Eruptive ulcerative follicular spicules heralding progression of smoldering multiple myeloma

Carlo Tomasini, Andrea Michelerio, Valeria Brazzelli

Research output: Contribution to journalArticle

Abstract

Herein, we describe a patient with immunoglobulin G (IgG)-lambda smoldering multiple myeloma with translocation t(4:14) who developed widespread ulcerative horny-like spicules, heralding rapid progression to overt myeloma requiring aggressive chemotherapy and autologous stem cell transplantation. The serum abnormal immunoglobulin in the blood was cryoglobulin, which typically precipitates in the tissues at low temperatures causing inflammation and tissue damage. Histopathological changes, observed in lesions at different evolutionary stages, evidenced columns of horny-like eosinophilic homogeneous material, immunoreactive for IgG lambda, protruding from the dilated and/or distorted follicular openings or acrosyringia and small vessel thrombotic vasculopathy and vasculitis in concert with an inflammatory neutrophilic and lymphocytic reaction. Biochemical investigations on material from a spicule and ulcero-necrotic lesion revealed cryoprecipitates containing IgG-lambda with electrophoretic characteristics identical to those of the serum dysprotein. Our findings suggest that the formation of spicules and development of ulcerative lesions are a part of the same clinical spectrum where the cold-dependent precipitation of the immunogenic dysprotein, both in the skin vessels and hair follicle infundibula, play a major pathogenetic role. Whether this highly characteristic paraneoplastic dermatosis can identify patients with high-risk cytogenetic abnormalities and be incorporated into prognostic models, applicable early on in the course of myeloma, warrants further investigation.

Original languageEnglish
JournalJournal of Cutaneous Pathology
DOIs
Publication statusPublished - Jan 1 2019

Fingerprint

Multiple Myeloma
Immunoglobulin G
Cryoglobulins
Hair Follicle
Stem Cell Transplantation
Pituitary Gland
Vasculitis
Serum
Skin Diseases
Chromosome Aberrations
Immunoglobulins
Inflammation
Drug Therapy
Skin
Temperature

Keywords

  • cryoglobulinemia
  • follicular spicules
  • myeloma
  • skin ulcers: t (4, 14)

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

Cite this

@article{83c7022dba174ac7a9f2500d6c25e420,
title = "Eruptive ulcerative follicular spicules heralding progression of smoldering multiple myeloma",
abstract = "Herein, we describe a patient with immunoglobulin G (IgG)-lambda smoldering multiple myeloma with translocation t(4:14) who developed widespread ulcerative horny-like spicules, heralding rapid progression to overt myeloma requiring aggressive chemotherapy and autologous stem cell transplantation. The serum abnormal immunoglobulin in the blood was cryoglobulin, which typically precipitates in the tissues at low temperatures causing inflammation and tissue damage. Histopathological changes, observed in lesions at different evolutionary stages, evidenced columns of horny-like eosinophilic homogeneous material, immunoreactive for IgG lambda, protruding from the dilated and/or distorted follicular openings or acrosyringia and small vessel thrombotic vasculopathy and vasculitis in concert with an inflammatory neutrophilic and lymphocytic reaction. Biochemical investigations on material from a spicule and ulcero-necrotic lesion revealed cryoprecipitates containing IgG-lambda with electrophoretic characteristics identical to those of the serum dysprotein. Our findings suggest that the formation of spicules and development of ulcerative lesions are a part of the same clinical spectrum where the cold-dependent precipitation of the immunogenic dysprotein, both in the skin vessels and hair follicle infundibula, play a major pathogenetic role. Whether this highly characteristic paraneoplastic dermatosis can identify patients with high-risk cytogenetic abnormalities and be incorporated into prognostic models, applicable early on in the course of myeloma, warrants further investigation.",
keywords = "cryoglobulinemia, follicular spicules, myeloma, skin ulcers: t (4, 14)",
author = "Carlo Tomasini and Andrea Michelerio and Valeria Brazzelli",
year = "2019",
month = "1",
day = "1",
doi = "10.1111/cup.13523",
language = "English",
journal = "Journal of Cutaneous Pathology",
issn = "0303-6987",
publisher = "Wiley-Blackwell",

