Showing papers in "Archives of Dermatology in 1987"

Xeroderma Pigmentosum: Cutaneous, Ocular, and Neurologic Abnormalities in 830 Published Cases

Abstract: • Quantitative frequencies of clinical abnormalities in xeroderma pigmentosum were estimated by abstracting published descriptions of 830 patients in 297 articles obtained from a survey of the medical literature from 1874 to 1982. The median patient age was 12 years with nearly equal numbers of male and female patients. Cutaneous symptoms (sun sensitivity or freckling) had a median age of onset of between 1 and 2 years. Forty-five percent of the patients described had basal cell carcinoma or squamous cell carcinoma of the skin. The median age of first nonmelanoma skin cancer among patients with xeroderma pigmentosum was 8 years, more than 50 years less than that among patients with skin cancer in the United States. Melanomas were reported in 5% of patients. Ninety-seven percent of the reported basal and squamous cell carcinomas and 65% of the melanomas in patients with xeroderma pigmentosum occurred on the face, head, or neck. Seventy percent probability of survival was attained at age 40 years, a 28-year reduction in comparison with the US general population. Ocular abnormalities were reported in 40% of the patients described and were restricted to tissues exposed to ultraviolet radiation (lid, conjunctiva, and cornea) and included ectropion, corneal opacity leading to blindness, and neoplasms. Neurologic abnormalities were found in 18% of the cases reported, consisting of progressive mental deterioration, hyporeflexia or areflexia, and progressive deafness in some patients in association with dwarfism and immature sexual development. There was scant information concerning the efficacy of any therapeutic regimen. (Arch Dermatol1987;123:241-250)

Toxic epidermal necrolysis. Clinical findings and prognosis factors in 87 patients.

Abstract: • Eighty-seven patients with toxic epidermal necrolysis were observed at Hopital Henri Mondor in Creteil, France, over the last 12 years. The mean percentage of body surface area involved was 39%. Erosive mucous membrane lesions, identical to those of Stevens-Johnson syndrome, were present in all but three cases. Necrolysis was sometimes generalized within 24 hours but usually spread progressively after a Stevens-Johnson syndrome—like aspect at the onset. Mortality was 25%. Infection, mainly with Staphylococus aureus and Pseudomonas aeruginosa , was the first cause of death, clearly responsible in ten of 20 cases. Age, extension of necrolysis, idiopathic nature of toxic epidermal necrolysis, ingestion of many drugs, elevation of urea, creatinine, and glucose levels, neutropenia, lymphopenia, and thrombocytopenia were statistically linked to a bad prognosis. A multivariant analysis showed that three of these prognosis factors are of paramount importance, namely: age, area of necrolysis, and serum urea level. Pigmentary changes and sicca syndrome were frequently observed sequelae in survivors. ( Arch Dermatol 1987;123:1160-1165)

Surgical margins for basal cell carcinoma.

Abstract: • Basal cell carcinomas frequently extend beyond their visible borders. Therefore, the goal of surgical therapy must be to eradicate both the clinically apparent tumor and its microscopic extension into the surrounding normal-appearing skin. This entails excising the tumor along with a margin of clinically normal skin. Unfortunately, there is no agreement as to the optimal width of surgical margins. We therefore studied 117 cases of previously untreated, well-demarcated basal cell carcinoma. Prior to excision, the normal-appearing skin surrounding the tumor was marked in 2-mm increments. The tumor was then excised using Mohs micrographic surgery. The extent of the subclinical tumor invasion was calculated from the presurgical skin markings. For tumors with a diameter less than 2 cm, a minimum margin of 4 mm was necessary to totally eradicate the tumor in more than 95% of cases. ( Arch Dermatol 1987;123:340-344)

Cellular retinoid-binding proteins.

