Buenas noches Doctor Juan Macaluso.
Le escribe la Dra Yuranis Tirado residente de Oncología Pediátrica, envío imágenes de paciente de 15 años con cuadro clínico de 2 años de evolución manejada con múltiples medidas convencionales por servicio de dermatología e infectología; posterior a un tiempo se envía muestra a patología quienes reportan diagnóstico de Micosis Fungoide, que aunque la literatura expresa ser unos de los tipos de linfomas cutaneos de células T más frecuentes, en 2 años ha sido el primero que observo y quise compartirles. De todas maneras se va a realizar un nuevo estudio anatomopatológico con inmunohistoquímica para determinar con exactitud el diagnóstico
Actualmente paciente en espera de estudio
inmunohistoquímico.
Dra. Yuranis Tirado.
Maracaibo. Venezuela.
Opinión: Muy interesante el caso Yuranis por varios motivos. Siempre tuve el concepto de que micosis fungoide (MF), era una enfermedad poco frecuente y de adultos. Cuando leí tu caso me fui a actualizar información en UpToDate y encontré que la incidencia de MF en Europa y los Estados Unidos es de aproximadamente seis casos por millón por año, lo que representa alrededor del 4 por ciento de todos los casos de linfoma no Hodgkin. Por otro lado, la edad pico de presentación es de 55 a 60 años, con una proporción hombre:mujer de 2:1. La enfermedad es más común en poblaciones negras. Aunque la MF es una enfermedad principalmente de pacientes mayores, se puede observar en pacientes menores de 35 años con hallazgos clínicos y evolución similares (39-41).
Actualización:
La Dra. Yuranis Tirado envió Buen día Dr. Envío
reporte de inmunohistoquímica de la paciente con sospecha diagnóstica de
Micosis fungoide; según reporte inmunohistoquímico es una Histiocitosis de Células
De Langerhans.
Opinión: La histiocitosis de
células de Langerhans (LСH) es una neoplasia mieloide inflamatoria que afecta
más comúnmente a los huesos y la piel, pero también puede afectar la médula
ósea, el hígado, el bazo, los pulmones, la glándula pituitaria/sistema nervioso
central y otros órganos. Es considerablemente más común en niños (especialmente
en niños pequeños) que en adultos. Las manifestaciones a nivel de piel y mucosa
oral se ven en 40% de los niños y 20% de los adultos y cuando solamente afecta
la piel, es importante excluir la afectación de otros órganos en pacientes con
aparente LCH que afecta solo a la piel.
Las manifestaciones cutáneas más comunes son una
erupción eccematosa similar a una infección por cándida y/o pápulas de color
marrón a violáceo; otras lesiones cutáneas pueden ser pustulosas, purpúricas,
petequiales, vesiculares o papulonodulares.
Pápulas.
Los bebés pueden presentar pápulas de color marrón a
violáceo en cualquier parte del cuerpo; esto se conoce como
reticulohistiocitosis congénita autocurativa o enfermedad de Hashimoto-Pritzker
(dos términos que deben abandonarse) ( imagen 4 ).
Imagen 4. En el tronco de este bebé con
reticulohistiocitosis congénita autocurativa se observan pápulas de color
marrón rojizo.
Si es solitaria, esta manifestación es benigna y las
lesiones desaparecen durante el primer año de vida sin tratamiento. Sin
embargo, los neonatos con afectación cutánea aparentemente aislada necesitan
una evaluación exhaustiva y un seguimiento cercano para confirmar que no haya
otros sitios afectados.
