Lymphomatoid papulosis (LyP) is a rare
skin disorder. The overall prevalence rate of lymphomatoid papulosis is estimated at 1.2 to 1.9 cases per 1,000,000 population. [This is a widespread misinterpretation of a 1992 study saying "the period prevalence rate of lymphomatoid papulosis was estimated to be 1.9 per 1,000,000 population for Massachusetts and 1.2 per 1,000,000 population for Pennsylvania". The authors of that study said clearly "Our estimate of 1.2-1.9 cases per 1,000,000 population should be considered a minimum estimate of the prevalence rate". That estimate was based on the 78 patients involved in the study, not the LvP population. The study recruited 11 patients from Massachusetts and 15 from Pennsylvania
]. This rare condition has only been studied in depth since 1968.
Presentation
It can appear very similar to anaplastic large cell lymphoma.
Type "A" is
CD30
CD30, also known as TNFRSF8 ( TNF receptor superfamily member 8), is a cell membrane protein of the tumor necrosis factor receptor family and a tumor marker.
Function
This receptor is expressed by activated, but not by resting, T and B cel ...
positive, while type "B" is CD30 negative.
It has been described as "clinically benign but histologically malignant."
Treatment
It may respond to
methotrexate or PUVA.
Prognosis
It can evolve into
lymphoma
Lymphoma is a group of blood and lymph tumors that develop from lymphocytes (a type of white blood cell). In current usage the name usually refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enla ...
.
See also
*
Cutaneous T-cell lymphoma
*
Parapsoriasis
*
Secondary cutaneous CD30+ large cell lymphoma
*
List of cutaneous conditions
References
External links
{{Papulosquamous disorders
Rare diseases
Lymphoid-related cutaneous conditions