Abstract:
:Based upon case reports and small case series, it has been known for many years that some types and subtypes of inherited epidermolysis bullosa (EB) may be at risk for developing one or more extracutaneous complications. Many of these are associated with considerable morbidity; some may result in death. Only over the past few years have there been data generated from large, well characterized cohorts. However, these data, to date, have been published almost exclusively in the nondermatologic literature. Our objective is to provide dermatologists with a comprehensive review of each major extracutaneous complication with a summary of the pertinent literature and recommendations for evaluation and optimal management. Part I highlights epithelial associated tissues, and part II addresses other organs. Based on these reviews, the readership should gain a greater understanding of the types of complications that may occur, when they are most likely to develop, and the range of medical and surgical interventions that are currently available. It should also be possible for the reader to develop surveillance strategies based on an understanding of the published evidence-based data. The breadth and range of severity of complications that arise in some EB types and subtypes within the external eye, ear, nose, upper airway, and gastrointestinal and genitourinary tracts suggest that optimal management must be multidisciplinary. Given the unique knowledge that dermatologists have of this disease, we believe that the care of the EB patient should be under the direction of his or her dermatologist, who can best assist in timely referrals to those specialists who are most experienced in the care of specific extracutaneous problems.
journal_name
J Am Acad Dermatoljournal_title
Journal of the American Academy of Dermatologyauthors
Fine JD,Mellerio JEdoi
10.1016/j.jaad.2009.03.052subject
Has Abstractpub_date
2009-09-01 00:00:00pages
367-84; quiz 385-6issue
3eissn
0190-9622issn
1097-6787pii
S0190-9622(09)00705-1journal_volume
61pub_type
杂志文章,评审abstract:BACKGROUND:After oral intake, 5-methoxypsoralen (5-MOP) is as effective as 8-MOP for PUVA therapy for psoriasis, with a lower incidence of acute cutaneous side effects. OBJECTIVE:We compared bath-water delivery of 5-MOP and 8-MOP for photochemotherapy of psoriasis. METHODS:Twenty-two patients underwent phototesting w...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(97)80278-2
更新日期:1997-06-01 00:00:00
abstract::Palliative care has been shown to improve quality of life, symptom, and caregiver burden for a range of life-limiting diseases. Palliative care use among patients with severe dermatologic disease remain relatively unexplored, but the limited available data suggest significant unmet care needs and low rates of palliati...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/j.jaad.2020.08.029
更新日期:2020-08-12 00:00:00
abstract::In a pilot study the therapeutic effect and side effect profile of low-dose interferon alfa-2b in combination with a retinoid for the treatment of cutaneous T cell lymphoma were evaluated. Seven patients (four women, three men) with histologically confirmed cutaneous T cell lymphoma were included. Four patients had re...
journal_title:Journal of the American Academy of Dermatology
pub_type: 临床试验,杂志文章
doi:10.1016/0190-9622(91)70036-2
更新日期:1991-02-01 00:00:00
abstract:BACKGROUND:Onychomycosis is a prevalent infection of the nail caused primarily by dermatophytes. Fluconazole is active in vitro against the most common pathogens, penetrates into the nail bed, and is clinically effective in the treatment of a wide variety of fungal infections. OBJECTIVE:The purpose of this study was t...