Una serie de casos de LCΗ neonatal con afectación
cutánea informó que 12 de 19 niños también tenían enfermedad multisistémica que
afectaba otros órganos. En otro informe de 61 casos neonatales de 1069
pacientes en la base de datos de la Histiocyte Society, el 59 por ciento tenía
enfermedad multisistémica y el 42 por ciento tenía afectación de órganos de
"riesgo". En otros estudios, el 40 por ciento de los neonatos que
tenían LCΗ cutáneo solitario en la presentación tenían enfermedad
multisistémica cuando se realizó una estadificación integral o desarrollaron
posteriormente afectación multisistémica. Los niños con LCΗ cutáneo verdadero
solo tenían generalmente menos de un año de edad, mientras que aquellos con LCΗ
multisistémico eran mayores de 18 meses. Aquellos con enfermedad cutánea solo
tenían una supervivencia libre de progresión (SLP) del 89 por ciento, mientras
que la SLP de los pacientes multisistémicos fue solo del 23 por ciento.
Erupción eccematosa.
La dermatitis seborreica puede manifestarse en niños y
adultos como una erupción papulosa eritematosa que se presenta en la ingle, el
abdomen, la espalda o el pecho y se asemeja a una dermatitis del pañal difusa
por cándida. Las lesiones varían en tamaño de 1 a 10 mm de diámetro. La
afectación seborreica del cuero cabelludo puede confundirse con una
"costra láctea" prolongada en los bebés o una dermatitis seborreica
grave en personas mayores. Las lesiones ulcerativas detrás de las orejas, en el
cuero cabelludo, los genitales o la región perianal son especialmente
problemáticas, ya que a menudo se diagnostican erróneamente como lesiones
bacterianas o fúngicas.
Lesiones orales.
Las manifestaciones orales más comunes de LCH son
masas intraorales, gingivitis, úlceras mucosas y dientes flojos causados por
lesiones de los huesos o tejidos blandos. Algunos niños con ԼCH presentan una
erupción dental temprana.
Referencias
UpToDate 2025.
39) Crowley JJ, Nikko A, Varghese A, et al. Mycosis
fungoides in young patients: clinical characteristics and outcome. J Am Acad
Dermatol 1998; 38:696.
40) Quaglino P, Zaccagna A, Verrone A, et al. Mycosis
fungoides in patients under 20 years of age: report of 7 cases, review of the
literature and study of the clinical course. Dermatology 1999; 199:8.
41) Pope E, Weitzman S, Ngan B, et al. Mycosis
fungoides in the pediatric population: report from an international Childhood
Registry of Cutaneous Lymphoma. J Cutan Med Surg 2010; 14:1.
ABSTRACTS
Abstracts for References 39-41 of 'Clinical
manifestations, pathologic features, and diagnosis of mycosis fungoides'
39
PubMed
TI
Mycosis fungoides in young patients: clinical
characteristics and outcome.
AU
Crowley JJ, Nikko A, Varghese A, Hoppe RT, Kim YH
SO
J Am Acad Dermatol. 1998;38(5 Pt 1):696.
BACKGROUNDMycosis fungoides (MF) can begin as early as
the first decade of life. Few studies have reviewed MF in younger patients and
none has been large enough to assess prognosis and outcome.
OBJECTIVEWe reviewed the clinical characteristics,
prognosis, factors related to disease progression, and therapy in patients with
MF younger than 35 years of age.
METHODSFifty-eight patients were entered into this
retrospective cohort analysis.
RESULTS Significantly fewer patients with MF who are
younger than 35 years presented with erythroderma (T4) and more with limited
patch/plaque (T1) disease than older patients. Duration of skin disease before
diagnosis of MF did not vary between the two groups. The long-term survival of
younger patients with MF is significantly decreased when compared with a race-,
age-, and sex-matched control population (p<0.001). Disease-specific
survivals (DSS) of younger and older patients are similar, but young patients
show a slight but significantly better overall DSS (p<0.02). However, DSS
comparison of generalized patch/plaque (T2) and tumor stage (T3) patients with
MF showed no significant difference between young and old patients (p=0.47, p=0.59).
