3.3.13 Lymphomatoide Papulose
Grading & Level of Importance: C
Continuing self-healing eruption, clinically benign, histologically malignant
Prevalence 0.1-0.2 /100'000; all age groups; predominantly young adults; M>F.
Chronic recurrent , self-healing papulo-nodular skin eruption with histologic features of a malignant anaplastic lymphoma; CD30 (Ki-1) positive.
Nosologic relationship to Pityriasis lichenoides et varioliformis acuta (PLEVA), to other CD30 positive malignant T-cell lymphomas and to Hodgkin’s disease.
Aetiology & Pathogenesis
Signs & Symptoms
Disseminated papules and/or small nodules, which within days or a few weeks become red‐brown, hemorrhagic or pustular and finally undergo ulceration, followed by complete spontaneous regression of the lesion, occasionally leaving behind hyper‐ or hypopigmented varioliform scars.
Trunk, buttocks, extremities.
Histological and immunocytochemical subtypes: Types A-E and Type 6p25.3 (genetic).
Laboratory & other workups
Biopsy; immunocytochemistry (CD30).
Broad spectrum with variably dense infiltrates of medium‐sized to large atypical pleomorphic CD30+ cells are the hallmarks of the disease. Depending on the lesion's stage of evolution, the histological presentation is different. In fresh lesions, there is a wedge‐shaped infiltrate of tumor cells with ulceration.
Various subtypes (see classification).
Chronic recurrent over decades.
Transformation to Hodkin’s disease. Threat of overtreatment due to confusion with malignant anaplastic lymphoma.
Clinically; typical spontaneous regressions and recurrences of lesions.
Malignant anaplastic T-cell ymphoma (CD30 positive); CD30-positive pseudolymphomatous reactions (scabies); Hodgkin’s disease (histology).
Prevention & Therapy
Prevention ot possible. The prognosis is quad vitam excellent; permanent healing not possible, only temporaring clearing.Follow up with respect to possible transformation to Hodgkin’s disease (very rare).
Temporar clearing: Methotrexat (10-20mg/week); photo- or photochemotherapy.
Skin care and prevention of superinfection; erosive lesions may be treated with antibiotic weak glucocorticosteroid crème.
It is important to avoid aggressive systemic (cytostatics, bone marrow transplantation) therapy, based on misdiagnosis.
Wichtig ist die Vermeidung aggressiver, systemischer (Zytostatika; Knochenmarktransplanation) Behandlungen bei Fehldiagnose.
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