TITLE

Usual Interstitial Pneumonia Complicating Dyskeratosis Congenita

AUTHOR(S)
Utz, James P.; Ryu, Jay H.; Myers, Jeffrey L.; Michels, Virginia V.
PUB. DATE
June 2005
SOURCE
Mayo Clinic Proceedings;Jun2005, Vol. 80 Issue 6, p817
SOURCE TYPE
Academic Journal
DOC. TYPE
Article
ABSTRACT
Dyskeratosis congenita (DC) )s a rare disorder characterized by skin hyperpigmentation, nail dystrophy, and leukoplakia of mucous membranes. Pulmonary complications occur in approximately 20% of patients, although the specific histopathologic features, the temporal relationship between the diagnosis of DC and the development of pulmonary fibrosis, and the response to specific treatment are largely undefined. We describe 2 patients with DC who developed usual interstitial pneumonia. Pulmonary fibrosis developed 18 and 38 years after the original manifestations of DC. Both patients died of respiratory failure, 4 and 6 months after lung biopsy. Pulmonary fibrosis in patients with DC may be linked to underlying abnormalities of fibroblast function.
ACCESSION #
17341386

 

Related Articles

  • Vermilionectomy Using the W-Plasty Technique. Vozmediano, J. M. Fernandez; Cabrera, M. A. Romero; Cerquero, A. Carrascosa // Journal of Dermatologic Surgery & Oncology;Jun89, Vol. 15 Issue 6, p627 

    We describe a variation of the conventional vermilionectomy consisting of incisions using the W-plasty technique, which allows a more distensible scar with better cosmetic and functional results. Its primary indications are actinic cheilitis and leukoplakia of the lower lip. It is a more...

  • Oral Tissue Color Changes (III). Dummett, Clifton O. // Quintessence International;Jan1980, Vol. 11 Issue 1, p49 

    The article presents several diseases which are considered as contributing factors for oral skin discoloration. A generalized silver toxicosis results to a bluish black discoloration of intra and extra oral mucosae. A chronic mercurialism provides a generalized stomatitis and gray black tissues...

  • HLA Class II (DRB, DQA1 and DQB1) Allele and Haplotype Frequencies in the Patients with Pemphigus Vulgaris. Sheida Shams; Maryam Yousefi; Nima Rezaei; Ghasem Solgi; Farideh Khosravi; Bita Ansaripour; Batoul Moradi // Journal of Clinical Immunology;Mar2009, Vol. 29 Issue 2, p175 

    Abstract Introduction  Pemphigus vulgaris (PV), an autoimmune disease affecting the skin and mucous membranes, is associated with some human leukocyte antigen (HLA) class II alleles and haplotypes. Materials and Methods  In order to evaluate the association of HLA-DR and DQ...

  • GP Registrar: Pictorial - Case Study. Hensler, Paula // GP: General Practitioner;3/25/2005, p56 

    The article reports that a 40-year old South Asian woman presented with whitish-yellow patches on her arms and legs that started to expand gradually. On examination the localised patches were symmetrical with irregular borders. The woman explained that other members of her family were...

  • nevus.  // Taber's Cyclopedic Medical Dictionary (2009);2009, Issue 21, p1582 

    An encyclopedia entry for "nevus" is presented. It refers to a congenital discoloration of a circumscribed area of the skin due to pigmentation. Also given are definitions of various terms with the word "nevus" which include "nevus araneus," "blue nevus" and "junction nevus," are presented....

  • Common changes in skin pigmentation. Dickson, Terrina // GP: General Practitioner;6/16/2006, p30 

    The article presents a case study on two possible causes of change in pigmentation. Chloasma or melasma occurs in genetically predisposed females and is more common in darker skin. Increased pigmentation develops slowly, most commonly affecting the forehead, cheeks, upper lip and chin. Patients...

  • ask dr. day. Day, Doris J. // Redbook;Oct2004, Vol. 203 Issue 4, p40 

    Addresses a question about facial pigmentation. Information on melasma, a skin condition triggered by a skin combination of fluctuating hormones and sun exposure; Ways to reduce production of skin-darkening melanin.

  • ICHTHYOSIFORM DERMATOSES. Frost, Phillip // Journal of Investigative Dermatology;Jun73, Vol. 60 Issue 6, p541 

    Ichthyosiform dermatoses represent deviations from smooth supple skin to dry scaly skin. Ichthyosis comes from the Greek ichthys meaning fish, although the resemblance of human skin to fish skin in these conditions is, at best, superficial. In this group of skin disorders, the scales are...

  • Ask DR. CRUTCHFIELD: SKIN CARE ADVICE FROM AN EXPERT. Crutchfield, Charles E. // Minnesota Monthly;Feb2016, Vol. 50 Issue 2, p11 

    The article offers information on the skin condition vitiligo. A brief definition of Vitiligo is also presented. Topics discussed include its causes which might either be genetic, immune system-related, or environmental, prevalence, and diagnosis through skin biopsy or wood's lamp examination....

Share

Read the Article

Courtesy of VIRGINIA BEACH PUBLIC LIBRARY AND SYSTEM

Sorry, but this item is not currently available from your library.

Try another library?
Sign out of this library

Other Topics