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

Suranagi, Vijayalaxmi Veerabasappa; Siddramappa, BS; Bannur, Hema B; Patil, Pradeep; Davangeri, Reshma

doi:10.4103/0019-5154.147868

Cited by 4 publications

(6 citation statements)

References 22 publications

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“…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.…”

Section: Bullous Amyloidosis (Keratinic Variant)mentioning

confidence: 97%

“…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.…”

Section: Bullous Amyloidosis (Keratinic Variant)mentioning

confidence: 99%

“…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.…”

Section: Bullous Amyloidosis (Keratinic Variant)mentioning

confidence: 99%

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

et al. 2021

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Amyloid is a protein derived from at least 20 different substances. Once misfolded, it results in a group of cutaneous and systemic conditions. Primary localized cutaneous amyloidosis of keratinocyte origin is a very common subtype that can manifest either as lichen or macular amyloidosis, lacking systemic involvement. Lichen amyloidosis often presents as multiple hyperpigmented papules on the lower extremities whereas macular amyloidosis is classically characterized by dark brown rippled macules on the interscapular area. Review of the literature reveals that in addition to the classical presentation of primary localized cutaneous amyloidosis there exists a plethora of various manifestations that can be grouped into either geographic or morphologic categories. This review provides clinicians with the intimate knowledge of these presentations and summarizes the available treatment modalities. Key PointsPrimary localized cutaneous amyloidosis is a frequently encountered skin condition with a heterogeneous spectrum of clinical manifestations.Classifying the clinical spectrum into geographic and morphological categories assists dermatologists in the timely recognition of the correct diagnosis.

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Section: Bullous Amyloidosis (Keratinic Variant)mentioning

confidence: 97%

“…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.…”

Section: Bullous Amyloidosis (Keratinic Variant)mentioning

confidence: 99%

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

et al. 2021

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“…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.…”

Section: Discussionmentioning

confidence: 99%

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

Rosado¹,

Neves²,

Cruz³

et al. 2015

Gal. Clin.

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The nodular primary localized cutaneous amyloidosis is rare and characterized by skin deposition of amyloid substance. There are cases of progression to invasive disease. The type of amyloid substance is not specific to this disease, also being found in primary systemic amyloidosis or systemic amyloidosis associated with multiple myeloma. The authors describe two cases of nodular primary localized cutaneous amyloidosis. A 70-year-old man underwent excision of papule on the mouth left commissure, and a 39-year-old woman with a vulva's labia majora swelling that was excised, in which the histology revealed a nodular amyloidosis, both without systemic involvement. Although the prognosis is benign, the follow-up of the patient is required for early detection of a possible evolution to systemic disease, or exclusion of an underlying systemic amyloidosis.

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“…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.…”

Section: The Diagnosismentioning

confidence: 99%

Asymptomatic Hemorrhagic Lesions in an Anemic Woman

Patel

Gillooly

Brown

et al. 2021

Cutis

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Eligible for 1 MOC SA Credit From the ABD This Photo Challenge in our print edition is eligible for 1 self-assessment credit for Maintenance of Certification from the American Board of Dermatology (ABD). After completing this activity, diplomates can visit the ABD website (http://www.abderm.org) to self-report the credits under the activity title "Cutis Photo Challenge." You may report the credit after each activity is completed or after accumulating multiple credits.A 67-year-old woman with a medical history of type 2 diabetes mellitus, unspecified leukocytosis, and anemia presented to the dermatology clinic with asymptomatic hemorrhagic bullae on the face, chest, and tongue, as well as a large, tender, tense, hemorrhagic bulla on the groin of 3 to 4 months' duration. A review of systems was negative for fever, chills, night sweats, malaise, shortness of breath, and dyspnea on exertion. A complete blood cell count showed mild leukocytosis, anemia, and thrombocytopenia. Her creatinine level was slightly elevated. Chest computed tomography showed early pulmonary fibrosis and coronary artery calcification. An echocardiogram showed diastolic dysfunction with moderate left ventricle thickening. A serum and urine electrophoresis demonstrated elevated free λ light chains with an M-spike. A punch biopsy was performed. WHAT'S YOUR DIAGNOSIS?a. bullous amyloidosis b. bullous drug eruption c. bullous pemphigoid d. epidermolysis bullosa acquisita e. traumatic purpura

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

Cited by 4 publications

References 22 publications

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

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

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

Asymptomatic Hemorrhagic Lesions in an Anemic Woman

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