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Follicular lymphomatoid papulosis revisited: a study of 11 cases, with new histopathological findings.
J Am Acad Dermatol. 2013 May; 68(5):809-16.JA

Abstract

BACKGROUND

Follicular lymphomatoid papulosis (LyP) describes a variant of LyP with perifollicular infiltrates and some degree of folliculotropism of CD30(+) atypical lymphocytes. So far, only a few cases of follicular LyP have been described.

OBJECTIVE

Our goal was to study the clinicopathologic features of follicular LyP in a series of 11 cases (9 male, 2 female; age range 7-78 years, mean age 50 years).

METHODS

In all, 113 cases of LyP were reviewed to select cases showing follicular involvement. Histology was correlated with the clinical data to exclude cases of CD30(+) anaplastic large-cell lymphoma or folliculotropic mycosis fungoides.

RESULTS

Six cases were classified as type C and 4 as type A, whereas the remaining case manifested epidermotropism of small lymphocytes in a background of a typical type A lesion (overlapping type A/B). Perifollicular infiltrates of CD30(+) atypical lymphoid cells were seen in all 11 cases, with infiltration of the follicular epithelium in 8 cases. Hyperplasia of the follicular epithelium was observed in 4 cases; ruptured hair follicles, in 3 cases; and follicular mucinosis, in 2 cases. In addition to hair follicle infiltration, atypical cells were recognized within sebaceous glands in 2 lesions. New findings were presence of numerous intrafollicular neutrophils in 2 patients, who clinically had pustules in addition to papules. Other histopathological features encountered included perieccrine infiltration (n = 5), focal subcutaneous involvement (n = 4), granulomatous inflammation (n = 3), epidermal hyperplasia (n = 2), and 1 each of infiltration of muscle bundles, numerous eosinophils in the infiltrate, and angiocentricity.

LIMITATIONS

This was a retrospective study.

CONCLUSIONS

Follicular LyP is a variant of LyP with involvement of hair follicles, mostly in the form of perifollicular infiltrate with variable degree of folliculotropism. Other changes including hyperplasia of the follicular epithelium, rupture of hair follicle, and follicular mucinosis are less common. Rarely, intrafollicular pustules can be seen in the follicular epithelium; such lesions manifest clinically as pustules.

Authors+Show Affiliations

Kempf und Pfaltz, Histologische Diagnostik, Zürich, Switzerland. kempf@kempf-pfaltz.chNo affiliation info availableNo affiliation info availableNo affiliation info available

Pub Type(s)

Case Reports
Journal Article

Language

eng

PubMed ID

23375516

Citation

Kempf, Werner, et al. "Follicular Lymphomatoid Papulosis Revisited: a Study of 11 Cases, With New Histopathological Findings." Journal of the American Academy of Dermatology, vol. 68, no. 5, 2013, pp. 809-16.
Kempf W, Kazakov DV, Baumgartner HP, et al. Follicular lymphomatoid papulosis revisited: a study of 11 cases, with new histopathological findings. J Am Acad Dermatol. 2013;68(5):809-16.
Kempf, W., Kazakov, D. V., Baumgartner, H. P., & Kutzner, H. (2013). Follicular lymphomatoid papulosis revisited: a study of 11 cases, with new histopathological findings. Journal of the American Academy of Dermatology, 68(5), 809-16. https://doi.org/10.1016/j.jaad.2012.12.952
Kempf W, et al. Follicular Lymphomatoid Papulosis Revisited: a Study of 11 Cases, With New Histopathological Findings. J Am Acad Dermatol. 2013;68(5):809-16. PubMed PMID: 23375516.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Follicular lymphomatoid papulosis revisited: a study of 11 cases, with new histopathological findings. AU - Kempf,Werner, AU - Kazakov,Dmitry V, AU - Baumgartner,Hans-Peter, AU - Kutzner,Heinz, Y1 - 2013/01/30/ PY - 2012/07/24/received PY - 2012/11/03/revised PY - 2012/12/03/accepted PY - 2013/2/5/entrez PY - 2013/2/5/pubmed PY - 2013/6/28/medline SP - 809 EP - 16 JF - Journal of the American Academy of Dermatology JO - J Am Acad Dermatol VL - 68 IS - 5 N2 - BACKGROUND: Follicular lymphomatoid papulosis (LyP) describes a variant of LyP with perifollicular infiltrates and some degree of folliculotropism of CD30(+) atypical lymphocytes. So far, only a few cases of follicular LyP have been described. OBJECTIVE: Our goal was to study the clinicopathologic features of follicular LyP in a series of 11 cases (9 male, 2 female; age range 7-78 years, mean age 50 years). METHODS: In all, 113 cases of LyP were reviewed to select cases showing follicular involvement. Histology was correlated with the clinical data to exclude cases of CD30(+) anaplastic large-cell lymphoma or folliculotropic mycosis fungoides. RESULTS: Six cases were classified as type C and 4 as type A, whereas the remaining case manifested epidermotropism of small lymphocytes in a background of a typical type A lesion (overlapping type A/B). Perifollicular infiltrates of CD30(+) atypical lymphoid cells were seen in all 11 cases, with infiltration of the follicular epithelium in 8 cases. Hyperplasia of the follicular epithelium was observed in 4 cases; ruptured hair follicles, in 3 cases; and follicular mucinosis, in 2 cases. In addition to hair follicle infiltration, atypical cells were recognized within sebaceous glands in 2 lesions. New findings were presence of numerous intrafollicular neutrophils in 2 patients, who clinically had pustules in addition to papules. Other histopathological features encountered included perieccrine infiltration (n = 5), focal subcutaneous involvement (n = 4), granulomatous inflammation (n = 3), epidermal hyperplasia (n = 2), and 1 each of infiltration of muscle bundles, numerous eosinophils in the infiltrate, and angiocentricity. LIMITATIONS: This was a retrospective study. CONCLUSIONS: Follicular LyP is a variant of LyP with involvement of hair follicles, mostly in the form of perifollicular infiltrate with variable degree of folliculotropism. Other changes including hyperplasia of the follicular epithelium, rupture of hair follicle, and follicular mucinosis are less common. Rarely, intrafollicular pustules can be seen in the follicular epithelium; such lesions manifest clinically as pustules. SN - 1097-6787 UR - https://www.unboundmedicine.com/medline/citation/23375516/Follicular_lymphomatoid_papulosis_revisited:_a_study_of_11_cases_with_new_histopathological_findings_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S0190-9622(12)02331-6 DB - PRIME DP - Unbound Medicine ER -