Cases reported "Alopecia"

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1/288. Diffuse alopecia with stem cell folliculitis: chronic diffuse alopecia areata or a distinct entity?

    A 34-year-old woman presented with an 8-year history of slowly progressive diffuse nonscarring alopecia with loss of hair density. scalp biopsy specimens showed increased miniaturized follicles and an asymmetric wedge-shaped lymphocytic infiltrate concentrated on the stem cell-rich region at the point of entry of sebaceous ducts and at bulge-like regions of multiple follicles. Several hair bulbs emerging at the stem cell compartment also were inflamed, but the hair bulbs in the deeper dermis and subcutis were spared. I speculate whether these findings may represent a stem cell folliculitis similar to the reaction pattern previously observed in graft versus host disease and in androgenetic alopecia. The additional presence of peribulbar lymphocytic inflammation could indicate that the patient had a variant of alopecia areata. The clinical presentation of a slowly progressive diffuse alopecia without progression to clinically recognizable alopecia areata and the prominent lymphocytic inflammation involving the stem cell compartment may prompt a reexamination of similar cases currently classified as chronic diffuse alopecia areata. The concept that lymphocytes can inhibit stem cell function without destroying the stem cells themselves needs consideration.
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2/288. celiac disease-associated alopecia in childhood.

    We report the association of celiac disease and alopecia in 3 children. In one, the alopecia developed after 4 years' nonadherence to a gluten-free diet; the other 2 patients presented with alopecia. Administration of a gluten-free diet resulted in partial regrowth of hair in the first child and complete hair growth in the others.
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3/288. Prevention and treatment of wide scar and alopecia in the scalp: wedge excision and double relaxation suture.

    The visible linear scar of the scalp is a cosmetically serious complication of a scalp incision in scalp surgery, forehead lift, and craniofacial surgery, especially on the temporal scalp. Its causes are cicatrical alopecia and scar widening. To solve this problem, we performed the wedge excision of the scalp and the double relaxation suture of the galea in 2 patients undergoing facial surgery through the coronal approach and in 15 patients with scalp alopecia ranging from 0.5 to 3.0 cm in width. The wedge excision using the beveling incision at an angle of 30 degrees to the hair follicles preserves the deep hair follicles of the flap margins and allows the hair to grow into the scar, eventually preventing cicatricial alopecia and camouflaging the linear scar. The double relaxation suture of the trimmed galea with nonabsorbable suture with or without the relaxation incision minimizes skin tension for a long time, eventually preventing scar widening. This procedure was followed by the superficial skin suture for maintaining the skin sutures for a long time and avoiding the injury of the superficial hair follicles. In all patients, we observed an excellent cosmetic result of unnoticed scar line without complications during the follow-up period of 10 weeks to 6 months.
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4/288. pityriasis amiantacea.

    pityriasis amiantacea is an easily recognizable cutaneous disease that is characterized by scaling of the scalp and temporary alopecia. The scaling, described as thick and asbestos-like, binds down tufts of hair. The alopecia may be temporary or scarring. Despite a well-established clinical description, no definitive etiology has been uncovered. This disease may represent seborrheic dermatitis, superficial fungal or pyogenic infections, lichen planus, or psoriasis. Histologically, the disease is characterized by eczematous features. We report a case of pityriasis amiantacea in an 11-year-old girl with simultaneous findings of psoriasis as well as review the literature on the etiology of this disease.
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5/288. Cutaneous telangiectasias, sparse hair, and type I membranoproliferative glomerulonephritis.

    We report the unusual association of normocomplementemic type I membranoproliferative glomerulonephritis in a 10-year-old girl with sparse red hair, absent eyebrows and eyelashes, cutaneous telangiectasias, and an atrial septal defect.
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6/288. 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.
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7/288. Cardiofaciocutaneous (CFC) syndrome.

    An 11-year-old boy affected by mental retardation and seizures demonstrates congenital heart defect, many dysmorphic features and dry skin. His hair is sparse over the vertex with alopecia of the eyebrows and eyelashes. There are horny small papules evident in those areas. The diagnosis of cardiofaciocutaneous syndrome has been made. The relationship between cardiofaciocutaneous and Noonan syndrome is discussed.
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8/288. Linear lesions reflecting lyonization in women heterozygous for IFAP syndrome (ichthyosis follicularis with atrichia and photophobia).

    A diagnosis of IFAP (ichthyosis follicularis with atrichia and photophobia) syndrome was established in a 1-year-old boy with congenital hairlessness, generalized ichthyotic skin changes with follicular hyperkeratoses, and photophobia. IFAP syndrome is considered to be an X-linked recessive trait. The phenotype present in female carriers has so far not been delineated. A 2-year-old sister had atrophoderma and ichthyotic skin lesions arranged in a linear pattern and a large noncicatrical bald patch on her scalp. Similarly, the mother had linear lesions of scaling and atrophy as well as circumscribed hairless areas involving the scalp, the axillary region, and the lower legs. sweat testing by means of iodine starch-reaction visualized hypohidrotic linear lesions corresponding to the areas of hyperkeratosis and atrophy. In both mother and daughter the lesions followed the lines of Blaschko, whereas the boy was diffusely affected. family history showed that the boy's maternal uncle who had died at age 1 year was likewise affected with the same disorder. Moreover, the maternal grandmother had reportedly bald patches on her scalp and very dry skin. This is the first report to document linear skin lesions visualizing lyonization in women heterozygous for IFAP syndrome.
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9/288. Effect of IVIG on the hair regrowth in a common variable immune deficiency patient with alopecia universalis.

    Common variable immune deficiency (CVID) is associated with a variety of autoimmune diseases. Alopecia universalis (AU), believed to have an autoimmune basis, has been found in 1.6% of patients with CVID. Intravenous immunoglobulin (IVIG) therapy is used in various immunodeficiency disorders including CVID, and benefit has been shown in the therapy of autoimmune diseases. We report a patient with CVID and AU treated with IVIG who experienced significant hair regrowth. An 8-year-old girl with CVID and AU was treated with IVIG 400 mg/kg every 4 weeks. Since her second dose of IVIG, regrowth of eyelashes, eyebrows, body and scalp hair was observed in this patient. At present, about 1 year treat-meant of IVIG, significant hair regrowth is noted with 5-6 cm of scalp hair. We believe that IVIG may be beneficial in the treatment of AU, at least in patients with CVID.
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10/288. Tufted hair folliculitis: a pattern of scarring alopecia?

    We present a patient with pemphigus vulgaris who, over the years, experienced the development of tufted hair folliculitis as a result of scalp involvement. Multiple hairs emerged from widely dilated follicular ostia surrounded by indurated, scarred skin. Histopathologic findings were typical for tufted hair folliculitis. We believe that because a specific host response to scalp injury might be crucial to the development of this rare disorder, it should be regarded as a type of scarring alopecia.
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