clinical case a 18 years old female presented with a 3 years- history of polymorphic skin lesions,...
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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