Two sporadic cases of adult-onset progressive mucinous histiocytosis

A. Young*, J. Olivere, S. Yoo, C. Martins, T. Barrett

*Corresponding author for this work

Research output: Contribution to journalArticle

12 Citations (Scopus)

Abstract

Progressive mucinous histiocytosis is a rare, benign, non-Langerhans' cell histiocytosis limited to the skin. Ten cases - all women - in four families and one sporadic case have been described in the literature. The disorder usually begins in childhood and progresses slowly. We report two sporadic cases of adult-onset progressive mucinous histiocytosis in unrelated African-American women, aged 48 and 55 years, respectively, who developed red-brown and flesh-coloured, asymptomatic papules on the face, the arms and the legs without truncal, mucosal or visceral involvement. The lesions showed no spontaneous regression. Both patients lacked associated systemic symptoms, including polyuria, polydipsia or seizures. There was no underlying hyperlipidaemia, paraproteinaemia or lymphoproliferative disease. No family history of similar lesions could be identified. Light microscopy revealed dermal proliferation of spindle-shaped histiocytes with abundant mucin deposition. Electron microscopy demonstrated a high number of myelin figures or zebra bodies in the cytoplasm of histiocytes. On immunohistochemistry, positive staining with macrophage markers - CD68, HAM56 and lysozyme - and factor XIIIa, a transglutaminase present in dermal dendrocytes, and negative staining with Langerhans' cell markers - CD1a and S100 - and CD34, a marker present in dermal dendritic cells derived from uncommitted mesenchymal cells, were observed.

Original languageEnglish (US)
Pages (from-to)166-170
Number of pages5
JournalJournal of Cutaneous Pathology
Volume33
Issue number2
DOIs
StatePublished - Feb 1 2006

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Histiocytes
Langerhans Cells
Skin
Non-Langerhans-Cell Histiocytosis
Factor XIIIa
Polydipsia
Polyuria
Negative Staining
Paraproteinemias
Transglutaminases
Equidae
Mucins
Muramidase
Myelin Sheath
Hyperlipidemias
African Americans
Microscopy
Leg
Electron Microscopy
Cytoplasm

ASJC Scopus subject areas

  • Pathology and Forensic Medicine
  • Histology
  • Dermatology

Cite this

Young, A. ; Olivere, J. ; Yoo, S. ; Martins, C. ; Barrett, T. / Two sporadic cases of adult-onset progressive mucinous histiocytosis. In: Journal of Cutaneous Pathology. 2006 ; Vol. 33, No. 2. pp. 166-170.
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Two sporadic cases of adult-onset progressive mucinous histiocytosis. / Young, A.; Olivere, J.; Yoo, S.; Martins, C.; Barrett, T.

In: Journal of Cutaneous Pathology, Vol. 33, No. 2, 01.02.2006, p. 166-170.

Research output: Contribution to journalArticle

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