Lymphomatoids papulose C86.6

Author: Prof. Dr. med. Peter Altmeyer

Co-Autor: Florian Burkhart

All authors of this article

Last updated on: 26.01.2021

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Synonym(s)

Anaplastic lymphomatoid papulose; Angiocentric lympomatoid papulose; Epitheliotropic lymphomatoid papulose; LyP; Mixed cell lymphomatoid papulose; Papulose lymphomatoids; papulosis lymphomatoid; T-cell pseudolymphoma

History
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Dupont, 1956; Macauly, 1968

Definition
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Low malignant, CD30-positive, particularly benign T-cell lymphoma (?) with a recurrent course over many years, characterized by single or multiple, painless, papulo-nodular or ulcerative skin changes with spontaneous healing and months of possibly even years of latency.

Remark: Some authors consider the clinical picture as benign!

Occurrence/Epidemiology
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Prevalence 0.1/100,000 inhabitants/year. Worldwide occurrence. Predominantly among members of the Caucasian ethnic groups, the proportion of CTCL is about 18%;

Etiopathogenesis
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Unknown. Viral genesis? In adolescent patients there is an increased association with atopic diseases.

Manifestation
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Mainly adults; age: 7-83 years, mean age: 39 years (Xiong J et al. 2015), f:m=2,3:1 (according to other sources: 1:1); very rarely the disease is also found in children and adolescents.

Localization
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Trunk>Extremities>Face. The oral cavity is rarely affected.

Clinical features
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Single (rarely more than 5, disseminated occurrence is possible but the exception) red to red-brown or brown-violet, soft-elastic, painless, flat, red papules forming within a few days (eruption phase). These develop (surprisingly quickly) into red, always painless nodules (up to 2 cm Ø) with an initially smooth surface.

The central parts of the nodules regularly disintegrate torpidly under initial hemorrhagic crust formation. This leads to a change in morphology! In this phase of development, the only clinical impression is an ulcer of varying depth (which cannot be explained to the examiner), which is usually (surprisingly) slightly painful. Danger of secondary infection (picture of pyoderma). Healing with formation of a less spectacular, hyper(hypo)pigmented scar.

After 3-8 weeks spontaneous regression with the formation of scaly crusts.

Histology
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Type A: Dense, diffuse, subcutaneous, mixed-cell infiltrate of pleomorphic and anaplastic lymphoid cells and numerous eosinophilic and neutrophilic granulocytes, histiocytes and small lymphocytes. Numerous mitoses.

Type B: Band-shaped dermal epidermotropic infiltrate of small to medium-sized lymphoid cells with chromatin-tight cerebriform nuclei.

Type C: Dense, diffuse infiltrate of large anaplastic cells, reaching down to the subcutaneous tissue. Only a few eosinophil or neutrophil granulocytes, histiocytes and small lymphocytes. Numerous mitoses.

Type D: Dense, diffuse subcutaneous infiltrate of small to medium-sized CD8+ and CD30+ lymphocytes. Distinct epidermotropy.

Type E (angioinvasive type): Dense, angiocentric and angioinvasive, subcutaneous mixed cell infiltrate of medium pleomorphic and anaplastic lymphoid cells. Detection of necrosis and ulcer formation. Further eosinophil granulocytes.

Immunohistology: Tumor cells are positive for CD3, CD4, CD8, CD25. In type A and C the anaplastic cells express CD30 (see CD classification below) T-cell receptor gene rearrangement: Only in isolated cases detection of monoclonality of T-cell infiltrates.

Differential diagnosis
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  • Lymphoma, cutaneous T-cell lymphoma, large cell, CD30-positive: Mostly solitary larger nodules; rarely grouped nodules or nodules with a tendency to ulceration. Spontaneous regressions and recurrences are possible (in up to 25% of patients).
  • Pityriasis lichenoides chronica: Very polymorphic clinical picture; initially small (usually < 0.5 cm), calotte-shaped, reddened, rough, superficially blunt or specular papules covered by a fine scale. Alignment along the tension lines of the skin. Typical: Formation of a compact, oblate, covering, centrally adherent scale. The disease causes no symptoms except for a slight to moderate itching. Histology is generally diagnostic.
  • Prurigo simplex subacuta: Mostly colourful clinical picture with efflorescences of different acuity "healed from fresh to scarred". Typical is a punctual, unpleasantly piercing itching, which is answered with a typical scratching reaction (spooning); after that the itching stops abruptly. Histology reliably excludes the diagnosis "lymphomatoid papulosis".

    Notice!

    It is not the "disturbing efflorescences" but the tormenting itching that leads to the doctor!
  • Pseudolymphomas: the clinical picture is variable; mostly surface stable, not ulcerated, less symptomatic papules. Histology: Mostly mixed, ripe cell, B- and T-cell pattern. No CD30+ infiltrate.
  • Insect bites: Mostly itchy "artificial" looking, urticarial papules and plaques. Mostly short anamnesis. Difficult to differentiate in persistent arthropod reactions. Histology: as in pseudolymphomas.
  • Syphilis: history; histology and serology are diagnostic.
  • Eosinophilic ulcer of the oral mucosa: this DD can only be clarified histologically.