Patient age was not a significant predictor of survival when controlled for
T-stage. Sixteen of 58 young patients with MF have died, 13 because of MF
(22%), compared with 138 of 500 older patients (28%) who died as a result of
MF. All younger patients with MF who progressed had at least T2 disease at
presentation. Fifty of 56 young patients with MF and T1-T3 disease were treated
initially with total skin electron beam or topical nitrogen mustard. The
response to therapy was similar in younger and older patients with MF.
CONCLUSIONT1 disease is more common and T4 disease is
unusual in young patients with MF compared with an older population of patients
with MF. Young patients with T1 disease, all of whom were treated with either
topical nitrogen mustard or total skin electron beam therapy, or both
therapies, showed no disease progression. Overall, young patients with MF
showed slightly better DSS, but this was because of differences in stage
distribution.
AD
Department of Dermatology, Stanford University School
of Medicine, California, USA.
PMID
9591813
40
PubMed
TI
Mycosis fungoides in patients under 20 years of age:
report of 7 cases, review of the literature and study of the clinical course.
AU
Quaglino P, Zaccagna A, Verrone A, Dardano F, Bernengo
MG
SO
Dermatology. 1999;199(1):8.
BACKGROUNDMycosis fungoides (MF) is rare in young
patients. Its clinical behavior is still uncertain, as some reports have
suggested that it has a more aggressive course than does the adult-onset type.
AIMTo ascertain if early-onset MF represents a
heterogeneous group of cutaneous T cell lymphomas.
MATERIALS AND METHODSClinical, immunohistopathological
and follow-up data of early-onset (<20 years of age) MF cases reported in
the literature (n = 42) plus 7 described herein were compared with those of a
cohort of adult-onset MF patients (n = 252) diagnosed at our institution since
1975.
RESULTS The majority of the 49 early-onset MF patients
had patch-plaque stage disease at diagnosis. Ten had hypopigmented lesions. The
predominant phenotype was CD3+ CD4+CD7-CD8-. Seven patients had a stage
progression, 6 with extracutaneous involvement. Five- and 10-year survival
rates were 93 and 74%, respectively.
CONCLUSIONSNo statistically significant differences
were found in the disease course between early- and adult-onset MF.
AD
Section of Dermatology, Department of Medical and
Surgical Specialties, University of Turin, Italy.
PMID
10449950
41
PubMed
TI
Mycosis fungoides in the pediatric population: report
from an international Childhood Registry of Cutaneous Lymphoma.
AU
Pope E, Weitzman S, Ngan B, Walsh S, Morel K, Williams
J, Stein S, Garzon M, Knobler E, Lieber C, Turchan K, Wargon O, Tsuchiya A
SO
J Cutan Med Surg. 2010 Jan;14(1):1-6.
BACKGROUND/OBJECTIVESThere are limited data on the
clinical presentation and progression of pediatric cutaneous lymphoma. This
study focuses on the clinical characteristics of pediatric patients with
mycosis fungoides (MF).
MATERIALS AND METHODS This descriptive study presents
clinical characteristics of 22 pediatric patients with MF, enrolled in the
international Childhood Registry for Cutaneous Lymphomas (CRCL).
RESULTS The mean ages at onset and at diagnosis were
7.5 (SD 3.8 years) years and 9.9 (SD 3.4) years, respectively. The most common
MF presentation was patch stage (68%), followed by hypopigmentation (59%) and
plaque stage disease (50%). Epidermotropism and lymphocytic atypia were the
most common pathologic features, found in 89% and 85%, respectively.
Cerebriform nuclei were noted in 42%, and Pautrier microabscesses were seen in
16% of cases. A cytotoxic pattern was more commonly seen (67% vs 33%), and
clonality was detected in 21% (3 of 14) of patients. All patients presented
with early-stage disease and received skin-directed therapy (topical steroids,
73%; light therapy, 54%; or combination therapy, 35%).
