1/267. poems syndrome: cicatricial alopecia as an unusual cutaneous manifestation associated with an underlying plasmacytoma.poems syndrome is a rare condition with cutaneous manifestations commonly including angiomas, hypertrichosis, hyperpigmentation, and thickening of the skin. We describe a male patient with a 2-year history of cervical lymphadenopathy, erythematous thickening of the skin on the neck, and progressive walking difficulties. The patient had an occipital erythema with scarring alopecia and sparse follicular pustules at the edge of the lesion. Further investigation revealed symmetric polyneuropathy, hepatosplenomegaly, monoclonal gammopathy, subclinical thyreopathy, and an osteolytic bone lesion of the skull. Histologically, a plasmacytoma with lambda cell restriction was found. The overlying skin showed marked fibrosis, with loss of hair follicles, and a plasma cell infiltrate of polyclonal origin. The cervical lymph nodes showed histologic characteristics of multicentric Castleman's disease, and the skin of the neck showed thickening and vasoproliferation. There was no evidence of further plamacytomas. After excision of the plasmacytoma and postoperative irradiation, the symptoms gradually resolved within a few months. A cicatricial lesion remained on the occiput without further folliculitis or hair loss on the rest of the scalp. This case illustrates the reactive character of poems syndrome as a paraneoplastic syndrome in myeloma patients.- - - - - - - - - - ranking = 1keywords = hair (Clic here for more details about this article) |
2/267. Pili torti with congenital deafness (Bjornstad syndrome): a case report.We report Bjornstad syndrome in a 5-year-old girl with severe bilateral congenital loss of hearing and pili torti. The mode of inheritance of this rare syndrome seems to be heterogeneous. A maternal uncle of the patient was deaf from birth and his hair had shown the same abnormalities at the same age; an autosomal recessive transmission can be assumed.- - - - - - - - - - ranking = 0.5keywords = hair (Clic here for more details about this article) |
3/267. Eruptive vellus hair cyst in a patient with pachyonychia congenita.pachyonychia congenita is characterized by symmetrical nail dystrophy, palmoplantar keratoderma, oral leukokeratosis, and follicular hyperkeratosis. In addition to these features, multiple cutaneous cysts of various kinds have been described. We report a case of pachyonychia congenita associated with eruptive vellus hair cyst.- - - - - - - - - - ranking = 2.5keywords = hair (Clic here for more details about this article) |
4/267. monilethrix--improvement by hormonal influences?monilethrix is a hereditary hair disorder that occurs monosymptomatically or as a monilethrix syndrome combined with other ectodermal anomalies. We report two siblings with the triple combination of monilethrix, keratosis pilaris, and koilonychia. In one of our patients, the hair disorder improved, with maximum hair growth up to 8 cm in length after her first menstrual period occurred, suggesting that hormonal influences may improve the hair disorders in monilethrix.- - - - - - - - - - ranking = 2keywords = hair (Clic here for more details about this article) |
5/267. Acquired partial curly hair.We report 6 adolescent females apparently affected by diffuse partial woolly hair. The patients complained of thinning and curling of some hair of the scalp. Examination revealed two distinct hair populations. The first hair population was straight, normally pigmented and of normal length, thus considered "normal" hair. The second hair population was wavy, curly, thinner and apparently slightly shorter, thus considered abnormal. Direct examination of the abnormal hair population under a light microscope revealed a curled pattern of the hair shaft, and single torsions. With light microscopy the clinically normal hair had no abnormalities. Under scanning electron microscopy (SEM) the abnormal hair had curly and flattened hair shafts of 30-60 mum in diameter, oval shaped sections, canalicular formations, single torsions and cuticular weathering. With SEM, clinically "normal" hair had a diameter between 60-80 mum, with no major relevant abnormalities except for weathering. These results are suggestive of a new variant of acquired kinking (curling) of hair simulating diffuse partial woolly hair. The absence of clinical alterations in the proximal area of the affected hair and the spontaneous improvement with time, suggests that this variant may result from environmental exposure (weathering, cosmetic procedures) in predisposed individuals.- - - - - - - - - - ranking = 9.5keywords = hair (Clic here for more details about this article) |
