosome 18. In fact the patient has several signs of the 18p-syndrome. As pointed out by Maserati et al. (1986) there is increasing evidence that the XO condition may not exist but is either due to hidden or undetected mosaicism or to the translocation of Yp material which can be detected by revealing the presence of specific Y-DNA sequences, or cytogenetically as in the case reported by Schempp et al. (1985). There are two cases of XO males that have been studied by us which are likely to be further instances of unbalanced Yp/ autosome translocations resulting in severe phenotypic disturbances. The first case was reported by Fraccaro et al. (1966) and is referred to as B.N. in that paper in which the reader can find photographs of the patient aged 6 years. He was born on January 6, 1956. An intracranial hemorrhage and hypospadias with urethra opening at the base of a small penis were noted at birth. Operative reconstructions of the hypospadias were carried out at 3 and 6 years of age. Psychomotor development was retarded, and at the age of 61/2 years the following additional observations were made: mental retardation (IQ 58), shuffling gait, slightly impaired muscular coordination, and peculiar facial characteristics. A chromosome analysis of two blood cultures and of fibroblasts from one skin biopsy and one testis biopsy revealed a 45,X chromosome constitution. In 1963 the patient was operated upon because of sclerosis of the bladder neck. He also suffered from repeated infections of the urinary tract and developed a chronic and progressive pyelonephritis with uremia. Moderate development of the secondary sex characteristics was noted in 1970. On the same occasion hypertension was diagnosed. In 1971 he had epileptic seizures of the grand real type and the electroencephalogram showed abnormal features characteristic of epilepsia. He was treated with anti-epileptic drugs but had several seizures over the years despite this. He died in November 1975 due to ventricular fibrillation probably caused by hyperkalemia which developed after anesthesia for cystoscopy and intravenous pyelography. Autopsy was denied by the parents. The second case was reported by Lo Curto et al. (1974). The patient was born on March 19, 1971 and the clinical description in the paper was based on examinations made at 7 months of age (Fig. 1 in the paper). The head was relatively large and he had large ears and a broad nose flattened at the bridge. The hands and feet were short and broad. There was a single bilateral transverse palmar crease. The abdomen was prominent and there was a small umbilical hernia and bilateral inguinal hernias extending into the scrotum. The gonads, normal in size, were in the scrotum. There were several disturbances of the skeletal system which led to Lo Curto et al. (1974) to conclude that "the patient is affected with a form of 'chondroosteodysplasia' which we were unable to define more precisely". The karyotype was consistently 45,X in blood cultures and in flbroblasts from one testicular biopsy and one skin biopsy. We have re-examined some of the old slides with the same results. The tiny, fluorescent body seen at the lower right end of Fig. 2 in the paper of Lo Curto et al. (1974) was not composed of chromosome material. The patient was reexamined in 1972 when he was i year and 7 months old and his mental development was still that of a 4-month-old child. Shortly after the patient was reported to have died in Sicily and no further information is available. We conclude that is very likely that both of these patients were carriers of an unbalanced Y/autosome translocation which we are unable to identify retrospectively on the basis of their phenotypic appearance alone. This strengthens the point made by Maserati et al. (1986), namely that all cases of "XO males" are probably either hidden mosaics or carry Y-chromosome material (and the male-determining factor(s)). The only exception known to us seems to be that of the male reported by de la Chapelle et al. (1986) who was negative with six single-copy DNA (Taql) , two repeated Y-specific (HaeIII) probes, and 45,X in blood and skin fibroblast cultures.

A comparative study of unusual dermatoglyphic and palmar patterns revealed significant differences between the frequencies of certain patterns among 200 congenitally affected mentally retarded children and 500 normal controls. A scoring method demonstrating the significance of eight unusual patterns as non-specific indicators of early intra-uterine fetal insult was devised. 10 per cent of the children previously classified as idiopathically mentally retarded were shown to have been exposed to early intra-uterine insult. Dermatoglyphic and palmar-crease analysis should be included as a routine investigation for children with mental retardation of unknown cause.

The palmar flexion creases of fully diagnosed patients of throat cancer (n = 120) and tuberculosis (n = 80) have been compared with a control group (n = 150) of the same stock. The palmar flexion creases of cancer and tuberculosis patients ate significantly different from those of the control population.

