Lichen amyloidosus with subepidermal blister formation

ARTICLE

Auteur(s) : Kei KURODA¹, Masako MIZOGUCHI²

¹ Department of Dermatology, National Defence Medical College, 3-2 Namiki, Tokorozawa 359-8513, Japan
² Department of Dermatology, Shimotsuga General Hospital, Tochigi, Japan

Article accepted on 26/01/2004

Lichen amyloidosus (LA) is a primary localized cutaneous amyloidosis characterized clinically by a persistent, pruritic eruption of multiple discrete hyperkeratotic papules and histologically by amyloid deposits confined to the papillary dermis [1]. Lesions are most commonly located on the legs, and there may be spread to the trunk or upper extremities. Although bullous amyloidosis has been reported in systemic amyloidosis, bullous lesions associated with LA are very rare [2, 3]. In this report, we describe a case of LA with subepidermal blister formation.

Case report

A 74-year-old Japanese man presented with a 10-year history of persistent pruritic eruptions. Physical examination revealed multiple, discrete, keratotic papules on the trunk and extremities. Papules coalesced to form thickened plaques on the shins, the extensor aspect of the thighs, buttocks, and forearms (Fig. 1A). Erosions and vesicles with hyperkeratosis were intermingled on plaque lesions on the extremities. Crusted or erosive, reddish papules were disseminated on the trunk (Fig. 1B). Depigmented patches were also found. No oral lesions were present. His family members were unaffected. There was no other medical history of note. The family and personal history disclosed no features of atopy. Laboratory study disclosed an elevated level of serum IgE (1,500 IU/ml, normal values < 250 IU/ml). No specific IgE antibodies to common environmental and food antigens were detected. Other laboratory examinations, including complete blood count, blood chemistry profile, antinuclear antibodies, blood and urine porphyrins, serum immunoglobulins (IgA, IgG, and IgM) and Bence Jones protein were normal or negative. Histopathologic examination of skin biopsy specimens taken from the forearm, the upper back, and the lower leg showed a subepidermal blister with irregular acanthosis and hyperkeratosis of the overlying epidermis. A few eosinophils were found in the blister. A moderate cellular infiltrate composed of lymphocytes and eosinophils was present in the upper dermis. Amyloid deposits were visible in the dermal papillae on both dylon [4] and Congo red stainings but not around blood vessels or adnexal structures (Fig. 2). Direct immunofluorescence testing showed no staining of immunoglobulins or complement along the epidermal basement zone. An indirect immunofluorescence on 1 M NaCl-split normal skin using the patient’s serum gave negative results. Potent topical steroid had a mild effect on trunk lesions, although lesions on the extremities responded poorly.

Discussion

LA is characterized by closely set, discrete, pigmented papules, which may coalesce into thickened plaques. Besides the typical features of a classc LA, our case showed bullous lesions which histologically demonstrated subepidermal blisters. Due to marked hyperkeratosis, some bullous lesions were not clinically distinct. Although the clinical differential diagnosis includes bullous pemphigoid, epidermolysis bullosa dystrophica, epidermolysis bullosa acquisita, and the porphyrias, our investigations excluded these conditions.
In our case, crusted or erosive papules were somewhat reminiscent of chronic prurigo, although histological studies clearly demonstrated amyloid deposits and a subepidermal blister. LA may closely simulate chronic prurigo or prurigo nodularis [5] and is occasionally associated with pruritic diseases such as atopic dermatitis and venous insufficiency [6, 7]. Although our case showed marked elevated serum IgE levels and tissue eosinophil infiltrates, other findings of atopic dermatitis including typical cutaneous lesions and family history of atopy were not present. We cannot state whether IgE-elevation and eosinophil infiltrate are associated with amyloidosis or blister formation. Previously, a case of bullous amyloidosis with elevated serum IgE has been reported [8].
Almost all cases previously described as bullous amyloidosis are systemic amyloidosis [2]. So far three cases without systemic involvement have been reported [3, 8, 9]. Two of the three cases seem to have no hyperkeratotic papules or plaques of LA [8, 9]. In this respect, our patient may resemble the case described as a bullous variant of LA by Khoo and Tay [3].
The pathogenesis of LA includes friction, genetic predisposition, and environmental factors [1]. Recent studies show that chronic scratching and Epstein-Barr virus infection is responsible for the formation of LA [10, 11]. Although the exact mechanism in which bullae are formed remains unknown, rubbing or scratching induced by persistent pruritus appears to act as the localized precipitating factor of blister formation in our case. n

References

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