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Showing papers in "Archives of Dermatology in 1980"


Journal ArticleDOI
TL;DR: Clinical and pathologic features were reviewed in 35 cases of acral lentiginous melanoma of the palms and soles, which probably represents the most common expression of melanoma in blacks; two thirds of the patients in this study were black.
Abstract: • Acral lentiginous melanoma represents a fourth variant of malignant melanoma in company with lentigo maligna melanoma, superficial spreading melanoma, and nodular melanoma. It is characterized by a lentiginous (radial) growth phase that evolves over months or years to a dermal (vertical) invasive stage. Clinical and pathologic features were reviewed in 35 cases of acral lentiginous melanoma of the palms and soles. This variant of melanoma probably represents the most common expression of melanoma in blacks; two thirds of the patients in our study were black. The average three-year survival rate was 11%. (Arch Dermatol116:773-776, 1980)

211 citations


Journal ArticleDOI
TL;DR: Two-dimensional electrophoresis on the two protein fractions of the abnormal hair confirmed that the abnormality is caused by decreased synthesis of high-sulfur matrix proteins, which may account for the various disease features in these persons.
Abstract: Trichothiodystrophy, or sulfur-deficient brittle hair, is a clinical marker for a neuroectodermal symptom complex that usually features mental and physical retardation and may also include nail dystrophy, lamellar ichthyosis, ocular dysplasia, dental caries, and decreased fertility. Cystine-deficient hair is common to all patients. The hairs from two new patients were studied, and the most distinctive microscopic hair findings were striking bright and dark bands seen with polarizing microscopy using crossed polarizers. To date, all hair samples showing this banding have had an abnormally low sulfur content. Two-dimensional electrophoresis on the two protein fractions of the abnormal hair confirmed that the abnormality is caused by decreased synthesis of high-sulfur matrix proteins. Disturbances of the transport or utilization of sulfur-containing amino acids in other neuroectodermal tissues may be proposed to account for the various disease features in these persons.

204 citations


Journal ArticleDOI
TL;DR: Solar exposure was the most important risk factor for both basal and squamous cell carcinoma; however, the ability to tan was shown to be of special importance even at low levels of exposure, and subjects over 60 years of age were shows to be at higher risk for nonmelanoma carcinoma than those at younger ages.
Abstract: • The relative importance of risk factors such as solar exposure, complexion, age, and the ability to tan were examined for their relationships to the development of basal and squamous cell carcinoma. Three samples were studied: patients with basal cell carcinoma (N = 366), patients with squamous cell carcinoma (N = 58), and control subjects (N = 294). The technique of logistic regression was used to estimate the relative risk of each type of carcinoma. Solar exposure was the most important risk factor for both basal and squamous cell carcinoma; however, the ability to tan was shown to be of special importance even at low levels of exposure. Given the same level of cumulative lifetime solar exposure, subjects over 60 years of age were shown to be at higher risk for nonmelanoma carcinoma than those at younger ages. Complexion was only shown to be significant for basal cell carcinoma. ( Arch Dermatol 116:454-456, 1980)

166 citations


Journal ArticleDOI
TL;DR: It is concluded that methotrexate administration may result in severe oligospermia and that these inhibiting effects on spermatogenesis are reversible.
Abstract: • A young man with severe psoriasis was treated intermittently with methotrexate. On two different occasions, severe oligospermia documented by serial seminal fluid analyses was present coincident with methotrexate administration. Discontinuation of methotrexate resulted in normal sperm concentration. Measurements of serum gonadotrophin and testosterone levels were normal during and after methotrexate treatment. It is concluded that methotrexate administration may result in severe oligospermia and that these inhibiting effects on spermatogenesis are reversible. (Arch Dermatol116:215-217, 1980)

