Chemoprevention of squamous cell carcinoma in recessive dystrophic epidermolysis bullosa: results of a phase 1 trial of systemic isotretinoin

Fine, Jo David; Johnson, Lorraine; Weiner, Madeline; Stein, Amy; Suchindran, Chirayath

doi:10.1016/j.jaad.2003.08.008

Cited by 39 publications

(18 citation statements)

References 24 publications

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“…This can usually be carried out at the time of surgical excision of the primary SCC. Sentinel lymph node biopsy (SLNB) may be undertaken in patients with EB presenting with SCC . However, all reports of SLNB in patients with EB have so far been negative for nodal SCC . As yet, there is no evidence that SLNB results inform prognosis in EB SCCs, nor that undertaking regional lymph node clearance in SLNB‐positive patients influences the eventual clinical outcome. Regional lymph node clearance should be undertaken if there is evidence of nodal disease on FNA or biopsy, although there is no evidence that it affects prognosis.…”

Section: Tumour Evaluation and Stagingmentioning

confidence: 99%

Management of cutaneous squamous cell carcinoma in patients with epidermolysis bullosa: best clinical practice guidelines

et al. 2015

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SummaryThis article summarizes recommendations reached following a systematic literature review and expert consensus on the diagnosis and management of cutaneous squamous cell carcinomas in people with epidermolysis bullosa. The guidelines are intended to help inform decision making by clinicians dealing with this complex complication of a devastating disease.What's already known about this topic?• Some subtypes of epidermolysis bullosa (EB), particularly severe generalized recessive dystrophic EB, are associated with the development of mucocutaneous squamous cell carcinomas (SCCs).• These tumours behave aggressively and are a leading cause of morbidity and mortality in at-risk patients with EB.What does this study add?• These guidelines will assist clinicians in the diagnosis, management and staging of EB-associated cutaneous SCCs based on available evidence and expert consensus.• They highlight the importance of a holistic multidisciplinary approach to the management of EB-associated SCCs, where patient involvement in decision making is paramount.

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Section: Tumour Evaluation and Stagingmentioning

confidence: 99%

Management of cutaneous squamous cell carcinoma in patients with epidermolysis bullosa: best clinical practice guidelines

et al. 2015

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“…Because SCCs are the most common cause of death in RDEB, efforts have been made to identify possible means of chemoprevention. In 2004, results of a phase I trial of systemic isotretinoin in 20 RDEB patients were reported 73 . This drug was chosen because systemic retinoids have been shown to potentially influence SCCs and keratoacanthomas arising in several diseases, to include xeroderma pigmentosa and transplant patients.…”

Section: S To 2009mentioning

confidence: 99%

Inherited epidermolysis bullosa: past, present, and future

Fine

2010

Annals of the New York Academy of Sciences

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Inherited epidermolysis bullosa encompasses dozens of diseases characterized by mechanical fragility of the skin, blister formation, and abnormal wound healing. Most of the more severe subtypes are associated with clinically significant extracutaneous complications. Some subtypes may lead to death, even in early infancy. Over the past two decades substantial advances have been made to our understanding of the underlying molecular basis for each member of this protean group of diseases. Research has now shifted toward the identification of therapeutic interventions, to include gene therapy, recombinant protein infusions, intradermal injection of allogeneic fibroblasts, and stem cell transplantation, that might eventually lead to a definitive cure for this disease. Other developing therapies being explored are directed toward the enhancement of wound healing and the prevention of potentially life-threatening skin cancers in these patients.

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“…Meanwhile, isotretinoin has fewer toxic effects on bone density than etretinate. According to Kraemer et al, all the XP patients on long-term isotretinoin (up to 3 years) experienced mucocutaneous toxic effects, and triglyceride, liver function, or skeletal abnormalities developed in some on highdose oral isotretinoin, but it was found to be effective in the chemoprophylaxis of skin cancers in patients with XP (15). Therefore, isotretinoin treatment seems to be a good approach for cancer prevention in conditions with a high predisposition to skin cancer, such as in XP-V, and in most of the literature systemic isotretinoin in the XP pediatric population is justified.…”

Section: Discussionmentioning

confidence: 99%

Xeroderma pigmentosum and lentigo maligna in identical twins

Faghihi¹,

Radan

2006

Journal of Dermatological Treatment

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Xeroderma pigmentosum (XP) is a rare autosomal recessive genodermatosis. Skin abnormalities result from an inability to repair UV-damaged DNA. Clinically, XP presents with early onset cutaneous changes (severe photosensitivity, actinic keratoses, and telangiectasias) and an increase of developing cutaneous malignancies beginning in early childhood, but lentigo maligna and melanomas are relatively rare. Here we report on homozygote twins in whom there was no positive family history. They showed subnormal physical growth. On ophthalmological examination, both had photophobia and decreased visual acuity. Since birth, several excisions had been performed for skin neoplasms. In one of them a pigmented patch developed over the frontal area which proved to be lentigo maligna and she was referred to a dermato-oncology center. They have been given isotretinoin and physical sunscreen since then. The follow-up period was extended to 2 years and no serious complications occurred from the above treatment. This is an interesting report about XP in twins with the presentation of the rare neoplasm lentigo maligna.

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Chemoprevention of squamous cell carcinoma in recessive dystrophic epidermolysis bullosa: results of a phase 1 trial of systemic isotretinoin

Cited by 39 publications

References 24 publications

Management of cutaneous squamous cell carcinoma in patients with epidermolysis bullosa: best clinical practice guidelines

Management of cutaneous squamous cell carcinoma in patients with epidermolysis bullosa: best clinical practice guidelines

Inherited epidermolysis bullosa: past, present, and future

Xeroderma pigmentosum and lentigo maligna in identical twins

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