Alopecia Universalis

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Renu Kandpal - One of the best experts on this subject based on the ideXlab platform.

  • rare presentation of Alopecia Universalis congenita and twenty nail dystrophy in siblings
    International Journal of Trichology, 2017
    Co-Authors: Debdeep Mitra, Reetu Agarwal, Ajay Chopra, Renu Kandpal
    Abstract:

    Congenital Alopecia Universalis is one of the rarest anomaly which involves skin and appendages. The inheritance pattern can be autosomal recessive, X-linked recessive, or autosomal dominant. However, the most common is autosomal recessive form and it is the most severe phenotype. Twenty-nail dystrophy refers to the condition in which all the twenty nails are affected in the form of excessive ridging and nail plate roughness leading to unsightly lustureless nails. We report a rare case of two siblings with Alopecia Universalis congenita with twenty-nail dystrophy. To the best of our knowledge, this case is the first case to be reported with such association in both siblings. This case reports highlights the fact that Alopecia areata is an autoimmune disease with a genetic predisposition as in our case both siblings had Alopecia Universalis and nail dystrophy. There was no evidence of any other ectodermal dyplasia and had normal teeth and seat glands. The skin biopsy ruled out congenital atrichia and was suggestive of Alopecia areata.

Toshio Hamada - One of the best experts on this subject based on the ideXlab platform.

Debdeep Mitra - One of the best experts on this subject based on the ideXlab platform.

  • rare presentation of Alopecia Universalis congenita and twenty nail dystrophy in siblings
    International Journal of Trichology, 2017
    Co-Authors: Debdeep Mitra, Reetu Agarwal, Ajay Chopra, Renu Kandpal
    Abstract:

    Congenital Alopecia Universalis is one of the rarest anomaly which involves skin and appendages. The inheritance pattern can be autosomal recessive, X-linked recessive, or autosomal dominant. However, the most common is autosomal recessive form and it is the most severe phenotype. Twenty-nail dystrophy refers to the condition in which all the twenty nails are affected in the form of excessive ridging and nail plate roughness leading to unsightly lustureless nails. We report a rare case of two siblings with Alopecia Universalis congenita with twenty-nail dystrophy. To the best of our knowledge, this case is the first case to be reported with such association in both siblings. This case reports highlights the fact that Alopecia areata is an autoimmune disease with a genetic predisposition as in our case both siblings had Alopecia Universalis and nail dystrophy. There was no evidence of any other ectodermal dyplasia and had normal teeth and seat glands. The skin biopsy ruled out congenital atrichia and was suggestive of Alopecia areata.

Shoji Taniguchi - One of the best experts on this subject based on the ideXlab platform.

Antonella Tosti - One of the best experts on this subject based on the ideXlab platform.

  • Alopecia Universalis associated with cutaneous t cell lymphoma
    Dermatology, 2014
    Co-Authors: Mariya Miteva, Laila El Shabrawicaelen, Regina Finkpuches, Christine Behamschmid, Paolo Romanelli, Francisco A Kerdel, Antonella Tosti
    Abstract:

    Background: Alopecia areata-like hair loss may occur in the context of cutaneous T cell lymphoma (CTCL) and can very rarely evolve to Alopecia Universalis-like presentation. The dermoscopic findings of CTCL-related Alopecia have not been described. Methods: Two patients with Alopecia areata Universalis-like hair loss occurring in the context of preexisting, pathology-proven CTCL are presented. Results: Clinical examination showed subtotal scalp Alopecia with sparse fine hair or total scalp Alopecia with loss of eyebrows, eyelashes and body hair. On dermoscopy there was follicular or diffuse scaling, reduced number of follicular openings with broken hairs, short hairs or keratotic filiform spicules. Pathology confirmed the diagnosis of CTCL-related Alopecia. One patient had almost complete hair regrowth after treatment. Conclusion: CTCL-related Alopecia Universalis is a rare non-scarring form of hair loss which simulates Alopecia areata Universalis. We provide clues to distinguish both based on clinical, dermoscopic and pathologic findings.

  • Alopecia areata a long term follow up study of 191 patients
    Journal of The American Academy of Dermatology, 2006
    Co-Authors: Antonella Tosti, S Bellavista, Matilde Iorizzo
    Abstract:

