Letters to the editor
1651
In conclusion, NWPT and treatment with NCPM are equally effective in ulcer debridement and wound bed preparation for successful skin grafting. While NWPT was faster in achieving a clean granulating wound bed than NCPM, handling was easier and much less painful with the latter and, theoretically, could also have been performed by the patient herself. An advantage of NWPT is its usefulness in securing skin grafts in a difficult recipient bed. The uniform distribution of the negative pressure over the surface improves the immobilization of a fresh placed mesh-graft, ensuring a positive contact between the undersurface of the skin graft and the recipient bed by minimizing the chance of shearing and exudative fluid collection (Table 1).10 J. Deluca, B. Gatscher, L. Tappeiner, M. Pichler, K. Eisendle* Department of Dermatology, Venerology and Allergology, Academic Teaching Department of Innsbruck Medical University, Central Teaching Hospital of Bolzano, Bozen, Italy *Correspondence: K. Eisendle. E-mail: [email protected]
Periorbital papules and erythematous-brownish plaques on the neck and axilla Editor A 65-year-old man presented with recurrent swellings of eyelids, lips, tongue and on course with erythematous-brownish papules around the eyelids as well as plaques on neck and axilla (Fig. 1). Four years ago multiple myeloma (according to Durie and Salmon Stage IIA) had been diagnosed and treated with chemotherapy (Bortezomid, Doxorubicin, Dexamethason). Previous to referral for evaluation of the skins signs, lambda light chains in the urine increased and the patient developed pronounced Bence-Jones proteinuria and immunoglobulin deficit. There was no hepatomegaly, but a slight left ventricular hypertrophy diagnosed by echocardiography and bone marrow infiltration by the myeloma was 15%. Histological examination from multiple skin and mucosal sites (tongue, abdomen, neck and axilla) showed orthokeratosis, atrophic epidermis and typical fissuring in the dermis. Hyaline
References 1 Barendse-Hofmann MG, van Doorn L, Steenvoorde P. Circumferential application of VAC on a large degloving injury on the lower extremity. J Wound Care 2009; 18: 79–82. 2 DeFranzo AJ, Marks MW, Argenta LC, Genecov DG. Vacuum-assisted closure for the treatment of degloving injuries. Plast Reconstr Surg 1999; 104: 2145–2148. 3 Molnar JA, DeFranzo AJ, Marks MW. Single-staged approach to skin grafting the exposed skull. Plast Reconstr Surg 2000; 105: 174– 177. 4 Scherer LA, Shive S, Chang M, Meredith JW, Owings JT. The vacuum assisted closure device: a method of securing skin grafts and improving graft survival. Arch Surg 2002; 137: 930–933. 5 K€ orber A, Franckson T, Grabbe S, Dissemond J. Vacuum assisted closure device improves the take of mesh grafts in chronic leg ulcer patients. Dermatology 2008; 216: 250–256. 6 Moisidis E, Heath T, Boorer C, Ho K, Deva AK. A prospective, blinded, randomized, controlled clinical trial of topical negative pressure use in skin grafting. Plast Reconstr Surg 2004; 114: 917–922. 7 Guest JF, Sladkevicius E, Panca M. Cost-effectiveness of using Polyheal compared with surgery in the management of chronic wounds with exposed bones and/or tendons due to trauma in France, Germany and the UK. Int Wound J 2013. doi: 10.1111/iwj.12055. [Epub ahead of print] 8 Wu L, Mockros NE, Casperson ME et al. Effects of electrically charged particles in enhancement of rat wound healing. J Surg Res 1999 Jul; 85: 43–50. 9 Shoham Y, Kogan L , Weiss J et al. Wound ‘dechronification’ with negatively-charged polystyrene microspheres: a double-blind RCT. J Wound Care 2013; 22: 144–146, 148, 150-2 passim 10 Schneider AM, Morykwas MJ, Argenta LC. A new and reliable method of securing skin grafts to the difficult recipient bed. Plast Reconstr Surg 1998; 102: 1195–1198. DOI: 10.1111/jdv.12527
JEADV 2015, 29, 1641–1657
(a)
(b)
Figure 1 A 65-year-old man presented with recurrent swellings of eyelids, lips, tongue and erythematous-brownish papules around the eyelids (a) as well as plaques on neck and axilla (b).
© 2014 European Academy of Dermatology and Venereology
1652
(a)
(b1)
(b2) Figure 2 (a) Histological examination showed orthokeratosis, atrophic epidermis and typical fissuring in the dermis. Hyaline deposits were found especially perivascular. (b) At work up immunohistochemistry showed AL-amyloid (lambda-chains) in the skin as well as in the bone marrow. Bone marrow infiltration by the myeloma is 15%.
