Excision and split thickness skin grafting in the treatment of nodular primary localized cutaneous amyloidosis

ARTICLE

Auteur(s) : Kresimir BOZIKOV, Tomaz JANEZIC

Department of Plastic Surgery, University Medical Centre, Ljubljana, Slovenia.
<kresimirbozikov@yahoo.com>

Nodular primary localized cutaneous amyloidosis (NPLCA) is the rarest of the cutaneous amyloidoses. The amyloid is derived from immunoglobulin light chains (AL protein) produced by local plasma cells [1]. Histological findings are indistinguishable from those of primary systemic amyloidosis, so additional clinical and laboratory evaluation must be performed, and careful follow up is recommended. There is no consistent treatment for NPLCA, particularly when large areas are involved, and local recurrence rate is high [2].
A 68-year-old Caucasian woman was referred for possible surgical treatment to our department in 1996. For the preceding three years she had had skin nodules on the anterior tibial region of her right leg, gradually increasing in size and number. The area of the skin involved measured 23 × 15 centimetres. The nodules were painful and frequently ulcerated and infected, causing the patient distress. Physical examination revealed five firm, non fixed, ovoid, shiny, brown-pink nodules on the anterior tibial region of right leg, which ranged in size up to 5 cm in diameter and which were surrounded by healthy skin. The overlying skin on three of the nodules was ulcerated.
One of the ulcerated lesions was surgically treated with excision and split thickness skin grafting, under local anaesthesia. Microscopic examination revealed large deposits of amphophilic to eosinophilic amorphous material in the dermis and subcutaneous tissue. The material stained positively with Congo red and demonstrated apple-green birefringence with polarizing microscopy. The immunohistochemical studies revealed that the amyloid proteins were of κ light chain origin. Additional extensive clinical and laboratory evaluation by a rheumatologist revealed no systemic involvement. The patient was diagnosed as having NPLCA.
The patient presented again at the beginning of 1999. There were no new lesions. Previous non-excised nodules had increased in size and were up to 7 centimetres in diameter, and the skin over several nodules was ulcerated and painful (figure 1A).
Total excision of all involved skin and subcutaneous tissue over the anteromedial side of right shin was performed under regional anaesthesia. Increased friability of clinically changed tissue was experienced during the operation (figure 1B). Altogether the disease involved 3% of the patient’s total body surface area. The soft tissue defect was covered with split thickness skin grafts meshed 1 to 1.5, taken from the left thigh. After one week 60% of the skin grafts were healed. The wounds completely healed in one month. A six year follow up shows no signs of local recurrence of the disease or systemic progression (figure 1C).

Discussion

There are three main forms of localized cutaneous amyloidosis: lichen and macular amyloidosis, which are felt to originate from keratin filament degeneration, and nodular amyloidosis which may represent a localized plasma cell dyscrasia that can be associated with a monoclonal gammopathy or multiple myeloma [1]. Therefore careful follow up is recommended for asymptomatic lesions of nodular amyloidosis [3].
Progression to systemic disease is unlikely and happens in only 7% of cases [2]. Nodules can present anywhere on the skin, vary in size from a few millimetres to a few centimetres and are usually asymptomatic and tend not to ulcerate.
Numerous surgical procedures have been employed to treat lesions of nodular amyloidosis [3-6].
Larger areas affected by NPLCA are rare and are usually treated conservatively [2]. Persistent painful ulcerated nodules with the threat of infection were the reason for a radical surgical procedure in the case presented, with acceptable results and no recurrence during long term follow up.
Extensive NPLCA of the extremities can be successfully treated with total excision of involved tissue and split thickness skin grafting, to achieve good local control of disease and a satisfactory cosmetic result. n

1. Touart DM, Sau P. Cutaneous deposition diseases. Part I. J Am Acad Dermatol 1998; 39(2 Pt 1): 149-71.

2. Woollons A, Black MM. Nodular localized primary cutaneous amyloidosis: A long term follow up study. Br J Dermatol 2001; 145(1): 105-9.

3. Truhan AP, Garden JM, Roenigk HH Jr. Nodular primary localized cutaneous amyloidosis: immunohistochemical evaluation and treatment with carbon dioxide laser. J Am Acad Dermatol 1986; 14: 1058-62.

4. Hamzavi I, Lui H. Excess tissue friability during CO2 laser vaporization of nodular amyloidosis. Dermatol Surg 1999; 25(9): 726-8.

5. Lien MH, Railan D, Nelson BR. The efficacy of dermabrasion in the treatment of nodular amyloidosis. J Am Acad Dermatol 1997; 36(2 Pt 2): 315-6.

6. Vestey JP, Tidman MJ, Mclaren KM. Primary nodular cutaneous amyloidosis: long term follow up and treatment. Clin Exp Dermatol 1994; 19(2): 159-62.