165 citations


Journal ArticleDOI
TL;DR: A significantly greater frequency of malignancy was found with dermatomyositis than with polymyositis, and the prognosis is similar in the two forms of myositis.
Abstract: • The association of malignancy with dermatomyositis and polymyositis has been questioned. During the last 20 years (1956 to 1975), we have studied 58 cases of myositis that met predefined diagnostic criteria. These cases were analyzed for the frequency of malignancy, prognosis, and the value of a diagnostic test series for malignancy. A significantly greater frequency of malignancy was found with dermatomyositis than with polymyositis. The prognosis of dermatomyositis and polymyositis appears to be altered in the presence of malignancy. In the absence of malignancy, the prognosis is similar in the two forms of myositis. Lastly, the value of a screening laboratory and roentgenographic investigation for the presence of occult malignancy beyond a thorough history, physical examination, and the use of basic laboratory tests such as complete blood count, stool guaiac test, urinalysis, multiphasic analysis, and chest roentgenogram was not documented by this study. (Arch Dermatol116:295-298, 1980)

153 citations


Journal ArticleDOI
TL;DR: Historical data, the results of skin tests, and the known pharmacology of captpril suggest that these captopril-induced eruptions may be the result of potentiation of kinin-mediated cutaneous reactions.
Abstract: • Captopril, an orally active dipeptidylcarboxypeptidase inhibitor, is a promising new antihypertensive agent. Cutaneous reactions are the most common side effects of this therapy. The data from 15 previous cases and seven new ones are reviewed. The cutaneous eruptions appear to be pharmacologic in nature, occurring mostly at higher dosage schedules and resolving at lower dosage levels. Historical data, the results of skin tests, and the known pharmacology of captopril suggest that these captopril-induced eruptions may be the result of potentiation of kinin-mediated cutaneous reactions. (Arch Dermatol116:902-905, 1980)

142 citations


Journal ArticleDOI
TL;DR: Ten patients with variegate porphyria were uniformly found to have distinctive plasma porphyrin fluorescence wavelength maxima in saline-diluted plasma specimens, indicating that plasma fluorescence emission that is maximal at 626 +/- 1 nm is a diagnostic marker for varie Gate Porphyria.
Abstract: • Ten patients with variegate porphyria were uniformly found to have distinctive plasma porphyrin fluorescence wavelength maxima in saline-diluted plasma specimens. The porphyrin complex in each of these plasma samples had a fluorescence emission maximum at 626 ± 1 nm. Twelve patients with porphyria cutanea tarda, eight patients with etythropoietic protoporphyria, one patient with congenital erythropoietic porphyria, two patients with acute intermittent porphyria, and four patients with hereditary coproporphyria, whose plasma specimens were similarly examined, had plasma fluorescence characteristics that were different from those of the patients with variegate porphyria. Plasma fluorescence emission that is maximal at 626 ± 1 nm is a diagnostic marker for variegate porphyria. ( Arch Dermatol 116:543-547, 1980)

136 citations


Journal ArticleDOI
TL;DR: Fourteen children with generalized morphea involving all levels of the skin and soft tissues were examined and Pulmonary changes in five patients and esophageal changes in one imply that acral pansclerotic morphea may be assoicated with mild nonprogressive visceral change.
Abstract: • Fourteen children with generalized morphea involving all levels of the skin and soft tissues were examined. The term "acral pansclerotic morphea" describes the distribution and the multiple levels of sclerosis. Lymphocytic inflammation and hyaline panniculitis were observed on biopsy specimens in some cases. Laboratory data were characterized by a polyclonal elevation of γ-globulin level and by peripheral eosinophilia. Pulmonary changes in five patients and esophageal changes in one imply that acral pansclerotic morphea may be associated with mild nonporgressive visceral change. Although cyclophosphamide may retard the process, no satisfactory treatment for progressive, mutilating acral pansclerotic morphea has been found. ( Arch Dermatol 116:169-173, 1980)

