[Rapidly growing sternal nodule. Granuloma teleangiectaticum].
Explore the source record for details and available documents.
Adolescent↗
Biomedical subjects
R M Trüeb
Publications and source records attributed to R M Trüeb.
[Hair diseases: diagnosis and therapy].
Explore the source record for details and available documents.
Female↗
[Cicatricial alopecias: diagnosis and therapy].
The cicatricial alopecias often are both a diagnostic and therapeutic challenge to the practitioner. Where there is no obvious physical/chemical injury or acute infectious etiology, clinical differential diagnosis of scarring alopecia is often difficult. The loss of follicular orifices in an area of alopecia points to a permanent loss of hair. In all of these cases a scalp biopsy is indicated. Primary and secondary scarring alopecia are differentiated: While the former is due to preferential destruction of the follicle, the latter results from events outside the follicle, which eventually impinge upon and eradicate the follicle. These include infiltrative processes such as granulomatous inflammation or neoplastic disease. In the group of primary scarring alopecia, well-defined chronic-inflammatory diseases of the scalp amenable to specific therapies are differentiated microscopically on the basis of the pattern of inflammation and the type of inflammatory cell that predominates. Thus, accurate diagnosis based on histopathology is a prerequisite to any rational therapy. Management of the less well classified diseases is much more problematic. Where end-stage fibrosis is established, surgical treatment and/or prosthetic help are taken into consideration. With the expanding knowledge of the immunology and molecular biology of the hair follicle, there is hope for the feasibility of therapeutic interventions that interfere early in the course of the pathogenetic processes ultimately leading to the permanent loss of hair.
Alopecia↗
[Idiopathic eosinophilia].
Peripheral and tissue eosinophilia are associated with a wide variety of inflammatory syndromes. These include both multisystem and limited diseases with vasculitis or non-vasculitic tissue damage and variable expression of end-stage-fibrosis. The idiopathic hypereosinophilic syndrome (IHS) represents a multisystem disorder defined by sustained eosinophilia of an undetectable cause with significant organ system dysfunction. Although not specified as such in the criteria for the diagnosis of IHS, there are idiopathic eosinophilic syndromes that are clinically distinct from IHS by virtue of the fact that the eosinophilic inflammation is limited to specific tissues (such as the skin) with an overall good prognosis. The pathogenic role of the eosinophilic granulocyte in these conditions is attested by evidence of eosinophil activation and degranulation at sites of tissue injury. The recruitment and localization of eosinophils to specific sites of tissue inflammation involves cytokines with haematopoietic growth factor activity, adhesion molecules expressed both by the vascular endothelium and eosinophils, and chemoattractants that stimulate eosinophil migration. Recently, overexpression of IL-5 in transgenic mice was shown to lead to both peripheral blood eosinophilia and tissue eosinophilia. With the advances in our understanding of cytokine-dependent regulatory mechanisms that control the peripheral eosinophil number as well as the recruitment and survivability of eosinophils at sites of inflammation, more targeted ways of manipulating the eosinophil reaction can be expected in the future.
Animals↗
[Dapsone in granulomatous rosacea].
We report on two patients with granulomatous rosacea and another patient with granulomatous perioral dermatitis who responded well to dapsone. Dapsone has a pharmacological double function as both an antibiotic and an antiphlogistic drug. Before the introduction of isotretinoin, dapsone had its place in the treatment of severe acne. To date, its use in granulomatous rosacea has not been described. When hematologic parameters are monitored, dapsone is considered a safe and cost-effective drug, especially in countries where isotretinoin is not readily available. However, the definite value of dapsone in granulomatous rosacea should be established by a controlled study.
Adult↗
[Tufted hair folliculitis].
A case of tufted hair folliculutis presenting as circumscribed, tender and inflamed areas in the occiput with residual tufted follicles in a 28-year old man is reported. Tufted hair folliculitis is a characteristic localized scarring bacterial folliculitis of the scalp due to Staphylococcus aureus. Histopathological studies reveal perifollicular inflammation around the upper portions of the follicles sparing the hair root level. Within areas of inflammation, several follicles converge toward a common follicular duct with a widely dilated opening. Currently, tufted hair folliculitis is considered a variant of folliculitis decalvans of Quinquaud. Staphylococcal infection is believed to be an initial causative factor, and underlying differences in follicular anatomy or host response may be important in determining which reaction pattern occurs in an affected individual. The development of atrophy with loss of adnexal structures (in folliculitis decalvans) or of hair tufts (in tufting folliculitis) may depend upon the depth and destructive potential of the inflammatory process. The therapeutic approach is problematic; prolonged treatment with oral antibiotics may stabilize the disease, but good and at times more definitive results (as in the presented case) have been reported after radical surgical excision of the involved areas.