Three strictly endogamous groups of Indian, Brahmin, Bhangi, and Punjabi Khattri, were analyzed to test the authenticity of palmar flexion creases as a criterion of human classification, according to the method of Bali and Chaube ('71), which has certain advantages over the existing classification. The significant frequency variation of palmar creases among different Indian populations and the bisexual and bimanual differences in the frequencies of different crease types indicate a regular distribution trend of the trait.

Fifty-three patients with 60 injuries within the digital sheath, which were treated with primary tendon repair by the Kleinert technique, had follow-up of 6 to 36 months after operation. Seventeen digits had isolated lesions of the profundus tendon and regained an average 38 degrees range of motion (ROM) in the distal interphalangeal (DIP) joint. Forty-three digits had injuries to both the profundus and superficialis tendons, 35 of which flexed within 2 cm from the distal palmar crease (81%), and 21 had a total active motion of at least 200 degrees (49%). The average active ROM in the DIP joint was 30 degrees. The results were superior to our own results with primary repair by the Verdan technique. Comparison with our own results after secondary tendon repair by the Kleinert technique showed that repair of isolated profundus lesions could be done primarily or secondarily within 1 month of injury with equally good results. Primary repair of double tendon lesions gave better results than secondary repair.

A dermatoglyphic and palmar crease analysis was carried out in 100 children aged six months to five years with psychomotor retardation (excluding Down's syndrome). When compared with 121 of the parents and a random group of 168 school-children, certain unusual features were found to be twice as common in the retarded children, demonstrating that these features may indicate an 'at risk' infant if found during routine examination of the newborn.

An accessory flexor digitorum profundus indicis was found during a recent hand exploration. It was a separate muscle-tendon unit lying ulnar to the main flexor digitorum profundus indicis and joined the latter at the level of the distal palmar crease. Its insertion was by both tendon and a small muscle belly.

Digital and palmar dermatoglyphics of 184 male and 224 female normal American Negroes were evaluated for digital patterns, digital ridge counts, palmar patterns, palmar main line terminations, accessory triradii and palmar creases. All subjects were seven year olds examined and found free of chronic or other genetic diseases. The results were presented for the left and right hand separately as well as in terms of bilateral symmetry. The present results were for the most part comparable to those of the African and other American Negro groups reported earlier. The distributions of the various dermatoglyphic features among the Negroes taken as a group were compared to those of the other racial groups and their differences were discussed.

Forty-six consecutive patients requiring flexor tendon grafts were evaluated at 4, 6, 8, 10, 12, 16, 20, 22, 24, 28, 34, and 52 weeks after grafting. The patients were divided into two groups: those who did not require a silicone rubber rod prior to tendon grafting (Group I), and those who did require a silicone rubber rod prior to grafting (Group II). Analysis of the rate of functional recovery as measured by total active motion, gross grip strength, and pinch grip strength showed no significant difference between the two groups. Analysis of the extent of functional recovery at one year, as measured by total active motion and distance of digital pulp from distal palmar crease, showed no significant differences between Groups I and II.

The palmar fascial ligaments have been examined by microdissection using an operating microscope in fresh and preserved cadaveric hands. The palmar fascia is seen to be a precise three dimensional system of skin ligaments having discreet transverse, longitudinal and vertical fibre systems. In the normal hand there is relative motion between the ligament systems on movement. The longitudinal fibres provide a system of skin anchorage which operates irrespective of the position of the underlying joints and acts particularly to resist shearing forces in gripping. The distribution of the lesions of Dupuytren's Disease has been recorded in a series of clinical cases; nodules, skin pits, distortion of the palmar creases, cords and joint contractures, and the pathogenesis of the disease is related to the anatomy of the palmar ligaments. The disease is described as a process of contracture along anatomical pathways. A hypothesis for the development of Dupuytren's Disease is presented whereby the loss of normal motion between palmar fascial ligaments gives rise on use of the hand to stress concentrations which stimulate fibrous tissue deposition and contracture.

Thirty-five patients with grades II through V flexor tendon damage in 42 fingers were treated first with a silicone rubber rod prosthesis and later with a flexor tendon allograft. The final results were evaluated by recording the distance each finger lacked in flexing to the distal palmar crease and by recording the total active motion of each finger. By the first measurement, 63% had good flexion, 21% fair, and 16% poor; and 8% had good total active motion, 71% fair, and 21% poor. An allograft can be used successfully in a two-stage reconstruction of flexor tendons, but based on other reports, an autograft may provide somewhat better results.

