Lymphomatoide Papulose

ICD-11

LP4; LP44

Synonyms

Continuing self-healing eruption, clinically benign, histologically malignant

Epidemiology

Prevalence 0.1-0.2 /100'000; all age groups; predominantly young adults; M>F.

Definition

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

Unknown.

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.

Localisation

Trunk, buttocks, extremities.

Classification

Histological and immunocytochemical subtypes: Types A-E and Type 6p25.3 (genetic).

Laboratory & other workups

Biopsy; immunocytochemistry (CD30).

Dermatopathology

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).

Course

Chronic recurrent over decades.

Complications

Transformation to Hodkin’s disease. Threat of overtreatment due to confusion with malignant anaplastic lymphoma.

Diagnosis

Clinically; typical spontaneous regressions and recurrences of lesions.

Differential diagnosis

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.

Special

It is important to avoid aggressive systemic (cytostatics, bone marrow transplantation) therapy, based on misdiagnosis.

3.3.13 Lymphomatoide Papulose