Abstract: • A number of specific carrier proteins for members of the vitamin A family have been discovered. Two of these proteins bind all-trans-retinol and are found within cells important in vitamin A metabolism or function. These two proteins have considerable sequence homology and have been named cellular retinol-binding protein (CRBP) and cellular retinol-binding protein, type II (CRBP [II] ). A third intracellular protein, cellular retinoic acid-binding protein (CRABP) also is structurally similar but binds only retinoic acid. Although retinol appears to be bound quite similarly by the two retinol-binding proteins, subtle differences are apparent that appear to be related to the different functions of the two proteins. That, coupled with the specific cellular locations of the two proteins, suggests their roles. Cellular retinol-binding protein appears to have several roles, including (1) delivering retinol to specific binding sites within the nucleus and (2) participating in the transepithelial movement of retinol across certain blood-organ barriers. In contrast, CRBP (II) appears to be involved in the intestinal absorption of vitamin A and, in particular, may direct retinol to a specific esterifying enzyme, resulting in the production of fatty acyl esters of retinol that are incorporated into chylomicrons for release to the lymph. Like CRBP, CRABP can deliver its ligand retinoic acid to specific binding sites within the nucleus, sites different from those for retinol. The nuclear binding of retinol and retinoic acid may be part of the mechanism by which vitamin A directs the state of differentiation of epithelial tissue. (Arch Dermatol1987;123:1693-1695a)

Genetic susceptibility to toxic epidermal necrolysis.

Abstract: • The pathophysiologic events leading to toxic epidermal necrolysis (TEN) remain unknown. With the idea of an immunologically mediated reaction occurring in predisposed subjects we performed HLA-A, -B and -DR typing in 44 patients surviving TEN. We observed a significant increase of only HLA-B12, previously found associated with ocular complications of Stevens-Johnson syndrome. When patients were stratified according to the drugs involved as causes for their TEN, we found other HLA phenotypes associated with B12, varying with each category of drugs. Sulfonamide-related cases of TEN were linked to A29, B12, and DR7, while oxicam-related cases of TEN were linked to A2 and B12. These results suggest that a genetic background, related to the major histocompatibility complex, may contribute to severe blistering drug reactions. (Arch Dermatol1987;123:1171-1173)

Acquired Immunodeficiency Syndrome— Associated Psoriasis and Reiter's Syndrome

Abstract: • Human immunodeficiency virus (HIV) causes a spectrum of immunodysfunction, the most severe of which is the acquired immunodeficiency syndrome (AIDS). We have followed the course of psoriasis in 13 patients over 2 ½ years in a population of more than 1000 HIV-positive individuals. Four patients had a history of mild psoriasis that became severe and uncontrollable as symptoms of immunodeficiency developed. Psoriasis and HIV positivity, AIDS-related complex, or AIDS simultaneously developed in nine patients. In addition to psoriasis, Reiter's syndrome (arthritis, urethritis, and conjunctivitis) developed in one patient in the first group and three patients in the second group. Opportunistic infections, especially candidiasis andStaphylococcus, drugs, and an altered immune system may contribute to the development or flare of psoriasis in these patients. The appearance of severe psoriasis (especially in a patient with other risk factors for HIV) should prompt evaluation for HIV, and may be a poor prognostic indicator in HIV-positive patients, since nine of our 13 patients have died. Immunosuppressive therapy with methotrexate is contraindicated in this group of patients. Newer forms of drug therapy including etretinate show promising results for the management of AIDS-associated psoriasis. (Arch Dermatol1987;123:1622-1632)

The culprit drugs in 87 cases of toxic epidermal necrolysis (Lyell's syndrome).

