Skin grafting as a therapeutic approach in pretibially restricted junctional epidermolysis bullosa

Abstract

among those patients. The development of cutaneous MFH in an immunocompromised host may be coincidental. However, it is also conceivable that long-term immunosuppression and, in addition, fair complexion and a stay in the tropics represent cofactors for tumour development. Although cutaneous MFHs in RTRs are very rare, the case reported here shows that cutaneous MFH with a poor prognosis may arise after RT, especially in patients with several risk factors. To our knowledge, this is the first reported case of cutaneous MFH of the scalp in an RTR. References1 Hartevelt MM, Bouwes Bavinck JN, Kootte AMM et al. Incidence of skin cancer after renal transplantation in the Netherlands. Transplantation 1990; 49:506-9. 2 Euvrard S, Kanitakis J, Claudy A. Skin cancers after organ transplantation. N Engl J Med 2003; 348:1681-91. 3 Penn I. Sarcomas in organ allograft recipients. Transplantation 1995; 60:1485-91. 4 Weiss SW, Goldblum JR. Malignant fibrohistiocytic tumors. In: Enzinger and Weiss's Soft Tissue Tumors (Weiss SW, Goldblum JR, eds), 4th edn. St Louis: Mosby CV 2001; 535-69. 5 Hafner J, Künzi W, Weinreich T. Malignant fibrous histiocytoma and atypical fibroxanthoma in renal transplant recipients. Dermatology 1999; 198:29-32. 6 Kanitakis J, Euvrard S, Montazeri A et al. Atypical fibroxanthoma in a renal graft recipient. J Am Acad Dermatol 1996; 35:262-4. 7 Camacho FM, Moreno JC, Murga M et al. Malignant fibrous histiocytoma of the scalp. Multidisciplinary treatment. J Eur Acad Dermatol Venereol 1999; 13:175-82. 8 Leite C, Goodwin JW, Sinkovics JG et al. Chemotherapy of malignant fibrous histiocytoma. A Southwest Oncology Group Report.

Punch grafting of chronic ulcers in patients with laminin-332–deficient, non-Herlitz junctional epidermolysis bullosa

“…28 For patients with EB the advantage of punch grafting over other autologous grafts is that the grafts are smaller and thicker; larger grafts are meshed and thinner, and therefore can take longer to heal in deep ulcers or induce prolonged blistering at the donor site. 10,28 For cultured grafts, fewer or no injuries are made by harvesting donor grafts compared with punch grafting; however, producing these cultured grafts is time-consuming, expensive, laborious, and needs special facilities. 28 Although the limitations of this study are the retrospective design, small number of patients, absence of a control group, and follow-up and ulcer measurement that were not standardized, we conclude that punch grafting can be used as a first-line treatment in long-standing ulcers with no healing tendency in patients with JEB-nH.…”

“…In the past, tissue-engineered artificial allografts, 4,5 cultured allografts, 3,6 cultured autografts, 7,8 and full-thickness or split-thickness autografts 9,10 have been successfully applied in patients with EB. In a Cochrane review concerning venous leg ulcers in a non-EB population, no differences in efficacy were seen in the different types of skin grafting.…”

“…Skin grafting has been successfully applied in patients with EB, ranging from tissue-engineered artificial allografts, 4,5 cultured allografts, 3,6 cultured autografts, 7,8 and full-thickness or split-thickness autografts. 9, 10 We use the simple and inexpensive punch grafting method in patients with EB. A previous publication in a French journal showed the successful application of this technique in patients with recessive dystrophic EB.…”

Targeting of the hNC16A collagen domain to dendritic cells induces tolerance to human type XVII collagen

“…Aged epidermis is thin and progressively loses its capacity of renewal. The non-sulphated glycosaminoglycan hyaluronic acid (HA), described to mediate keratinocyte activation upon retinoid treatment or during wounding (3,4), is also reported to decrease with age (5,6). HA is synthesized by the hyaluronan synthases (HAS) through in the plasma membrane of the basal and spinous keratinocytes and is secreted under a high molecular mass between 10 2 and 10 4 kDa.…”

“…Mutations in the 16th non-collagenous domain A (NC16A) (3) are associated with junctional epidermolysis bullosa (JEB) (4,5). Beyond the pivotal role in this inherited skin disease, type XVII collagen is involved in various acquired subepidermal autoimmune blistering diseases, the most common being bullous pemphigoid (BP) (6,7).…”