6/267. Pilomatricoma associated with several hair follicles.We report two cases of pilomatricoma in which the neoplasm was connected with several hair follicles. A 17-year-old boy developed an erythematous nodule, 1 cm in diameter, in the right temporal region, histologically showing basophilic cells connected with at least two hair follicles. In the other case, a 20-year-old man had a centrally ulcerated tumor, 2 cm in diameter, in the left lower back. Basophilic cells proliferated in connection with at least six hair follicle-like structures, accompanied by the development of shadow cells underneath. The histopathologic findings in these two cases suggest the existence of a subset of pilomatricomas that involve several pre-existing hair follicles.- - - - - - - - - - ranking = 4keywords = hair (Clic here for more details about this article) |
7/267. Unilesional follicular mycosis fungoides.Follicular mycosis fungoides (FMF) is an unusual clinical and histological variant of MF, characterized by selective involvement of hair follicles by atypical lymphocytes. We describe a female patient who had follicular papules located only on the medial aspect of her right thigh. To the best of our knowledge, this patient represents the first reported case of unilesional FMF.- - - - - - - - - - ranking = 0.5keywords = hair (Clic here for more details about this article) |
8/267. Treatment of multiple eruptive hair cysts with erbium:YAG laser.BACKGROUND: Eruptive vellus hair cysts (EVHC) frequently resist a variety of treatment modalities. While pulsed carbon dioxide (CO2) laser has been used effectively for facial EVHC, this laser presents significant risks for hypertrophic scarring when used on truncal sites. Due to absorption of 2940 nm energy by both tissue water and protein, the erbium:yttrium-aluminum-garnet (Er:YAG) laser ablates more cleanly and creates less residual thermal injury in the wound bed. This laser might prove efficacious and safe in treating nonfacial EVHC. OBJECTIVE: To assess treatment efficacy and wound healing after Er:YAG laser ablation of EVHC. methods: Two patients with 32 truncal EVHC were treated with pulsed Er:YAG laser using a drilling technique followed by second intention healing. RESULTS: Laser treatment sites healed without permanent dyspigmentation or hypertrophic scarring. No lesion recurrence was observed. CONCLUSION: Er:YAG laser ablation is an effective method for treating EVHC at anatomic sites prone to hypertrophic scar formation.- - - - - - - - - - ranking = 2.5keywords = hair (Clic here for more details about this article) |
9/267. Unusual multiple pilomatrixomata: case report and review of the literature.Pilomatrixomata are rare, benign, calcifying cutaneous tumours of the hair matrix cells. They usually present in childhood with a pre-surgical diagnosis of sebaceous cyst, because of their size and appearance. The usual sites of occurrence are the scalp, head and neck. Treatment is usually surgical excision. We report a case of unusual, multiple, benign pilomatrixomata in an Indian adult. The plastic surgeon should be aware of the various guises of pilomatrixoma and be alert to the important, although rare, occurrence of malignancy.- - - - - - - - - - ranking = 0.5keywords = hair (Clic here for more details about this article) |
10/267. cartilage-hair hypoplasia syndrome: increased apoptosis of T lymphocytes is associated with altered expression of Fas (CD95), FasL (CD95L), IAP, Bax, and Bcl2.cartilage-hair hypoplasia (CHH) is a rare autosomal recessive short-limbed dwarfism associated with thin and sparse hair and cell mediated or combined immunodeficiency. However, the basis of immune deficiency in CHH is unclear. In this study, we investigated a role of apoptosis in immunodeficiency in a patient with CHH. An increased apoptosis of both CD4 and CD8 T cells, as determined by TUNEL assay, was observed in CHH compared to an age-matched healthy dwarf control. Increased apoptosis in CHH was associated with increased expression of Fas (CD95), CD95L, and Bax and decreased expression of Bcl-2 and inhibitor of apoptosis protein (IAP) compared to the control. These data suggest that lymphopenia and immunodeficiency in CHH may be, at least in part, due to increased apoptosis of T cells, possibly through the Fas/ FasL signaling pathway.- - - - - - - - - - ranking = 3keywords = hair (Clic here for more details about this article) |
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