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Biomedical subjects

R M Trüeb

Publications and source records attributed to R M Trüeb.

90 records · Page 5Linked to original sources
PubMed1995

Alopecia areata in a patient with candidiasis-endocrinopathy syndrome: unsuccessful treatment trial with diphenylcyclopropenone.

The association of chronic mucocutaneous candidiasis with endocrine dysfunction (candidiasis-endocrinopathy syndrome, CES) is a complex immune disorder, in which autoimmunity is found besides selective impairment of cellular immunity. We report a case of CES associated with alopecia areata. The histology of a scalp biopsy demonstrated minor inflammation with profound atrophy of the hair follicles and sclerotic whorls in the region of the hair papillae. A treatment trial with topical immunotherapy using diphenylcyclopropenone was unsuccessful, which might be explained by both long-standing alopecia areata and the underlying immune deficiency with an impaired delayed hypersensitivity reaction. Furthermore, the patient presented with agenesis of the spleen. Atrophy of the spleen in patients suffering from CES has formerly been described in the literature, but not asplenia. Its significance in the setting of the syndrome is discussed.

Adolescent↗
PubMed1995

Trichorhinophalangeal syndrome.

Trichorhinophalangeal syndrome (TRPS) comprises a distinctive combination of hair, facial and bony abnormalities with variable expression. A 20-year-old man with TRPS was seen because of marked androgenetic alopecia. Scanning electron-microscopic studies of the hair revealed flattened hair with an elliptoid transverse section pattern. Mechanical behavior of the hair was abnormal with a significant increase in the viscous parameter, indicating a decreased intermolecular bridging within the keratin matrix. The dermatologist confronted with premature or marked alopecia in young adults should always consider the possibility of an underlying congenital syndrome involving the hair and prompt further investigation.

Adult↗
PubMed1995

"Chignon alopecia": a distinctive type of nonmarginal traction alopecia.

Localized occipital alopecia may result from traction of the hair necessary to form and maintain a bun (chignon) in the occipital region. Marginal alopecia in the patient whose hair is drawn firmly back is more familiar, although traction may lead to loss of hair at any site of the scalp depending on the hairdressing techniques used. Three women were seen with localized alopecia of the occipital scalp associated with the wearing of a chignon. They had previously been uniformly misdiagnosed as having alopecia areata. "Chignon alopecia" should be ruled out when the diagnosis of persistent alopecia areata at the level of the lambda is considered. A distinctive histopathologic finding is perifollicular fibrosis extending into the subcutaneous fat. Traumatic alopecia is classified with respect to the type of trauma, localization, and pattern within the hairy scalp.

Alopecia↗
PubMed1994

[Prepubertal hypertrichosis].

Hypertrichosis must be assessed in the light of the type of hair involved, the pattern of abnormal hair growth, associated abnormalities, age of onset, and family history including ethnic background. Associated diseases and the associated medication must be considered, as drug-induced hair growth is not uncommon. Although disorders of the hair usually have little medical significance and involve no interference with the general physical condition, they may be present in congenital syndromes or be associated with other abnormalities and metabolic disorders. The individual psychological impact of excessive hair growth also has a bearing on the problem with regard to its management. We describe in a 7-year-old girl and an 8-year-old boy a state of prepubertal hypertrichosis, characterized in otherwise normal children by a form of generalized excessive terminal hair growth which does not conform to the pattern of (androgen-mediated) hirsutism. The nosology of this form of excessive hair growth is discussed. The question whether it represents an atavistic trait is controverted.

Algorithms↗
PubMed1994

[Pili torti et canaliculi in ectodermal dysplasia].