109 citations


Journal ArticleDOI
TL;DR: Chloroquine phosphate and hydroxychloroquine sulfate are substituted 4-amino quinoline compounds that differ only by a hydroxy group that are shown to be effective in the treatment of lupus erythematosus (LE).
Abstract: Chloroquine phosphate and hydroxychloroquine sulfate are substituted 4-amino quinoline compounds that differ only by a hydroxy group. Quinacrine hydrochloride also has the 4-amino quinoline radical but has, in addition, a benzene ring; it is classified as an acridine compound. The 4-amino quinoline radical is present in all antimalarial compounds shown to be effective in the treatment of lupus erythematosus (LE) (Figure).1 HISTORY Quinacrine was introduced for malaria therapy in 1930. Its superiority over quinine was established during World War II, when it became the official drug for the treatment of malaria. The toxicity of quinacrine and its inability either to cure malaria or to act as an effective prophylactic, however, spurred continued research for better drugs. A large series of 4-amino quinolines were investigated. Of these, chloroquine proved to be the most promising and was released for field trial.2By the end of World War II, it had

107 citations


Journal ArticleDOI
TL;DR: Age of the patient and duration of therapy have been found to be significant factors in those patients receiving only the weekly oral dosage schedule and fibrosis of the liver developed to a degree that contraindicated further treatment with methotrexate.
Abstract: † Fibrosis of the liver developed to a degree that contraindicated further treatment with methotrexate in 11 of 43 patients who had been receiving maintenance therapy with methotrexate for psoriasis. Liver biopsy had been performed prior to initiation of methotrexate therapy and was repeated at 12- to 18-month intervals. In this retrospective study, age of the patient and duration of therapy have been found to be significant factors in those patients receiving only the weekly oral dosage schedule. Yearly biopsies of the liver are recommended for patients who receive methotrexate throughout their courses of therapy. ( Arch Dermatol 116:413-415, 1980)

103 citations


Journal ArticleDOI
TL;DR: The distribution of lesions in the authors' patients may have been caused, in part, by the use of tight-fitting, uninsulated riding pants, which may have slowed blood flow through the skin, thereby further reducing tissue temperature.
Abstract: • We describe four patients with panniculitis attributable to a combination of cold exposure and equestrian activities. All were young, healthy women who rode horses for at least two consecutive hours per day throughout the winter. Initially, several small, erythematous, pruritic papules appeared on the superior-lateral portions of one or both thighs. During one week, the lesions progressed to indurated, red-to-violaceous, tender plaques and nodules. Studies for cryofibrinogens and cryoglobulins were negative. The histologic picture was that of a panniculitis with prominent inflammation of veins most notable at the dermal-subcutaneous fat junction. Cold panniculitis is not limited to infancy and childhood. The distribution of lesions in our patients may have been caused, in part, by the use of tightfitting, uninsulated riding pants. Such attire may have slowed blood flow through the skin, thereby further reducing tissue temperature. ( Arch Dermatol 116:1025-1027, 1980)

Journal ArticleDOI
TL;DR: Topical application of 0.05% fluocinonide in an adhesive base was investigated in patients with oral vesiculoerosive diseases and each patient's condition continued to respond to the treatment when its use was required.
Abstract: Patients with oral vesiculoerosive diseases frequently require corticosteroid treatment to induce healing and reduce pain. Because of the adverse effects associated with systemic use, topical application of 0.05% fluocinonide in an adhesive base was investigated. The study group was comprised of 89 patients with erythema multiforme, lichen planus, benign mucous membrane pemphigoid, or pemphigus. In the double-blind phase involving 15 patients, seven responded completely and eight partially to the topical ointment. In the open study that included 74 patients, 35 had complete disappearance of signs and symptoms, while 39 experienced partial benefit. There were no adverse side effects. All 89 patients were observed for three to 35 months and each patient's condition continued to respond to the treatment when its use was required.

