1/17. The wide spectrum of clinical expression in Adams-Oliver syndrome: a report of two cases.Two children are described with the combination of aplasia cutis congenita (ACC) and transverse limb defects known as Adams-Oliver syndrome. Whereas in the first child the typical features of ACC, syndactyly and transverse nail dystrophy were only mildly expressed and associated defects of the central nervous system and cardiac malformations were absent, the second child suffered from a very severe expression of the syndrome, with a combination of ACC, syndactyly, cutis marmorata telangiectatica congenita and multiple cardiac and central nervous system malformations which resulted in fatal central respiratory insufficiency.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
2/17. hyperplasia of the subcutaneous adipose tissue is the primary histopathologic abnormality in lipedematous scalp.A 51-year-old white woman presented with thickening of the scalp located at the vertex and left lateral occiput without hair abnormalities or alopecia. skin biopsies of the thickened scalp showed thickening of the subcutaneous tissue with proliferation of mature subcutaneous fat cells but no signs of inflammation or hair abnormalities. During 2.5 years of follow-up, scalp thickening progressed over the entire hair-bearing scalp and persisted without signs of further progression at 3.5 year follow-up. Lipedematous scalp is an extremely rare diagnosis. It is defined by a thickening of the subcutaneous layer of the scalp and can be distinguished from lipedematous alopecia, in which subcutaneous thickening is associated with diffuse alopecia and shortening of scalp hairs. A total of seven cases of lipedematous alopecia and two cases of lipedematous scalp have been reported. We report the third case of lipedematous scalp in a 51-year-old white woman associated with early symptoms of meningitis. Additional features described in the literature include pruritus, pain, and paresthesia of the scalp as well as associated medical problems such as hyperelasticity of skin and laxity of joints, renal failure, and diabetes mellitus. This sporadic disorder is predominantly located at the vertex and occiput. The etiology and pathogenesis of lipedematous scalp and alopecia remain unclear. The treatment is symptomatic.- - - - - - - - - - ranking = 1926.015625keywords = subcutaneous fat, fat (Clic here for more details about this article) |
3/17. Linear lupus erythematosus profundus on the scalp following the lines of Blaschko.We describe a 10-year-old Japanese girl presenting linear alopecia on the scalp and forehead. Histological examination showed fat degeneration with mucin deposit and periappendageal infiltrate of mononuclear cells. We diagnosed her as having linear lupus erythematodes profundus with a linear configuration following the lines of Blaschko.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
4/17. nevus psiloliparus and aplasia cutis: a further possible example of didymosis.nevus psiloliparus is a distinct type of mesodermal nevus of the scalp characterized by absence or paucity of hair, and presence of an excessive amount of fatty tissue. It is considered a hallmark of encephalocraniocutaneous lipomatosis, a rare disorder comprising a variety of cutaneous, ophthalmologic, and neurologic defects. We report two infants with encephalocraniocutaneous lipomatosis with nevus psiloliparus on the scalp in close association with aplasia cutis congenita. This unusual association may be considered a further example of didymosis, for which we propose the term, didymosis aplasticopsilolipara.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
5/17. Lipedematous alopecia of the scalp.Lipedematous scalp is a rare disorder, mainly described in adult African-American females. We report 2 adult caucasian males with lipedematous scalp associated with androgenetic alopecia. patients were studied by dermoscopy and histopathology; they were treated with finasteride 1 mg. In our patients, lipedematous scalp affected the occipital and the vertex areas and pathologically exhibited mild edema and thickening of the adipose subcutaneous layer. At videodermoscopy, lipedematous scalp areas showed linear areas of teleangiectasia within the scalp creases, possibly caused by compression of the superficial blood capillaries by the increased volume of the subcutaneous fat layer within the thickened scalp. finasteride at a dose of 1 mg per day for 1 year induced mild improvement of androgenetic alopecia in one patient and stabilization of the disease in the other. The lipedematous scalps remain unchanged. Lipedematous scalp is apparently a rare disease even though the condition is probably underdiagnosed. As a matter of fact, we diagnosed lipedematous scalp in our patients during a clinical examination for androgenetic alopecia, which was the patients' complaint. The association of lipedematous scalp and androgenetic alopecia in our two patients appears to be coincidental.- - - - - - - - - - ranking = 1926.015625keywords = subcutaneous fat, fat (Clic here for more details about this article) |
6/17. Lipedematous scalp in a child.A nine year old Maori girl was noted to have a spongy consistency to her posterior scalp. The overlying skin appeared normal, as was hair growth in that area. The abnormal findings became more extensive over the subsequent year. A computerized tomography scan showed diffuse thickening of subcutaneous tissues posteriorly on the scalp. A skin biopsy specimen showed a normal epidermis with a thick layer of mature subcutaneous fat and some disruption of the subcutaneous architecture. The clinical features and investigations were consistent with a diagnosis of lipedematous scalp, which has been described predominantly in adult women and is usually associated with alopecia. This condition has not been reported previously in the pediatric literature.- - - - - - - - - - ranking = 1926.015625keywords = subcutaneous fat, fat (Clic here for more details about this article) |
7/17. Primary localized cutaneous nodular amyloidosis: case report and biochemical analysis of amyloid.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.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
8/17. Fatal streptococcus viridans septicemia and meningitis: relationship to fetal scalp electrode monitoring.viridans streptococci have been reported as an increasingly frequent pathogen in neonatal sepsis. These infections have appeared to be less virulent than those associated with other bacteria. The case of an infant is reported who developed a scalp abscess secondary to an internal fetal monitoring electrode caused by viridans streptococci. This infection evolved into a rapidly fatal septicemia and meningitis. viridans streptococci may be responsible for virulent fatal infections in neonates.- - - - - - - - - - ranking = 2keywords = fat (Clic here for more details about this article) |
9/17. Tufted hair folliculitis.A case of scarring alopecia presenting as two circumscribed, tender and inflamed areas in the occiput with residual tufted follicles is reported. Each tuft comprised 10 to 15 normal-appearing hairs arising from individual hair follicles in the reticular dermis or subcutaneous fat, converging toward a single orifice in the epidermis. The initial pathologic finding was inflammation and scarring of the papillary and mid dermis with almost total sparing of the hair follicles in the subcutaneous fat. Successful treatment was achieved by surgical excision of the two areas. It is suggested that the areas of tufting represented nevoid lesions that underwent inflammation and scarring.- - - - - - - - - - ranking = 3852.03125keywords = subcutaneous fat, fat (Clic here for more details about this article) |
10/17. Craniocervical necrotizing fasciitis: critical factors in management.Necrotizing fasciitis involving the head and neck is rare. The authors describe two such patients treated at their institution and analyse 39 cases reported in the literature. This entity may be divided into two groups based on the site of origin of the infection: group 1 (13 cases) infections, originating in the scalp and eyelids, mostly secondary to trauma, do not progress rapidly, respond well to medical and operative measures and result in minimal permanent disability. These infections usually are caused by hemolytic streptococci and staphylococcus aureus. Group 2 (28 cases) infections, originating in the face or neck and mostly complications of dental and pharyngeal sepsis, progress rapidly to adjoining sites including the chest wall and mediastinum. These infections are caused by a wide variety of microorganisms including anaerobes; fatal complications are frequent and the death rate is high (32%). Early and very aggressive debridement and drainage are mandatory and should be repeated if warranted.- - - - - - - - - - ranking = 1keywords = fat (Clic here for more details about this article) |
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