Uncombable hair is a heterogeneous symptom with a partially genetic background. In order to make an exact diagnosis, it is mandatory to recognize associated abnormalities, do pedigree analyses, and perform scanning electron microscopic studies of the hair. In a case of ectodermal dysplasia, ectrodactyly, cleft lip/palate (EEC) syndrome and in a patient with familial tricho-odonto-onychial ectodermal dysplasia with syndactyly, scanning electron microscopy demonstrated pili torti et canaliculi, helicotrichia and cuticular dystrophy. Congenital pili torti et canaliculi must be differentiated from pili torti and from pili trianguli et canaliculi ("cheveux incoiffables"), inasmuch as they may present in hypotrichosis congenita hereditaria of Marie-Unna or as part of complex ectodermal dysplasia syndromes with clefting of the lip/palate and/or limb defects. It is noteworthy that some of those patients show a dysmorphic facies and an atopic constitution in addition to the aforementioned abnormalities. We discuss the possible relationship of these syndromes to each other, with special respect to the Hay-Wells or ankyloblepharon, ectodermal dysplasia, cleft lip/palate (AEC) and the Rapp-Hodgkin (ectodermal dysplasia, midfacial hypoplasia, cleft lip/palate) syndromes.

Abnormalities, Multiple↗
PubMed1994

Recurrent Pseudomonas folliculitis.

Pseudomonas aeruginosa has attracted the attention of the dermatologic literature through repeatedly reported outbreaks of folliculitis occurring in small epidemics related to contaminated whirlpools, hot tubs, and swimming pools. In contrast, sporadic cases without these recreational exposures may present a diagnostic challenge. Two sisters, age 8 and 4 years, had sporadic Pseudomonas folliculitis. It is important to recognize the organism as the cause of this recurrent, papulopustular, follicular eruption on the trunk and gluteal regions, also called nonrecreational Pseudomonas folliculitis, to avoid misdiagnosis and mismanagment. Inadvertent therapy with steroids may result in rapid spread of the lesions. Unless a meticulous investigation of contaminant sources in the environment leads to the detection of the vehicle of infection, the dermatitis tends to recur and become chronic.

Baths↗
PubMed1993

[Trichomegaly in HIV infection].

Hypertrichosis of the eyelashes was first described as "trichomegaly" in the setting of rare congenital syndromes. Recently numerous cases of acquired trichomegaly of the eyelashes have been described, especially in patients with advanced human immunodeficiency virus type I infection. The pathomechanisms leading to acquired trichomegaly remain largely unknown. Direct effects of the virus on the hair follicle, immune dysregulation, and a multifactorial pathogenesis are discussed. We report a case of acquired trichomegaly in advanced HIV-I infection in order to present this unusual clinical finding and other hair problems in AIDS patients. It is suggested that acquired trichomegaly of the eyelashes may act as a useful clinical marker for assessment of severity of HIV-I infection.

AIDS-Related Opportunistic Infections↗
PubMed1993

Idiopathic middermal elastolysis.

A 42-year-old white woman experienced widespread fine wrinkling and perifollicular protrusions in areas of erythematous patches with obvious photodistribution. Histological examination of the affected skin revealed loss of elastic tissue limited to the middermis. The clinical and histological presentation meets the criteria for the diagnosis of idiopathic middermal elastolysis. These findings support the previously postulated inflammatory pathogenesis for the loss of middermal elastic tissue and suggest that sun exposure may be a contributing factor. Idiopathic middermal elastolysis may represent only a variant in a spectrum of acquired wrinkling due to elastolysis with varying histological and clinical findings.

Adult↗
PubMed1993

Acute generalized exanthematous pustulosis due to doxycycline.

Sterile epidermal neutrophilic pustulation can be observed in a variety of diseases. Though drug hypersensitivity is an uncommon cause, it is yet a known entity to be considered in the differential diagnosis of generalized pustulosis. In a 40-year-old woman, who developed a generalized pustular eruption after starting on doxycycline therapy of bronchitis, the rash was concluded to be drug induced after exclusion of other pustular dermatoses. Sensitization to doxycycline was demonstrated by in vitro lymphocyte testing and correlated with clinical drug hypersensitivity after recurrence of the pustular eruption on nonintentional rechallenge with doxycycline.

Acute Disease↗
PubMed1993

Cutaneous ossification in Albright's hereditary osteodystrophy.

