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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 24  |  Issue : 2  |  Page : 120-122

Papular mycosis fungoides: Three case reports and a comprehensive literature review


Department of Hematology, Jinhua Municipal Central Hospital, Jinhua, Zhejiang Province, PR China

Date of Web Publication4-Sep-2018

Correspondence Address:
Dr. Huixian Hu
Department of Hematology, Jinhua Municipal Central Hospital, 365, Ren Min Road, Jinhua 321000, Zhejiang Province
PR China
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/indianjotol.INDIANJOTOL_133_17

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  Abstract 


This study was to investigate the clinicopathological characteristics, treatments, and prognoses of papillary mycosis fungus (PMF). From May 2004 to September 2015, three cases of papular mycosis fungoides were analyzed at Jinhua Municipal Central Hospital. Diagnostic criteria refer to the World Health Organization 2005 Classification of Cutaneous Lymphoma. Factors that influenced patient survival and prognoses were analyzed. Skin papules were reduced, and skin color was lightened in three patients after a short treatment regimen. Follow-up for 4–7 years revealed few changes in pigmentation, no increase in skin papules, and neither organ nor lymph node involvement. Skin papular mycosis fungal disease can heal itself, and there is no need for excessive intervention.

Keywords: Clinical analysis, mycosis fungoides, popular


How to cite this article:
Zhang J, Zhao M, Hu H. Papular mycosis fungoides: Three case reports and a comprehensive literature review. Indian J Otol 2018;24:120-2

How to cite this URL:
Zhang J, Zhao M, Hu H. Papular mycosis fungoides: Three case reports and a comprehensive literature review. Indian J Otol [serial online] 2018 [cited 2018 Dec 13];24:120-2. Available from: http://www.indianjotol.org/text.asp?2018/24/2/120/240563




  Introduction Top


Papular mycosis fungoides (PMF) is a primary cutaneous T-cell lymphoma and a special subtype of mycosis fungoides (MF).[1] Long-term skin papules are typical clinical manifestations and are generally neither painful nor itching. Dermal lymphoid cell infiltration is its major pathological feature, followed by partial invasion of the epidermis leading to  Pautrier microabscess More Details, epidermotropism, and CD4-positive and abnormal T-cell proliferation. PMF is rare in clinical practice and has pathological manifestations of MF. We summarize the clinical features, treatments, and prognoses of three patients with PMF to improve our understanding of PMF patients.


  Case Report Top


From May 2004 to September 2012, three cases of PMF were admitted to the Department of Hematology, Jinhua Hospital, Zhejiang University. Diagnostic criteria refer to the World Health Organization (WHO) 2005 Classification of Cutaneous Lymphoma. The main diagnostic indicators in this study are dermal layer with atypical T-lymphocyte infiltration of an existing Pautrier microabscess and immunohistochemical findings. The three cases included two males and one female. The age at diagnosis was 22–48 years, with a median of 38 years. It was the first time for each of them to receive treatment, and the patients only exhibited skin involvement when diagnosed. The first case presented with dense, miliary-sized dark red papules on the chest and both lower extremities and no pain; the incidence of papules gradually increased, the epidermis occasionally exhibited scaling, and the pimples never faded. The second case presented with dark red papules scattered on the body, and the size and nature of the papules were similar to those of the first case. The third case presented with papules concentrated on the hips that were the size of a grain and with dark red, scaling skin, and occasional itching.

Pathological examination

The three cases had repeatedly undergone biopsy from onset to diagnosis. These cases exhibited epidermal hyperkeratosis, superficial dermal abnormal lymphocyte infiltration, and partial implantation of the skin on a Pautrier microabscess [Figure 1].
Figure 1: Papule biopsy shows typical Pautrier microabscess (indicated by the arrow)

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Immunohistochemistry

Immunohistochemistry display CD3 (+), CD8 (+), CD8 (+), CD45RO (+), CD20 (-), CD79 (-), CD68 (-), CD30 (-).

Auxiliary examination

The blood, urine, lung film, electrocardiography, and liver and kidney function tests for the three cases were normal. The superficial lymph nodes of the whole body and retroperitoneal lymph nodes of the thoracic cavity were not enlarged.

Treatment

The first patient was administered daily isotretinoin gel once a day topically and weekly narrow-band ultraviolet B (UVB) for local irradiation. After 1 month of treatment, the papules subsided and the color faded, so the patient stopped treatment. The third patient did not receive any treatment because of the small range of the papules.

Follow-up

The first patient was followed up for 7 years after diagnosis; the skin papules still exists in the chest area, the color faded, and the papules were smaller [Figure 2]. Papules on the lower limbs disappeared and had a dark red pigmentation. New pimples were not found on the body. The second patient was followed up for 5 years, and the papules were lighter than when diagnosed, but the others did not change significantly. The third patient was followed up for 4 years. The skin papules on the buttocks disappeared, showed little pigmentation, and had partially increased visible dander.
Figure 2: Patients with chest area skin pimples. Left: in September 2009. Right: in October 2017, has not been any treatment in patients