Abnormalities of the haemopoietic system may produce anaemia, polycythaemia, lymphadenopathy, hepatosplenomegaly, or purpura alone or in combination. Pallor is the outstanding physical sign of anaemia and is best confirmed by inspection of the conjunctivae and palmar creases. Cardiovascular manifestations of anaemia consist of a high output state with tachycardia and perhaps cardiac failure. If the face and mucous membranes appear plethoric, polycythaemia is suggested and should be confirmed by haemoglobin estimation. A relative polycythaemia may occur in the presence of dehydration. In lymphadenopathy, examination is first carried out from behind, with the patient's neck flexed to relax the sternomastoid muscles. Lymphadenopathy may occur in relationship to the jugular vessels, submandibular and submental regions, and anterior and posterior triangles of the neck. Purpuric spots are spontaneous petechiae or ecchymoses, which represent bleeding into or under the skin. The spots do not blanch on pressure and on fading leave a bluish discolouration as with a bruise. Purpura may be thrombocytopenic or non-thrombocytopenic; it is differentiated by performing a platelet count.

The Ehlers-Danlos syndrome (E-D) is a well recognized genetic disorder of connective tissue of unknown etiology. In the classical form of the disease, skin and joint changes involving the hand often suggest the clinical diagnosis. By studying three affected members in three generations of a family with E-D by means of fingerprint analysis, we have shown the evolution of secondary palmar creases. The possible etiology and significance of these secondary skin creases are discussed.

Inspection of palm creases should be a routine part of the pediatric physical examination. Any variation in the basic configuration should be cause for concern, irrespective of the age of the child. Is the "nor mal" child with an SPC, SL, or other type C palm really normal? Will time or psychologic evaluation uncover an unsuspected abnormality? An otherwise normal infant with palmar crease abnormalities should be considered at risk of developmental problems appearing later.

The rare variegate form of porphyria (inherited as a Mendelian dominant character) which was manifested after 1 week of oral contraceptive treatment with Minovlar in a previously asymptomatic 20-year old woman is reported. Symptoms persisting from July-September 1970 included abdominal pain low backache obstinate constipation vomiting anorexia and weight loss; 7 more days of contraceptive treatment were followed by redness and swelling of face after exposure to sunlight blisters and erosions on the hands and intense itching on both face and hands. In September pigmentation of face neck dorsa of hands and palmar creases was observed with more blisters and scarring. Laboratory investigations showed initial jaundice and transient biochemical findings of liver derangement; urinary and faecal porphyrin values were abnormally high. It is thought that the oestrogenic component of the oral contraceptive caused the patients change from latent to symptomatic variegate porphyria. After the case report current literature of variegate and acute intermittent porphyria is discussed with mention of porphyria cutanea tarda. X-porphyrins present in abnormally high amounts in individuals with variegate porphyria can help distinguish such cases. Excessive activity of hepatic delta-aminolaevulic acid synthetase which determines rate of porphyrin synthesis has been found with variegate porphyria. Acute non-cutaneous symptoms of variegate porphyria must be due to a disturbance of cellular or subcellular metabolism as yet undetermined; mechanism of skin damage is unknown. Oral contraceptives along with several other drugs including sulphonamides and barbiturates should be avoided in cases of variegate porphyria to prevent drug-induced exacerbation.

SEVERAL important facts regarding the management of severe hand injuries are underscored by the following case. Case Report J.B., a 40-year-old cabinetmaker, was admitted to the Boston City Hospital on July 9, 1958, 10 minutes after sustaining an injury to the left hand on a shaping machine at work. Examination revealed a near-transmetacarpal amputation of the distal portion of a hand that remained attached to the proximal portion by a dorsal pedicle (Fig. 1 and 2). The wound was located at the level of the proximal palmar crease as illustrated in the accompanying drawing of the left hand. In the operating . . .

Keratosis punctata of the palmar creases (KPPC) is a variant of keratosis punctata of palms and soles in which lesions are confined to palmar creases. We present a patient with KPPC and study the prevalence of the disease in Caucasians. The survey included 1,001 white patients, that were examined for palmar lesions. We could not find any case that fulfilled the diagnostic criteria for KPPC. We conclude that KPPC is a rare disease among Caucasians but rather common in the black race.