Abstract: Between 1972 and 1985, 87 patients with toxic epidermal necrolysis (TEN) were admitted to the dermatological intensive care unit at Hopital Henri Mondor, Creteil, France. The culpable drug was determined by standardized criteria. Only three patients had received no drugs before the onset of TEN. Most patients (71 of 87) were receiving more than one drug. Patients had taken an average of 4.4 +/- 3.4 drugs each. A culpable drug was determined in 67 patients (77%). The mean time from first drug administration to onset of TEN was 13.6 +/- 8.4 days. The culprit drugs included the following: sulfonamides, 18 cases, and especially sulfamethoxazole and trimethoprim, 12; anticonvulsants, seven (barbiturates and carbamazepine only); nonsteroidal anti-inflammatory drugs, 29 (especially the phenylbutazone derivative, 16, and oxicam derivatives, 10); allopurinol, three; chlormezanone, three; and others, seven. Aspirin, antipyretics, and antibiotics are infrequently implicated in this series. The pattern of culprit drugs changed with years. The level of sulfonamide-related TEN remained the same, while incidence of nonsteroidal anti-inflammatory drug-induced TEN increased sharply, the introduction of oxicam derivatives being in part responsible.

Complications of Cutaneous Laser Surgery: A Survey

Abstract: We surveyed a selected group of 139 dermatologists and plastic surgeons about their experience with the complications of cutaneous laser surgery. Reported complication rates varied from 0% to 35%, with means of 3.2% for dermatologists and 6.2% for plastic surgeons using the argon laser, and 4.2% for dermatologists and 2.8% for plastic surgeons using the carbon dioxide laser. Hypertrophic scarring was the complication noted most frequently; 69% of physicians using the argon laser and 64% of physicians using the carbon dioxide laser have seen at least one case of hypertrophic scarring. Complications noted less frequently include infection, pain, atrophic scarring, intraoperative or postoperative hemorrhage, and prolonged wound healing. Environmental accidents were few. No procedure-related deaths, ocular damage, or secondary cutaneous malignant neoplasms were reported. We conclude that cutaneous laser surgery has an acceptable risk profile but that complications are not uncommon.

Prognostic significance of hypopigmentation in malignant melanoma.

Abstract: • It has been suggested that the presence of cutaneous hypopigmentation favorably influences the prognosis of patients with malignant melanoma (MM). To examine this possibility, we have compared the actual with the predicted survival of 46 patients with MM and hypopigmentation who were among 1130 patients with MM entered in a long-term prospective study of MM at the New York University Medical Center. The actual average five-year survival rate of the patients with MM and hypopigmentation (86.3%) was significantly better than predicted (74.8%) on the basis of the risk factors present in each patient at the time of entry into the study. The findings suggest that hypopigmentation is a factor that beneficially influences the prognosis of MM, and that the mechanisms that inhibit or destroy normal melanocytes in patients with MM may also slow the growth of this cancer. ( Arch Dermatol 1987;123:1053-1055)

A survey of skin problems and skin care regimens in the elderly.

Abstract: • In an attempt to provide clinically relevant data regarding both dermatologic disease and skin care needs in the elderly, 68 noninstitutionalized volunteers, aged 50 to 91 years (average age, 74 years), were enrolled in a study consisting of a 33-item questionnaire and a total cutaneous examination. Two thirds of the entire group and 83% of the 23 octogenarians reported medical concerns regarding their skin, with pruritus as the most frequent complaint. On examination, all subjects had at least one cutaneous abnormality, and symptomatic and/ or medically significant disorders were present in 64.7%. In decreasing order of prevalence, disorders for which dermatologic therapy was judged desirable included actinic keratoses, tinea pedis, contact dermatitis, seborrheic dermatitis, stasis dermatitis, and skin cancer. Overall, there was rather poor correlation between the subjects' complaints and perceptions and objective physical findings. Further, despite a high prevalence and long average duration of dermatologic concerns, very few subjects had consulted a physician for these problems, and no complaints other than "rashes" and pruritus had ever been discussed with any health care professional. Review of skin care regimens revealed substantial limitations with regard to bathing, shampooing, and nail care, particularly for subjects aged 80 years or older. Despite a small sample size and possibility of selection bias among the subjects, these data strongly suggest that skin problems are common among the elderly and that at present their dermatologic needs are largely unmet. (Arch Dermatol1987;123:1638-1643)

