PMID- 33675561
OWN - NLM
STAT- MEDLINE
DCOM- 20211228
LR  - 20211228
IS  - 1600-0560 (Electronic)
IS  - 0303-6987 (Linking)
VI  - 48
IP  - 9
DP  - 2021 Sep
TI  - Spiky/keratosis-pilaris-like early follicular mycosis fungoides: A 
      clinicopathologic study of 20 cases with extended follow-up.
PG  - 1124-1132
LID - 10.1111/cup.14002 [doi]
AB  - BACKGROUNDS: Follicular mycosis fungoides (FMFs) is a distinct form of T-cell 
      lymphoma whose course is considered aggressive. METHODS: A retrospective study 
      with long-term follow-up of 20 patients diagnosed with 
      spiky/keratosis-pilaris-like FMF between 2008 and 2017 was conducted. RESULTS: 
      Twelve males and eight females were identified, with a mean age at first 
      diagnosis of 59 years (range 42-86). Hyperkeratotic follicular papules were the 
      sole clinical finding in 16 of 20 patients. A diagnostic delay between first 
      symptom development and initial diagnosis was frequent (mean 42 months). The 
      head/neck region was concurrently affected only in two patients. Disease stage at 
      diagnosis was IA in two patients (10%) and IB in 18 (90%). Five patients had 
      almost complete lesion regression, whilst there was only a slight improvement, 
      without regression in 14. Two patients developed infiltrated papules, comedones, 
      and small cysts during follow-up. Only one patient progressed to tumor stage 
      (IIB) five years after the first diagnosis. The mean follow-up was seven years 
      (range: 12-180 months). None of them died of cutaneous lymphoma. CONCLUSIONS: FMF 
      presenting with only spiky/keratosis-pilaris-like lesions have an excellent 
      prognosis at medium-term follow-up. Early recognition of patients with this 
      peculiar FMF presentation might lead to identifying prognostic factors.
CI  - (c) 2021 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.
FAU - Tomasini, Carlo
AU  - Tomasini C
AUID- ORCID: 0000-0001-8383-3443
AD  - Dermatology Clinic, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
AD  - Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of 
      Pavia, Pavia, Italy.
FAU - Michelerio, Andrea
AU  - Michelerio A
AD  - Dermatology Clinic, Fondazione IRCCS Policlinico San Matteo, Pavia, Italy.
AD  - Department of Clinical-Surgical, Diagnostic and Pediatric Sciences, University of 
      Pavia, Pavia, Italy.
FAU - Quaglino, Pietro
AU  - Quaglino P
AD  - Department of Medical Sciences, Dermatologic Clinic, University of Turin, Turin, 
      Italy.
LA  - eng
PT  - Journal Article
DEP - 20210323
PL  - United States
TA  - J Cutan Pathol
JT  - Journal of cutaneous pathology
JID - 0425124
RN  - Burnett Schwartz Berberian syndrome
SB  - IM
MH  - Abnormalities, Multiple/*diagnosis/pathology
MH  - Adult
MH  - Aged
MH  - Aged, 80 and over
MH  - Darier Disease/*diagnosis/pathology
MH  - Delayed Diagnosis
MH  - Disease Progression
MH  - Eyebrows/*abnormalities/pathology
MH  - Female
MH  - Follow-Up Studies
MH  - Humans
MH  - Lymphoma, T-Cell/pathology
MH  - Male
MH  - Middle Aged
MH  - Mycosis Fungoides/*diagnosis/pathology
MH  - Neoplasm Staging/methods
MH  - Prognosis
MH  - Retrospective Studies
MH  - Skin Neoplasms/*pathology
OTO - NOTNLM
OT  - cutaneous T-cell lymphoma
OT  - follicular mycosis fungoides
OT  - hyperkeratotic spicules
OT  - mycosis fungoides
OT  - spiky
EDAT- 2021/03/07 06:00
MHDA- 2021/12/29 06:00
CRDT- 2021/03/06 12:08
PHST- 2020/12/21 00:00 [revised]
PHST- 2020/10/26 00:00 [received]
PHST- 2021/03/01 00:00 [accepted]
PHST- 2021/03/07 06:00 [pubmed]
PHST- 2021/12/29 06:00 [medline]
PHST- 2021/03/06 12:08 [entrez]
AID - 10.1111/cup.14002 [doi]
PST - ppublish
SO  - J Cutan Pathol. 2021 Sep;48(9):1124-1132. doi: 10.1111/cup.14002. Epub 2021 Mar 
      23.