A 23-year-old woman presented with subcutaneous ossification, which together with short stature, stocky physique, round face and brachydactyly suggested Albright's hereditary osteodystrophy (AHO). Serum calcium and phosphorus levels were normal. AHO refers to the phenotype of the syndromes of pseudo-hypoparathyroidism (PHP) type Ia and pseudopseudohypoparathyroidism (PPHP), both considered genetically related variants with a defect of the alpha subunit of the stimulatory G protein of adenylate cyclase, necessary for the action of parathyroid and other hormones using cyclic AMP as an intracellular second messenger. PPHP differs from PHP in that it lacks parathyroid hormone resistance manifesting itself as hypocalcemia. Other endocrine end organ unresponsiveness, e.g. hypothyroidism and hypogonadism, may also be found with PHP. Both PHP and PPHP usually exhibit characteristic phenotypic abnormalities, of which subcutaneous ossification may be a presenting feature. The differential diagnosis of cutaneous calcification and ossification is outlined.

Adult↗
PubMed1993

[Pseudomonas aeruginosa folliculitis after epilation].

We report on a rare case of Pseudomonas aeruginosa folliculitis following depilation of the legs in a 33-year-old woman. Recently attention has been drawn to Pseudomonas-folliculitis acquired from whirlpools. The possibility of P. aeruginosa folliculitis occurring after skin depilation is less well known. The source of infection may be contaminated cosmetics or the hospital environment. The conditions favouring Pseudomonas invasion of the skin, and the clinical picture, course and of treatment P. aeruginosa folliculitis are discussed.

Administration, Topical↗
PubMed1993

[Unilateral "nevoid" spider nevi].

A case of unilateral spider telangiectasia on the left side of the face initiating at puberty is reported. With respect to its therapy and prognosis this alteration must be distinguished from other telangiectasias. With unilateral nevoid telangiectasia it shares the unilateral distribution of the lesions and the predilection for the female sex during hormonal instability periods. Both of these types of acquired telangiectasia seem to occur in relation with endocrine influences. Unilateral nevoid telangiectasia may also show typical spider telangiectasia but occurs in combination with other telangiectatic alterations. Unilateral "nevoid" spider telangiectasia consists exclusively of spider telangiectasia. Pathogenesis and further differential diagnosis of this peculiar type of primary telangiectasia are outlined.

Adolescent↗
PubMed1992

[Loose anagen hair].

We report on a 5-year-old girl whose hair could be pulled out with no effort and without causing pain. Occipital alopecia in a patchy pattern and with no sign of scalp inflammation or scarring was noted. A trichogram from the clinically involved area disclosed a striking predominance of anagen hair with absence of external root sheaths. Histological examination of the hair suggests abnormal premature keratinization of the inner root sheath, resulting in the loss of its anchoring function between the hair shaft and the follicle. Zaun first reported this new type of hair loss in children in 1984. The name loose anagen hair of childhood has recently been suggested for this disease. The differential diagnosis includes alopecia areata, trichotillomania, and congenital hypotrichosis.

Alopecia Areata↗
PubMedDate not supplied

Uncombable hair syndrome with angel-shaped phalango-epiphyseal dysplasia.

Uncombable hair syndrome or "cheveux incoiffables" is due to a characteristic longitudinal grooving of the hair shaft resulting in a triangular cross section (pili trianguli et canaliculi). In the majority of cases the abnormality is an isolated finding, although uncombable hair-type changes have been observed in conjunction with other features of ectodermal dysplasia. Ultrastructural studies in the latter have revealed more complex changes of the hair shaft, such as longitudinal grooving in combination with torsion, suggesting classification as a different entity. We describe a 6-year-old girl with typical "cheveux incoiffables," as confirmed by scanning electron microscopy, in combination with angel-shaped phalango-epiphyseal dysplasia. The relationship to a previously described syndrome of uncombable hair in combination with retinal dystrophy, juvenile cataract, and brachydactyly, in which both hair and skeletal abnormalities are common, remains to be further elucidated.

Abnormalities, Multiple↗