Thin malignant melanomas with regression and metastases

Abstract: • The significance of partial regression in thin malignant melanomas (0.76 mm or less) of the skin was evaluated to determine if the regression was associated with the later development of metastases in patients who previously were considered to have a favorable prognosis. Of 575 patients with primary cutaneous melanoma treated and followed up by the Division of Surgical Oncology at the University of Illinois, Chicago, we found that 103 (18%) had tumors that measured less than 0.76 mm. Of these, 30 (29%) showed histologic evidence of partial regression. In six (20%) of the 30 patients, visceral metastases developed and the patients died. All six had more than 77% regression of their primary tumors. Of the remaining 24 patients, only one had regression greater than 77% and she is still alive three years after diagnosis. Most of these 24 (83%) patients had regression of less than 50% (mean, 29.9%). No metastasis occurred in the 73 patients who had thin melanomas without histologic evidence of regression. It is apparent from this study that patients with thin melanomas who show partial regression cannot be included in the "low-risk" group if the extent of regression is 75% to 80% or more. (Arch Dermatol1987;123:1326-1330)

Acute Graft-vs-Host Disease: Development Following Autologous and Syngeneic Bone Marrow Transplantation

Abstract: • Graft-vs-host disease (GVHD) is a frequent complication of allogeneic bone marrow transplantation but has been infrequently reported following autologous or syngeneic bone marrow transplantation. Ninety-six autologous and 19 syngeneic marrow transplants were performed at our institution between July 1977 and March 1984. We report acute cutaneous GVHD occurring in seven patients who received autologous marrow and two patients who received marrow from an identical twin. All nine patients had clinically detectable eruptions and had skin biopsy specimens with histologic changes of grade 2 acute GVHD. Although most cases were mild and self-limiting, four patients required systemic corticosteroids to treat their disease. Thus, acute cutaneous GVHD was seen in approximately 8% of patients receiving autologous or syngeneic bone marrow transplants at our institution. ( Arch Dermatol 1987;123:745-750)

Skin as the Site of Vitamin D Synthesis and Target Tissue for 1,25-Dihydroxyvitamin D3: Use of Calcitriol (1,25-Dihydroxyvitamin D3) for Treatment of Psoriasis

Abstract: • Vitamin D is a hormone, not a vitamin. The skin is responsible for producing vitamin D. During exposure to sunlight, ultraviolet radiation penetrates into the epidermis and photolyzes provitamin D3to previtamin D3. Previtamin D3can either isomerize to vitamin D3or be photolyzed to lymisterol and tachysterol. Vitamin D is also sensitive to sunlight and is photolyzed to 5,6-transvitamin D3, suprasterol I, and suprasterol II. In Boston, solar irradiation only produces previtamin D3in the skin between the months of March and October. Aging, sunscreens, and melanin all diminish the capacity of the skin to produce previtamin D3. Once formed, vitamin D3enters the circulation and is sequentially metabolized to 25-hydroxyvitamin D3and 1,25-dihydroxyvitamin D3(1,25-[OH]2-D3). The epidermis possesses receptors for 1,25-(OH)2-D3.1,25-(OH)2-D3D3inhibits the proliferation of cultured keratinocytes and induces them to terminally differentiate. The topical or oral administration of 1,25-(OH)2-D3has proved to be effective for the treatment of psoriasis. Therefore, the skin is the site for the synthesis of vitamin D and a target tissue for its active metabolite. The successful use of 1,25-(OH)2-D3for the treatment of psoriasis heralds a new approach for the treatment of this enigmatic disorder. (Arch Dermatol1987;123:1677-1683a)

Control of growth and differentiation in vitro of human keratinocytes cultured in serum-free medium.

Abstract: • Normal human proliferative keratinocytes can be serially cultured in serum-free medium. The medium and culture conditions that have been developed support the growth of these cells in the absence of a feeder layer and in the absence of contaminating fibroblasts. Using this system, the nutritional and hormonal factors that affect the growth and differentiation of these cells have been studied in the absence of undefined supplements. The data suggest that the control of proliferation and differentiation in keratinocytes may take place by unique mechanisms. The defined serum-free conditions that have been developed provide an excellent system for the study of nutritional disorders that affect human epidermal cells. In addition, the culture system can be used to propagate large numbers of cells for use in autografts for cases of severe burns. (Arch Dermatol1987;123:1541a-1544a)

Keratitis, ichthyosis, and deafness (KID) syndrome. Vertical transmission and death from multiple squamous cell carcinomas.

