From the Guidelines
Perivascular lymphoplasmacytic dermatitis is a skin condition characterized by inflammation around blood vessels in the skin, specifically involving lymphocytes and plasma cells, as seen in conditions like adult onset Still's disease (AOSD) 1. This inflammatory pattern is not a specific diagnosis but rather a descriptive term used in dermatopathology (skin pathology) to describe what is seen under the microscope when examining skin biopsies. The inflammation typically occurs in the dermis layer of the skin, surrounding small blood vessels. This pattern can be found in various skin disorders including drug reactions, insect bite reactions, some viral exanthems, and certain autoimmune conditions, such as AOSD, which is characterized by high-spiking fevers, a characteristic rash, and arthritis/arthralgias 1. Some key features of AOSD include:
- High-spiking fevers exceeding 39°C, which are transient and typically last under 4 hours 1
- A characteristic rash that is an evanescent, salmon-pink, maculopapular eruption, predominantly found on the proximal limbs and trunk 1
- Arthralgia and arthritis, which are found in the majority of patients with AOSD, with incidences ranging from 64% to 100% 1 Patients may present with rashes, redness, or other skin manifestations depending on the underlying cause. Treatment is directed at the primary condition causing the inflammation rather than the dermatitis pattern itself, and may include topical corticosteroids, antihistamines, or other medications depending on the specific diagnosis. Understanding this pattern helps dermatologists and pathologists narrow down potential causes of skin disease and develop appropriate treatment plans for patients. In the context of AOSD, the presence of perivascular lymphoplasmacytic dermatitis is an important diagnostic clue, and treatment should be focused on managing the underlying disease process 1.
From the Research
Definition and Characteristics
- Perivascular lymphoplasmacytic dermatitis is a condition characterized by the presence of lymphoplasmacytic infiltrates in the skin, specifically around blood vessels 2.
- This condition is often associated with cutaneous plasmacytosis, a rare skin disorder marked by multiple reddish-brown nodules with polyclonal plasma cell proliferation 2.
- The infiltrates typically consist of a mix of lymphocytes and plasma cells, with the plasma cells being polyclonal in nature 2.
Clinical Features
- The clinical presentation of perivascular lymphoplasmacytic dermatitis can vary, but it often includes the presence of reddish-brown nodules or plaques on the skin, particularly on the trunk, face, and extremities 2.
- The lesions are usually asymptomatic, but may be associated with hypergammaglobulinemia and other systemic symptoms 2.
Histopathological Features
- The histopathological features of perivascular lymphoplasmacytic dermatitis include perivascular plasma cell infiltrates without light chain restriction, as well as mast cell infiltrates 2.
- The presence of acanthosis and interface dermatitis may also be observed in some cases 3.
- Granulomas and giant cells may be present in a subset of cases, but are not a universal feature 3.
Differential Diagnosis
- The differential diagnosis for perivascular lymphoplasmacytic dermatitis includes other conditions characterized by lymphoplasmacytic infiltrates, such as lymphoplasmacytic plaque and cutaneous plasmacytosis 3, 2.
- A thorough diagnostic work-up, including immunohistochemistry and molecular studies, may be necessary to distinguish between these conditions 3, 4.