Journal ArticleDOI
TL;DR: Using a new protocol with varied exposure increments, it is found that, in 18 of 20 patients with psoriasis vulgaris who were given ultraviolet (UV)-B phototherapy three times a week, the disease completely cleared.
Abstract: • Using a new protocol with varied exposure increments, we found that, in 18 of 20 patients with psoriasis vulgaris who were given ultraviolet (UV)-B phototherapy three times a week, the disease completely cleared. One patient's condition substantially improved, and one patient's condition failed to respond. Ten to 38 treatments were required during a three- to 13-week period. The only topical treatment agent used was white petrolatum. The results are compared with a schedule using five treatments a week and fixed UV-B-exposure increments. Infrequent (three times weekly) treatments, avoidance of hospitalization and crude coal tar treatment, and fewer episodes of UV-radiation burn may offer advantages for selected patients. Long-term effects and maintenance requirements for this treatment regimen are not known. ( Arch Dermatol 1981;117:623-626)

Journal ArticleDOI
TL;DR: The hypothesis that Jewish people are at higher risk than others for the development of pemphigus is supported and provides a basis for comparison with the results of other studies of this disease.
Abstract: • An investigation of a possible cluster of pemphigus cases that were diagnosed in Hartford County, Connecticut, in 1977 provided data for estimating the incidence of this disease in a defined population for the years 1972 to 1977. The average annual incidence (new cases per population per year) estimated for the overall adult population (over the age of 20 years) was 0.42 cases per 100,000 people, whereas that for Jewish adults was 3.2 cases per 100,000. Thus, the results of this report both support the hypothesis that Jewish people are at higher risk than others for the development of pemphigus and provide a basis for comparison with the results of other studies of this disease. ( Arch Dermatol 116:1035-1037, 1980)

Journal ArticleDOI
TL;DR: In spite of this relationship, serial titers were not found to be consistent enough to be used reliably as a guide to therapy or prognosis in pemphigus.
Abstract: • Twenty cases of pemphigus extracted from the literature and 19 patients from UCLA with pemphigus were studied in detail regarding the possible correlation of pemphigus titer and disease activity. A statistically significant relationship between titer and disease activity, as well as change in titer and change in disease activity, was found. However, in spite of this relationship, serial titers were not found to be consistent enough to be used reliably as a guide to therapy or prognosis in pemphigus. (Arch Dermatol116:285-290, 1980)

Journal ArticleDOI
TL;DR: Thirty-nine well-known European, mainly German, dermatologic academicians worked together to create a treatise that emphasizes the important aspects of morphology, offers numerous well-chosen illustrations for an easy comprehension of the text, and discusses modern pathophysiology and treatment.
Abstract: Volume 2 of Dermatologie in Praxis und Klinik , edited by Prof Korting, director of the skin clinic of the University of Mainz, West Germany, is the second volume in a series of four up-to-date German-language textbooks. Thirty-nine well-known European, mainly German, dermatologic academicians and their collaborators worked together to create a treatise that emphasizes the important aspects of morphology, offers numerous well-chosen illustrations for an easy comprehension of the text, and discusses modern pathophysiology and treatment. Three surgeons describe the current management of burns, cold injuries, and other physically caused skin problems. Individual chapters deal with acute infectious erythemas and exanthemas, drug eruptions, erythema multiforme, erythema nodosum, erythema elevatum diutinum, psoriasis, the parapsoriasis group of disease, pityriasis rubra pilaris, the eczemas, occupational dermatoses, dyshidrosis, noninfectious pustuloses, physically caused skin diseases, xeroderma pigmentosum, urticaria, prurigo, lichen planus, lichen nitidus, Darier's disease, pyodermas, mycobacterial infections, sarcoidosis, Melkersson-Rosenthal syndrome, leprosy, leishmaniasis, viral