    Background The prognosis of Alopecia areata (AA) is difficult to predict. Few studies report long-term follow-up of AA patients. Objective The purpose of this study is to better assess the long-term evolution of AA and the possible relationship between disease severity and treatment response with long-term prognosis. Methods One hundred ninety-one patients with AA who presented with a new diagnosis of AA between 1983 and 1990 were subsequently contacted by phone. Patients were queried regarding current disease status, treatments, and disease course. Results Severity of AA at first consultation ranged from mild (128 patients) to severe (63 patients). Fifty-five of 191 patients were affected by concomitant autoimmune or related inflammatory disease. Sixty-six of 191 patients were presently disease free (follow-up duration, 15-22 years; mean 17.74 years). These include 41 of 60 patients with S1 disease (68.3%), 22 of 68 patients with S2 disease (32.3%), 1 of 11 patients with S3 disease (9%), 1 of 14 patients with S4 disease (7.1%), and 1 of 11 patients with Alopecia totalis (AT) (9.1%). Sixty-nine of 191 patients (36-1%) were presently affected by AT or Alopecia Universalis. There was a statistically significant tendency of severe patterns of AA to worsen over time. In children, 18 of 39 (13 with ≤S2 disease and 5 with ≥S3 disease) with AA had developed AT or Alopecia Universalis at long-term follow-up. In children, however, this trend was not statistically significant. Patients with severe AA who responded to topical immunotherapy seem to have a better prognosis than nonresponders. Limitations Follow-up was only performed by phone. Conclusions Severity of AA at time of first consultation is an important prognostic factor. Response to therapy (topical immunotherapy) may be associated with better prognosis. In children, the prognosis is worse; our study found that AA worsens over time.

  • Clobetasol propionate 0.05% under occlusion in the treatment of Alopecia totalis/Universalis.
    Journal of The American Academy of Dermatology, 2003
    Co-Authors: Antonella Tosti, Massimiliano Pazzaglia, Bianca Maria Piraccini, Colombina Vincenzi
    Abstract:

    Abstract Background: Efficacy of topical steroids in Alopecia areata is still discussed. Objective: The purpose of this study was to evaluate the efficacy of clobetasol propionate 0.05% ointment under occlusion in 28 patients with Alopecia areata totalis (AT) or AT/Alopecia Universalis. Methods: A total of 28 patients were instructed to apply 2.5 g of clobetasol propionate to the right side of the scalp every night under occlusion with a plastic film. Treatment was performed 6 days a week for 6 months. When regrowth of terminal hair occurred, treatment was extended over the entire scalp. All patients were followed up for another 6 months. Results: Of the 28 patients included in the study, 8 were treated successfully (28.5%). Regrowth of terminal hair began on the treated side 6 to 14 weeks after the start of treatment. Of these 8 patients, 3 had a relapse and were not able to maintain hair regrowth. Conclusion: Our study shows that clobetasol propionate 0.05% under occlusion is effective in inducing hair regrowth in patients with AT or AT/Alopecia Universalis. Occurrence of hair regrowth only on the treated half of the scalp clearly shows that efficacy of treatment is a result of a local and not systemic effect of the drug. Although only 17.8% of patients had long-term benefit by treatment, our results were obtained in a population of patients with severe and refractory forms of the disease. (J Am Acad Dermatol 2003;49:96-8.)

  • clobetasol propionate 0 05 under occlusion in the treatment of Alopecia totalis Universalis
    Journal of The American Academy of Dermatology, 2003
    Co-Authors: Antonella Tosti, Massimiliano Pazzaglia, Bianca Maria Piraccini, Colombina Vincenzi
    Abstract:

    Abstract Background: Efficacy of topical steroids in Alopecia areata is still discussed. Objective: The purpose of this study was to evaluate the efficacy of clobetasol propionate 0.05% ointment under occlusion in 28 patients with Alopecia areata totalis (AT) or AT/Alopecia Universalis. Methods: A total of 28 patients were instructed to apply 2.5 g of clobetasol propionate to the right side of the scalp every night under occlusion with a plastic film. Treatment was performed 6 days a week for 6 months. When regrowth of terminal hair occurred, treatment was extended over the entire scalp. All patients were followed up for another 6 months. Results: Of the 28 patients included in the study, 8 were treated successfully (28.5%). Regrowth of terminal hair began on the treated side 6 to 14 weeks after the start of treatment. Of these 8 patients, 3 had a relapse and were not able to maintain hair regrowth. Conclusion: Our study shows that clobetasol propionate 0.05% under occlusion is effective in inducing hair regrowth in patients with AT or AT/Alopecia Universalis. Occurrence of hair regrowth only on the treated half of the scalp clearly shows that efficacy of treatment is a result of a local and not systemic effect of the drug. Although only 17.8% of patients had long-term benefit by treatment, our results were obtained in a population of patients with severe and refractory forms of the disease. (J Am Acad Dermatol 2003;49:96-8.)

  • Prevalence of Nail Abnormalities in Children With Alopecia Areata
    Pediatric Dermatology, 1994
    Co-Authors: Antonella Tosti, R. Morelli, Federico Bardazzi, A. M. Peluso
    Abstract:

    : We assessed the prevalence of nail abnormalities in 272 children with Alopecia areata who were seen in our department during an eight-year period. Of these, 126 (46%; 50 girls, 76 boys) had nail abnormalities that were related to Alopecia areata. Nail pitting was detected in 92 patients, including 37 with Alopecia totalis or Alopecia Universalis. Three patients experienced an onychomadesis of all 20 nails during the acute onset of Alopecia areata Universalis. Thirty-two (11.7%) had nail thinning and severe nail plate surface abnormalities that were consistent with a diagnosis of trachyonychia.