A 76-year-old man with multiple erythematous lesions on his palms and soles which appeared following bullous pemphigoid (BP) is the subject of this case report. The lesions were not raised above the normal skin level, and there were no nodules on the erythematous lesions. The lesions had the histologic appearance of eccrine syringofibroadenoma. This condition is considered to be not a true tumor but a hyperplasia of eccrine sweat ducts following recurrent subepidermal blister formation in BP.
59 patients suffering from vitiligo were investigated anamnestically and clinically with intradermal (prick tests) and laboratory tests (RAST and total IgE count) for the presence of atopy. Clinical manifestations (allergic rhinitis, asthma) and intense positive prick tests and RAST with an increase in total IgE count were found in 13 patients (22%). This frequency was significantly higher than that found in the normal population in our area (11.9%; p = 0.0212). These patients had a significantly higher incidence of vitiligo in their families (76.9 vs. 29.7% of the non-atopic; p less than 0.025), an earlier onset (14.1 vs. 24 years of the nonatopic) and a rapid worsening of the disease.
We report a patient with scalp lesions of primary localized cutaneous nodular amyloidosis. The extensive examination revealed no systemic involvement. Analysis of glycosaminoglycans (GAGs) in amyloid deposits showed a twofold increase as compared with normal skin, which was due to the increase in dermatan sulfate. Local disorders of GAG metabolism may be related to the amyloid fibril formation. Amyloid fibrils were purified and identified electron-microscopically, which consisted of two major 12,000- and 13,000-dalton and minor 29,000- and 48,000-dalton peptides. Western blotting analysis showed a minor 29,000-dalton peptide reactive with antibodies against both kappa and lambda-light chains of immunoglobulin. There is a possibility that some components of amyloid in some cases of primary localized cutaneous nodular amyloidosis may consist of both kappa and lambda-immunoglobulin light chains.
The presented case-control study with 204 melanoma patients and 200 control persons assesses the significance of melanoma risk factors for an ethnically homogeneous population from a geographically small region. In a multivariate analysis of the data the total number of benign naevi proved to be the most predictive parameter with a relative risk (RR) of 14.9 (total number of naevi higher than 50). The constitutional factors red hair colour and skin type 1 were less predictive with an RR of 2.9 and 4.9, respectively. Occupational and recreational sun exposure were of ancillary importance (RR 1.8 and 2.1). The assessment of the risk for the subtypes of melanoma however showed a clear difference in the predictive value of the mentioned risk factors. The risk of developing superficial spreading melanoma is nearly exclusively defined by the number of benign naevi (RR 24.8), red hair colour was of subordinate importance (RR 4.2), whereas the risk of lentigo maligna melanoma is dependent on skin type 1 (RR 12.9) and sun exposure (RR 3.4).
A patient with Sjögren’s syndrome and seronegative polyarthritis is reported. After piroxicam intake and sun exposure she developed subacute cutaneous lupus erythematosus lesions with Ro antibodies. Despite drug withdrawal, typical cutaneous lesions and serological markers of systemic lupus erythematosus (SLE) progressively appeared. The use of piroxicam and other nonsteroidal anti-inflammatory drugs with photosensitizing potential in patients with Sjögren’s syndrome, sicca syndrome or a high suspicion of a collagen disorder should be avoided because these drugs may trigger a latent SLE.
We report the cases of 4 male subjects, 29, 32, 41 and 44 years old, presenting isolated seropositivities for the human immunodeficiency virus (HIV), or full-blown acquired immunodeficiency syndrome, associated with a typical porphyria cutanea tarda (PCT). The 4 patients are in the usual risk groups for HIV infection. Viral hepatitis was observed in 3 of the 4 cases. Over the past 3 years, 15 cases associating HIV infection and PCT have been reported; almost all had the usual risk factors for HIV infection and hepatopathy. We speculate that HIV infection may have favored the occurrence of early PCT in these cases by altering the metabolism of the porphyrins, either directly or by means of the associated hepatopathy.
During 1988, the Gruppo Italiano Studi Epidemiologici in Dermatologia (GISED) coordinated a pilot study aimed at evaluating the feasibility of a system for spontaneous monitoring of adverse drug reactions in dermatological practice in Italy. Approximately 400 dermatologists were asked to collaborate, and 141 agreed to the study. Procedures similar to those well established in other surveillance programs (including the use of standard forms and standardized assessment procedure) were adopted. In a 2-month period 775 reports were collected, of which 711 were maintained after careful evaluation. The general profile of the adverse reactions reported was in accordance with the experience derived by other spontaneous surveillance programs. The main purpose of spontaneous reporting systems is the identification of new reactions, and a model analysis was proposed, in our study, with reference to skin reactions to bamifylline. The demonstration of the feasibility of a drug-monitoring program in Italy, where little tradition exists in the area, is the most important result of our study.
