Cutaneous macroglobulinosis. A case report with unique ultrastructural findings

Lowe, Lori; Je, Fitzpatrick; Jc, Huff; Pf, Shanley; Le, Golitz

doi:10.1001/archderm.128.3.377

Cited by 18 publications

(16 citation statements)

References 22 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…The microscopic differential diagnosis of cutaneous macroglobulinosis includes conditions characterized by the deposition of eosinophilic, amorphous material in the dermis. 6 In addition, as shown by the pathologic findings in this and prior reports, monoclonal cryoglobulin vasculopathy should also be considered. 4 Lipoid proteinosis is a rare autosomal-recessive deposition disorder due to mutations in the extracellular matrix protein 1 (ECM1) gene that manifests clinically with facial papules, nodules and pitted scars and verrucous lesions favoring the elbows, knees and hands.…”

Section: Discussionmentioning

confidence: 69%

Cutaneous macroglobulinosis: a case series

Camp

Magro

2012

J Cutan Pathol

View full text Add to dashboard Cite

Cutaneous macroglobulinosis is a rare skin manifestation of Waldenstrom macroglobulinemia. It is characterized by the deposition of eosinophilic, immunoglobulin‐derived material in the dermis. It typically presents as pink or skin‐colored papules favoring the extensor surfaces of the extremities. There are 11 reported cases of cutaneous macroglobulinosis in the literature. In our consultative dermatopathology practice we encountered three additional cases. In Case 1, a 41‐year‐old female with a monoclonal immunoglobulin M (IgM)‐kappa gammopathy developed skin‐colored papules on her extensor extremities, forehead and back. Histopathologic and immunohistochemical analyses revealed periodic acid‐Schiff (PAS)‐positive pink material in the dermis that stained with IgM. In Case 2, an 83‐year‐old female with a monoclonal IgM‐lambda paraproteinemia developed non‐blanching papules and plaques on the lower extremities and cheeks. Skin biopsies showed a striking occlusion of the vessels with a PAS‐positive eosinophilic precipitate, which was also found in the extravascular spaces and stained with IgM using direct immunofluorescence (DIF) and immunohistochemical stains. In Case 3, an 80‐year‐old male with Waldenstrom macroglobulinemia developed ulcerated papules and nodules on the lower extremities. The biopsy findings were similar to those of Case 2. We present a series of three patients with cutaneous macroglobulinosis and explore variations in the clinical and histopathological findings of this uncommon entity.

show abstract

Section: Discussionmentioning

confidence: 69%

Cutaneous macroglobulinosis: a case series

Camp

Magro

2012

J Cutan Pathol

View full text Add to dashboard Cite

show abstract

“…Direct immunofluorescence shows strong stain for immunoglobulin M. Ultrastructurally, the deposits are composed of amorphous or granular and sometimes filamentous material without periodicity of amyloid. 19 In summary, we present a rare case of primary localized cutaneous nodular amyloidosis with a unique histological picture of amyloid elastosis, a rare disease described only in patients with primary systemic amyloidosis.…”

Section: Discussionmentioning

confidence: 88%

Primary Cutaneous Localized Amyloid Elastosis

Santos‐Briz¹,

Cañueto²,

Antunez³

et al. 2010

The American Journal of Dermatopathology

View full text Add to dashboard Cite

show abstract

“…A variety of skin manifestations have been observed in association with IgM antibodies reacting to the BMZ (summarized in Table 1). IgM deposits have been demonstrated in the pruritic papular skin lesions in rare cases of Waldenstrom's macroglobulinaemia (10)(11)(12)(13)(14). This eruption is called an IgM storage papule or cutaneous macroglobulinosis, which is histologically characterized by a massive dermal collection of amorphous, eosinophilic and fissured material.…”

Section: Discussionmentioning

confidence: 99%

A Case of Acquired Autoimmune Bullous Disease Associated with IgM Macroglobulinaemia

Morita

Horiuchi

Yamamoto

et al. 1999

The Journal of Dermatology

View full text Add to dashboard Cite

A variety of autoimmune bullous dermatoses have been reported to develop in association with lymphoproliferative disorders. We report a patient with IgM macroglobulinaemia, who presented with a skin fragility similar to but somewhat milder than that seen in epidermolysis bullosa acquisita. Immunofluorescence detected circulating IgM autoantibodies reacting with the basement membrane zone, which reacted predominantly with dermal side of 1 M NaCl-split skin. Immunoblotting of the epidermal and dermal extracts with the patient's serum showed no specific reactivity. Further studies are needed to identify the antigenic molecules responsible for the IgM deposition.

show abstract

Cutaneous macroglobulinosis. A case report with unique ultrastructural findings

Cited by 18 publications

References 22 publications

Cutaneous macroglobulinosis: a case series

Cutaneous macroglobulinosis: a case series

Primary Cutaneous Localized Amyloid Elastosis

A Case of Acquired Autoimmune Bullous Disease Associated with IgM Macroglobulinaemia

Contact Info

Product

Resources

About