Abstract: • A father and daughter had typical clinical features of the keratitis, ichthyosis, and deafness (KID) syndrome, as described by Skinner et al in 1981. To our knowledge, ours is the first observation of a vertical transmission of this syndrome. The mechanism of inheritance is uncertain. These two patients as well as the 26 previously described exhibited a typical hyperkeratotic eruption, which should not be confused with ichthyosis. The characteristic features are diffuse hyperkeratosis, keratotic plaques, reticulated hyperkeratosis on the face, peribuccal grooves, and heavy-grain leatherlike keratoderma. The occurrence of multiple squamous cell carcinomas underlines the seriousness of this congenital ectodermal defect. Etretinate can relieve the lesions without actually improving the chances of survival. (Arch Dermatol1987;123:777-782)

Isotretinoin and Cutaneous Helper T-Cell Lymphoma (Mycosis Fungoides)

Abstract: Retinoids, including isotretinoin, have demonstrated antiproliferative and antineoplastic activity in laboratory and clinical trials. In a phase II trial, 25 patients with extensive mycosis fungoides were evaluated for response to isotretinoin. There was a 44% (11 patients) objective clinical response rate with three clinical complete responses without concomitant evidence of pathologic clearing of the disease. An additional 24% (six patients) showed a minor degree of clinical improvement. The median time to response was two months (range, 0.5 to eight months) and the median response duration was eight months or longer (range, one to 25 months). Chronic toxic reactions consisted primarily of drying of the skin and mucous membranes and resulted in dose reduction in the majority of patients. It is concluded that isotretinoin produces significant clinical benefit to some patients with mycosis fungoides.

Essential fatty acids and epidermal integrity.

Abstract: • The intercellular spaces of the stratum corneum contain multilamellar lipid sheets derived from the extruded contents of lamellar granules. In the absence of linoleic acid, lamellar granules appear empty, and only fragmentary extracellular sheets are found. This defective differentiation is attributable to substitution of oleate for linoleate inO-acylsphingolipids. Normally, linoleate is ester-linked to 30- to 34-carbon ω-hydroxyacids, which, in turn, are amide-linked to sphingosine. Acylglucosylceramides, bearing a β-D-glucosyl moiety on the sphingosine, may provide the driving force for lamellar granule assembly. The ω-hydroxyacyl chains are long enough to span a lipid bilayer, while the linoleate inserts into an adjacent bilayer. This interaction could promote assembly of lamellar granules. It has also been proposed that acylceramides may stabilize the extracellular sheets by a similar mechanism. In addition, the horny cell has been found to possess a covalently bound lipid envelope consisting principally of ω-hydroxyacylsphingosines derived fromO-acylsphingolipids. (Arch Dermatol1987;123:1381-1384)

Livedo reticularis associated with increased titers of anticardiolipin antibodies in systemic lupus erythematosus.

Abstract: • Seventy-eight consecutive patients with systemic lupus erythematosus were assessed for the presence of livedo reticularis. The possible association of livedo reticularis with other clinical and laboratory features including anticardiolipin antibodies was explored. Thirty-eight patients had livedo reticularis. Four cases were severe, 11 moderate, and 23 mild. There was a statistically significant association between the combined moderate and severe livedo reticularis group and elevated levels of anticardiolipin antibodies. The recognized association of anticardiolipin antibodies with thrombotic events suggests a possible pathogenetic role. The presence or history of central nervous system disease, renal disease, vasculitis, or lupus inhibitor was significantly associated with the moderate and severe livedo reticularis group. Livedo reticularis may be a cutaneous marker for the later development of important systemic events in systemic lupus erythematosus. ( Arch Dermatol 1987;123:596-600)

