What Is Lymphomatoid Papulosis?
Lymphoma-like papulopathy is a chronic, recurrent, self-healing, papular necrosis, or papular nodular skin disease. The basic damage is pimples and small nodules, which are clinically similar to acute pimples-like pityriasis, but have histological characteristics of skin T-cell lymphoma. Another 10% to 20% of patients may develop another type of lymphoma one after the other. The latest WHO-EORTC classification considers the disease to be a low-grade cutaneous T-cell lymphoma. In the new WHO hematopoietic and lymphoid tissue tumor classification, it belongs to the CD30 + skin lymphoproliferative disease lineage.
Basic Information
- English name
- lymphomatoid papulosis
- Visiting department
- dermatology
- Multiple groups
- 20 to 40 years old
- Common locations
- Occurs on the trunk and limbs
- Common symptoms
- Skin red pimples
Causes of lymphoma-like papules
- The etiology is unknown and the pathogenesis is unknown.
Clinical manifestations of lymphoma-like papules
- It is common in adults 20 to 40 years old. The primary lesion is a red pimples, up to about 1 cm in diameter, which can progress to papular blistering, papular pustular, or hemorrhagic. After several days and weeks, surface necrosis. Typical damage can heal within 8 weeks. Most chronic processes are untreated, new lesions are constantly appearing, so skin lesions of different periods can coexist. Acne-like, hyperpigmented, or hypopigmented scars remain after the damage has healed. Symptoms are mild, with a course of 3 months to 40 years. 10% to 20% of patients with lymphoma-like papulopathy can develop MF-CD30 skin T-cell lymphoma or Hodgkin disease.
Lymphoma-like papules
- It mainly depends on skin histopathological examination, which has diagnostic and typing value for this disease. The pathological manifestations are divided into A, B, and C types. The three types can appear in a patient at the same time, or appear at different stages of the disease, and there are individual skin lesions that can have the characteristics of the three types.
- 1. Type A (histocellular type)
- Intradermal infiltration is wedge-shaped, consisting of scattered or clustered anaplastic large cells mixed with small lymphocytes, histiocytes, neutrophils, and eosinophils. Large anaplastic cells do not exceed 50% of the infiltrating cells. Rich in quality, similar to tissue-like cells, sometimes multinucleated giant cells similar to RS cells can be seen, mitotic phases are common, and epidermis-like phenomenon is optional.
- 2.B type (mushroom-like granulomatous type)
- This type is rare (less than 10%). The epidermis is often atrophic, and the dermal infiltration is wedge-shaped or band-shaped. The infiltrating cells are composed of small to medium-sized polymorphic lymphocytes, which are irregular in shape, mostly cerebral gyrus, deeply stained, epidermal, and pathologically similar to mushroom Like granuloma, diagnosis needs to be combined with clinical.
- 3. Type C (anaplastic large cell lymphoma-like type)
- This type is considered the borderline of lymphoma-like papulopathy-anaplastic large cell lymphoma. It is characterized by more monomorphic, CD30 + large T cell infiltration, and less inflammatory cells. It is difficult to distinguish pathologically from anaplastic large cell lymphoma.
- Immunohistochemistry: Type A and C lymphoma-like papules disease tumor cells express CD30, cluster or large pieces, and most tumor cells are CD3 +, CD4 +, and CD8-. Type B tumor cells are only CD4 + and do not express CD30. PCR gene clone rearrangement was seen in more than 40% of lymphoma-like papules.
Lymphoma-like papulosis diagnosis
- According to clinical manifestations, skin lesion characteristics, histopathological characteristics, immunohistochemical examination can diagnose.
Lymphoma-like papules disease treatment
- Generally does not require treatment. There is no evidence that treatment can prevent the development of secondary lymphoma. But if the patient has symptoms, it can be treated appropriately. Such as potent corticosteroids; PUVA systemic or local treatment. Carmustine (carbazide) is applied topically to suppress damage without bone marrow suppression. Methotrexate (MTX) can significantly improve symptoms in 90% of patients.
Prognosis of lymphoma-like papules
- Typical damage can heal within 8 weeks. Most tend to be chronic, without treatment, new lesions are constantly appearing, so skin lesions of different periods can coexist. Acne-like, hyperpigmented, or hypopigmented scars remain after the damage has healed. Symptoms are mild, with a course of 3 months to 40 years.