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Showing papers in "Archives of Dermatology in 1967"


Journal ArticleDOI
TL;DR: This is a report of a case of nevoid basal cell carcinoma syndrome occurring in a patient with the following new features: gross deformities of bones of the lower extremities, asymmetry of the skull, degenerative changes of shins and vertebrae, increased fragility of bones due to general demineralization, and infected jaw cysts.
Abstract: This is a report of a case of nevoid basal cell carcinoma syndrome occurring in a patient with the following new features: gross deformities of bones of the lower extremities, asymmetry of the skull, degenerative changes of shins and vertebrae, increased fragility of bones due to general demineralization, and infected jaw cysts.

446 citations


Journal ArticleDOI
TL;DR: Methotrexate is used for psoriasis by the majority of teaching services of dermatology in the United States and is contraindicated in pregnancy, hematological disorders, peptic ulceration, and liver disease.
Abstract: Methotrexate is used for psoriasis by the majority of teaching services of dermatology in the United States. Three modes of administration were compared: small daily doses by mouth in interrupted schedules, single large weekly oral doses, and parenteral doses. All methods were effective and had toxicity, but the parenteral route showed the greatest toxicity. The drug is contraindicated in pregnancy, hematological disorders, peptic ulceration, and liver disease. Chromosomal breaks have been reported with greater frequency than normal. Renal clearance is vital. The drug should be considered only for very severe forms of psoriasis.

197 citations


Journal ArticleDOI
TL;DR: Primary changes of the overlying epidermis eventually lead to complete elimination of the altered connective tissue.
Abstract: A case is presented exhibiting an unusual reaction of the skin to superficial trauma. In response to minor trauma, certain changes take place within the papillary layer of the corium characterized by affinity of the connective tissue for hematoxylin. Secondary changes of the overlying epidermis eventually lead to complete elimination of the altered connective tissue.

177 citations


Journal ArticleDOI
TL;DR: Exacerbation following natural or experimental ultraviolet light radiation, distribution of lesions limited to sun-exposed areas of skin, and frequent clinical and histologic similarities to true actinic keratoses indicate that actinic radiation plays an important role in the pathogenesis of DSAP.
Abstract: This study of 31 patients presents disseminated superficial actinic porokeratosis (DSAP) as a distinctive and recognizable entity characterized by many uniformly small, minimal, annular, anhidrotic, keratotic lesions developing during the third or fourth decade of life on sun-exposed areas of skin. It is not a rare condition. While many clinical features of DSAP differ from the classic type of porokeratosis (Mibelli), the histologic features are essentially the same including the typical cornoid lamella, but the features are often minimal. Of six attempts to autotransplant portions of lesions, three were successful. Exacerbation following natural or experimental ultraviolet light radiation, distribution of lesions limited to sun-exposed areas of skin, and frequent clinical and histologic similarities to true actinic keratoses indicate that actinic radiation plays an important role in the pathogenesis of DSAP.

167 citations


Journal ArticleDOI
TL;DR: It was concluded that before 1 year of age pigmentation increases, and after the age of 2 it may show a regression.
Abstract: A statistical study of 240 patients with nevus of Ota was carried out. The onset was before 1 year of age in 119 and after the age of 2 in 121. Onset between the ages of 1 and 11 years is distinctly rare. In type 3, 77% of the cases began before 1 year of age. Color is influenced by fatigue, menstruation, insomnia, and weather. The association of ocular and buccal melanosis and of Mongolian spots and blue nevus was discussed. Among 207 families, there were two familial incidences and 15 patients whose parents were related, but heredity was not suggested. No malignant change was observed. Clinical features in childhood, in adolescence, and in old age were described. Spontaneous regression did not occur. The clinical course in childhood was followed, and subtle changes of extent, tone, and intensity of color were observed. It was concluded that before 1 year of age pigmentation increases, and after the age of 2 it may show a regression.

161 citations


Journal ArticleDOI
TL;DR: Investigations of the biology of melanin have increased in number and diversity during the last decade and have clinical significance because problems associated with melanocytic changes are constantly encountered in internal medicine, surgery, pediatrics, and neurology, as well as in dermatology.
Abstract: Investigations of the biology of melanin have increased in number and diversity during the last decade. Melanocytes, the cellular units of melanin formation, are easily identified by light and electron microscopy and can be grown in tissue culture. Studies of melanocytes have clinical significance because problems associated with melanocytic changes are constantly encountered in internal medicine, surgery, pediatrics, and neurology, as well as in dermatology.

