Primary cutaneous amyloidosis involving the external ears along with the classical sites

Introduction

Primary cutaneous amyloidosis manifests in various clinical forms. Macular, papular / lichenoid or a combination of the two are the common pre-sentation. Other rare forms are nodular or tumefactive, poikiloderma- like, bullous and vitiligi-nous.[1] Apart from the morphological variations, there are variations in the sites involved. The classical sites for lichen amyloidosis are shins, forearms and arms and for macular amyloidosis the upper back and upper arms.[1] We report lesions of amyloidosis in-volving both ears along with the lesions on the fore-arms and the shins in a young woman.

Case Report

A 26- year- old woman had multiple itchy persistent papules on the forearms that developed 10 years back. Four years later she noticed similar lesions on the shins. This was followed by appearance of mildly itchy papular lesions symmetrically on both the ear lobes. There were no systemic or con-stitutional, symptoms.

Exami-nation revealed multiple, monomorphic, discrete hyper-pigmented, firm, 2-3 mm p a pules localised on the extensor as-pects of both forearms [Figure - 1] and both legs (shins). Similar lesions were present inside the con-cha and the outer surface of upper part of pinna of both the ears. The other areas of the body, mucosae and scalp were normal. Examination of other sys-tems revealed no abnormalities. Investigations re-vealed haemoglobin -12 gm, total leucocyte count - 8000/mm3 with 64% neutrophils, 33% lymphocytes and 3% eosinophils. The ESR was 44 mm in first hour. Biochemical tests for renal and liver functions were within normal limits. Skin biopsy [Figure - 2] taken from the forearm skin as well as the ear lesions showed hyperkeratosis with mild acanthosis and amorphous hyalinized material in multiple adja-cent papillae. The stain with Congo red showed orange- red aggregated fibrillar material in the papillae un-der light microscopy and a bright green birefringence on polarising.

The patient was treated with cyclophosphamide 50 mg and cetirizine 10 mg daily orally along with topical clobetasol propionate 0.05% twice daily. Within 3 months, further progression of the disease was arrested along with reduction in the size of the lesions and significant relief in pruri-tus. Thereafter cetirizine was needed only occasion-ally. After 6 months of therapy there was more than 50% improvement in the extent of involvement and almost complete control of pruritus. No side- effects were observed with this treatment.

Discussion

The sites commonly involved in lichen amy-loidosis are pretibial areas, thighs, calves, ankles and dorsa of feet, while macular amyloidosis occurs mainly on the upper back (scapular and interscapular ar-eas), neck, clavicular areas and also the arms, fore-arms, thighs and shins.[1] In extensive cases, involve-ment of the face, chest, abdomen and anosacral region has also been recorded. The involvement of auricular concha in primary cutaneous amyloidosis is very rare. Perhaps, the first case in English litera-ture was reported in 1983 by Sanchez, as collag-enous papules on the aural conchae which were ac-tually primary cutaneous amyloidosis. Dupre et al[3] reported another case of aural lesions in 1984 where a similar case by Mascaro in Spanish literature was also referred. Hickes et al,[4] reported four such cases in 1988. In all these cases the amyloidosis was confined to ears and the other sites were uninvolved. However, in our case there was involvement of the forearms and shins along with the ex-ternal ears. The lesions were in au-ricular concha as well as on outer surface of pinna. This rare presenta-tion suggests that pathogenetically the process may be same but the in-volvement of various sites in one in-dividual and the variations in the dis-tribution in different individuals may possibly be because of the triggers pertaining to the affected sites or some kind of constitutional predis-position for these sites.