Lymphomatoid Papulosis

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Contents 1 Introduction 2 Etiology 3 Epidemiology 4 Pathology 5 Clinical-manifestations 6 Laboratory 7 Differential-diagnosis 8 Management 9 More General Terms 10 References

Introduction

• Asymptomatic, chronic, self- healing polymorphous eruption.

Etiology

• idiopathic
• considered to be a low grade lymphoma or a pseudolymphoma involving T-helper cells without systemic involvement

Epidemiology

• incidence: 1.2-1.9/million
• affects all ages; mean age 40 years
• affects both sexes equally
• occurs sporadically

Pathology

• lesions limited to skin
• low, but real risk of malignant transformation

• occasional progression to Hodgkin's disease or cutaneous T-cell lymphoma

• superficial or deep, perivascular or interstitial mixed cell infiltrate

• atypical cells may comprise 50% of infiltrate
• large, atypical histiocytic lymphocytes; occasional Reed-Sternberg like cells
• smaller, atypical lymphocytes

• epidermis:

• focal spongiosis
• mild exocytosis
• necrotic keratinocytes
• extravasated erythrocytes are frequent

• small vessel lymphocytic vasculitis (10%)

Clinical-manifestations

• generally asymptomatic
• occasionally lesions are pruritic, tender or painful
• NO weight loss, fever, night sweats
• lesions appear in crops of recurrent, self- healing eruptions
• lesions may resolve spontaneously at any point in their evolution
• some lesions may persist & produce scarring
• individual lesions evolve over 2-8 week period
• primary lesions are erythematous to red-brown with central hemorrhage & necrosis (black)
• lesions are papular with diameter 2 mm to 3 cm, a few to hundreds in number
• lesions are usually random, but may be grouped
• lesions occur primarily on trunk & extremities; rarely on oral or genital mucosa
• lesions may ulcerate
• atrophic scars may persist
• post- inflammatory hyperpigmentation or hypopigmentation may occur

Laboratory

• skin biopsy

• immunohistochemistry
• IL-2 receptor positive
• HLA- DR-positive
• CD30 positive
• CD4 positive

• negative workup for systemic involvement

Differential-diagnosis

• lymphoma cutis
• scabies
• histiocytosis X
• papular urticaria
• viral exanthem

Management

• may remit in 3 weeks or continue for decades
• may be periods or remission or continuous repetitive outbreaks
• no treatment is consistently effective
• pharmaceutical agents

• corticosteroids
• carmustine ( BCNU)
• tetracycline
• retinoids
• methotrexate
• chlorambucil
• cyclophosphamide
• cyclosporin
• interferon alpha-2b

• electron-beam radiation
• PUVA may control disease, but does NOT affect long-term prognosis

More General Terms

• skin disease (dermatologic disorder, dermatopathy, dermatosis)
• lymphoproliferative disorder

References

Color Atlas & Synopsis of Clinical Dermatology, Common & Serious Diseases, 3rd ed, Fitzpatrick et al, McGraw Hill, NY, 1997, pg 570-71

lymphomatoid papulosis