148 citations


Journal ArticleDOI
TL;DR: Nine selected cases of a chronic, recurrent segmental hyalinizing vasculitis of the dermis of unknown cause with cutaneous findings which resemble atrophie blanche are presented.
Abstract: Nine selected cases of a chronic, recurrent. segmental hyalinizing vasculitis of the dermis are presented. Heretofore, many of these cases had been classified as livedo reticularis with ulceration, but none of the patients in this series had livedo. The cutaneous findings are limited to the lower extremities and may include a patchy livedo-like pattern of purpura, ulcers, and, in the healed phase, scars which resemble atrophie blanche. Pathologically, this is a process of relatively noninflammatory endothelial proliferation, subintimal hyalinization, and focal thrombosis in the vessels of the middle dermis of unknown cause.

129 citations


Journal ArticleDOI
TL;DR: A technique for accurately measuring transepidermal water diffusion in vivo depends upon electrically monitoring the changing humidity of a stream of dry air passed through a skin chamber, finding that the loss is constant for any given area under standard ambient conditions of humidity and temperature.
Abstract: A technique for accurately measuring transepidermal water diffusion in vivo depends upon electrically monitoring the changing humidity of a stream of dry air passed through a skin chamber. The test is performed on skin in which the sweat glands have been pharmacologically inactivated. The rate of diffusional water loss was found to be 0.2 to 0.3 mg/hr -1 cm -2 for most areas of the human trunk. The loss is constant for any given area under standard ambient conditions of humidity and temperature. Considerable regional variation was noted in certain areas, even after the readings had been corrected for varying horny layer thickness and expressed as diffusion constants. Compared with that of the back, the diffusion constant is four times greater on the forehead, nine times greater on the back of the hand, and one hundred times greater through the palm. Vasoconstriction, vasodilation, or whealing in the underlying dermis did not influence diffusive water loss. Removing the horny layer by stripping with cellophane tape abolished the barrier, increasing water loss 50-fold. Sodium lauryl sulfate and dimethyl sulfoxide directly altered the horny layer, immediately increasing outward water diffusion. Superficial defatting of the horny layer had virtually no effect on water loss.

119 citations


Journal ArticleDOI
TL;DR: Recurrence is frequent in verrucous hemangioma because of the involvement of the deeper tissue and possibly because of altered hemodynamics opening up preexisting noncanalized malformed vessels.
Abstract: Verrucous hemangioma is a structural variant of capillary or cavernous hemangioma in which reactive epidermal acanthosis, papillomatosis, and hyperkeratosis develop secondarily. The clinical appearance of verrucous hemangioma may simulate that of angiokeratoma and the separation of the two lesions is important, since the histologic and clinical behavior are different. Verrucous hemangiomas are vascular malformations, whereas the angiokeratomas are telangiectasias, Verrucous hemangioma usually involves the lower extremities and spreads slowly. As the lesion spreads, satellite nodules may develop. Verrucous hemangioma involves the dermis and the subcutaneous fat, but angiokeratoma involves only the papillary dermis. Recurrence is frequent in verrucous hemangioma because of the involvement of the deeper tissue and possibly because of altered hemodynamics opening up preexisting noncanalized malformed vessels. Verrucous hemangioma should be excised while still small to prevent large and unsightly scars.

117 citations


Journal ArticleDOI
TL;DR: The ultrastructural identity of these dermal macrophages to epidermal Langerhans' cells supports the hypothesis that the epider mal LangerHans' cell is a macrophage.
Abstract: Biopsy specimens of cutaneous lesions from two patients with histiocytosis X of the infantile type (Letterer-Siwe disease) were examined with the electron microscope. More than one half of the dermal macrophages in both specimens contained organelles identical to epidermal Langerhans' cell granules. These organelles appear to arise during the process of endocytosis of extracellular material and are ultimately found in various stages of degradation within Iysosomelike granules. The ultrastructural identity of these dermal macrophages to epidermal Langerhans' cells supports the hypothesis that the epidermal Langerhans' cell is a macrophage.