Adult↗
Eosinophilic myositis/perimyositis: frequency and spectrum of cutaneous manifestations.
BACKGROUND: Eosinophilic myositis/perimyositis (EM/P) are a group of rare idiopathic muscle disorders associated with eosinophilia. OBJECTIVE: We describe the frequency and spectrum of cutaneous manifestations in EM/P and compare them with the idiopathic hypereosinophilic syndrome (HES). METHODS: We review the literature on EM/P and describe an additional case associated with angioedema. RESULTS: Of a total of 26 reported patients with EM/P, cutaneous manifestations were observed in 10. These were, in order of frequency, deep subcutaneous induration, erythema, angioedema, urticaria, and papular lesions. CONCLUSION: Skin lesions occur less frequently in EM/P than in HES. Although erythematous papulonodular lesions and urticaria/angioedema are most commonly observed in HES, the most frequent skin manifestations of EM/P are subcutaneous induration and erythema. In HES, angioedema has been correlated with a favorable prognosis. At least some of these patients apparently have an idiopathic eosinophilic disorder distinct from HES, including EM/P. In contrast to HES, the overall prognosis of EM/P is good, particularly when muscle lesions are focal, and the principal histopathologic finding is perimysial infiltrates.
Adult↗
Expression of intercellular adhesion molecule 3 (CDw50) on endothelial cells in cutaneous lymphomas. A comparative study between nodal and cutaneous lymphomas.
Advances in the molecular definition of surface proteins (adhesion molecules) involved in tumor metastasis may help to explain the invasive behavior of malignant tumors, that is, the migration of tumor cells involving reversible adhesive contacts, their release in the circulation, and their extravasation into distant sites. Intercellular adhesion molecule-3 (ICAM-3), the third receptor for the lymphocyte function-associated antigen molecule-1 (LFA-1) was recently characterized. We investigated fresh frozen skin biopsies from 10 patients with mycosis fungoides, four with pleomorphic T-cell lymphoma, six with Sézary syndrome, 10 with primary cutaneous B-cell lymphoma, and 10 with eczematous lesions as controls. The biopsies were compared with lymph node biopsies of five patients with known cutaneous T-cell lymphoma (CTCL), 10 with primary nodal B-cell lymphoma, and 11 with lymph-node specimens showing dermatopathic lymphadenopathy as controls. The specimens were stained with ICAM-3 antibody (Bender Medical Science) using the alkaline phosphatase antialkaline phosphatase method. Using cytomorphologic criteria, neoplastic lymphocytes could be differentiated from smaller reactive cells. Staining intensities were classified semiquantitatively as follows: 4, strong expression in 75 to 100% of the tumor cells; 3, 50 to 75%; 2, 25 to 50%; 1, 5 to 25%; and 0 fewer than 5% of the tumor cells. The endothelial cells in skin biopsies of seven of 30 primary cutaneous lymphomas expressed ICAM-3. In contrast, no expression of ICAM-3 could be demonstrated on endothelial cells in lymph nodes infiltrated with tumor cells of CTCL. Finally, endothelial cells of lymph nodes infiltrated with primary nodal B-cell lymphomas showed expression of ICAM-3 in three of 10 patients. The endothelial cells in the 11 control patients presenting with both eczematous lesions and dermatopathic lymphadenopathy showed no staining for ICAM-3. Every patient who expressed ICAM-3 on endothelial cells showed systemic spread of this disease. The findings suggest that ICAM-3 expression may be induced on endothelial cells in late-stage cutaneous lymphomas, probably by a cytokine-mediated mechanism.
Adult↗
Lupus profundus/panniculitis.