Journal ArticleDOI
TL;DR: Abundant perivascular pigmented material was present at all levels of the dermis below the upper papillary portion and most likely represents a metabolic derivative of minocycline.
Abstract: • Localized brown to blue-black discoloration of the skin occurred in three patients receiving long-term minocycline hydrochloride therapy. Abundant perivascular pigmented material was present at all levels of the dermis below the upper papillary portion. Histochemical studies demonstrated reactivity with the Prussian blue stain and the Fontana-Masson silver technique. The granules were brightly refractile by dark-field illumination. Ultrastructurally, there were membranebound dense intracellular inclusions differing from melanin and iron but identical to those known to occur in the thyroid glands of minocycline-primed laboratory animals. The abnormal pigment most likely represents a metabolic derivative of minocycline. (Arch Dermatol116:1262-1265, 1980)

Journal ArticleDOI
TL;DR: A 57-year-old man with prurigo nodularis, unresponsive to conventional therapy, was successfully treated with thalidomide.
Abstract: • A 57-year-old man with prurigo nodularis, unresponsive to conventional therapy, was successfully treated with thalidomide. The pharmacology and side effects of the drug are briefly reviewed. Why thalidomide is effective for prurigo nodularis is unclear, but possible mechanisms of action are discussed. (Arch Dermatol116:571-572, 1980)

Journal ArticleDOI
TL;DR: The existence of a local defect in cell-mediated immunity in skin altered by long-term sun exposure is suggested, and Responses to intradermally injected common antigens were less intense in sun-damaged skin that in nonexposed skin.
Abstract: In previously sensitized volunteers, higher concentrations of 2,4-dinitrochlorobenzene were necessary to elicit positive patch test reactions on the sun-damaged skin of the neck than on the protected skin of the upper part of the back. This difference was not found in subjects without evidence of sun damage, and there was no difference between sun-damaged and nondamaged skin in response to a primary irritant. Responses to intradermally injected common antigens were also less intense in sun-damaged skin that in nonexposed skin. These findings suggest the existence of a local defect in cell-mediated immunity in skin altered by long-term sun exposure.

Journal ArticleDOI
TL;DR: Hypersensitivity, manifested by generalized nummular eczematous and papular dermatitis, and presumably by contact urticaria, developed in a 47-year-old man after four years of using oral and topical aloe.
Abstract: • Hypersensitivity, manifested by generalized nummular eczematous and papular dermatitis, and presumably by contact urticaria, developed in a 47-year-old man after four years of using oral and topical aloe. Patch tests for aloe were positive in this patient. ( Arch Dermatol 116:1064-1065, 1980)

Journal ArticleDOI
TL;DR: From 1950 through 1975, 27 patients at the Mayo Clinic, Rochester, Minn, had coexistent psoriasis and lupus erythematosus (LE), in whom the disorders were severe and complicated by a third disease.
Abstract: From 1950 through 1975, 27 patients at the Mayo Clinic, Rochester, Minn, had coexistent psoriasis and lupus erythematosus (LE). Of the 27 patients, ten had systemic LE (SLE), 13 had discoid LE, and four had drug-induced LE or an SLE-like syndrome. The onset of psoriasis preceded LE in ten patients and was concomitant with it in six. Photosensitivity was noted in 23 patients. In 20 patients, the lesions of psoriasis and LE remained clinically distinct. Morphologic overlap and clinical interaction occurred in seven patients in whom the disorders were severe and complicated by a third disease. Generally, this latter group had extensive psoriasis, prominent photosensitivity, and a poor response to therapy.