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  Discussion Top


The 2016 version of the WHO Non-Hodgkin's Lymphoma Classification treats MF as an independent subtype of cutaneous peripheral T-cell lymphoma.[2] MF accounts for approximately 60% of all skin peripheral T-cell lymphoma cases. Typical clinical MF includes manifestations of three types of lesions, namely erythema, plaques, and tumor stage. Early lesions appear as flat, pink, scaly patches. Late lesions are dark red, thick, pad-like, ring-shaped, or irregularly shaped uplift plaques. Surface scabs can appear shell-like because of repeated exudation, and some can be expressed as moss-like papules, blisters, or hypopigmentation. Clinical progress of MF is slow, often lasts for several years or decades, and can affect internal organs in the tumor stage.[3] Papillary MF (PMF) was first reported in 2005 by Kodama et al.,[4] and PMF papules showed no evidence of erythema or other classic MF lesions, with bright red, miliary papules as the first presentation. Papules can vary from 1 to 10 mm in size and are usually focally and symmetrically distributed. PMF mainly occurs on the trunk, upper arm, and thigh.[5] Similar to MF, histopathological examinations revealed a typical Pautrier microabscess, and immunohistochemistry showed a CD3-, CD4-, CD8-positive T-cell immunophenotype. In clinical practice, physicians often classify PMF as general MF and give overtreatment because its pathological manifestations are the same to that of MF. However, if the new papules appear on the basis of MF erythema, it could be considered a signal of MF disease progression that indicates transformation to a plaque or tumor and suggests a poor prognosis, which is significantly different from the clinical manifestations of PMF.[3]

Skin papules consistent from the beginning to end of the treatment are typical manifestations of PMF in a clinic. The clinical and pathological aspects of disease that are similar to PMF are as follows: (1) Follicular MF (FMF) – Papules are mainly distributed on the head and neck, followed by the upper part of the trunk and extremities. Typical skin lesions are follicular and acne-like papules. Papules often exhibit ulceration and crusting and can be associated with alopecia areata, especially in the eyebrows. FMF prognosis is poor with a 5-year survival rate of 60%. (2) Lymphomatoid papulosis – It manifests as generalized skin lesions, and the typical signs of the disease are erythema, papules, nodules, ulcers, necrosis, and scabbing. (3) Lymphoma-like papulosis – Papules, nodules, lumps, necrosis, and scab pleomorphic are the major clinical manifestations. Pimples and PMF can be similar, but Pautrier microabscess has not been observed as pathological features.

The pathogenesis of PMF is currently unclear. It is currently thought that auxiliary T2 cells, CD8+ cells, interleukin-4, and interleukin-10 are abundant in PMF papules in the early stages of MF and that these factors control malignant clonal proliferation. In the advanced stage of MF, CD8+ cells were decreased, whereas T2 cytokines, interleukin-4, and interleukin-10 increased [6] with an increasing number of tumor cells. However, other researchers have argued that PMF is an independent subtype of MF and that the most important diagnosis of PMF is not associated with erythema or rupture of the dense, dark red papules. The incidence of PMF is very low, and its treatment refers to the MF erythema period, for which there is only the “expectant therapy”[4] that involves treating the skin with topical glucocorticoid and isotretinoin gel as a symptomatic treatment. It has also been reported that the use of narrow-band UVB local radiation can achieve a good effect, but PMF generally does not require the use of systemic chemotherapy unless a clear lesion involving the internal organs is present.[7] The three patients in this group exhibited red miliary papules with no obvious incentive. The papules were round or oval with a hard texture and nonfusion, and they had a flaky distribution, on which some dander could be seen on the surface of the papules of the cases. No patients experienced pain or itching, and on multiple biopsies, they showed findings similar to those of MF. After short-term treatment, two of the three cases had no obvious regression of the papules. On follow-up for 4–7 years, none of the three patients had new papules, while the original papules flattened, the color faded, and some disappeared. Follow-up indicated a good prognosis, which suggests that PMF does not require active treatment.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patients have given their consent for their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Acknowledgment

This work was supported by the Zhejiang Province Public Technology Research Projects (Grant No. 2016F81SA700015) and by a Jinhua Science and Technology Research Project (Grant No. LY15H160049).

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Vonderheid EC, Kadin ME. Papular mycosis fungoides: A variant of mycosis fungoides or lymphomatoid papulosis? J Am Acad Dermatol 2006;55:177-80.  Back to cited text no. 1
    
2.
Swerdlow SH, Campo E, Pileri SA, Harris NL, Stein H, Siebert R, et al. The 2016 revision of the World Health Organization classification of lymphoid neoplasms. Blood 2016;127:2375-90.  Back to cited text no. 2
    
3.
Colomo L, López-Guillermo A, Perales M, Rives S, Martínez A, Bosch F, et al. Clinical impact of the differentiation profile assessed by immunophenotyping in patients with diffuse large B-cell lymphoma. Blood 2003;101:78-84.  Back to cited text no. 3
    
4.
Kodama K, Fink-Puches R, Massone C, Kerl H, Cerroni L. Papular mycosis fungoides: A new clinical variant of early mycosis fungoides. J Am Acad Dermatol 2005;52:694-8.  Back to cited text no. 4
    
5.
Martorell-Calatayud A, Botella-Estrada R, Sanmartín-Jimenez O, Requena C, Guillén-Barona C, Sangòeza OP, et al. Papular mycosis fungoides: Two new cases of a recently described clinicopathological variant of early mycosis fungoides. J Cutan Pathol 2010;37:330-5.  Back to cited text no. 5
    
6.
Tomasini D, Zampatti C, Palmedo G, Bonfacini V, Sangalli G, Kutzner H. Cytotoxic mycosis fungoides evolving from pityriasis lichenoides chronica in a seventeen-year-old girl. Report of a case. Dermatology 2002;205:176-9.  Back to cited text no. 6
    
7.
Vonderheid EC, Kadin ME, Telang GH. Commentary about papular mycosis fungoides, lymphomatoid papulosis and lymphomatoid pityriasis lichenoides: More similarities than differences. J Cutan Pathol 2016;43:303-12.  Back to cited text no. 7
    


    Figures

  [Figure 1], [Figure 2]



 

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