Evaluation of anthralin in the treatment of alopecia areata

Abstract: • Anthralin cream 0.5% to 1.0% was used to treat 68 patients with severe alopecia areata. Therapy was relatively well tolerated, although all patients experienced pruritus and local erythema and scaling. Cosmetic response was seen in 17 (25%) of the patients, and was maintained during therapy in 12 (71%) of the 17 cosmetic responders. For the patients treated with 0.5% anthralin, the mean time to response (44 of 66 patients) was 11 weeks; the mean time to cosmetic response (13 of 66 patients) was 23 weeks. Duration of the current episode of hair loss did not correlate with cosmetic response. Compared with other currently available topical treatments, anthralin appears to be a reasonable therapeutic option for severe alopecia areata. (Arch Dermatol1987;123:1491-1493)

Mast Cell Numbers in Diffuse Scleroderma

Abstract: • Numbers of mast cells were quantitated in the lesions of diffuse scleroderma and morphea. Mast cells increased and then decreased in number in the papillary dermis of diffuse scleroderma. No significant change of mast cell numbers was noted in the reticular dermis. Mast cells increased in the papillary dermis with fine collagen bundles (grade 2 skin of scleroderma) and decreased in the papillary dermis with homogeneous collagen bundles (grade 3 skin of scleroderma). The total number of cells increased in the papillary dermis of grade 1 and 2 skin of scleroderma and decreased in the grade 3 skin of scleroderma. In morphea a reduced number of mast cells was noted in grade 3 lesions. It is suggested that mast cells play an important role in fibrotic process of scleroderma skin. (Arch Dermatol1987;123:205-208)

Efficacy of low-dose cyclosporine in severe atopic skin disease.

Abstract: To the Editor.— Cyclosporine is a drug that is new to dermatology and, therefore, awaits further study. 1,2 Its use, however, must be closely monitored because of possible side effects. 1 Recently, in psoriasis the favorable effect of low-dose cyclosporine (≤5 mg/ kg/d) treatment in patients with severe drug-resistant psoriasis has been reported. 3,4 Studies on cyclosporine therapy in severe cases of drug-resistant atopic dermatitis have not yet been reported, to our knowledge. Report of a Case.— We have observed the temporary effect of short-term cyclosporine treatment in two women (Table), with severe, drug-resistant, pruritic, activedisseminated, chronic prurigolike lesions and eczema. Case 1 was classified as atopic dermatitis with serum IgE levels greater than or equal to 20000 IU/mL (≥48000 μg/L). Clinical Data on Cyclosporine Treatment in Two Patients With Severe Drug Resistant Atopic Dermatitis Body Area Affected, % Improve-Patient ment Cyclosporine No./ After 4 After 4 Blood Age, y/ Start

Neurotropic Melanoma. A variant of desmoplastic melanoma.

Abstract: • In ten cases of neurotropic melanoma, patients presented with nodules composed of amelanotic, deeply infiltrating neuroid fascicles. Only four cases were clinically suggestive of melanoma. In eight of the tumors, a precursor lesion was found histologically and provided a major clue to the diagnosis. In seven cases, the dysplastic precursor was lentiginous, while a superficial spreading pattern was present in one. Initial surgery was often inadequate because of the difficulty in defining tumor margins and the lack of pigment. In seven of the tumors, S100 protein was demonstrated within the invasive spindle cell component by the immunoperoxidase method. This finding was negative in three cases, two of which showed positive staining of the precursor and nerve filaments, indicating that the absence of S100 protein cannot be used as an exclusion criterion for neurotropic melanoma. ( Arch Dermatol 1987;123:907-912)

Dermatologic signs in anorexia nervosa and bulimia nervosa.