Journal ArticleDOI
TL;DR: Solar lentigines were reinvestigated by a number of different methods to gain a better perspective on their structure and the melanosome complexes inside the keratinocytes were much larger than those found in noninvolved skin.
Abstract: • Solar lentigines were reinvestigated by a number of different methods to gain a better perspective on their structure. In histologic sections and dopa preparations of split skin, large numbers of melanocytes were seen crowded at the base of the clubbed, budding rete ridges. In splitskin preparations, the scanning electron microscope showed complex systems of ridges, columns, and craters on the underside of the lentiginous epidermis. Oval bodies measuring 15 to 30 μ, with dendrites, were numerous at the apices of the complex epidermal ridges; these bodies were presumed to be melanocytes. In transmission electron micrographs of lentigines, the melanosome complexes inside the keratinocytes were much larger than those found in noninvolved skin. The complex and distinctive architecture of these maculae is probably the result of concurrent proliferation of melanocytes and keratinocytes. ( Arch Dermatol 116:1151-1154, 1980)

Journal ArticleDOI
TL;DR: Ten patients with disorders of keratinization were treated with oral isotretinoin (13-cis-retinoic acid) on an investigational protocol to test the efficacy, safety, and optimal dosage schedule for using the drug in these rare disorders.
Abstract: • Ten patients with disorders of keratinization were treated with oral isotretinoin (13- cis -retinoic acid) on an investigational protocol to test the efficacy, safety, and optimal dosage schedule for using the drug in these rare disorders. Elevations of serum triglyceride levels above the highest normal levels developed in seven of the ten patients, while they maintained normal levels of serum cholesterol. This effect was found to be dose and/or time related and reversible. Moderate elevations of serum triglyceride levels have not been clearly established as a risk factor for the development of coronary artery disease. High levels, however, may precipitate acute pancreatitis. For this reason, the conditions of patients receiving retinoids must be carefully monitored for triglyceride abnormalities throughout their courses of treatment. ( Arch Dermatol 116:1369-1372, 1980)

Journal ArticleDOI
TL;DR: A case of eruptive xanthomas and increased blood triglyceride levels that developed in a patient taking isotretinoin is reported here and it is reported that the patient had had Darier's disease since he was 12 years old.
Abstract: Isotretinoin (13-cis-retinoic acid) has been shown to be of benefit in treating disorders of keratinization, such as Darier's disease and ichthyosis 1,2 and severe cystic acne. 3 Increased blood triglyceride levels have been reported in rats being given isotretinoin 4,5 and have now been noted in patients taking isotretinoin as part of a multicenter study for disorders of keratinization (Hoffmann-LaRoche Inc, Nutley, NJ, written communication, July 27, 1979). We report here a case of eruptive xanthomas and increased blood triglyceride levels that developed in a patient taking isotretinoin. Report of a Case A 28-year-old man had had Darier's disease since he was 12 years old. Examination at the Mayo Clinic, Rochester, Minn, showed confluent keratotic papules with extensive crusting on the scalp, forehead, and ears. The keratotic papules were also extensive but more discrete on the face, upper part of the trunk, and upper extremities. There were only scattered papules

Journal ArticleDOI
TL;DR: Pathologic findings and reports of lipodystrophies associated with nephritis, hypocomplementemia, and other inflammatory or immunologic disorders offer an opportunity for histologic and immunologic study of patients with localized lipoatrophy.
Abstract: • Four patients with localized lipoatrophy were observed. All were female, and three were children. Lymphocytic panniculitis was observed in early biopsy specimens of all four cases, with focal histiocytic granulomas in one case. Later biopsy specimens in three patients showed that the inflammation was transient. These pathologic findings and reports of lipodystrophies associated with nephritis, hypocomplementemia, and other inflammatory or immunologic disorders offer an opportunity for histologic and immunologic study of such patients. Localized lipoatrophy may represent the end stage of panniculitis in a localized expression of connective tissue disease. ( Arch Dermatol 116:1363-1368, 1980)

Journal ArticleDOI
TL;DR: The management of congenital melanocytic nevi (CMN) has become a controversial subject and some dermatopathologists believe CMN are histologically distinct from lesions developing after birth, but at least two issues remain to be resolved.
Abstract: The management of congenital melanocytic nevi (CMN) has become a controversial subject. Since the studies of Greeley et al, 1 it has become apparent that patients with giant ("bathing trunk" and others) nevi have a relatively increased incidence of malignant melanoma developing in their lesions. Now the subject of debate has been enlarged to include all CMN. I stress the term "congenital" because CMN occur at a much earlier time in development than nevi appearing after birth. The presence of such lesions on the upper and lower eyelids (the "wink" nevus) suggests that they may be formed between 50 and 53 days of embryologic age. Furthermore, some dermatopathologists 2 believe CMN are histologically distinct from lesions developing after birth. At least two issues remain to be resolved. Can CMN be classified by size (or any other way) into those that should be removed and those that may be safely left

