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Biomedical subjects

Rudolf Happle

Publications and source records attributed to Rudolf Happle.

77 records · Page 5Linked to original sources

Trichorrhizophagia.

Trichotillomania is a form of hair loss characterized by an irresistible compulsion to pull out or twist and break off one's own hair. Extracted hairs are often manipulated, examined, or played with before being discarded or eaten. We report an obsessive-compulsive 65-year-old lady who exclusively ate the root of the hair she plucked, in a very selective type of trichophagia. We suggest the new term trichorrhizophagia which means "eating the root of the hair" (from the Greek rhiza rho iota zeta alpha = root) to name this condition.

Aged↗

Nevus psiloliparus: report of two nonsyndromic cases.

A diagnosis of nevus psiloliparus was made both clinically and histopathologically in two otherwise healthy girls, one being 4 years and the other one being 1 year old. A congenital hairless patch with a round or oblong shape and a soft surface was noted on the scalp. In one case the lesion was yellowish and flat, whereas in the other case it was skin colored and somewhat elevated. In both cases, histopathological examination showed the absence of mature hair follicles and the presence of undeveloped follicular structures as well as orphaned arrector pili muscles in the dermis. As a new histopathological feature of this type of nevus, we found arrested anlagen of hair bulbs in both cases. The fatty tissue was abundant and also involved, in the form of aberrant lobules, the lower portion of the dermis. Clinical examination did not show any associated extracutaneous abnormality, and during a follow-up period of 2 years in either case, respectively, the children developed without any complication. Although neurological abnormalities could not be excluded by imaging techniques, such extracutaneous involvement is highly unlikely because the nevi psilolipari were of rather limited size. When clinicians and dermatohistopathologists have become familiar with this new entity, they will most likely recognize it as a nonsyndromic skin disorder more often than as a cutaneous sign of encephalocraniocutaneous lipomatosis.

Adipose Tissue↗

Mucocutaneous telangiectases of the head and neck in individuals with hereditary hemorrhagic telangiectasia -- analysis of distribution and symptoms.

Telangiectases are a diagnostic clue of hereditary hemorrhagic telangiectasia (HHT, Rendu-Osler-Weber disease), but they are not specific to HHT. The characteristic features of telangiectases were studied in a group of 70 individuals with this disorder. The files, including photo and video documentation of these patients, were reviewed with regard to mucocutaneous vascular lesions. Telangiectases could be identified within the nasal mucosa in 90% of the HHT patients. Extranasal telangiectases were identified in descending order in the oral cavity, the facial skin, the hands, the auricles and the thorax. The vascular lesions showed considerable variation in size and shape, and on the nasal mucosa they were most commonly hemorrhagic. The earliest onset of cutaneous telangiectases was documented at the age of 6 years. Contrary to previous communications, more than 25% of patients had hemorrhages outside the nose. These hemorrhages were self-limiting in most cases. Prolonged hemorrhages requiring treatment were observed in 12% of cases. Such severe hemorrhages originated from telangiectases of the base of the tongue, the body of the tongue, the fingers and the skin of the supraclavicular fossa. We conclude that telangiectases occur at an earlier age than generally thought and are a hallmark of a serious disorder rather than a cosmetic problem.

Adolescent↗

Nevus roseus: a distinct vascular birthmark.

The new term nevus roseus is proposed to denote a lateralized telangiectatic birthmark with a light-red or pale-pink color, unlike the dark hue of nevus flammeus. It appears to be a distinct entity rather than just a color variant of nevus flammeus. Remarkably, nevus flammeus is a characteristic component of phacomatosis pigmentovascularis type II ("phacomatosis cesioflammea"), whereas nevus roseus represents a distinguishing feature of phacomatosis pigmentovascularis type III ("phacomatosis spilorosea"). In analogy to "port-wine stain" that is used as a synonym for nevus flammeus, nevus roseus could also be called "rosé-wine stain". This lateralized vascular birthmark should be distinguished from the salmon patch that always involves the midline of the body. Nevus roseus belongs to the rather broad category of telangiectatic nevi, as well as to the even larger group of "capillary malformations". For obvious reasons these terms are not suitable to designate any specific type of vascular birthmark such as nevus roseus.

Adult↗

Delineation of the various shapes and patterns of nevi.

It has been proposed that all nevi reflect mosaicism, and this concept has been corroborated at the molecular level in a number of nevi. If the concept of mosaicism holds true, one would expect that the various types of nevi should be characterized by intrinsic shapes or patterns. A photographic review of 1,188 recognizable images of a list of nevi of the skin was undertaken in order to delineate a classification of the various shapes and patterns of nevi. We disclosed three distinct shapes and two so far undescribed archetypical patterns, in addition to the well-known Blaschko-linear, flag-like and extensive garment-like patterns, to which all lesions could be allocated. The newly delineated shapes are called round or oval shape; patches with indented borders; and teardrop or triangular shape. The newly proposed archetypical configurations are the agminated pattern and the diffuse patchy pattern. Other mosaic patterns, such as the phylloid pattern or the lateralization pattern, were not observed in any of the images reviewed. We conclude that the various types of nevi can be assigned to specific shapes or patterns. Admittedly, however, a given nevus may manifest itself in several different shapes, whereas its archetypical pattern tends to be the same. This 'archetypical' pattern can be taken as the perfect or ideal model of a given type of nevus, whereas the newly described shapes are subordinated to the archetypical patterns and are best considered to be 'nonarchetypical'. Our work gives more consistency to the idea that nevi reflect mosaicism, because they display repetitive shapes and patterns.

Child↗