article in PDF
Our Dermatol Online. 2014; 5(4): 408
Date of submission:  23.09.2014 / acceptance: 27.09.2014
Conflicts of interest: None

Gladys Alejandra Paguaga, Orlando Rodas Pernillo, Helga María Sarti


Ass. Prof. Małgorzata Sokołowska-Wojdyło MD, PhD, MSc

Department of Dermatology, Venereology and Allergology, Medical University of Gdansk, Gdansk, Poland
How to cite this article: Sokołowska-Wojdyło M. Comment: Type D Lymphomatoid Papulosis: An uncommon Variant. A case report and review of the literature. Our Dermatol Online. 2014; 5(4): 408.

The interesting article touches the unfamiliar for most of doctors problem: the existence of skin lymphoproliferative disease which is malignant in histopathology but mild in course: lymphomatoid papulosis (LyP) It presents with papules in the skin of the trunk and the extremities. Those papules can appear scaly and typically develop superficial, central necrosis. They also tend to heal spontaneously (most often in 3-12 weeks). LyP mainly affects adults (the median age at diagnosis is 45 years), with a slight predominance of males but children are also affected [1]. Histologically, what was mentioned by Paguaga et al., LyP is generally divided into the following 4 subtypes:
– type A is characterized by large CD30+ atypical cells intermingled with a prominent inflammatory infiltrate consisting of histiocytes, small lymphocytes, granulocytes and eosinophiles;
– type B is characterized epidermotropism and infiltration with smaller atypical cells with hyperchromatic cerebriform nuclei resembling the atypical lymphocytes in Mycosis fungoides (MF), and of antigen composition of C-ALCL.;
– type C is characterized by sheets of CD30+ anaplastic large lymphocytes;
– type D is characterised by infiltrates similar to those in CD8+ aggressive epidermotropic lymphoma or/and resembling pagetoid reticulosis (CD8+ CD30+, sometimes CD4+, CD56+).
The malignant histopathological features do not correlate with chronic / mild course. That is why the diagnose can not be determined only on the base of them. The clinical symptoms analysis is necessary to avoid the overtreatment because the diagnose of Hodgkin disease, MF or aggressive epidermotropic lymphoma instead LyP. That is why the every case of unusual or rare type of LyP is worth of publication – to remind the dermatologists, oncologists, haematologists and pediatricins that this dermatose exist. The problem is even more difficult because other histopathological subtypes of LyP, not mentioned in the publication of Paguaga et al., were described so far ex. granulomatous and eccrinotropic [2-4]. Those two subtypes can be discussed as subtypes of typ B LyP – analogously to MF and ex. pilotropic, syringotropic subtype. Type D of LyP can even imitate the pagetoid reticulosis type of MF (before known as Woringer-Kolopp type). There is also possibility that, as in Mycosis fungoides – where 31 clinical subtypes were described, there are also other subtypes of LyP, not only those four named A-D [5]. The authors mentioned also other important problem – LyP have an increased risk for developing a nonlymphoid tumour or, more commonly, a lymphoma (10-20%). It can be primary cutaneous lymphoma [6]. That is why there are cases of coexistence of LyP and CD30+ cutaneous or systemic T-cell lymphoma with secondary cutaneous involvement, which should be not misdiagnosed [7]. The authors rightly noted that there are no clinical or pathological features predicting increased risk for developing malignancy. That is why the observation of LyP patients should be very vigilant.
1. Weaver J, Mahindra AK, Pohlman B, Jin T, Hsi ED. Non-mycosis fungoides cutaneous T-cell lymphoma: reclassification according to the WHO-EORTC classification. J Cutan Pathol. 2010;37:516-24.
2. Jain N, Gutte R, Jadhav P, Khopkar U. Granulomatous and eccrinotropic lymphomatoid papulosis. Indian J Dermatol Venereol Leprol. 2012;78:82-4.
3. Kempf W. CD30+ lymphoproliferative disorders: histopathology, differential diagnosis, new variants, and simulators. J Cutan Pathol. 2006;33 Suppl 1:58-70.
4. Morimura S, Sugaya M, Tamaki Z, Takekoshi T, Sato S. Acta Derm Venereol. 2011;91:712-3.
5. Requena L, González-Guerra E, Angulo J, DeVore AE, Sangueza OP. Anetodermic mycosis fungoides: a new clinicopathological variant of mycosis fungoides. Br J Dermatol. 2008;158:157-62.
6. Min JA, Oh ST, Kim JE, Cho BK, Chung NG, Park HJ. Lymphomatoid papulosis followed by anaplastic large cell lymphoma in a pediatric patient. Ann Dermatol. 2010;22:447-51.
7. Marschalkó M, Eros N, Holló P, Hársing J, Bottlik G, Bátai A, et al. Secondary ALK negative anaplastic large cell lymphoma in a patient with lymphomatoid papulosis of 40 years duration. Am J Dermatopathol. 2010;32:708-12.

Comments are closed.