| Lymphomatoid papulosis | |
|---|---|
| Specialty | Dermatology  |
Lymphomatoid papulosis (LyP) is a rare skin disorder.
| Lymphomatoid papulosis | |
|---|---|
| Specialty | Dermatology |
Lymphomatoid papulosis (LyP) is a rare skin disorder.
The overall prevalence rate of lymphomatoid papulosis is estimated at at least 1.2 cases per 1,000,000 population. [1] This rare condition has only been studied in depth since 1968. [2]
It can appear very similar to anaplastic large cell lymphoma. [3] Type "A" is CD30 positive, while type "B" is CD30 negative. [4]
It has been described as "clinically benign but histologically malignant." [5]
| A | Wedge-shaped clusters of large atypical lymphocytes that are CD30+, interspersed with a mixed inflammatory infiltrate of neutrophils, histiocytes, and eosinophils. |
|---|---|
| B | Similar to mycosis fungoides, with bandlike infiltrate and epidermotropism of smaller atypical lymphocytes that may be CD30-. |
| C | Similar to cutaneous anaplastic large-cell lymphoma, with larger clusters or sheets of large anaplastic CD30+ cells without the interspersed mixed infiltrate of Type A. |
| D | Similar to CD8+ epidermotropic cutaneous T-cell lymphoma, with large CD8+ and CD30+ lymphocytes that often stain with cytotoxic markers (TIA-1, granzyme, perforin). |
| E | Angioinvasive with small to large angiocentric CD30+ atypical lymphocytes that invade walls of small to medium vessels in dermis or subcutaneously. |
| F | Perifollicular infiltrates of CD30+ atypical cells with folliculotrophism with or without follicular mucinosis. |
It may respond to methotrexate or PUVA. [7]
Lymphoma is a group of blood and lymph tumors that develop from lymphocytes. The name typically refers to just the cancerous versions rather than all such tumours. Signs and symptoms may include enlarged lymph nodes, fever, drenching sweats, unintended weight loss, itching, and constantly feeling tired. The enlarged lymph nodes are usually painless. The sweats are most common at night.
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Panniculitis is a group of diseases whose hallmark is inflammation of subcutaneous adipose tissue. Symptoms include tender skin nodules, and systemic signs such as weight loss and fatigue.
Anaplastic large-cell lymphoma (ALCL) refers to a group of non-Hodgkin lymphomas in which aberrant T cells proliferate uncontrollably. Considered as a single entity, ALCL is the most common type of peripheral lymphoma and represents ~10% of all peripheral lymphomas in children. The incidence of ALCL is estimated to be 0.25 cases per 100,000 people in the United States of America. There are four distinct types of anaplastic large-cell lymphomas that on microscopic examination share certain key histopathological features and tumor marker proteins. However, the four types have very different clinical presentations, gene abnormalities, prognoses, and/or treatments.
A seborrheic keratosis is a non-cancerous (benign) skin tumour that originates from cells, namely keratinocytes, in the outer layer of the skin called the epidermis. Like liver spots, seborrheic keratoses are seen more often as people age.
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A dysplastic nevus or atypical mole is a nevus (mole) whose appearance is different from that of common moles. In 1992, the NIH recommended that the term "dysplastic nevus" be avoided in favor of the term "atypical mole". An atypical mole may also be referred to as an atypical melanocytic nevus, atypical nevus, B-K mole, Clark's nevus, dysplastic melanocytic nevus, or nevus with architectural disorder.
Parapsoriasis refers to one of a group of skin disorders that are characterized primarily by their resemblance to psoriasis, rather than by their underlying cause.
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Intravascular lymphomas (IVL) are rare cancers in which malignant lymphocytes proliferate and accumulate within blood vessels. Almost all other types of lymphoma involve the proliferation and accumulation of malignant lymphocytes in lymph nodes, other parts of the lymphatic system, and various non-lymphatic organs but not in blood vessels.
Lymphomatoid granulomatosis (LYG or LG) is a very rare lymphoproliferative disorder first characterized in 1972. Lymphomatoid means lymphoma-like and granulomatosis denotes the microscopic characteristic of the presence of granulomas with polymorphic lymphoid infiltrates and focal necrosis within it.
Florid cutaneous papillomatosis (FCP), is an obligate paraneoplastic syndrome.
An arsenical keratosis is a growth of keratin on the skin caused by arsenic, which occurs naturally in the Earth's crust and is widely distributed in the environment, Arsenical compounds are used in industrial, agricultural, and medicinal substances. Arsenic is also found to be an environmental contaminant in drinking water and an occupational hazard for miners and glass workers. Arsenic may also causes other conditions including: Bowen's disease, cardiovascular diseases, developmental abnormalities, neurologic and neurobehavioral disorders, diabetes, hearing loss, hematologic disorders, and various types of cancer. Arsenical keratoses may persist indefinitely, and some may develop into invasive squamous cell carcinoma. Metastatic arsenic squamous cell carcinoma and arsenic-induced malignancies in internal organs such as the bladder, kidney, skin, liver, and colon, may result in death.
Bowenoid papulosis is a cutaneous condition characterized by the presence of pigmented verrucous papules on the body of the penis. They are associated with human papillomavirus, the causative agent of genital warts. The lesions have a typical dysplastic histology and are generally considered benign, although a small percentage will develop malignant characteristics.
Secondary cutaneous CD30+ large-cell lymphoma is a cutaneous condition that may arise in cases of mycosis fungoides, and in patients with lymphomatoid papulosis.
Extranodal NK/T-cell lymphoma, nasal type (ENKTCL-NT) is a rare type of lymphoma that commonly involves midline areas of the nasal cavity, oral cavity, and/or pharynx At these sites, the disease often takes the form of massive, necrotic, and extremely disfiguring lesions. However, ENKTCL-NT can also involve the eye, larynx, lung, gastrointestinal tract, skin, and various other tissues. ENKTCL-NT mainly affects adults; it is relatively common in Asia and to lesser extents Mexico, Central America, and South America but is rare in Europe and North America. In Korea, ENKTCL-NT often involves the skin and is reported to be the most common form of cutaneous lymphoma after mycosis fungoides.
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Günter Burg is a German dermatologist. Born in Mayen, Germany, he holds German and Swiss citizenship. He has been married to Dr. Doris Burg-Nicklas, a neurologist, since 1968. They have two sons: Andreas and Thomas.