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Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis.
J Cutan Pathol. 2010 Jan; 37(1):15-9.JC

Abstract

OBJECTIVES

To determine (i) whether primary (idiopathic) follicular mucinosis (PFM) and lymphoma-associated follicular mucinosis (LAFM) are distinct or related entities and whether there are reliable criteria that allow the two forms to be distinguished, (ii) the histochemical properties and consequently the type of mucin that accumulates in the follicle in PFM and LAFM, and (iii) whether there is any difference between the staining properties of mucin in patients with PFM and LAFM.

METHODS

Thirty-one patients were divided into two groups. Group 1 comprised 20 patients with no associated mycosis fungoides or Sézary syndrome (PFM) and group 2 was made up of the other 11 patients who had clinicopathological evidence of cutaneous T-cell lymphoma (LAFM). The biopsy specimens of the patients were studied with histopathological, histochemical and immunohistochemical methods. Molecular biology studies were also performed.

RESULTS

Patients with PFM were more frequently younger (mean age 39 years), women (F:M=3:1), and presented with a solitary lesion involving the head/neck area more often than patients with LAFM who were older (mean age 54 years), men (M:F=2:1), and presented with multiple lesions on areas of the body other than the head/neck area. As for histopathological findings, large cystic spaces filled with mucin and a slight perivascular and periadnexal polyclonal infiltrate of mostly non-atypical lymphocytes without epidermotropism and with an equivalent CD4+/CD8+ cell rate were more suggestive of PFM. On the contrary, patients with LAFM were more probably to present with a dense band-like infiltrate with some atypical lymphocytes and sign of epidermotropism, a prominent CD4+ immunophenotype and a monoclonal rearrangement of the infiltrate. Mucin proved to be a dermal-type mucin, composed of both hyaluronic acid and sulfated glycosaminoglycans. No differences were found in the composition of the follicular mucin in the PFM compared with LAFM.

CONCLUSIONS

Although no single, indisputable feature can reliably differentiate PFM from LAFM and a considerable overlapping among the two groups exists, the use of multiple clinical, histological and immunopathological criteria associated with gene rearrangement analysis can be useful in evaluation of those patients.

Authors+Show Affiliations

Section of Dermatology, DISEM, University of Genoa, Genoa, Italy. franco.rongioletti@unige.itNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Comparative Study
Journal Article

Language

eng

PubMed ID

19615011

Citation

Rongioletti, Franco, et al. "Follicular Mucinosis: a Clinicopathologic, Histochemical, Immunohistochemical and Molecular Study Comparing the Primary Benign Form and the Mycosis Fungoides-associated Follicular Mucinosis." Journal of Cutaneous Pathology, vol. 37, no. 1, 2010, pp. 15-9.
Rongioletti F, De Lucchi S, Meyes D, et al. Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis. J Cutan Pathol. 2010;37(1):15-9.
Rongioletti, F., De Lucchi, S., Meyes, D., Mora, M., Rebora, A., Zupo, S., Cerruti, G., & Patterson, J. W. (2010). Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis. Journal of Cutaneous Pathology, 37(1), 15-9. https://doi.org/10.1111/j.1600-0560.2009.01338.x
Rongioletti F, et al. Follicular Mucinosis: a Clinicopathologic, Histochemical, Immunohistochemical and Molecular Study Comparing the Primary Benign Form and the Mycosis Fungoides-associated Follicular Mucinosis. J Cutan Pathol. 2010;37(1):15-9. PubMed PMID: 19615011.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Follicular mucinosis: a clinicopathologic, histochemical, immunohistochemical and molecular study comparing the primary benign form and the mycosis fungoides-associated follicular mucinosis. AU - Rongioletti,Franco, AU - De Lucchi,Simona, AU - Meyes,Dan, AU - Mora,Marco, AU - Rebora,Alfredo, AU - Zupo,Simonetta, AU - Cerruti,Giannamaria, AU - Patterson,James W, Y1 - 2009/07/13/ PY - 2009/7/21/entrez PY - 2009/7/21/pubmed PY - 2011/7/13/medline SP - 15 EP - 9 JF - Journal of cutaneous pathology JO - J Cutan Pathol VL - 37 IS - 1 N2 - OBJECTIVES: To determine (i) whether primary (idiopathic) follicular mucinosis (PFM) and lymphoma-associated follicular mucinosis (LAFM) are distinct or related entities and whether there are reliable criteria that allow the two forms to be distinguished, (ii) the histochemical properties and consequently the type of mucin that accumulates in the follicle in PFM and LAFM, and (iii) whether there is any difference between the staining properties of mucin in patients with PFM and LAFM. METHODS: Thirty-one patients were divided into two groups. Group 1 comprised 20 patients with no associated mycosis fungoides or Sézary syndrome (PFM) and group 2 was made up of the other 11 patients who had clinicopathological evidence of cutaneous T-cell lymphoma (LAFM). The biopsy specimens of the patients were studied with histopathological, histochemical and immunohistochemical methods. Molecular biology studies were also performed. RESULTS: Patients with PFM were more frequently younger (mean age 39 years), women (F:M=3:1), and presented with a solitary lesion involving the head/neck area more often than patients with LAFM who were older (mean age 54 years), men (M:F=2:1), and presented with multiple lesions on areas of the body other than the head/neck area. As for histopathological findings, large cystic spaces filled with mucin and a slight perivascular and periadnexal polyclonal infiltrate of mostly non-atypical lymphocytes without epidermotropism and with an equivalent CD4+/CD8+ cell rate were more suggestive of PFM. On the contrary, patients with LAFM were more probably to present with a dense band-like infiltrate with some atypical lymphocytes and sign of epidermotropism, a prominent CD4+ immunophenotype and a monoclonal rearrangement of the infiltrate. Mucin proved to be a dermal-type mucin, composed of both hyaluronic acid and sulfated glycosaminoglycans. No differences were found in the composition of the follicular mucin in the PFM compared with LAFM. CONCLUSIONS: Although no single, indisputable feature can reliably differentiate PFM from LAFM and a considerable overlapping among the two groups exists, the use of multiple clinical, histological and immunopathological criteria associated with gene rearrangement analysis can be useful in evaluation of those patients. SN - 1600-0560 UR - https://www.unboundmedicine.com/medline/citation/19615011/Follicular_mucinosis:_a_clinicopathologic_histochemical_immunohistochemical_and_molecular_study_comparing_the_primary_benign_form_and_the_mycosis_fungoides_associated_follicular_mucinosis_ L2 - https://doi.org/10.1111/j.1600-0560.2009.01338.x DB - PRIME DP - Unbound Medicine ER -