Abstract: • The dermatologic changes in anorexia nervosa and bulimia nervosa may be the first signs to give the clinician a clue that an eating disorder is present, as many of these patients either deny their symptoms or secretly refuse to comply with treatment. The dermatologic signs are a result of (1) starvation or malnutrition, eg, lanugolike body hair, asteatotic skin, brittle hair and nails, and carotenodermia; (2) self-induced vomiting, eg, hand calluses, dental enamel erosion, gingivitis, and a Sjogrenlike syndrome; (3) use of laxatives, diuretics, or emetics and their dermatologic side effects; and (4) other concomitant psychiatric illness, eg, hand dermatitis from compulsive handwashing. Further, as most of the cutaneous signs are not specific to anorexia nervosa and bulimia nervosa, failure to include eating disorders in the differential diagnosis may lead to misdiagnosis of the cutaneous symptoms. ( Arch Dermatol 1987;123:1386-1390)

Panniculitis Associated With Severe a1-Antitrypsin Deficiency: Treatment and Review of the Literature

Abstract: • Panniculitis associated with homozygous severe α 1 -antitrypsin deficiency was documented in three women hospitalized for painful cutaneous and subcutaneous ulcerations (severe panniculitis with spontaneous ulceration and drainage of clear or serosanguineous fluid). None had a history of trauma or infection. One patient responded rapidly and completely to treatment with dapsone. One patient, who had more extensive disease, failed to respond to prednisone plus dapsone; infusions of α 1 -proteinase inhibitor concentrate led to resolution of her panniculitis. One patient who had severe and extensive panniculitis and pleural effusions failed to respond to corticosteroids but did well when both dapsone and infusions of α 1 -proteinase inhibitor concentrate were added to her treatment program. ( Arch Dermatol 1987;123:1655-1661)

Mast-cell stabilization to decrease neurofibroma growth. Preliminary experience with ketotifen.

Abstract: • Based on (1) the large numbers of mast cells present in neurofibromas, (2) the possibility that these mast cells contribute directly to neurofibroma growth, and (3) the ability of ketotifen therapy to stabilize (ie, block) mast-cell secretion, treatment with ketotifen was started in a patient with severe neurofibromatosis (NF) in August 1983. Subsequently, ten additional patients with one or more symptomatic neurofibromas were treated with comparable doses of ketotifen, 2 to 4 mg/d, orally administered for 30 to 43 months. This represents a total of 389 patient-months or 32.4 patient-years. All of these patients showed an unequivocal decrease in neurofibroma-associated pruritus and/or pain and tenderness; a consistent, but less-uniform, decrease in the rate of neurofibroma growth; and an unexpected improvement in overall sense of well-being, productivity, and general performance. It appears likely that mast-cell secretions do contribute to the growth and associated symptoms of neurofibromas, and that mast-cell blockers, such as ketotifen therapy, can retard this growth. (Arch Dermatol1987;123:1011-1016)

Improved pressure sore healing with hydrocolloid dressings.

Abstract: † We prospectively followed inpatients receiving treatment for pressure sores to identify the better of two local treatment regimens. Twenty-seven patients with 76 pressure sores received treatment with hydrocolloid dressings (HCDs) and 25 patients with 52 pressure sores received treatment with Dakin's solution (chloramine-T)-soaked wet-to-dry dressings. Thirty-eight (73%) patients initially had severe nutritional depletion. The mean serum albumin value of the pressure sore treatment groups was lower than that of an age-matched group without pressure sores. In the HCD group, 66 (86.8%) pressure sores improved compared with 36 (69.2%) pressure sores in the wet-to-dry dressings group. The HCD regimen was more efficacious even in a subgroup of patients who received inadequate nutritional support during treatment. Adequate nutritional intake during the study was associated with better healing in both local treatment groups. (Arch Dermatol1987;123:766-771)

The relationship of mild hirsutism or acne in women to androgens.