}

TY - JOUR

T1 - Eruptive ulcerative follicular spicules heralding progression of smoldering multiple myeloma

AU - Tomasini, Carlo

AU - Michelerio, Andrea

AU - Brazzelli, Valeria

PY - 2019/1/1

Y1 - 2019/1/1

N2 - Herein, we describe a patient with immunoglobulin G (IgG)-lambda smoldering multiple myeloma with translocation t(4:14) who developed widespread ulcerative horny-like spicules, heralding rapid progression to overt myeloma requiring aggressive chemotherapy and autologous stem cell transplantation. The serum abnormal immunoglobulin in the blood was cryoglobulin, which typically precipitates in the tissues at low temperatures causing inflammation and tissue damage. Histopathological changes, observed in lesions at different evolutionary stages, evidenced columns of horny-like eosinophilic homogeneous material, immunoreactive for IgG lambda, protruding from the dilated and/or distorted follicular openings or acrosyringia and small vessel thrombotic vasculopathy and vasculitis in concert with an inflammatory neutrophilic and lymphocytic reaction. Biochemical investigations on material from a spicule and ulcero-necrotic lesion revealed cryoprecipitates containing IgG-lambda with electrophoretic characteristics identical to those of the serum dysprotein. Our findings suggest that the formation of spicules and development of ulcerative lesions are a part of the same clinical spectrum where the cold-dependent precipitation of the immunogenic dysprotein, both in the skin vessels and hair follicle infundibula, play a major pathogenetic role. Whether this highly characteristic paraneoplastic dermatosis can identify patients with high-risk cytogenetic abnormalities and be incorporated into prognostic models, applicable early on in the course of myeloma, warrants further investigation.

AB - Herein, we describe a patient with immunoglobulin G (IgG)-lambda smoldering multiple myeloma with translocation t(4:14) who developed widespread ulcerative horny-like spicules, heralding rapid progression to overt myeloma requiring aggressive chemotherapy and autologous stem cell transplantation. The serum abnormal immunoglobulin in the blood was cryoglobulin, which typically precipitates in the tissues at low temperatures causing inflammation and tissue damage. Histopathological changes, observed in lesions at different evolutionary stages, evidenced columns of horny-like eosinophilic homogeneous material, immunoreactive for IgG lambda, protruding from the dilated and/or distorted follicular openings or acrosyringia and small vessel thrombotic vasculopathy and vasculitis in concert with an inflammatory neutrophilic and lymphocytic reaction. Biochemical investigations on material from a spicule and ulcero-necrotic lesion revealed cryoprecipitates containing IgG-lambda with electrophoretic characteristics identical to those of the serum dysprotein. Our findings suggest that the formation of spicules and development of ulcerative lesions are a part of the same clinical spectrum where the cold-dependent precipitation of the immunogenic dysprotein, both in the skin vessels and hair follicle infundibula, play a major pathogenetic role. Whether this highly characteristic paraneoplastic dermatosis can identify patients with high-risk cytogenetic abnormalities and be incorporated into prognostic models, applicable early on in the course of myeloma, warrants further investigation.

KW - cryoglobulinemia

KW - follicular spicules

KW - myeloma

KW - skin ulcers: t (4, 14)

UR - http://www.scopus.com/inward/record.url?scp=85068147375&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85068147375&partnerID=8YFLogxK

U2 - 10.1111/cup.13523

DO - 10.1111/cup.13523

M3 - Article

AN - SCOPUS:85068147375

JO - Journal of Cutaneous Pathology

JF - Journal of Cutaneous Pathology

SN - 0303-6987

ER -