JEADV 2015, 29, 1641–1657
Letters to the Editor
deposits were found especially perivascular (Fig. 2a). Immunhistochemistry showed AL-amyloid (lambda-chains) in the skin as well as in the bone marrow (Fig. 2b). Thus, Lambda-type amyloidosis associated with myeloma was diagnosed. Because of disease progression the patient received autologous stem cell transplantation and maintenance therapy with follow and concomitant lenalidomid treatment. Amyloidosis is caused by extracellular or rarely intracellular deposition of insoluble fibrillary proteins and eventually leading to organ dysfunction.1 Diagnosis is based on histopathology (e.g. congo red) plus immunohistochemistry. About 30 immunohistochemical different amyloid types exist.2 Our patient displayed amyloidosis of AL-type with underlying lymphoproliferative disease, which is associated in 20–40% of cases with skin manifestations.3 At work up, immunohistochemistry showed AL-amyloid (lambda-chains) in the skin as well as in the bone marrow (Fig. 2b). Nomenclature follows: (i) the progenitor protein; and (ii) clinical presentation (localized vs. systemic, hereditary vs. chronic inflammation vs. tumour associated). In primary localized cutaneous amyloidosis, amyloid is deposited in previously apparently normal skin with no evidence of deposits in any of internal organs (e.g. lichenoid amyloidosis, macular amyloidosis, nodular amyloidosis, familial primary cutaneous amyloidosis).4 Secondary localized cutaneous amyloidosis can develop with, e.g. skin tumours or discoid lupus erythematosus. Macular amyloidosis often presents as light brown patches with focal areas of dark red and brown. Lichen amyloidosis is described as hyperpigmented papules that are located on the shins. Both are associated with pruritus. Nodular amyloid is very rare and is characterized by skin-coloured nodules.5 The risk of progression from nodular to systemic amyloidosis ranges from 7 to 50%.6 Skin may also be involved in systemic amyloidosis. There are three main types of systemic amyloidosis: AL-amyloidosis, AAamyloidosis and TTR-hereditary amyloidosis. AL-amyloidosis occurs in patients with monoclonal gammopathies. AA-amyloidosis may occur in chronic infections or rheumatic or inflammatory diseases, whereas the familial types are autosomal dominant diseases in which a genetically inherited variant form of a normal protein constitutes the amyloid fibril and accounts for
1651
In conclusion, NWPT and treatment with NCPM are equally effective in ulcer debridement and wound bed preparation for successful skin grafting. While NWPT was faster in achieving a clean granulating wound bed than NCPM, handling was easier and much less painful with the latter and, theoretically, could also have been performed by the patient herself. An advantage of NWPT is its usefulness in securing skin grafts in a difficult recipient bed. The uniform distribution of the negative pressure over the surface improves the immobilization of a fresh placed mesh-graft, ensuring a positive contact between the undersurface of the skin graft and the recipient bed by minimizing the chance of shearing and exudative fluid collection (Table 1).10 J. Deluca, B. Gatscher, L. Tappeiner, M. Pichler, K. Eisendle* Department of Dermatology, Venerology and Allergology, Academic Teaching Department of Innsbruck Medical University, Central Teaching Hospital of Bolzano, Bozen, Italy *Correspondence: K. Eisendle. E-mail: [email protected]
Periorbital papules and erythematous-brownish plaques on the neck and axilla Editor A 65-year-old man presented with recurrent swellings of eyelids, lips, tongue and on course with erythematous-brownish papules around the eyelids as well as plaques on neck and axilla (Fig. 1). Four years ago multiple myeloma (according to Durie and Salmon Stage IIA) had been diagnosed and treated with chemotherapy (Bortezomid, Doxorubicin, Dexamethason). Previous to referral for evaluation of the skins signs, lambda light chains in the urine increased and the patient developed pronounced Bence-Jones proteinuria and immunoglobulin deficit. There was no hepatomegaly, but a slight left ventricular hypertrophy diagnosed by echocardiography and bone marrow infiltration by the myeloma was 15%. Histological examination from multiple skin and mucosal sites (tongue, abdomen, neck and axilla) showed orthokeratosis, atrophic epidermis and typical fissuring in the dermis. Hyaline