CONCLUSIONS Pediatric patients with MF present in the
first decade of life, with early-stage disease and unusual forms such as
hypopigmented variant. Further patient enrollment will provide information
regarding natural history, treatment response, and overall prognosis of
pediatric cutaneous T-cell lymphoma (CTCL).
AD
Section of Dermatology, The Hospital for Sick
Children, Toronto, ON, Canada. Elena.pope@sickkids.ca
PMID
20128983
PubMed
TI
Langerhans cell histiocytosis of the skin.
AU
Munn S, Chu AC
SO
Hematol Oncol Clin North Am. 1998;12(2):269.
Cutaneous involvement in Langerhans cell histiocytosis
(LCH) occurs in 50% of cases and may be the presenting feature. It is,
therefore, important to recognize the wide spectrum of clinical disease that
this disorder may adopt in the skin. Cutaneous involvement is not necessarily a
benign feature and many patients progress to multi-system disease. There are a
number of treatments available for cutaneous LCH. The rationale is to start
with the simplest treatment and progress to systemic or interventional therapy
as needed.
AD
Imperial College School of Medicine, London, United
Kingdom.
PMID
9561900
55
PubMed
TI
Congenital self-healing reticulohistiocytosis
(Hashimoto-Pritzker disease): ten-year experience at Dallas Children's Medical
Center.
AU
Kapur P, Erickson C, Rakheja D, Carder KR, Hoang MP
SO
J Am Acad Dermatol. 2007;56(2):290.
The real incidence of congenital self-healing
reticulohistiocytosis (CSHR) may be underreported because of its high rate of spontaneous
resolution and lack of clinical recognition. Currently, there are no criteria
other than clinical that can reliably distinguish CSHR from cutaneous
involvement by disseminated Langerhans cell histiocytosis (LCH). In this study
we investigate the role of E-cadherin, Ki-67, and phosphorylated histone H3
(PHH3) immunohistochemical stains in distinguishing CSHR from disseminated LCH.
We found that no significant difference was seen in the histologic features and
the expression of E-cadherin, Ki-67, and PHH3 between the two groups; thus
supporting the theory that CSHR and LCH represent different ends of a spectrum
of the same condition.
AD
Department of Pathology, Children's Medical Center
Dallas, Texas, USA.
. PubMed
TI
Langerhans cell histiocytosis presenting in the
neonatal period: a retrospective case series.
AU
Stein SL, Paller AS, Haut PR, Mancini AJ
SO
Arch Pediatr Adolesc Med. 2001;155(7):778.
OBJECTIVESTo describe the morphologic characteristics
of skin lesions, extent of extracutaneous disease, and outcomes in patients
with neonatal presentation of Langerhans cell histiocytosis (LCH), and to
examine clinical predictors of disease prognosis.
DESIGNRetrospective validation cohort study. Maximum
duration of follow-up was 10 years.
SETTINGA tertiary care children's hospital in Chicago,
Ill.
PATIENTSNineteen children with cutaneous findings in
the first 4 weeks of life and subsequently diagnosed with LCH based on
compatible tissue histologic analysis, confirmed by electron microscopy and/or
immunohistochemical analysis.
MAIN OUTCOME MEASURECutaneous lesion morphologic
characteristics, extracutaneous manifestations, treatments, and outcomes were
tabulated and compared.
RESULTSThe most common initial skin lesion was
erythematous, often crusted, vesiculopustules. Skin lesion morphologic traits did
not correlate with extent of extracutaneous disease. One third of patients had
disease limited to the skin and/or mucous membranes. All of these patients are
alive and well, and 1 has developed diabetes insipidus. Twelve of th..e 19
patients had multisystem disease, and 2 died of disease. The results of a
multiorgan workup performed at the time of diagnosis were predictive of which
patients in this cohort manifested multisystem disease. The overall incidence
of diabetes insipidus was 21%.
