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