Abstract: • The relationship in women between mild hirsutism or acne to androgen levels has not been well-defined. We investigated this in 62 Caucasian women, aged 18 to 21 years, by relating these pilosebaceous signs to plasma free testosterone (fT), the main circulating determinant of plasma androgenicity. Women with mild hirsutism (n = 13) had a significantly elevated fT (12.7 ± 5.5, SD, pg/mL [44.1 ± 19.1 pmol/L]) compared to normal controls (7.4 ± 2.7 pg/mL [25.7 ± 9.4 pmol/L]), as did subjects with minor acne (10.7 ± 4.25 pg/mL [37.1 ± 14.7 pmol/L]). The most important finding was the striking variability in the relationship between pilosebaceous overactivity and fT levels. In mildly hirsute subjects plasma fT was normal in half of the subjects, and the coefficient of variation of plasma fT was about twice what one would expect from individual variability. We could not demonstrate correlations among the variables of hirsutism, acne, and plasma fT. On the other hand, among 15 women with modest elevations of plasma fT levels (up to twofold), 27% had moderate hirsutism, 40% had mild hirsutism, and 33% had none. However, four of five of the latter patients (without hirsutism) had acne. The relationship of fT to acne severity varied similarly. To define the interactions between androgens and the pilosebaceous apparatus, we propose a model in which variation in apparent skin sensitivity and the level of androgen seem to contribute about equally to the pathogenesis of mild hirsutism and acne. The clinician should suspect that hyperandrogenemia will be/found in about half the women with mild cases of hirsutism, and one third with minor acne. ( Arch Dermatol 1987;123:209-212)

Treatment of Toxic Epidermal Necrolysis: Créteil's Experience

Abstract: For a long time, experience of the treatment of toxic epidermal necrolysis (TEN) was limited to occasional patients. The advent of specialized care units has led to larger series and consequent improvements in therapy. Since the principal causes of death in TEN are sepsis and hypovolemia, 1,2 control of infection and fluid balance are of vital importance. 3 The management of TEN and second-degree burns have often been compared, 4-9 and although many of the principles are the same, there are three important differences. First, in TEN, mucous membrane involvement usually antedates skin necrolysis, hindering eating and drinking for several days before admission and worsening the fluid deficit. Second, systemic involvement renders the patient with TEN sicker than a patient with equally extensive burns. Third, the dissemination of cutaneous lesions often leaves no healthy area for venous access, enhancing the risk of sepsis. FLUID BALANCE Approximate fluid loss is estimated

Dermal pericapillary fibrin in venous disease and venous ulceration.

Abstract: • Pericapillary fibrin deposition is thought to contribute to the pathogenesis of venous ulceration. To our knowledge, however, there is no previous evidence that pericapillary fibrin is deposited in the tissue adjacent to venous ulcers. We prospectively studied patients with ulcers of the lower extremities for the presence of dermal pericapillary fibrin in the skin adjacent to the ulcers. On direct immunofluorescence, pericapillary fibrin was found in 14 (93%) of the 15 patients with venous ulceration but in only one (7%) of the 14 subjects with ulcers due to other causes. We also confirmed the presence of dermal pericapillary fibrin in legs with venous disease without ulcerations. We conclude that the pericapillary fibrin is easily demonstrable in the dermis adjacent to venous ulcers. In the evaluation of ulcers due to uncertain causes, the presence of dermal pericapillary fibrin may provide additional diagnostic help. ( Arch Dermatol 1987;123:620-623)

A Case-Control Study of Possible Causative Factors in Mycosis Fungoides

Abstract: A detailed case control study was carried out on 53 patients (33 males and 20 females) with histologically proven mycosis fungoides and on an age- and sex-matched control population. Possible causative factors investigated included occupation, recreation, and exposure to petrochemicals, pesticides, insecticides, and potential carcinogens. Exposure to plants of the Compositae family, tanning history, and chronic sun exposure were also investigated, as were smoking history, drug ingestion history, and other skin disease. Personal and family histories of other malignancies were also investigated. The only statistically significant difference to emerge was that the patients with mycosis fungoides had significantly more family history of atopic dermatitis. In view of the absence of any significant difference between patients and controls with regard to personal history of atopic dermatitis, this difference may be the result of multiple statistical testing rather than a phenomenon of true biological significance.