A 25-year-old Caucasian woman developed two areas of tender panniculitis on her left thigh. While one lesion ulcerated, the other showed no overlying cutaneous changes. Histological examination showed lymphocytic perivascular and periappendageal infiltrates, and direct immunofluorescence revealed granular immunoglobulin deposits at the dermal-epidermal junction in the skin overlying the lesions. Laboratory tests revealed lymphopenia, anemia and an increased blood sedimentation rate. The serology for syphilis was false positive. For several months the patient had also been suffering from arthralgia with palpable synovitis of the wrist and several interphalangeal joints. Overall, these clinical findings are consistent with the diagnosis of lupus profundus/panniculitis associated with mild systemic LE.
Adult↗
Scarring folliculitis in the ectrodactyly-ectodermal dysplasia-clefting syndrome. Histologic, scanning electron-microscopic and biophysical studies of hair.
Several clinical syndromes are characterized by ectodermal dysplasia (ED) in association with clefting of the lip and/or palate. In these syndromes, alopecia is primarily due to abnormalities of the hair shaft associated with increased hair fragility. Scalp dermatitis is yet another peculiar finding, primarily seen in the ankyloblepharon-ED-clefting (AEC) syndrome. We report on a 16-year-old patient with ectrodactyly-ED-clefting (EEC) syndrome, who exhibited a scarring alopecia due to deep folliculitis. On scanning electron microscopy, irregular torsion and longitudinal grooving of the hair shaft (pili torti et canaliculi) were observed. Quantitative determinations of the elastic and viscous parameters of hair demonstrated a normal viscosity but a significantly reduced hair elasticity, indicating either an abnormal composition or a disordered arrangement of microfibrils within the apparently normal keratin matrix. In contrast to the erosive scalp dermatitis of early onset in the AEC syndrome, alopecia in this case of EEC syndrome demonstrated follicular scarring with onset during puberty. We question a possible role of the anatomical hair abnormality in the pathogenesis of chronic deep folliculitis in this and clinically related syndromes.
Adolescent↗
Eosinophilic myositis with eosinophilic cellulitislike skin lesions. Association with increased serum levels of eosinophil cationic protein and interleukin-5.
BACKGROUND: Peripheral and tissue eosinophilia are associated with a group of idiopathic inflammatory syndromes. The idiopathic hypereosinophilic syndrome represents a spectrum of disorders characterized by prolonged eosinophilia of an undetectable cause and significant organ dysfunction. The pathogenic role of the eosinophil in these conditions is attested to by evidence of eosinophil activation and degranulation at sites of tissue injury. Recently, an overlapping range of idiopathic eosinophilic muscle disease with an overall good prognosis has been described. RESULTS: We describe a patient with a syndrome of idiopathic myositis with eosinophilia and eosinophilic cellulitislike cutaneous manifestations. Histopathological studies of the skin and muscle revealed eosinophilic infiltration. Elevated serum levels of eosinophilic cationic protein and interleukin-5 paralleling disease activity were detected. CONCLUSIONS: This patient demonstrates clinical and laboratory features of eosinophilic myositis with eosinophilic cellulitislike skin lesions. The elevated serum levels of interleukin-5 and eosinophilic cationic protein may be responsible for the eosinophilia and tissue injury, respectively. With the advances in our understanding of cytokine-dependent regulatory mechanisms governing the eosinophil reaction, more targeted ways of manipulating eosinophilia as well as the entry and activation of eosinophils within specific tissues can be expected.
Adult↗
Juvenile amyopathic dermatomyositis.
We report a 15-year-old girl with a 10-year-old history of typical skin features of dermatomyositis (DM) without evidence of muscle involvement. Amyopathic dermatomyositis (ADM) is defined by the presence of biopsy confirmed classic cutaneous findings of dermatomyositis in the absence of any clinical or laboratory signs of muscle disease for at least 2 years after onset of skin pathology. To exclude muscle involvement muscle enzymes should be normal; moreover additional use of magnetic resonance imaging and muscle ultrasound is currently being proposed. It is as yet undetermined, whether early aggressive immunosuppressive treatment of ADM might prevent the development of myositis at a later date or influence the course of the skin disease. In a paediatric patient with ADM we advocate a more expectant attitude with careful and regular monitoring for possible development of muscle disease.
