Lymphomatoid papulosis
| Classification according to ICD-10 | |
|---|---|
| C86.6 | Papulosis lymphomatoides |
| ICD-10 online (WHO version 2019) | |
The lymphomatoid papulosis is a skin Affected benign T-cell lymphoma pseudo .
Skin changes in the form of papules are symptomatic . These can take on very different shapes: in color from pink to dark brown, sometimes with the formation of necrosis , rarely more than five in number. The papules usually heal on their own within a few weeks. However, this usually leaves a small scar. In contrast to other cutaneous T-cell lymphomas, the CD30 surface molecule is expressed in lymphomatoid papulosis (CD30 +).
Lymphomatoid papulosis was first described in 1968 by WL Macaulay as a disease that causes “ histologically malignant and clinically benign skin lesions” . In the meantime, three subtypes (A to C) of lymphomatoid papulosis are differentiated, the differentiation from other subspecies is the subject of current research.
Overall, it is a very rare disease. An incidence of around 1.2 to 1.9 new cases per 1,000,000 people is assumed. The resulting very low awareness of the disease and the mostly harmless course lead to a significant number of incorrectly diagnosed cases.
Differential diagnosis is Mycosis fungoides delimit, whereby a parallel occurrence is possible; furthermore chickenpox , syphilis , allergic vasculitis and many more
The prognosis is very good. Complete remissions are seen even with long-term follow-up. In a single-center study that retrospectively observed 299 patients with cutaneous lymphomas over the period from January 1980 to December 2005, an optimal 5-year survival rate of 100% was determined for lymphomatoid papulosis. On the other hand, the risk of developing malignant lymphoma appears to be slightly increased. A study by Bekkenk et al with 118 patients reported a progression after 10 years in 4%.
There is currently no curative treatment. The resulting skin changes can be combated with a PUVA treatment. Corticoids are also used with moderate success. Topical treatment attempts with various active ingredients are carried out, including the drugs imiquimod and tacrolimus . In severe relapsing cases, systemic treatment using low-dose methotrexate may be considered.
Web links
- Images of lymphomatoid papules
- Systematics, diagnostics and therapy of cutaneous lymphomas , guidelines (2016) including those of the German Dermatological Society (DDG) in the online portal of the Working Group of Scientific Medical Societies (AWMF) accessed on November 17, 2017
Sources / literature
- ↑ WL Macaulay: lymphomatoid papulosis. A continuing self-healing eruption, clinically benign - histologically malignant . In: Arch Dermatol . tape 97 , no. 1 , January 1968, p. 23-30 , doi : 10.1001 / archderm.97.1.23 , PMID 5634442 .
- ↑ D. Korpusik, T. Ruzicka: Clinical forms and therapy of lymphomatoid papulosis. In: The dermatologist . 58, 2007, pp. 870-881, doi: 10.1007 / s00105-007-1295-z .
- ↑ a b M. W. Bekkenk, FA Geelen, PC van Voorst Vader et al .: Primary and secondary cutaneous CD30 + lymphoproliferative disorders: a report from the Dutch Cutaneous Lymphoma Group on the long-term follow-up data of 219 patients and guidelines for diagnosis and treatment. In: Blood . 2000; 95, pp. 3653-3661.
- ↑ DermIS: Diagnosis of Lymphomatoid Papulosis
- ^ Christian Hallermann, Christoph Niermann, Rudolf-Josef Fischer, Hans-Joachim Schulze: Survival Data for 299 Patients with Primary Cutaneous Lymphomas: A Monocentre Study. In: Acta Dermato-Venereologica . 2011.
- ↑ A. Gyulay, T. Kahl et al. a .: Lymphomatoid papulosis: is imiquimod a therapeutic alternative? In: Current Dermatology. 30, 2004, doi : 10.1055 / s-2004-832552 .
