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                Myxoid Tumours of Soft Tissue

    Dr  Sampurna Roy  MD

 
 
     DermPath-India

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 Dr. Sampurna Roy MD

          

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Lymphomatoid papulosis (LyP) is a recurrent hemorrhagic papular skin eruption with a clinically benign course and histopathologic features of lymphoma.

The lesions usually present as papules and nodules that spontaneously involute.

nitially the lesions are smooth but later these lesions become crusted, ulcerated and necrotic.

Lymphomatoid papulosis can only be diagnosed accurately through a careful history in which the characteristic waxing and waning of the skin lesions is identified and through proper communication between clinicians and pathologists.

 Visit:  Primary  Cutaneous  Lymphoma ;  Cutaneous Pseudolymphoma Role of immuno-histochemistry  in Dermatopathology

Age: Usually occurs in the third and fourth decade of life. This lesion may also occur  in children.

Site: Trunk ; Proximal parts of the limb ; face ; scalp ; palms and sole .

Microscopic features:  Image Link(Dr Weems)   ;

 Subtypes-  LyP Type A ; LyP Type B ; LyP Type C.     

 LyP Type A : Wedge-shaped, superficial and deep mixed infiltrate of small lymphocytes, abnormal lymphocytes and, often, plasma cells, neutrophils, and eosinophils ; Abnormal lymphocytes with  large pleomorphic nuclei ; Binucleate abnormal lymphocytes that resemble Reed-Sternberg's cells and multinucleate abnormal lymphocytes; There is no epidermotropism .

 LyP Type B : Perivascular or band like dermal infiltrate; Small to medium sized lymphocytes with cerebriform nuclei;  There is epidermotropism. (Features resemble plaque stage mycosis fungoides).

 LyP Type C :  Monotonous population of large atypical cells ;  There are fewer inflammatory cells. (Features resemble ALCL).

 Image Link1  ; Image Link2 (CD30+cells)

Immunohistochemistry:

Large Atypical cells( LyP type A & C): CD30+ ; CD3+ ; CD2 -/+ ;  CD4 +/- ; CD8 - ; CD15 and EMA are usually negative.

Atypical cells (LyP typeB): CD3+ ; CD4+ ; CD8 - ; Cells do not express CD30.

Differential diagnosis: Differentiating between mycosis fungoides and anaplastic large cell lymphoma and Hodgkin's disease may be very difficult .

Mycosis fungoides consists of abnormal lymphocytes that may be identical to those of lymphomatoid papulosis, but infiltrates tend to be bandlike, not wedge-shaped. No neutrophils are present as a rule in mycosis fungoides.

Lesions of Hodgkin's disease usually do not display a wedge-shaped infiltrate and exhibits Reed-Sternberg's cells consistently.

Clinical, microscopic and immunohistochemical features of lymphomatoid papulosis and anaplastic large cell lymphoma (ALCL) may overlap.

Both Primary cutaneous CD30-positive ALCL and lymphomatoid papulosis are characterized by CD30-positive large atypical cells predominantly of T-cell origin.

Insect bites exhibit a wedge-shaped infiltrate composed of lymphocytes and eosinophils. Slightly abnormal lymphocytes may be present but these are not in mitosis.

 Pityriasis lichenoides et varioliformis acuta. Click here (Dr Weems)

              

Abstract:

CD8+ lymphomatoid papulosis and its differential diagnosis. Am J Clin Pathol. 2006;125(4):490-501.

Oral involvement in lymphomatoid papulosis. Report of two cases and review of the literature.Dermatology. 2005;210(1):53-7.

Lymphomatoid papulosis associated with recurrent cutaneous T-cell lymphoma.Ann Acad Med Singapore. 2004;33(1):110-2.

Lymphomatoid papulosis from childhood with anaplastic large-cell lymphoma of the small bowel.Clin Lymphoma. 2004;5(3):190-3.

Lymphomatoid papulosis associated with mycosis fungoides: clinicopathological and molecular studies of 12 cases.Acta Derm Venereol. 2004;84(6):463-8

Lymphomatoid papulosis: reappraisal of clinicopathologic presentation and classification into subtypes A, B, and C. Arch Dermatol. 2004 ;140(4): 441-7.

Lymphomatoid papulosis type C or transition to CD30+-T-cell lymphoma? A difficult differential diagnosis.Hautarzt. 2003;54(4):366-8.

Lymphomatoid papulosis and cutaneous CD30+ lymphoma. Am J Dermatopathol. 1996;18(3):221-35.

A comparison of clinical, morphological and immunohistochemical features of lymphomatoid papulosis and primary cutaneous CD30(Ki-1)-positive anaplastic large cell lymphoma.J Cutan Pathol. 1995;22(4):310-8.

Lymphomatoid papulosis in children.J Am Acad Dermatol. 1995;33(5 Pt 1):741-8.

Lymphomatoid papulosis: a T-cell dyscrasia with a propensity to transform into malignant lymphoma.Ann Intern Med. 1995;122(3):210-7.

Lymphomatoid papulosis: a clinical and histopathologic review of 53 cases with leukocyte immunophenotyping, DNA flow cytometry, and T-cell receptor gene rearrangement studies.J Am Acad Dermatol. 1994;30(2 Pt 1):210-8.

Lymphomatoid papulosis evolving into malignant lymphoma. Clinico-pathological and immunohistochemical study of 3 cases.Med Clin (Barc). 1993;100(6):220-2.

 

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