109 citations


Journal ArticleDOI
TL;DR: The diagnosis of angiokeratoma should be considered in the differential diagnosis of malignant melanoma, and is considered a telangiectasia rather than a hemangioma.
Abstract: Angiokeratomas from 116 patients were studied. Eighty-three percent of the patients had solitary lesions. Most solitary angiokeratomas are small, bluish to black, rough and warty growths occurring on all parts of the body but especially on the lower extremities. The diagnosis of angiokeratoma should be considered in the differential diagnosis of malignant melanoma. Fifteen percent, or 17, of the lesions were clinically diagnosed as melanoma and a wide surgical excision carried out. The lesion is classified as angiokeratoma but is considered distinctly different from the angiokeratomas of Mibelli, Fordyce, and Fabry by location and cause. It is considered a telangiectasia rather than a hemangioma.

Journal ArticleDOI
TL;DR: The majority of the clear cell tumors of the skin represent an immature eccrine sweat gland tumor and Ail tumor cells were positive for enzymes present in normal eccrine glands in a higher concentration than in normal apocrine glands.
Abstract: Clear cell hidradenoma was found to be composed of two main types of cells: (1) a fusiform, dense cell located at the periphery of the tumor strands contained a large number of tonofilaments and a small amount of glycogen; and (2) a large and "clear" cell, differentiated from the former toward the center of the tumor strands, was filled with glycogen but poor in tonofilaments. Ail tumor cells were positive for enzymes present in normal eccrine glands in a higher concentration than in normal apocrine glands. The luminal spaces were lined by one of four types of epithelium, ie, embryonic epidermal, dermal, transitional, or secretory. Some tubular lumina were found to be very similar to the unopened secretory coil of immature embryonic eccrine gland. The majority of the clear cell tumors of the skin represent an immature eccrine sweat gland tumor.

Journal ArticleDOI
TL;DR: The lipids from isolated human sebaceous glands have been studied by thin- layer chromatography and no thin-layer chromatographic spots were found that corresponded to the reference standards of hydrocarbon, sterol esters, free fatty acids, sterols, monoglycerides or diglycerides.
Abstract: The lipids from isolated human sebaceous glands have been studied by thin-layer chromatography. Lipids were present that corresponded to the reference standards of squalene, wax esters, and triglycerides. No thin-layer chromatographic spots were found that corresponded to the reference standards of hydrocarbon, sterol esters, free fatty acids, sterols, monoglycerides or diglycerides.

Journal ArticleDOI
TL;DR: A viewpoint is expressed that subclinical anomalous arteriovenous channels may preexist in the afflicted dermatitic areas, thus giving rise to the peculiar features of angiomatous stasis dermatitis.
Abstract: A case of an unusual angiomatous stasis dermatitis arising from a congenital arteriovenous (AV) malformation that morphologically and histologically simulated Kaposi's disease is reported. The clinical manifestations associated with congenital AV fistulae are reviewed, particularly along lines of coexistent cutaneous vascular abnormalities. The developmental pathophysiology of the stasis lesion is briefly discussed. A viewpoint is expressed that subclinical anomalous arteriovenous channels may preexist in the afflicted dermatitic areas, thus giving rise to the peculiar features.

Journal ArticleDOI
TL;DR: The confrontation between the "teacher-journal" and the "student-reader," either in quick encounters between chores or more formally and leisurely by assignment, is likely to provide the most stimulating and productive kind of learning.
Abstract: A JOURNAL is a unique educational modality, a classic medium for communication without sound and without moving pictures, bringing ideas and information to those desirous of learning. This instrument of conveying ideas and information is a sort of teacher, a quiet, silent, and provocative teacher. It is a precise teacher, bringing new tools and new insights to the reader. And it makes of that reader, desirous of learning, a student, disciplined, attentive, and inquiring. A journal is a demanding taskmaster, insistent on thinking and on deliberative contemplation of premises (sometimes expansive), and of findings (sometimes miniscule). The confrontation between the "teacher-journal" and the "student-reader," either in quick encounters between chores or more formally and leisurely by assignment, is likely to provide the most stimulating and productive kind of learning, and may indeed provide the opportunity for transition of student to scholar. If a journal does not offer easy exchange between

Journal ArticleDOI
TL;DR: The renewal time for the stratum corneum of the back was found to be about two weeks, for the back of the hand three weeks, and for the forehead one week, while the reduced forehead time may reflect a real increase in epidermal turnover rate at this site.
Abstract: A simple method for estimating the turnover time of the human stratum corneum is described. The horny layer is impregnated with a fluorescent dye and the site followed until the fluorescence disappears. The renewal time for the stratum corneum of the back was found to be about two weeks, for the back of the hand three weeks, and for the forehead one week. The increased time on the back of the hand is probably related to the increased thickness of the horny layer at this site. The reduced forehead time is not related to thickness and may reflect a real increase in epidermal turnover rate at this site.

