Cutaneous Amyloidoma: A Rare Case Report.

Dear Editor:

Amyloidosis comprises a group of diseases characterized by extracellular deposition of unique insoluble, fibrillar, proteinaceous material. On light microscopic examination, amyloid appears as an eosinophilic amorphous hyaline extracellular substance, which turns green-yellow after Congo-Red staining and examination under polarized light. Amyloidoma is defined as primary solitary amyloidosis in which no plasma-cell dyscrasia or abnormal serum proteins are detectable. We here in report an unusual case of skin amyloidoma.

A 65-year-old woman presented to Erciyes Universtiy Medical Faculty Dermatology Department Outpatient Clinic with two masses located on the right chest under the mammarian area. The lesions had grown in size over the past 5 years. The lesion had become ulcerated over the last month. Two biopsies had been performed previously (1 year and three years ago). Both of them were reported as squamous epitheliomatous hyperplasia. She had been diagnosed as having Sjögren syndrome 11 years ago. On examination there were two skin-colored firm nodules with a smooth surface, one of them had a hemorrhagic border and the other was ulcerated. Their measurements were found as approximately 2×2.5 cm and 3×3 cm (Fig. 1). Histologic examination revealed homogenization of the dermal connective tissue with deposits of amorphous material (Fig. 2A, B). Extensive amyloid deposits in the entire dermis were positive with Congo-red staining (Fig. 2C) with the characteristic apple-green birefringence under polarized light. The amyloid type was determined immunohistochemically as AA (secondary) amyloid (clone mc1; Dako) (Fig. 2D).

Fig. 1

Two skin-colored firm nodules with a smooth surface, one of them had a hemoragic border and the other was ulcerated.

Fig. 2

(A, B) Dermal connective tissue with deposits of amorphous material (H&E). (C) Extensive amyloid deposits in the entire dermis were positive with Congo-red staining. (D) The amyloid type was determined immunohistochemically as AA amyloid. (E) Axial computed tomography (CT) scan at the soft tissue window shows skin lesion (arrow). (F) Axial CT scan at the lung window shows cluster of multiple small nodules in the right lower lobe (circle).

The patient underwent further investigations for systemic amyloidosis. Axial computed tomography scan at the lung window shows cluster of multiple small nodules in the right lower lobe and at the soft tissue window showed skin lesion were suggestive of amyloidosis (Fig. 2E, F). Echocardiography, ultrasound of the abdomen, pelvis and lymph nodes of the neck, axilla and groin showed no signs of systemic amyloidosis. Blood analyses including immunoglobulins and immunoelectrophoresis were unremarkable. The patient was consultated with the pulmonary medicine department. The patient is currently being followed up for systemic amyloidosis.

Amyloidoma is defined as primary solitary amyloidosis1. It has been described in widely varied anatomic sites including the genitourinary tract, breast, respiratory tract, nasal sinuses, mediastinum, gastrointestinal tract, retroperitoneum, mesentery, eye, bone and joints, lymph node, spleen and parotid gland. Amyloidoma of the skin is a very rare condition, and only a few cases have been described in the literature so far23. We determined the AA type of amyloidosis. Sjögren syndrome was described with paranasal4 and pulmonary amyloidoma5 previously to the best of our knowledge AA type skin amyloidoma with Sjögren syndrome and with pulmonary involvement has not been reported before in the literature. We believe that amyloidoma might have been presented as a complication of Sjögren syndrome in this patient. Although local resection is curative for amyloidoma, patients must be evaluated for systemic involvement of amylodosis.