Bullous variant of familial biphasic lichen amyloidosis: A unique combination of three rare presentations

Abstract

A 55-year-old man presented with multiple, itchy papules and macules on the trunk and extremities. Histopathologic examination of biopsy specimens taken from three different lesions showed a subepidermal blister with amyloid deposits in the dermal papillae. No systemic disease or involvement of other organs was detected. The clinical and histological findings were compatible with a bullous variant of lichen amyloidosis (LA). Primary cutaneous localized amyloidosis usually presents with papular, macular or nodular lesions. Bullous lesions associated with LA are very rare. Furthermore, patient had seven other members in the family with similar lesions, which is also a rare occurrence. We report a case with a rare combination of biphasic, bullous variant of familial LA.

Nodular primary localized cutaneous amyloidosis: presentation of 2 case reports and literature review

“…The localized cutaneous amyloidosis is a rare type of amyloidosis in which extracellular amyloid substance deposits are located only in the skin, and includes three variants: lichenoid, macular and nodular amyloidosis. 6,8,9,10 The lichenoid and the macular amyloidosis are often grouped together, since both have constituents derived from keratin on amyloid deposits, that do not invade the blood vessels and do not extend below the papillary dermis. 6,8 The nodular amyloidosis is the rarest variant, 5,6,8,9 its amyloid deposits are formed by immunoglobulin light chains (AL amyloid | 171 | substance type) 6,8,9 produced by local plasma cells, 6,11 infiltrating the entire dermis, the subcutaneous layer and the blood vessel wall.…”

Primary Localized Cutaneous Amyloidosis of Keratinocyte Origin: An Update with Emphasis on Atypical Clinical Variants

“…Based on the clinicopathological findings, a diagnosis of lichen amyloidosis was established. Note that familial forms of bullous amyloidosis were observed emphasizing the importance of genetic factors in the pathogenesis of PLCA [37]. Bullous amyloidosis without systemic involvement is a rare entity.…”

“…A bullous variant of lichen amyloidosis has been described in a few case reports [36][37][38]. Cases presented with pruritic lichenoid papules and vesicles.…”

“…Bullous amyloidosis without systemic involvement is a rare entity. Actually, most cases (88%) [38] described as bullous amyloidosis were later diagnosed as systemic amyloidosis [37]; therefore, careful attention should be given to patients presenting with bullous lesions.…”

Asymptomatic Hemorrhagic Lesions in an Anemic Woman

“…1 Some cases have reported a familial linkage, suggesting there might be a genetic component to the disease. 4 A PubMed search of articles indexed for MEDLINE using the terms bullous amyloidosis, bullous, amyloidosis, and amyloid revealed fewer than 35 cases of bullous amyloidosis in the English-language literature. 5 Bullae can be located intradermally or subepidermally and commonly are hemorrhagic but also can be translucent, tender, and tense.…”