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Cutaneous macroglobulinosis: a case series.

Brendan J Camp¹, Cynthia M Magro

¹Department of Pathology and Laboratory Medicine, Weill Cornell Medical College, New York, NY 10065, USA.

Journal of Cutaneous Pathology

|August 14, 2012

Summary

Cutaneous macroglobulinosis, a rare Waldenstrom macroglobulinemia manifestation, presents as skin papules due to immunoglobulin deposits. This study details three new cases, highlighting clinical and histopathological variations of this uncommon skin condition.

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Area of Science:

Dermatology
Hematology
Pathology

Background:

Cutaneous macroglobulinosis is a rare skin manifestation linked to Waldenstrom macroglobulinemia.
It involves the deposition of immunoglobulin-derived material in the dermis, typically presenting as pink or skin-colored papules.
Only 11 cases have been previously reported in the literature.

Observation:

Three new cases of cutaneous macroglobulinosis were identified in a dermatopathology practice.
Case 1 involved a 41-year-old female with IgM-kappa gammopathy presenting with skin-colored papules.
Cases 2 and 3 involved older females with monoclonal IgM paraproteinemia and Waldenstrom macroglobulinemia, respectively, showing papules, plaques, and ulcerated lesions.

Findings:

Histopathologic and immunohistochemical analyses revealed periodic acid-Schiff (PAS)-positive material staining with IgM in the dermis.
Skin biopsies in Cases 2 and 3 demonstrated vessel occlusion by PAS-positive eosinophilic precipitate.
Direct immunofluorescence and immunohistochemical stains confirmed IgM deposition.

Implications:

This series expands the reported cases of cutaneous macroglobulinosis, contributing to the understanding of its clinical spectrum.
The findings highlight variations in clinical presentation and histopathological features of this rare entity.
Further research is warranted to elucidate the pathogenesis and optimize management strategies for cutaneous macroglobulinosis.