Journal ArticleDOI
TL;DR: Preliminary data concerning successful passive transfer in guinea pigs of photocontact hypersensitivity using mononuclear peritoneal exudates is presented, finding patterns of cross-reactivity similar to those observed in man.
Abstract: Photocontact dermatitis was observed in 32 patients who had been exposed to brominated salicylanilides or other closely related chemicals used as antimicrobial agents in soaps. Patterns of cross-reactivity between tribromsalan, dibromsalan, metabromsalan, TCSA, bithional, hexachlorophene, and trichlorcarbanilide were assessed. Action spectrum studies using monochromatic radiation indicated a peak reactivity in the 3,600Angstrom range. Twelve of the 32 patients were found to be persistent light reactors. Their clinical characteristics, abnormal reaction to sunburn radiation, and course are described. Contact photosensitivity was induced in Hartley strain albino guinea pigs with both TCSA and tribromsalan. Patterns of cross-reactivity were assessed and found to be similar to those observed in man. Preliminary data concerning successful passive transfer in guinea pigs of photocontact hypersensitivity using mononuclear peritoneal exudates is presented.

Journal ArticleDOI
TL;DR: Twenty-four patients were studied for Klein-Waardenburg syndrome, a congenital disorder of deafness, eye changes, and localized hypomelanosis, and two deaf patients with oculocutaneous albinism were studied and patients previously reported with "albinoidism" and Klein- Waitingburg syndrome were reexamined.
Abstract: Twenty-four patients were studied for Klein-Waardenburg syndrome, a congenital disorder of deafness, eye changes, and localized hypomelanosis. Two deaf patients with oculocutaneous albinism were studied and patients previously reported with "albinoidism" and Klein-Waardenburg syndrome were reexamined. Piebaldness with deafness alone may also be an associated disorder.

Journal ArticleDOI
TL;DR: Eruptive hidradenoma, a rare type of syringoma, was studied histologically, histochemically, and electron microscopically in two patients and it seemed that the lesion was undergoing constant destruction and regeneration.
Abstract: Eruptive hidradenoma, a rare type of syringoma, was studied histologically, histochemically, and electron microscopically in two patients. In spite of its peculiar clinical differences, such as occurrence of numerous lesions with limited distribution to anterior surfaces of the body, it showed features identical to the eyelid type syringoma. Direct connections were found between the lesions and epidermis and between individual lesions, but not between the lesions and underlying eccrine secretory segments. Also, cystic or tubular spaces were lined with an "immature eccrine intra-epidermal type" of epithelium which possessed a periluminal tonofilament band, lysosomes, keratohyaline granules, and often underwent complete keratinization. No secretory epithelium was found in the lesion. Dermal histiocytes with typical Langerhans cell granules and collagen fiber bundles were seen in parenchyma. It seemed that the lesion was undergoing constant destruction and regeneration.

Journal ArticleDOI
TL;DR: It would appear that the incidence of basal cell carcinoma in rhinophyma is significantly greater than in the skin of normal noses.
Abstract: Forty-seven patients with rhinophyma were studied, five of whom had basal cell carcinoma of the nose. The microscopic changes of rhinophyma are characterized by epidermal epithelial hyperplasia and pilosebaceous gland hyperplasia, fibrosis, inflammation, and telangiectasia of the corium. It would appear that the incidence of basal cell carcinoma in rhinophyma is significantly greater than in the skin of normal noses. This indication of greater incidence among rhinophyma patients should be examined further by a study of the incidence of rhinophyma among basal cell carcinoma patients.

Journal ArticleDOI
TL;DR: An unusual but characteristic bullous dermatosis occurring in diabetics, is described and a possible etiologic mechanism is suggested.
Abstract: An unusual but characteristic bullous dermatosis occurring in diabetics, is described. The diagnostic features which separate it from other bullous disorders are discussed, and a possible etiologic mechanism is suggested.