CONCLUSIONSVesiculopustular lesions are common in
congenital/neonatal LCH, but the morphologic characteristics of lesions are not
helpful in predicting the extent of disease. A multiorgan evaluation at the
time of diagnosis may be predictive of the probability of multisystem
involvement with LCH.
AD
Department of Dermatology, Northwestern University
Medical School, Chicago, IL 60614, USA.
PMID
11434843
. PubMed
TI
Cutaneous Langerhans cell histiocytosis in children
under one year.
AU
Lau L, Krafchik B, Trebo MM, Weitzman S
SO
Pediatr Blood Cancer. 2006;46(1):66.
BACKGROUNDTo evaluate the clinical course and outcome
of infants with Langerhans cell histiocytosis (LCH) involving skin and to
estimate the incidence of progression to multi-system (M-S) disease in those
with isolated skin involvement.
METHODSA retrospective review was conducted on 22 LCH
patients who were younger than 12 months at the onset of their skin eruption.
RESULTSTwelve patients had isolated skin involvement
at diagnosis and 10 were evaluable for progression. Four of the 10 (40%)
evaluable patients progressed to multi-system (M-S) disease. Of the 10 patients
with M-S disease at diagnosis, 5 had a history of a preceding skin eruption 2
to 13 months prior to diagnosis. Eleven of the 14 (79%) patients with M-S
disease had risk organ involvement. The mortality rate of M-S disease was 50%.
CONCLUSIONSIt is important for primary caregivers to
recognize that isolated cutaneous LCH in infants is not always a benign disorder.
The diagnosis of self-healing cutaneous LCH should only be made in retrospect.
Careful, albeit non-invasive, follow-up is recommended to monitor for disease
progression and development of long-term complications.
AD
Division of Haematology/Oncology, Department of
Paediatrics, The Hospital for Sick Children, Toronto, Ontario, Canada.
PMID
16261594
59
PubMed
TI
Differentiating skin-limited and multisystem
Langerhans cell histiocytosis.
AU
Simko SJ, Garmezy B, Abhyankar H, Lupo PJ, Chakraborty
R, Lim KP, Shih A, Hicks MJ, Wright TS, Levy ML, McClain KL, Allen CE
SO
J Pediatr. 2014;165(5):990. Epub 2014 Oct 21.
OBJECTIVETo identify features associated with
multisystem involvement and therapeutic failure in patients with skin
Langerhans cell histiocytosis (LCH).
STUDY DESIGNWe reviewed medical records of 71
consecutive patients with LCH with skin involvement evaluated at Texas
Children's Hospital and analyzed clinical features, laboratory results, and the
presence of circulating cells with the BRAF-V600E mutation with respect to
initial staging and clinical outcomes.
RESULTSSkin disease in patients older than 18 months
of age at diagnosis was associated with the presence of multisystem disease
(OR, 9.65; 95% CI, 1.17-79.4). Forty percent of patients referred for presumed
skin-limited LCH had underlying multisystem involvement, one-half of these with
risk-organ involvement. Patients with skin-limited LCH had a 3-year
progression-free survival of 89% after initial therapy, and none developed
multisystem disease. Patients with skin/multisystem involvement had a 3-year progression-free
survival of 44% with vinblastine/prednisone therapy, and risk-organ involvement
did not correlate with failure to achieve nonactive disease. Circulating cells
with BRAF-V600E were detected at higher frequency in patients with multisystem
involvement (8 of 11 skin/multisystem vs 1 of 13 skin-limited; P = .002).
CONCLUSIONSkin-limited LCH necessitates infrequent
therapeutic intervention and has a lower risk of progression relative to skin
plus multisystem LCH. The less-aggressive clinical course and lack of
circulating cells with the BRAF-V600E mutation in skin-limited LCH suggest a
different mechanism of disease origin compared with multisystem or risk-organ
disease.
AD
Texas Children's Cancer Center, Baylor College of
Medicine, Houston, TX. Electronic address: sjsimko@txch.org.
PMID
25441388