A patient with severe Raynaud's phenomenon (RP) associated with the presence of antibodies against extractable nuclear antigen (ribonucleoprotein) in the context of undifferentiated connective tissue syndrome is presented. The rapid course of digital necrosis in her case resulted in the surgical amputation of 9 of her fingers. We conclude that massive digital necrosis accompanied by severe RP could constitute the first manifestation of a connective tissue disease.
Ectodermal dysplasias are a large and heterogeneous groups of clinically and genetically distinct syndromes. We studied a family suffering from dystrophies of the distal part of the nails and trichodysplasia. Scalp, beard, pubic and axillary hair were broken off leaving a stubble 1-10 mm in length. Eyebrows, eyelashes and body hair were completely absent. Serum levels of copper and plasma levels of amino acids were within the normal range. Inheritance was autosomal recessive. Previous reports of ectodermal dysplasias and other complex syndromes with pili torti are reviewed.
Heiko Traupe, Department of Human Genetics, University of Nijmegen, PO Box 9101, NL-6500 HB Nijmegen (The Netherlands) In this issue of Dermatologica, Dr. Piergiacomo Calza-vara-Pinton in collaboration with Dr. Anna Carlino and Dr. Anna Benetti and Dr. Giuseppe De Panfilis from the Departments of Dermatology and Anatomopathology (II) of the University of Brescia, Italy, describe nothing less but a new genetic syndrome which is characterized by brittle hair breaking off to leave only stubbles of 1-10 mm in length, dystrophies of the distal nail plate and a distinct facies [1]. Scanning electron microscopy revealed focal flattening of the hair shafts and rotations along the axis of hair shafts. The hairs fracture in these twists. This latter finding may have been the reason why the authors decided to report their new hair shaft breakage syndrome under the designation of ‘pili torti and onychodysplasia’. The term ‘pili torti1 ‚ of course ‚ is a very old one and has the advantage of being deeply rooted in the medical literature. To me, the latin term ‘pili torti’ suggests that one deals with a very specific phenomenon that is pathogno-monic for a certain disease as is the case in trichorrhexis invaginata and the Comèl-Netherton syndrome. In contrast, ‘pili torti’ are rather nonspecific and can be found in quite a number of genetic syndromes affecting the hairs. Therefore, I prefer to speak of ‘twisted hairs’ instead. Nevertheless, these twisted hairs cause breakage of the hair shafts and result in a clinical picture resembling atri-chia in the disorder reported by Dr. Calzavara-Pinton and collaborators. The authors carefully delineate their new syndrome from atrichia with nail dystrophy, abnormal facies and retarded psychomotor development [2] which, at first glance, shows some resemblance because of marked alopecia and distal nail dystrophies, but has a different histopathology and therefore a different pathomechanism underlying the alopecia. The Beare syndrome [3] featuring ‘pili torti’ and nail dystrophies, the Clouston type of hidrotic ectodermal dysplasia and the Rapp-Hodgkin/ AEC syndrome, which also features ‘pili torti’ [4], can be immediately excluded because of autosomal dominant inheritance, whereas the disorder described by Dr. CalzavaraPinton follows an autosomal recessive inheritance that only mimicks dominant transmission due to consanguineous marriages. A normal banding pattern on polarizing microscopy rules out the heterogeneous group of trichothiodystrophy syndromes all of which are inherited in an autosomal recessive manner [5]. Many colleagues who describe a new syndrome then have the understandable urge to classify ‘their’ syndrome within the framework of a larger disease group. Dr. Calzavara-Pinton and coworkers could not resist this urge and decided to place their hair shaft breakage/nail dystrophy syndrome within the group of ‘ectodermal dysplasia’. Nothing is wrong with this, except that this group is already rather crowded. If one applies the very broad definition of ectodermal dysplasia as conditions featuring one of the following signs: (1) trichodysplasia; (2) dental defect; (3)
A 57-year-old man suffering from persistent light reaction with photocontact allergy to musk ambrette and contact allergy to fragrance mix was evaluated. A lowered minimal erythema dose to UV-B (MED-UV-B) was seen. Reactions to long-wave UV-A and visible radiation were normal. A skin biopsy from one MED-UV-B, taken 24 h after irradiation, showed acute spongiotic dermatitis.
We report a case of urticaria pigmentosa, acromegaly and acanthosis nigricans in a 25-year-old male. The patient exhibited multiple pigmented papules on the trunk and the extremities. Histological examinations of the papules revealed infiltrates of mast cells in the upper dermis. Ultrastructurally, the mast cells were fully matured and exhibited no atypical features. A typical appearance of acromegaly, frontal bossing, prominence of the jaw and bony overgrowth and cutaneous changes of acanthosis nigricans on the neck, the axillae and the groins were observed. Growth hormone hypersecretion and insulin resistance were detected in the patient. A pituitary tumor was found and resected surgically. After the operation, endocrinological abnormalities and cutaneous manifestations of acanthosis nigricans improved markedly. As far as we know, this is the first report of the coexistence of urticaria pigmentosa, acromegaly and acanthosis nigricans.