Journal ArticleDOI
TL;DR: It is believed that SIN either as Bowen's or non-Bowen's type may serve as an important marker for the presence of internal malignancy.
Abstract: † The relationship of Bowen's disease to internal malignancy is controversial. We have studied 72 cases of Bowen's disease and compared them with 58 cases of non-Bowen's squamous intraepidermal neoplasia (SIN). Clinical data were obtained in all cases in an attempt to identify carcinogens and to detect the occurrence of other cutaneous or internal malignancies. Internal malignancy was not found more commonly in patients with Bowen's disease than in those with other forms of SIN. However, 5.7% of all patients with SIN were found to have a concurrent internal malignancy. Other cutaneous malignancies were also more frequent in these groups and may relate to sun exposure. Evidence for arsenic ingestion was not found in most patients. We believe that SIN either as Bowen's or nonBowen's type may serve as an important marker for the presence of internal malignancy. ( Arch Dermatol 116:422-426, 1980)

Journal ArticleDOI
TL;DR: A 66-year old man with bronchial asthma who was receiving immunosuppressive medication and in whom Kaposi's sarcoma developed is described.
Abstract: • Immunosuppressed patients are at risk of acquiring Kaposi's sarcoma. We describe here a 66-year-old man with bronchial asthma who was receiving immunosuppressive medication (prednisone given for systemic effect) and in whom Kaposi's sarcoma developed. The literature on this subject is reviewed. ( Arch Dermatol 116:1280-1282, 1980)

Journal ArticleDOI
TL;DR: It is suggested that the term "ectodermal dysplasia" be limited to those disorders that are congenital, are diffusely present, are not progressive, and do involve the epidermis and at least one of the appendages.
Abstract: • The ectodermal dysplasias are a heterogeneous group of disorders that, in the past, has included conditions best classified as progeroid disorders. The inaccuracy of the terminology has led to a proliferation of syndromes in which the patients are said to have poorly defined "ectodermal dysplasia," and a real need exists to define that appellation further. We suggest that the term "ectodermal dysplasia" be limited to those disorders that are congenital, are diffusely present, are not progressive, and do involve the epidermis and at least one of the appendages. We recognize that a heterogeneous group of disorders remains that generically have certain similarities. Not enough is known about the defects in each of the elements of the skin affected in these conditions to classify them more accurately. Several recently described disorders appear to have some degree of ectodermal dysplasia. ( Arch Dermatol 116:1295,1299, 1980)

Journal ArticleDOI
TL;DR: Evaluation of bullous or porphyrialike dermatoses in patients treated with hemodialysis should include adequate testing for increased porphyrin levels, as this patient and three previously reported cases have been found to have abnormal p Morphyrin study results.
Abstract: • A fourth case of symptomatic porphyria associated with hemodialysis for chronic renal failure is reported. Subepidermal bullous dermatoses of patients who have undergone hemodialysis have not usually been associated with elevated porphyrin levels. However, this patient and three previously reported cases have been found to have abnormal porphyrin study results in association with skin lesions typical for porphyria cutanea tarda, occurring after hemodialysis. Hemodialysis does not effectively decrease circulating plasma uroporphyrin levels, although some dialysis of uroporphyrin into the dialysate could be measured in this case. Evaluation of bullous or porphyrialike dermatoses in patients treated with hemodialysis should include adequate testing for increased porphyrin levels. ( Arch Dermatol 116:191-195, 1980)