Journal ArticleDOI
TL;DR: The difficulty in evaluating a potential contactant, the industrial sources of which are reputed to be many, but unknown, and which is present in a popular topical medication together with two unknown contactants and triamcinolone is illustrated.
Abstract: Thirteen patients presenting with contact dermatitis and having positive results of closed patch tests to 1% ethylenediamine dihydrochloride solution were observed in a four-month period. A mixture containing triamcinolone acetonide, neomycin, gramicidin, and nystatin (Mycolog Cream) which contains ethylenediamine as a stabilizer had been recently used by 12 of these patients. Only four of the ten patients who were patch tested to Mycolog Cream had a positive response. Eleven of the patients presented with a history of rapid onset of a patchy eczematous generalized eruption. Two patients given aminophylline, which contains theophylline and ethylenediamine, developed an exacerbation of their generalized patchy eczematous eruptions. In all patients, the dermatitis cleared following the use of 1% hydrocortisone cream and avoidance of Mycolog Cream. This article illustrates the difficulty in evaluating a potential contactant, the industrial sources of which are reputed to be many, but unknown, and which is present in a popular topical medication together with two unknown contactants and triamcinolone.

Journal ArticleDOI
TL;DR: Two types of intranuclear structures which have not been found in normal material were seen and on morphological grounds there was no evidence that either of these was viral in nature, the latter, however, had an appearance similar to mycoplasma.
Abstract: Biopsy specimens from 11 patients with lichen planus have been studied by electron microscopy to see if changes could be revealed which would indicate the etiology of this condition. Apart from inflammatory cells no abnormality was detected in the dermis. The basal lamina frequently showed multiplication and breaks. Tonofibrils lost their electron density, regular arrangement, and in advanced stages attachment to desmosomes. Breakdown of desmosomes and half-desmosomes occurred at all stages of the disease. Two types of intranuclear structures which have not been found in normal material were seen. One of these was lamellar in appearance and the other consisted of a membrane-bound mass of granular material. On morphological grounds there was no evidence that either of these was viral in nature, the latter, however, had an appearance similar to mycoplasma.

Journal ArticleDOI
TL;DR: Cutaneous and visceral biopsies from three of these patients, plus autopsy specimens from another show that the lipoglycoprotein that characterizes the disease is also present in very small amounts outlining occasional blood vessels in the jejunum, urinary bladder, appendix, pancreas, lung, kidney, lymph node, and striated muscle.
Abstract: The literature about lipoid proteinosis has previously documented the presence of a peculiar, characteristic, and ill-defined chemical substance in certain inexplicable patterns of deposition in the dermis of the skin, submucosa of the mouth, tongue, tonsil, larynx, pharynx, trachea, esophagus, stomach, rectum, and vagina, and also in the testes, eye, and brain. Six cases were studied, four of them previously unreported. Cutaneous and visceral biopsies from three of these patients, plus autopsy specimens from another show that the lipoglycoprotein that characterizes the disease is also present in very small amounts outlining occasional blood vessels in the jejunum, urinary bladder, appendix, pancreas, lung, kidney, lymph node, and striated muscle. Lipoid proteinosis is unquestionably a generalized disorder involving many organs besides the skin and mucous membranes.

Journal ArticleDOI
TL;DR: The scalded skin syndrome, or toxic epidermal necrolysis of Lyell, is a nonspecific reaction pattern of the skin that may be lethal to infants less than 1 year of age, but children between 1 and 6 years of age usually recover rapidly, exhibiting a mortality of only 7%.
Abstract: The scalded skin syndrome, or toxic epidermal necrolysis of Lyell, is a nonspecific reaction pattern of the skin. In small children, the syndrome has been associated with group 2 staphylococcal infection, as has previously been described in Ritter's disease. We have observed this association in four children. Other cases of scalded skin syndrome are almost certainly due to drugs. Other infectious and toxic agents are also occasionally responsible for the syndrome. Although the scalded skin syndrome may be lethal to infants less than 1 year of age, children between 1 and 6 years of age usually recover rapidly, exhibiting a mortality of only 7%. In patients 6 or more years of age, the syndrome is fatal more than 40% of the time.