journal_title:Journal of the American Academy of Dermatology
pub_type: 临床试验,杂志文章,多中心研究,随机对照试验
doi:10.1016/s0190-9622(98)70491-8
更新日期:1998-06-01 00:00:00
abstract:BACKGROUND:Biologics targeting interleukin 17A (IL-17A) allow for rapid clearance of psoriatic plaques, with a clinically favorable safety profile. OBJECTIVES:To compare the safety and efficacy of ixekizumab, an IL-17A antagonist, with the safety and efficacy of the IL-12/23 inhibitor ustekinumab through 52 weeks of t...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,随机对照试验
doi:10.1016/j.jaad.2018.06.039
更新日期:2019-01-01 00:00:00
abstract::We describe a family with the unusual association of Cowden's disease and Bannayan-Riley-Ruvalcaba syndrome. The father has characteristic mucocutaneous features that are palmoplantar keratoses, multiple facial papules, oral papillomatoses, lipomas, and vitiligo with involvement of the thyroid and digestive tract. The...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/s0190-9622(00)90109-9
更新日期:2000-02-01 00:00:00
abstract::We reviewed all cases of malignant melanoma in children younger than 17 years of age who were evaluated at Sainte Justine Hospital, a tertiary care pediatric center, between 1980 and 2002. The medical records and histologic features of all cases were reviewed. Thirteen cases were identified, 4 boys and 9 girls. Fifty-...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2005.07.013
更新日期:2005-11-01 00:00:00
abstract::Lipedema is characterized by symmetric lower extremity enlargement secondary to the deposition of fat. Lipedema is not rare, but it is commonly misdiagnosed as lymphedema. We describe a 20-year-old woman with massive lower extremity enlargement that did not respond to compression therapy. Magnetic resonance imaging of...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2006.09.023
更新日期:2007-08-01 00:00:00
abstract:BACKGROUND:Despite improvements in melanoma mortality, disparities in melanoma survival persist. We evaluated possible sociodemographic and health care-based predictors of differences in melanoma survival in the United States by using the melanoma mortality-to-incidence ratio (MIR). METHODS:State-based MIRs were calcu...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2019.01.003
更新日期:2019-05-01 00:00:00
abstract:BACKGROUND:The use of immunotherapies in the treatment of metastatic cancers has significantly advanced oncology. However, due to safety concerns, solid organ transplant recipients (SOTRs) are routinely excluded from immunotherapy trials; thus, there is limited data for these agents in this population. METHODS:A syste...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2019.07.005
更新日期:2020-06-01 00:00:00
abstract:BACKGROUND:T-cell receptor (TCR) clonality may help establish a diagnosis of mycosis fungoides (MF). Routine clonality analysis is performed by using a polymerase chain reaction TCR- gamma assay, yet with this method, 10% to 50% of T-cell lymphomas escape detection. TCR- beta gene rearrangement is an additional assay. ...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2020.05.110
更新日期:2020-11-01 00:00:00
abstract:BACKGROUND:Recombinant human platelet derived growth factor-BB gel (PDGF gel) has been shown to be effective in the treatment of diabetic neuropathic ulcers. It is also being used off-label to speed wound healing of flaps, grafts, and wounds from Mohs micrographic surgery. OBJECTIVE:The purpose of the study was to com...
journal_title:Journal of the American Academy of Dermatology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1067/mjd.2001.117721
更新日期:2001-12-01 00:00:00
abstract:BACKGROUND:Reducing people's exposure to ultraviolet radiation is the primary strategy for skin cancer prevention. OBJECTIVE:We sought to provide comprehensive national data on preventive behaviors and risk assessment for Australia. METHODS:A national survey was conducted in summer 2003-2004. In 8 weekly cross-sectio...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2008.06.011
更新日期:2008-10-01 00:00:00
abstract::We report a case of drug-induced pemphigus caused by an angiotensin-converting enzyme inhibitor, captopril. The cutaneous reaction remitted after withdrawal of captopril therapy. Unique to this case, however, was the substitution of another angiotensin-converting enzyme inhibitor, enalapril, without exacerbation of th...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/0190-9622(92)70057-m
更新日期:1992-02-01 00:00:00
abstract::Prevention or modification of the onset of atopic dermatitis has been difficult to document through prolonged breast feeding or delayed introduction of solid foods. Dietary management of established atopic dermatitis is not routinely indicated for the majority of patients. Dietary management of atopic dermatitis shoul...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(86)70205-3
更新日期:1986-09-01 00:00:00
abstract:BACKGROUND:Numerous studies have analyzed the influence of psoriasis on the quality of life and psychosocial health of patients. However, few studies have addressed the effect of this disease on individuals living with these patients (cohabitants). OBJECTIVE:To analyze the influence of psoriasis on the levels of anxie...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2014.03.039
更新日期:2014-08-01 00:00:00
abstract::A patient who had a plaque on his forehead as the first sign of chronic myelomonocytic leukemia (CMML) is described. Histologic studies, which formerly led to the misdiagnosis of non-Hodgkin's lymphoma, revealed CMML with an unusual phenotype. This represents a rare type of CMML for the following reasons: (1) specific...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/s0190-9622(96)90088-2
更新日期:1996-11-01 00:00:00
abstract:BACKGROUND:The efficacy of combination therapy with topical corticosteroids and tacrolimus in the treatment of atopic dermatitis remains to be established. OBJECTIVE:Our aim was to determine whether a regimen of sequential application of topical corticosteroids and topical tacrolimus is effective in the treatment of p...