References 1 Barendse-Hofmann MG, van Doorn L, Steenvoorde P. Circumferential application of VAC on a large degloving injury on the lower extremity. J Wound Care 2009; 18: 79–82. 2 DeFranzo AJ, Marks MW, Argenta LC, Genecov DG. Vacuum-assisted closure for the treatment of degloving injuries. Plast Reconstr Surg 1999; 104: 2145–2148. 3 Molnar JA, DeFranzo AJ, Marks MW. Single-staged approach to skin grafting the exposed skull. Plast Reconstr Surg 2000; 105: 174– 177. 4 Scherer LA, Shive S, Chang M, Meredith JW, Owings JT. The vacuum assisted closure device: a method of securing skin grafts and improving graft survival. Arch Surg 2002; 137: 930–933. 5 K€ orber A, Franckson T, Grabbe S, Dissemond J. Vacuum assisted closure device improves the take of mesh grafts in chronic leg ulcer patients. Dermatology 2008; 216: 250–256. 6 Moisidis E, Heath T, Boorer C, Ho K, Deva AK. A prospective, blinded, randomized, controlled clinical trial of topical negative pressure use in skin grafting. Plast Reconstr Surg 2004; 114: 917–922. 7 Guest JF, Sladkevicius E, Panca M. Cost-effectiveness of using Polyheal compared with surgery in the management of chronic wounds with exposed bones and/or tendons due to trauma in France, Germany and the UK. Int Wound J 2013. doi: 10.1111/iwj.12055. [Epub ahead of print] 8 Wu L, Mockros NE, Casperson ME et al. Effects of electrically charged particles in enhancement of rat wound healing. J Surg Res 1999 Jul; 85: 43–50. 9 Shoham Y, Kogan L , Weiss J et al. Wound ‘dechronification’ with negatively-charged polystyrene microspheres: a double-blind RCT. J Wound Care 2013; 22: 144–146, 148, 150-2 passim 10 Schneider AM, Morykwas MJ, Argenta LC. A new and reliable method of securing skin grafts to the difficult recipient bed. Plast Reconstr Surg 1998; 102: 1195–1198. DOI: 10.1111/jdv.12527
JEADV 2015, 29, 1641–1657
(a)
(b)
Figure 1 A 65-year-old man presented with recurrent swellings of eyelids, lips, tongue and erythematous-brownish papules around the eyelids (a) as well as plaques on neck and axilla (b).
© 2014 European Academy of Dermatology and Venereology
1652
(a)
(b1)
(b2) Figure 2 (a) Histological examination showed orthokeratosis, atrophic epidermis and typical fissuring in the dermis. Hyaline deposits were found especially perivascular. (b) At work up immunohistochemistry showed AL-amyloid (lambda-chains) in the skin as well as in the bone marrow. Bone marrow infiltration by the myeloma is 15%.
JEADV 2015, 29, 1641–1657
Letters to the Editor
deposits were found especially perivascular (Fig. 2a). Immunhistochemistry showed AL-amyloid (lambda-chains) in the skin as well as in the bone marrow (Fig. 2b). Thus, Lambda-type amyloidosis associated with myeloma was diagnosed. Because of disease progression the patient received autologous stem cell transplantation and maintenance therapy with follow and concomitant lenalidomid treatment. Amyloidosis is caused by extracellular or rarely intracellular deposition of insoluble fibrillary proteins and eventually leading to organ dysfunction.1 Diagnosis is based on histopathology (e.g. congo red) plus immunohistochemistry. About 30 immunohistochemical different amyloid types exist.2 Our patient displayed amyloidosis of AL-type with underlying lymphoproliferative disease, which is associated in 20–40% of cases with skin manifestations.3 At work up, immunohistochemistry showed AL-amyloid (lambda-chains) in the skin as well as in the bone marrow (Fig. 2b). Nomenclature follows: (i) the progenitor protein; and (ii) clinical presentation (localized vs. systemic, hereditary vs. chronic inflammation vs. tumour associated). In primary localized cutaneous amyloidosis, amyloid is deposited in previously apparently normal skin with no evidence of deposits in any of internal organs (e.g. lichenoid amyloidosis, macular amyloidosis, nodular amyloidosis, familial primary cutaneous amyloidosis).4 Secondary localized cutaneous amyloidosis can develop with, e.g. skin tumours or discoid lupus erythematosus. Macular amyloidosis often presents as light brown patches with focal areas of dark red and brown. Lichen amyloidosis is described as hyperpigmented papules that are located on the shins. Both are associated with pruritus. Nodular amyloid is very rare and is characterized by skin-coloured nodules.5 The risk of progression from nodular to systemic amyloidosis ranges from 7 to 50%.6 Skin may also be involved in systemic amyloidosis. There are three main types of systemic amyloidosis: AL-amyloidosis, AAamyloidosis and TTR-hereditary amyloidosis. AL-amyloidosis occurs in patients with monoclonal gammopathies. AA-amyloidosis may occur in chronic infections or rheumatic or inflammatory diseases, whereas the familial types are autosomal dominant diseases in which a genetically inherited variant form of a normal protein constitutes the amyloid fibril and accounts for