Complication(s)
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  • 40-60% of patients develop another lymphoproliferative disease. In this case the rate of Mycosis fungoides is particularly high (average 78%). Patients with lymphomatoid papulosis type A are preferentially affected (> 90%). Mycosis fungoides may occur in parallel with lymphomatoid papulosis (30-40%) or precede it (60-70%).
  • Other lymphoproliferative diseases that may occur together or following lymphomatoid papulosis are large cell anaplastic lymphomas (12%). Occasionally, the occurrence of Hodgkin' s disease has been reported.

Therapy
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The therapy is primarily based on the clinical manifestation of the clinical picture. None of the available therapies influence the natural course of the disease. All therapy modalities should be used under the aspect of disease control and not cure.
  • In this respect, a prompt proactive local therapy with medium-strong topical glucocorticoids, if necessary under occlusion (glucocorticoids class II and III) such as 0.1% betamethasone-lotio R030, 0.1% triamcinolone cream R259 or intralesional administration of triamcinolone (e.g. Volon A 1:1 diluted with LA such as scandicain) is recommended in case of localized, land infestation.
  • Alternatively for multifocal spread:
  • Six-monthly check-ups and repeated skin biopsies are necessary in order to detect any transition to aggressive cutaneous T-cell lymphoma in time!

General therapy
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Overall rather poor response to the therapy. In this respect, symptomatic behaviour is indicated. Possible treatment of secondary infections.

Progression/forecast
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  • Mostly good, years of recurrent blandness, with months or years of freedom of appearance.
  • Reduction of life expectancy only by associated lymphomas (up to 20% of patients develop other lymphomas over the years: mycosis fungoides, immunoblastic or large-cell anaplastic lymphoma, Hodgkin's disease). The mortality rate is around 8%.
  • Patients who develop lymphomatoid papulosis in childhood are 200 times more likely to develop non-Hodgkin's lymphoma than the general population.

Literature
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  1. Dupont A, Thulliez A, Brosens M (1956) Reticulose histiomoncytaire et mycosis fungoide: remarques propos de quatre observations. Arch Belg Dermatol Syphil 12: 263-265
  2. Gan EY et al (2012) Lymphomatoid papulosis: is a second lymphoma commoner among East Asians? Clin Exp Dermatol 37:118-121
  3. Kempf W et al (2011) EORTC, ISCL, and USCLC consensus recommendations for the treatment of primary cutaneous CD30-positive lymphoproliferative disorders: lymphomatoid papulosis and primary cutaneous anaplastic large-cell lymphoma. Blood 118:4024-4035
  4. Kempf W et al (2014) Cutaneous lymphomas: an update. Part 1: T-cell and natural killer/t-cell lymphomas and related conditions. At J Dermatopathol 36:105-123
  5. Kempf W et al (2015) Paediatric cutaneous lymphomas: a review and comparison with adult counterparts. J Eur Acad Dermatol Venereol 29:1696-1709
  6. Kempf W, Cerroni L (2016) Cutaneous lymphomas. In: Cerroni L et al Histopathology of the skin. Springer-Verlag Berlin Heidelberg New-York S. 912-913
  7. Kiavash K et al (2015) New variant lymphomatoid papulosis type E preceding and coexisting with mycosis fungoides - a case report and review of theliterature. J Cutan Pathol doi: 10.1111/cup.12606
  8. Nashan D et al (2018) Primary cutaneous lymphoma - a case series of 163 patients. Dermatologist 69: 1014-1020
  9. Martorell-Calatayud A et al (2010) Lymphomatoid papulosis in children: report of 9 cases and review of the literature. Actas Dermosifiliogr 101:693-701
  10. Macaulay WL (1968) Lymphomatoid papulosis. A continuing self-healing eruption, clinically benign - histologically malignant. Arch Dermatol 97: 23-30
  11. Markeeva E et al. (2011) Lymphomatoid Papulose Type A: Is less (therapy) more? Act Dermatol 37: 19-21
  12. Martires KJ et al (2015) Characterization of Primary Cutaneous CD8+/CD30+ Lymphoproliferative Disorders. At J Dermatopathol 37:822-833
  13. Meena M et al (2014) Lymphomatoid papulosis type C of the eyelid in a young girl: a case report and review of literature. Orbit 33:395-398
  14. Nijsten T et al (2004) Lymphomatoid papulosis in children. A retrospective cohort study of 35 cases. Arch Dermatol 140: 306-312
  15. Xiong J et al (2015) Lymphomatoid papulosis: a clinicopathologic study of 22 cases.
    Zhonghua Yi Xue Za Zhi 95):3750-3752.
  16. Zackheim HS et al (2003) Lymphomatoid papulosis associated with mycosis fungoides: a study of 21 patients including analyses for clonality. J At dermatol 49: 620-623

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Last updated on: 26.01.2021