We have raised a polyclonal antibody to the 170-kD epidermal growth factor receptor. We found an intercellular pattern of immunoreactivity in the epidermis as well as a positivity of the cytoplasm of keratinocytes and eccrine secretory cells. In some samples, a nuclear labelling was evidenced in these type of cells. There is a close resemblance in the topographical distribution of these cells with nuclear labelling and those synthesizing DNA under phytohaemagglutinin stimulation.
We report an additional typical case of neutrophilic eccrine hidradenitis, which was observed in a 57-year-old man with acute myelogenous leukemia. He developed this eruption following chemotherapy with cytarabine and mitoxantrone. A short review of the literature is presented.
The number and affinity of glucocorticoid binding sites in peripheral mononuclear leukocytes of patients with atopic dermatitis (AD) and healthy controls were determined under baseline conditions and after a defined oral glucocorticoid treatment. Patients with AD (n = 15) exhibited significantly more glucocorticoid receptors (GR) per cell than the control group (n = 22), while the GR affinity did not differ. Methylprednisolone treatment resulted in a significant reduction of the GR sites per cell in the steroid-treated control group (n = 10) in contrast to the patients. The dissociation constant was not affected by methylprednisolone treatment in either group. In view of the therapeutic efficiency of glucocorticoids in AD and findings of abnormal cAMP and cAMP-phosphodiesterase activity, the elevated GR concentrations in AD lend support to the hypothesis of a compensatory GR upregulation due to an insufficient action of endogenous cortisol or to altered cAMP-induced GR expression.
Human keratinocytes are able to synthesize and express cell surface moieties characteristic of effector and/or accessory cells of the immune system (CD16, CD36, HLA-DR, intercellular adhesion molecule-1). In the present study, skin biopsies from healthy volunteers, from patients with psoriasis vulgaris (PV), mycosis fungoides (MF), purpura pigmentosa chronica (PPC), acute urticaria (AU) and from positive tuberculin skin tests were investigated with regard to the reactivity with the monoclonal antibodies to complement receptors CR1 CR2 and CR3 by means of a multistep immunoperoxidase method. In the clinically involved skin of all patients with PV, MF or PPC, and in biopsies obtained from positive tuberculin tests, specific epidermal intercellular staining with OKB7 and Leu anti-CR2 was seen on subcorneal keratinocytes. This finding suggests a differentiation-linked expression of CR2 on human keratinocytes in cytokine-mediated skin diseases whereas CR1 and CR3 are apparently not expressed.
A silver colloidal technique to demonstrate argyrophilic proteins of the nucleolar organizer regions (AgNORs) was performed on sections of 20 cases of malignant melanoma (MM) associated with underlying benign nevus (BN). In these cases, significant different AgNOR counts were found for MM and BN. In addition, this technique permitted the identification of melanocytic cells located between malignant and benign cells showing AgNOR scores intermediate (5.51) between BN (2.6) and MM (7.71) with a more complex and bizarre morphology than that observed in BN. The AgNOR technique can be suitable in the identification of residual nevus cells in MM, especially when their number is minimal and the common histologic criteria are unsatisfactory; it can also increase the understanding of the natural history of MM.
Fifty patients with the chief complaint of Raynaud's phenomenon (RP) presented at our scleroderma clinic from March to December 1990. Physical examination, routine laboratory tests (blood, urine and chest X-ray), determination of the pattern of RP, antinuclear antibody (ANA) tests and examination for nailfold bleeding were performed. Three patients were diagnosed as having systemic sclerosis sine scleroderma, 15 patients as having RP with positive anticentromere antibody and 6 patients as having an incomplete form of mixed connective tissue disease. Thus, a total of at least 24 patients out of 50 (48%) were shown to have a scleroderma spectrum disorder. A definite RP pattern (triphasic or biphasic and bilateral), positive ANA and positive nailfold bleeding were strongly correlated statistically, suggesting that these are simple useful findings for the early detection of scleroderma spectrum disorders in patients with RP. We expect that there are many undiagnosed patients with an early-stage scleroderma spectrum disorder in the general population.
There is evidence that topical tretinoin promotes wound healing, especially when the wound area is pretreated. In our study, 5 patients were pretreated for 2 weeks with 0.05% tretinoin cream to one groin or axilla, followed by electroepilation to both sides. Electroepilation created small, superficial wounds. Healing was defined as complete re-epithelialization. In all patients the pretreated side showed a significantly decreased healing time as compared to the nonpretreated side. Pretreatment of skin with topical tretinoin may be useful in reducing healing times of patients undergoing electroepilation.
