Recessive Epidermolysis Bullosa Dystrophicans (Hallopeau‐Siemens)—Improvement of Wound Healing by Autologous Epidermal Grafts on an Esterified Hyaluronic Acid Membrane

Wollina, Uwe; Konrad, H; Fischer, Tobias W.

doi:10.1111/j.1346-8138.2001.tb00120.x

Cited by 22 publications

(18 citation statements)

References 15 publications

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“…Since then, cultured autologous and allogeneic epithelia have been produced for the treatment of extensive full-thickness burns, with good results (10). In addition, these biological dressings have been used for patients with EB with some success (6). However, controversy remains as to whether the process of isolating and eulturing keratinocytes prior to transplantation may somehow induce genetic modifications or enhance cell stem properties, potentially generating an increased risk of tumourigenesis after transplantation.…”

Section: Discussionmentioning

confidence: 98%

Long-term Follow-up of Cultured Epidermal Autograft in a Patient with Recessive Dystrophic Epidermolysis Bullosa

Shinkuma¹,

Sawamura²,

Fujita³

et al. 2014

Acta Derm Venerol

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Section: Discussionmentioning

confidence: 98%

Long-term Follow-up of Cultured Epidermal Autograft in a Patient with Recessive Dystrophic Epidermolysis Bullosa

Shinkuma¹,

Sawamura²,

Fujita³

et al. 2014

Acta Derm Venerol

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“…[4][5][6][7] The challenge is not the radical excision but instead the wound closure. Different options for wound coverage in patients with EB are known such as secondary wound healing, local skin flap/microsurgical free flap, skin grafting either as full thickness 10,11 or split thickness, 5À7,12 application of autologous keratinocytes (epidermal autografts [13][14][15] ), artificial grafts, 4 and bioengineered allogeneic grafts. 16 In our patient, reepithelialization could be achieved with all procedures performed but time varied considerably.…”

Section: Discussionmentioning

confidence: 99%

Multiple Squamous Cell Carcinomas in Junctional Epidermolysis Bullosa: A Surgical Challenge

Mohr¹,

Lohmeyer²,

Mikhaimer³

et al. 2008

Dermatologic Surgery

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The authors have indicated no significant interest with commercial supporters.H ereditary epidermolysis bullosa (EB) is a diverse group of genetic skin disorders having blister formation mostly upon minor trauma as their common feature. They present with a wide clinical spectrum of manifestations because of different underlying molecular defects. EB arises from abnormalities in proteins of the dermoepidermal junction. Three major EB forms can be distinguished on the basis of ultrastructural blistering level: EB simplex, junctional EB (JEB) and dystrophic EB (DEB). EB simplex is a mild form since the defect is located in certain keratins expressed in the keratinocytes of the basal layer, thus causing only superficial blisters. JEB can be associated with defects in the basement membrane protein laminin 5 and thus presents with deeper wounds, which sometimes heal with a distinctive atrophic appearance. DEB is caused by mutations in Type VII collagen, a dermal protein which anchors into the basement membrane. The hallmark features of DEB are deep wounds associated with scarring and severe mutilations. 1 In addition, DEB patients suffer frequently from multiple squamous cell carcinomas (SCC). 1,2 These tumors exert a quite aggressive phenotype with early metastases. 1 Owing to the impaired wound healing and the tendency for mutilating scarring, surgical removal of these tumors is quite problematic.In contrast to DEB, multiple SCC are not a typical feature of JEB. 2 Here we report a patient with JEB who developed multiple SCC. His brother, who was also affected by JEB, died from multiple SCC due to extensive metastasis. Because of the rare association of multiple SCC and JEB, the surgical experiences in this unique setting are very limited. We compared different approaches of wound closure after extensive open excisions in this patient. Case ReportA patient with JEB developed SCC on the lower legs for the first time at the age of 39 years. Clinical diagnosis of JEB was made a few days after birth and confirmed by electron microscopy and antigen mapping. Skin defects by JEB extended mainly over his lower legs. The entire region was almost completely covered with blisters, which were impetiginized mostly by Staphylococcus aureus. Other areas affected but less severely were arms, trunk, and thighs. His younger brother also suffered from JEB with a more pronounced course of the disease. 3 He developed multiple SCC at the age of 32 years. Despite surgical treatment, the aggressive growth of these tumors led to metastasis involving the lymph nodes and the lung and finally to death at the age of 39.In the presented patient, SCC was first diagnosed 11 years previously. He presented with hyperkeratotic areas of approximately 10 Â 12 cm on both shins. Diagnosis was confirmed by multiple punch biopsies. We performed large excisions of the lesions with

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“…Until now, various kinds of biological dressings, including autologous and allogeneic skin grafts, have been employed for the treatment of the intractable ulcers in patients with junctional or the recessive dystrophic type of EB 7–14 with favorable results. However, autologous skin substitutes are very costly and time‐consuming to procure, and it may be impracticable to use them for the treatment of wide extensive and chronic ulcers in cases of RDEB.…”

Section: Discussionmentioning

confidence: 99%

Amnia for intractable skin ulcers with recessive dystrophic epidermolysis bullosa: Report of three cases

Hasegawa

Mizoguchi

Haruna

et al. 2007

The Journal of Dermatology

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Recessive dystrophic epidermolysis bullosa (RDEB) is a disease characterized by recurrent blistering and chronic ulceration of the skin. In these patients, recurrent blisters frequently result in intractable skin ulcers due to impaired wound healing caused by mutations in the type VII collagen gene and malnutrition as well as by increased collagenase activity. To evaluate the efficacy of amnia for intractable ulcers in RDEB, we treated RDEB patients with amnia. The amniotic membrane was simply placed on the cleansed wound surface. The procedure was repeated once a week for up to 10 weeks. As a result, wound conditions improved remarkably after treatment with amnia for 2-10 weeks in all the patients, resulting in total re-epithelization of the ulcers. Amnia could be an effective therapy for intractable skin ulcers in RDEB patients, and should be considered as a re-emerging therapeutic option for the disease.

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Recessive Epidermolysis Bullosa Dystrophicans (Hallopeau‐Siemens)—Improvement of Wound Healing by Autologous Epidermal Grafts on an Esterified Hyaluronic Acid Membrane

Cited by 22 publications

References 15 publications

Long-term Follow-up of Cultured Epidermal Autograft in a Patient with Recessive Dystrophic Epidermolysis Bullosa

Long-term Follow-up of Cultured Epidermal Autograft in a Patient with Recessive Dystrophic Epidermolysis Bullosa

Multiple Squamous Cell Carcinomas in Junctional Epidermolysis Bullosa: A Surgical Challenge

Amnia for intractable skin ulcers with recessive dystrophic epidermolysis bullosa: Report of three cases

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