The present study involving MZ and DZ twin pairs, as well as paired normal sibs, was undertaken to investigate further the genetic involvement in the formation of normal palmar crease patterns, as well as simian and Sydney patterns. Two variations of normal creases were investigated: the thenar type R and the distal type I. Both types cluster in families. The R is more frequent in the females. For both types, MZ twins are more concordant than DZ twins or sibs. Sib studies suggest strong genetic involvement in the development of both the R and I crease types. Parents with simian and/or Sydney creases have more children with these traits than offspring of parents without these traits; however, concordance rate comparisons of MZ or DZ twins and sibs do not suggest strong genetic involvement as in the case of the R and I crease types.

A 2-year-old female patient was admitted to the Department of Neurology of the University of Bari because of floppiness and easy fatigability after mild exertion. She had been born after a normal term pregnancy and delivery (birth weight 3250g) but her suction had always been poor because of generalized hypotonia. She had not been able to change her position spontaneously until 1 year of age. Physical examination revealed that the child was overweight and had significant lumbar lordosis with a large protruding abdomen, visceroptosis and a small umbilical hernia. The skin was redundant at the soles of the feet and unusually smooth. The palms were wrinkled with a large number of adventitious palmar creases. She was also noted to have 'pseudohypertrophy' of the calf muscles and joint hypermobility (Figure 1) in particular hyperextensible elbows, fingers, genu recurvatum and severe pes planus. The remainder of the examination revealed nothing remarkable.

A case of an avulsion of a flexor digitorum superficialis tendon insertion of a healthy white man is presented. Five months after the injury exploration showed that the chiasma of the superficialis had retracted to the region of the distal palmar crease. Excision of the distal portion of the superficialis tendon, the scar surrounding the palmar plate, and release of the palmar plate and collateral ligaments was done. Four months after the operation the active range of motion of the proximal interphalangeal joint ranged from 20 to 105 degrees.

Sir .—Maternal floor infarction (MFI) is an uncommon placental lesion associated with stillbirth and intrauterine growth retardation, with an apparent high rate of recurrence in subsequent pregnancies. 1-3 The condition has been infrequently discussed in the obstetric or pathology literature, and essentially no information is available in pediatric or genetic journals. Patient Report .—A 690-g male infant was born after a 36-week gestation. His birth length was 31.0 cm, and his head circumference was 26.0 cm. He had first-degree hypospadias, bilateral cryptorchidism, inguinal hernias, and bilateral bridged palmar creases. No other minor or major anomalies and no specific malformation syndrome were identified. Blood IgM levels, cytomegalovirus, herpes virus, and toxoplasmosis titers, and chromosomal analysis results were normal. The placenta weighed 155 g and showed extensive fibrosis on the maternal surface. Microscopic sections of the placenta confirmed the diagnosis of MFI. The patient's parents are nonconsanguineous and in good health. The

To the Editor.— Doxorubicin (adriamycin) an antitumor antibiotic, 1-3 is used widely in the treatment of childhood and adult tumors and leukemias. Its reported toxic side effects include alopecia, hematopoietic depression, nausea, vomiting, mucosal ulcerations, weight loss, urticaria, electrocardiographic changes, and fatal and nonfatal cardiomyopathy. Recently, we have noticed an additional side effect of this drug, an increase in melanin pigmentation in the nails, nailbeds, phalangeal, and other dermal creases, as shown in the Figure. These changes appear six or more weeks following initiation of doxorubicin administration and have been observed more frequently in black than in white children. Of 13 black children who received doxorubicin alone or in combination with other agents for six weeks or more, five showed increased pigmentation in the nails and nailbeds; three of these five children also had changes in the interphalangeal and palmar creases. In 39 white children, increased pigmentation was seen in

A 44-year-old man had mycosis fungoides and generalized plaque disease involving 80% of his skin surface with diffuse lymphadenopathy and alopecia of the scalp and groin. In addition, distal to the wrist, there were sclerodermatous changes involving the skin of the hands with associated sclerodactyly of all digits with loss of normal palmar creases. There were no subungual telangiectasis or digital ulcers. The changes in the hand that occurred in this case, no doubt arose as a result of the patient's neoplasm. Abnormalities of collagen biosynthesis and degradation probably occur with mycosis fungoides as a result of the extensive infiltration of the epidermis and dermis with malignant cells. To our knowledge, the association of sclerodactyly with mycosis fungoides has not been previously reported.

