Clinical case

A 18 years old female presented with a 3 years-history of polymorphic skin lesions, at inferior limbs

Clinical features

Laboratory

- an inflammatory syndrome

(VSH = 40 mm/h).

Lymphomatoid papulosis – a case reportDr Georgescu Mihaela*, Dr Margaritescu Irina***SUUMC “Carol Davila”, Bucharest, ** Domina Sana Medical Center, Bucharest

Skin biopsyThe pathology showed:- a mixed “wedge-shape” infiltrate containing large atypical cells admixed  with small lymphocytes, histiocytes and neutrophils, - the large cells present large, highly irregular, hyperchromatic, pleomorphic nuclei, - a periecrinal glands disposition,- a scar area at the reticular dermis without the lymphomatoid infiltrate, - the atypical lymphomatoid cells are CD 30+.

Treatment

- PUVA 3x/week

- topical steroidsFig.1 Clustered hemorrhagic and crusted papules of lymphomatoid papulosis on left leg.

Fig.2&3 Hyperpigmented macules and superficial atrophic (varioliform) scars of lymphomatoid papulosis on patient’s extremities

Diagnosis- lymphomatoid papulosis tip A

Histopathology

Figure 4,5 Fragment of cutaneous biopsy with mixed “wedge-shape” infiltrate containing large atypical cells admixed  with small lymphocytes, histiocytes and neutrophilsFigure 6. Lymphomatoid papulosis type A lesion with predominance of large atypical lymphoid cells, displaying polymorphic nuclei, prominent nucleoli, and abundant cytoplasmFigure 7,8 Numerous CD 30+ cells are present.

Figure 5. HEx200

Figure 6. HEx400

Figure 7. HEx200

Figure 8. HEx400

Lymphomatoid papulosis – a case reportDr Georgescu Mihaela*, Dr Margaritescu Irina***SUUMC “Carol Davila”, Bucharest, ** Domina Sana Medical Center, Bucharest Fig.4 HEx50

Lymphomatoid papulosis – a case reportDr Georgescu Mihaela*, Dr Margaritescu Irina***SUUMC “Carol Davila”, Bucharest, ** Domina Sana Medical Center, Bucharest

Introduction

Lymphomatoid papulosis is a peculiar condition, caractherized by a chronic, recurrent, self-healing papulonecrotic or papulonodular eruption with histopathological features suggestive of a (CD 30 – positive) malignant lymphoma

Clinical features

• typical skin lesions are red-brown papules and nodules that may develop central hemorrhage, necrosis and crusting, which disappear

within 3-8 weeks • characteristically, lesions are often at different stagesof development

The pathogenesis

• unknown

• a possible viral-driven etiology• a low-grade lymphoma induced by chronic antigenic stimulation• studies show a high rate of apoptosis contributing to regression, mediated by death-receptor pathway signaling via cell surface Fas (CD95) signaling and/or due to increased levels of the proapoptotic protein bax. Mutations of TGF-β signaling receptor genes results in disease progression

Treatment

- active treatment -not

necessary (few non-scarring lesions) - no treatment has proven consistently effective- beneficial effects from:• PUVA• topical mechlorethamine or carmustine• low-dose etoposide

Differential diagnosis

• viral infections (herpes virus, molluscum contagiosum, parapox virus (milker's nodule), Epstein-Barr virus, HTLV1, HIV )• scabies • syphilis • superficial fungal infections• pityriais lichenoides et varioliformis acuta• atopic dermatitis• drug reactions (particularly to anticonvulsants)• mycosis fungoides or a lymphomatoid drug reaction (type B  lymphomatoid papulosis)  

Histological types Differential diagnosis

Type A - Scattered CD30+, large Hodgkin lymphoma

Type B - Epidermotropic CD30-/+ small Mycosis fungoides

Type C - Cohesive sheets CD30+, large ALCL

Type D - Epidermotropic CD30+ CD8+ small AECTCL (Berti lymphoma)

Type E - Angioinvasive CD30+ CD8+>CD4+ Extranodal NK/T, GD-TCL

Conclusions

The disease is now classified as an indolent lymphoma

in the new WHO classification  

The clinicopathological and immunohistochemical correlation is essential in establishing the diagnosis

The evolution of the disease is characterized by recurrence

References1. McKee’s Pathology of the Skin, Chapter 29 – Cutaneous lymphoproliferative diseases and related disorders, John Goodlad, Eduardo Calonje, Lymphomatoid papulosis, 4th edition, Elsevier, 20122. Skin Lymphoma: The Illustrated Guide,Lorenzo Cerroni et al, CD30+ lymphoproliferative disorders. Lymphomatoid papulosis3rd Edition, Wiley, 20093. Dermatology, By By Jean L. Bolognia et al., Chapter 120. Primary cutaneous CD30-positive lymphoproliferative disorder. Lymphomatoid papulosis, 3rd Edition, Elsevier, 20124. Self assessmentCourse in Virtual Dermatopathology, Dr. Werner Kempf, Case 02, EADV Congress, Istanbul, 2-6 oct 2013

Lymphomatoid papulosis – a case reportDr Georgescu Mihaela*, Dr Margaritescu Irina***SUUMC “Carol Davila”, Bucharest, ** Domina Sana Medical Center, Bucharest