Pagetoid reticulosis
Editor-In-Chief: Prab R Tumpati, MD
Obesity, Sleep & Internal medicine
Founder, WikiMD Wellnesspedia &
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Pagetoid reticulosis | |
---|---|
Synonyms | Woringer-Kolopp disease |
Pronounce | N/A |
Specialty | N/A |
Symptoms | Skin lesions, erythematous patches |
Complications | Potential progression to cutaneous T-cell lymphoma |
Onset | Typically in adulthood |
Duration | Chronic |
Types | N/A |
Causes | Unknown |
Risks | Immunosuppression, genetic predisposition |
Diagnosis | Skin biopsy, histopathology |
Differential diagnosis | Mycosis fungoides, Paget's disease of the breast |
Prevention | N/A |
Treatment | Radiation therapy, topical corticosteroids |
Medication | N/A |
Prognosis | Generally good with treatment |
Frequency | Rare |
Deaths | N/A |
A rare form of cutaneous T-cell lymphoma
Pagetoid reticulosis is a rare form of cutaneous T-cell lymphoma characterized by the presence of localized patches or plaques on the skin. It is considered a variant of mycosis fungoides, which is the most common type of cutaneous T-cell lymphoma.
Clinical Presentation
Pagetoid reticulosis typically presents as a solitary lesion, often on the extremities. The lesion is usually a well-demarcated, scaly plaque that may be mistaken for other dermatological conditions such as psoriasis or eczema. Unlike mycosis fungoides, pagetoid reticulosis does not typically progress to involve other areas of the skin or internal organs.
Histopathology
The histological hallmark of pagetoid reticulosis is the presence of atypical T-cells with a pagetoid spread within the epidermis. This means that the atypical cells are scattered throughout the epidermis in a pattern reminiscent of Paget's disease of the breast. The cells are often CD4 positive, and immunohistochemical staining is used to confirm the diagnosis.
Diagnosis
Diagnosis of pagetoid reticulosis is based on clinical examination, histopathological analysis, and immunophenotyping. A skin biopsy is essential to observe the characteristic histological features. Differential diagnosis includes other forms of cutaneous T-cell lymphoma, Bowen's disease, and extramammary Paget's disease.
Treatment
Treatment options for pagetoid reticulosis are generally localized due to its indolent nature. These may include:
- Topical corticosteroids
- Phototherapy
- Localized radiation therapy
- Surgical excision
In most cases, the prognosis is excellent, with a low risk of progression or systemic involvement.
See also
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Contributors: Prab R. Tumpati, MD