1/6. Surgical treatment of aplasia cutis in the Adams-Oliver syndrome.Aplasia cutis is one of the features of the Adams-Oliver syndrome, beside limb anomalies. Aplasia cutis, congenital absence of skin, is a lesion that usually presents over the vertex of the skull. Management of aplasia cutis depends on the size of the skin defect and the child's physical condition. scalp defects larger than approximately 1 cm should be treated surgically. In patients with aplasia cutis, surgery should preferably be performed using rotation scalp flaps, and additional split skin grafts. The history of two babies with the Adams-Oliver syndrome is presented. In the Adams-Oliver syndrome, large rotation scalp flaps are not reliable due to the abnormal vascularity of the skin. skin grafting is the safest way, preventing hemorrhage and infection, in the operative treatment of aplasia cutis in these babies.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
2/6. Severe monilethrix associated with intractable scalp pruritus, posterior subcapsular cataract, brachiocephaly, and distinct facial features: a new variant of monilethrix syndrome?monilethrix is a rare developmental hair shaft defect characterized by small elliptical node-like deformities with increased hair fragility resulting in partial or diffuse alopecia. The disorder is usually transmitted in an autosomal dominant fashion with incomplete penetrance and variable expressivity, but autosomal recessive inheritance has also been reported. It is thought to be without systemic involvement, whereas keratosis pilaris and follicular papules are almost invariably associated features. We describe an instance of monilethrix in a 9-year-old boy from consanguineous parents, characterized by universal dystrophic alopecia associated with intractable scalp pruritus, diffuse keratosis pilaris, and bilateral posterior subcapsular cataracts. His disease was further characterized by physical underdevelopment and distinct features of hypertelorism, a wide-based nose, long philtrum, relatively large mouth with thick lower lip, enlarged forehead, small, receding chin, short neck, and rounded (ultrabrachycranial) skull. The findings in our patient suggest that "monilethrix syndrome" is an appropriate term for defining the instances of monilethrix associated with other abnormalities. We conclude that our patient may represent a new and severe, autosomal recessive variant of monilethrix syndrome.- - - - - - - - - - ranking = 1keywords = physical (Clic here for more details about this article) |
3/6. Atypical fibroxanthoma on a bald scalp.We present the clinical, histopathological and immunohistochemical findings of an atypical fibroxanthoma (AFX) on the bald scalp of an 81-year-old French man who had worked at a private high school in japan as a janitor for over 40 years. The patient had a history of basal cell carcinoma on the nape, and chronic solar radiation seemed to be a predisposing factor in the pathogenesis of this association. This case showed the typical clinical and histopathological characteristics of AFX, and the immunohistochemical results suggested differentiation of histiocytes and myofibroblasts. The AFX was completely resected, and the patient has not had tumor recurrence or metastasis for over four postoperative years. This case therefore provides further support to the theory that AFX displays a clinically benign course, even though it is essentially a malignant tumor histologically located in the dermis. Therefore, we must excise AFX completely with great care and perform regular physical examinations for several years after operation.- - - - - - - - - - ranking = 13.305118040651keywords = physical examination, physical (Clic here for more details about this article) |
4/6. Cutis verticis gyrata.When the scalp exhibits folds and furrows resembling gyri, it is termed cutis verticis gyrata. The possible etiologies may be categorized as primary essential, primary nonessential, and secondary cutis verticis gyrata. This is based on history, physical examination, and histologic criteria with or without laboratory examinations.- - - - - - - - - - ranking = 13.305118040651keywords = physical examination, physical (Clic here for more details about this article) |
5/6. 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.- - - - - - - - - - ranking = 4keywords = physical (Clic here for more details about this article) |
6/6. The "teepee" sign in inflammatory scalp disease.psoriasis and other diffuse inflammatory diseases of the scalp can cause a unique physical findings that we call the "teepee sign" because the hair forms pyramidal tufts at regular intervals that resemble an American Indian encampment. A pathological mechanism for this finding is proposed, with the description of two cases in Air Force basic trainees, one of a severe seborrheic dermatitis and the other psoriasis. The significance with regard to physical qualification for entry into military service is discussed.- - - - - - - - - - ranking = 2keywords = physical (Clic here for more details about this article) |