Adolescent↗
Necrotizing granulomatosis of the breast.
We describe a case of necrotizing granulomatosis of Wegener's type involving the breasts of a 40-year-old man. There were no signs of generalized disease. Involvement of the breast is rare in Wegener's granulomatosis (WG). To date, 17 cases have been reported, and all were women. They predominantly presented with a unilateral breast mass, and mammary malignancy was the principal concern. In the majority of cases, breast lesions of WG have been a presenting sign of, or preceded, disseminated disease. Our patient is unusual in that the necrotizing granulomas developed as an isolated finding in a site remote from those usually affected by WG, and, as far as we are aware, represents the first case of Wegener's type granulomatosis involving the male breast.
Adult↗
Trichotillomania in connection with alopecia areata.
Alopecia areata and trichotillomania present the most frequent causes of circumscribed hair loss in children. The connection between trichotillomania and alopecia areata has confused former observers. It may result from scratching at the site of alopecia areata that is symptomatic with pruritus, initiating a habit-forming behavior, or patients with a mental predisposition may artificially prolong the disfigurement as the hair on the bald patches of alopecia areata regrows. We have seen the concomitant occurrence of trichotillomania and alopecia areata in a 13-year-old boy, and confirmed the diagnosis by results of histopathologic examination. With regard to pathogenesis and prognosis, the differentiation of trichotillomania in connection with alopecia areata from the more common patterns of temporary localized childhood trichotillomania and severe adult trichotillomania is suggested.
Adolescent↗
[Linear focal elastosis (elastotic striae)].
Linear focal elastosis is the term used to denote asymptomatic, palpable, striae-like lines extending horizontally across the middle and lower back. Histologically these lesions present a focal increase in elastic fibres separating the dermal collagen bundles. To date, this disorder has been described only in male patients, with onset after 60 years of age. A case of linear focal elastosis in a young female patient is presented. In the differential diagnosis the clinical variants of connective tissue naevi, of the elastolyses, and of actinic elastosis are discussed. Considering that this disorder may be merely an unusual form of striae distensae with a distinctive process of "elastotic" regeneration, the term elastotic striae seems appropriate.
Adult↗
[Idiopathic elastolysis of the mid-dermis].
Idiopathic middermal elastolysis (IMDE) is a rare disorder with two different clinical presentations: type I lesions, with tiny wrinkles along skin cleavage lines in large confluent areas, and type II lesions, with small perifollicular papular protrusions. The histopathological picture is characteristic. IMDE is mainly seen in healthy young or middle-aged women. We describe a 60-year-old male patient with this disorder. The special interest of this case report is that there was no photosensitivity and no exacerbation with PUV therapy. IMDE is probably a special sort of elastolytic disorder, for which immunologic mechanisms seem possible.
Atrophy↗
[Overlap of Sjögren-lupus erythematosus syndrome].
Patients with anti-SSA(Ro)-positive Sjögren's syndrome (SS)/lupus erythematosus (LE) overlap are a immunogenetically, serologically and clinically homogeneous group; what they have in common is an increased frequency of the HLA-DR3 phenotype, demonstrating SSA(Ro) antibody activity and the typical annular, polycyclic, erythematous lesions of subacute cutaneous LE. The sicca symptoms of SS may develop long after the cutaneous lesions of LE have been present, or vice versa, as well as vasculitic, purpuric, and Sweet's syndrome-like lesions. The elderly, predominantly female patients are at enhanced risk for pulmonary and neurological disease, but glomerulonephritis occurs infrequently. We present an 81-year-old, SSA(Ro) antibody-positive and HLA-DR3-positive woman with SS/LE overlap syndrome, who had cutaneous LE for 30 years, developing sicca symptoms, vasculitic and Sweet's syndrome-like skin lesions only years after the LE symptoms. Although there is still some discussion as to whether phenotypical SS/LE overlap represents a distinct disease or is secondary SS in SLE or systemic SS with cutaneous involvement, the SS/LE overlap syndrome seems well enough delineated to be considered as a disease entity with separate implications for therapy and prognosis. Its position in the spectrum of anti-SSA(Ro) antibody- and HLA-DR3-positive diseases and its relationship to SS and to anti-SSA(Ro) antibody-positive LE are discussed.
Aged↗