The occasional patient whose illness is associated with generalized hyperpigmentation presents the physician with a challenging diagnostic problem. The increased deposition of melanin is characteristically diffuse and deepens the coloration of the palmar creases, intertriginous areas, oral mucous membranes, and recent scars. Often it accentuates pressure points such as the knuckles, elbows, and knees. The wide variety of disease processes that may cause such hyperpigmentation demands a working knowledge of pathogenesis and differential diagnosis (Table). Although not frequently cited, the observation that hyperpigmentation occurs in pernicious anemia (Ogbuawa et al, this issue of theArchives[p 388]), is not new. Herbert 1 has emphasized the occasional occurrence of hyperpigmentation in patients with a vitamin B 12 or folic acid deficiency. Vitamin B 12 deficiency with hyperpigmentation has been noted in whites, East Indians, Orientals, and in the black race. 2 If pernicious anemia in blacks is as rare as has

• Certain clinical findings, such as pallor of the conjunctivae, nail beds, lips, oral mucosa, and palmar creases have traditionally been used by physicians in the diagnosis of anemia. We prospectively examined 50 patients to determine whether there was any correlation between these clinical findings and hemoglobin concentration. We noted a statistically significant correlation between hemoglobin concentration and the following: color tint of the lower eyelid conjunctiva, nail-bed rubor, nail-bed blanching, and palmar crease rubor. Results from our study support the contention that the presence and degree of anemia can be estimated clinically by careful physical examination. (Arch Intern Med1988;148:831-832)

In 1973 Jones et al. were the first to report on the so-called fetal alcohol syndrome. Hitherto 16 cases have been published. Within the last 2 years our group has observed 12 additional cases. All infants were hypotrophic at birth; mean length=39 cm, mean weight=1880, mean gestational lenght=38 weeks. The main symptoms were: microcephaly /11/12/, micrognathia /10/, high arched palate /6/, epicanthus /7/; clinodactyly /6/, vitium cordis /4/, genital malformations /6/, pathological palmar creases /9/. Postnatally, growth, weight gain, statomotor and mental development were considerably retarded. The alterations of the heart, brain and dermatoglyphs which can be traced back to distinct gestational stages are ample evidence for damaging influences already occurring during the second and third month of pregnancy. Thus, the syndrome is an embryopathy rather than a fetopathy.

Our studies of skin creases in the early developing hand and in a variety of malformed hands show that these creases, which develop between 7 and 14 weeks of fetal life, are secondard to the early flexion planes in the developing hand. Thus with lack of development or flexion of a digital joint no underlying digital crease was found of a digital joint no underlying digital crease was found; and with lack of formations or oppositional function of the thumb there was no thenar palmar crease. The normal distal palmar sloping crease was found to be related to the sloping flexional plane of the 3rd, 4th, and 5th metacarpal-phalangeal joints and the usual mid-palmar crease represents the plane of skin folding between the sloping distal crease and the thenar crease. The occurrence of a single horizontal palmar crease, the simian crease, was found by roentogenographic studies to be related to a diminished slope of the 3rd, 4th, and 5th metacarpal-phalangeal joints such that only a single early horizontal plane of flexion existed in the skin of the upper palm. Thus hand creases provide a record of the early flexional planes of function in the developing hand and any crease abnormality should always be interpreted as an indication of a more primary defeat in form and/ or function of the early developing hand.

Unusual palmar flexion creases have been reported in a number of clinical syndromes including Down's syndrome, 1 trisomy D, 2 cri-du-chat, 3 and prenatal rubella. 4 These reports have awakened the interest of medical practitioners in a field formerly the province of chiromancers and gypsies. 5 Since palmar flexion creases develop in the embryo 6 and are readily evident at birth, the clinician may inspect them during the perinatal examination. If unusual configurations are present, he may be alerted to the possibility of other anomalies or his confidence in making an early diagnosis of a con genital defect may be strengthened. Systematic study of palmar creases reveals that some individuals who seem quite normal otherwise have unusual palmar flexion creases. Therefore, information on variation in palmar crease configuration in normal populations would

The second case of a variant of Kyrle's disease limited to the palmar creases of the hands is presented. Although the histopathologic picture is similar, the clinical features of Kyrle's original description and the present case are markedly different. The role of trauma in producing the disease is discussed.