journal_title:Journal of the American Academy of Dermatology
pub_type: 临床试验,杂志文章
doi:10.1016/j.jaad.2008.09.034
更新日期:2009-02-01 00:00:00
abstract::The epidermal nevus syndrome is characterized by the association of epidermal nevi with abnormalities of the skin, skeletal system, central nervous system, eyes, and cardiovascular system, as well as with malignant conditions. We describe a 2-year-old girl with an extensive epidermal nevus involving the left side of t...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(96)90098-5
更新日期:1996-11-01 00:00:00
abstract:BACKGROUND:Genetic and environmental components may contribute to acne causation. OBJECTIVE:We sought to assess the impact of family history, personal habits, dietary factors, and menstrual history on a new diagnosis of moderate to severe acne. METHODS:We conducted a case-control study in dermatologic outpatient clin...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,多中心研究
doi:10.1016/j.jaad.2012.02.018
更新日期:2012-12-01 00:00:00
abstract::Acute generalized exanthematous pustulosis is a severe cutaneous adverse reaction characterized by the rapid development of nonfollicular, sterile pustules on an erythematous base. It is attributed to drugs in the majority of cases. Antibiotics are the most common cause of acute generalized exanthematous pustulosis; h...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/j.jaad.2015.07.017
更新日期:2015-11-01 00:00:00
abstract:BACKGROUND:Pulsed dye laser treatment and other cosmetic procedures result in significant bruising. Claims have been made regarding the efficacy of topical vitamin K in both preventing and speeding the clearing of bruising; however, well-controlled studies are lacking. OBJECTIVE:The purpose of this study is to evaluat...
journal_title:Journal of the American Academy of Dermatology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1067/mjd.2002.120465
更新日期:2002-08-01 00:00:00
abstract::Transepithelial elimination of granulomas was observed in histologic specimens taken from involved skin of a patient with disseminated tuberculosis and two patients with sarcoidosis. This phenomenon, previously also observed in other granulomatous disorders, represents one of the skin's several mechanisms to rid itsel...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(86)70016-9
更新日期:1986-01-01 00:00:00
abstract::Critical to the proper management of pyoderma gangrenosum are correct diagnosis, identification and treatment of any underlying disorder, and the proper choice of topical and systemic therapy. Many agents are available for the treatment of pyoderma gangrenosum. We review the current therapeutic options, their efficacy...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/s0190-9622(96)90285-6
更新日期:1996-06-01 00:00:00
abstract::One hundred three patients with acne vulgaris were randomly designated to receive either a topical formulation of erythromycin plus zinc or a topical solution of 1% clindamycin phosphate (Cleocin-T). The patients treated themselves twice daily and were examined at 3, 6, 9, and 12 weeks after the start of therapy. By w...
journal_title:Journal of the American Academy of Dermatology
pub_type: 临床试验,杂志文章,随机对照试验
doi:10.1016/0190-9622(90)70069-t
更新日期:1990-03-01 00:00:00
abstract::More than 100,000 species of fungi have been identified worldwide, but only a few dozen are of medical significance. Because of the opportunistic nature of these organisms and the prevalence of immunodeficient conditions, the number of medically important fungi is increasing. Identification of the causative organism i...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1016/s0190-9622(89)70234-6
更新日期:1989-10-01 00:00:00
abstract::A tense yellow vesicle was noted on the back parietal scalp of a female newborn. This proved to be a bullous variant of aplasia cutis congenita. Only 16 cases of this apparently rare disorder have been previously reported. Histologic evaluation of such lesions reveals a distinct pattern containing fibrovascular stroma...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章,评审
doi:10.1067/mjd.2003.150
更新日期:2003-05-01 00:00:00
abstract:BACKGROUND:Confetti-like depigmentation was noted in patients reporting recent worsening of vitiligo. OBJECTIVE:We sought to determine if confetti-like depigmentation is a marker of rapidly progressing vitiligo. METHODS:Review of patient records and images of patients from a vitiligo registry resulted in 7 patients w...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/j.jaad.2015.05.014
更新日期:2015-08-01 00:00:00
abstract::Infantile acropustulosis (IA) is a syndrome characterized by recurrent crops of 1- to 2-mm intensely pruritic vesicopustules that are found primarily on the distal extremities of infants. It is reportedly responsive to sulfones and unresponsive to other therapy, but if left untreated spontaneously resolves at about 2 ...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(83)70187-8
更新日期:1983-11-01 00:00:00
abstract::We report a cluster of primary cutaneous aspergillosis in six children with hematologic malignancy. When first seen, they had hemorrhagic bullae caused by Aspergillus flavus, Aspergillus fumigatus, and Aspergillus niger at the sites of insertion of intravenous cannulas or where arm boards had been taped to the extremi...
journal_title:Journal of the American Academy of Dermatology
pub_type: 杂志文章
doi:10.1016/s0190-9622(85)80042-6
更新日期:1985-02-01 00:00:00