Journal ArticleDOI
TL;DR: Clinically normal skin of psoriatic patients showed histologic and enzymatic evidence of altered metabolism, as evidenced by glucose-6-phosphate dehydrogenase activity, and metabolism in the pentose-monophosphate shunt was markedly increased in the keratinizing zone in the psoriasis lesion.
Abstract: Sections from 100 psoriatic lesions commonly showed parakeratosis, Munro microabscesses, a diminished or absent granular layer, acanthosis, papillomatosis, tortuosity and dilatation of capillaries, edema, and chronic inflammation in the upper corium. Vacuolization, disruption and hydropic degeneration of the basal cells above the tips of the dermal papillae associated with exocytosis of polymorphonuclear leukocytes and spongiosis were found to be important features which have not been generally emphasized. Clinically normal skin of psoriatic patients showed histologic and enzymatic evidence of altered metabolism. Metabolism in the pentose-monophosphate shunt, as evidenced by glucose-6-phosphate dehydrogenase activity, was markedly increased in the keratinizing zone in the psoriatic lesion. This change was also found in certain other dermatoses and is not specific for psoriasis.

Journal ArticleDOI
TL;DR: Optical systems and staining procedures are described to facilitate examination of normal and abnormal horny cells, based upon removal of horny cells directly onto adhesive coated glass slides.
Abstract: A procedure for studying the cytology and morphology of the horny cells of the skin has been described. It is based upon removal of horny cells directly onto adhesive coated glass slides. By viewing the cells in their broad axes, many details may be seen which are concealed in traditional transverse sections. Optical systems and staining procedures are described to facilitate examination of normal and abnormal horny cells.

Journal ArticleDOI
TL;DR: Both localized and generalized photoallergies were noted in the same patient and the generalized sensitivity was transient, with emphasis placed upon the role that the abundant Australian sunshine must play.
Abstract: Photoallergy to fenticlor (S7) in three patients and to Multifungin in four patients is reported. Fenticlor (bis[2-hydroxy-5-chlorphenyl] sulfide), is related chemically to bithionol. Multifungin (5-bromo-4′-chlorosalicylanilide), is related chemically to tribromosalicylanilide. Fenticlor and Multifungin are used as antifungal applications in Australia. Photo-patch tests were carried out using direct sunlight. Cross-sensitization between fenticlor and hexachlorophene was demonstrated and there was also evidence to suggest crosssensitization between fenticlor and bithionol. Cross-sensitization between Multifungin and tribromosalicylanilide was found in all four cases. There was a close similarity between the patchtest responses to these two chemicals. Both localized and generalized photoallergies were noted in the same patient. The localized sensitivity might last for several months. The generalized sensitivity was transient. The possible mechanisms producing these responses are discussed in relation to the clinical findings, with emphasis placed upon the role that the abundant Australian sunshine must play.

Journal ArticleDOI
TL;DR: The hematoxylin and eosin and elastic tissue stains revealed a bizarre disturbance of the horny layer, and topical keratolytics, superficial surgery, cryotherapy, and desiccation were unacceptable forms of therapy.
Abstract: Multiple minute digitate hyperkeratoses are located on the chest, arms, and legs of a 32-yearold Negro soldier. The lesions do not resemble acrochordon (or "skin tags") or previously described epidermal defects. The hematoxylin and eosin and elastic tissue stains revealed a bizarre disturbance of the horny layer. Topical keratolytics, superficial surgery, cryotherapy, and desiccation were unacceptable forms of therapy.

Journal ArticleDOI
TL;DR: To the best of the knowledge, this is considered to be the first confirmed case of blue rubber bleb nevus in association with Maffucci's syndrome, also called Bean-Maffucci syndrome.
Abstract: A case is reported of a 31-year-old Japanese woman who exhibited numerous cavernous hemangiomas characteristic of blue rubber bleb nevus over almost the entire body surface. Erectile or polypoid hemangiomas were also found on the visible oropharyngeal and anal mucosa, and radiological findings strongly suggested that similar tumors were distributed in the mucosa of the esophagus and were grouped on that of the distal ileum. Chronic iron-deficiency type of anemia was also detected. In addition, she exhibited multiple enchondromas in the bones of fingers and toes. To the best of our knowledge, this is considered to be the first confirmed case of blue rubber bleb nevus in association with Maffucci's syndrome, also called Bean-Maffucci syndrome.