Cutaneous lymphoid hyperplasia

Cutaneous lymphoid hyperplasia
Synonyms	Borrelial lymphocytoma, lymphadenosis benigna cutis, lymphocytoma cutis, pseudolymphoma, pseudolymphoma of Spiegler and Fendt, sarcoidosis of Spiegler and Fendt, Spiegler–Fendt lymphoid hyperplasia, Spiegler–Fendt sarcoid
Pronounce
Field	Dermatology
Symptoms	Erythematous papules, nodules, skin plaques
Complications	Secondary infection, cosmetic concerns, rare progression to lymphoma
Onset	Can develop at any age
Duration	Chronic or recurrent
Types	Nodular, bandlike, perivascular
Causes	Immune response, infections, medications, unknown in some cases
Risks	Genetic predisposition, tick-borne infections, medication use
Diagnosis	Skin biopsy, immunohistochemistry, PCR testing
Differential diagnosis	Lymphoma, lupus erythematosus, cutaneous sarcoidosis
Prevention	Avoidance of tick bites, proper infection management
Treatment	Corticosteroids, antibiotics, immunosuppressants
Medication	Topical and systemic therapies
Prognosis	Generally benign, can be chronic
Frequency	Rare
Deaths	None reported directly from condition

Cutaneous lymphoid hyperplasia (CLH), also known as lymphocytoma cutis or pseudolymphoma, is a group of benign skin conditions characterized by localized accumulations of lymphocytes, macrophages, and dendritic cells in the skin. These conditions mimic cutaneous lymphoma but lack the malignant characteristics of true lymphomas.

Cutaneous lymphoid hyperplasia can arise as a reactive immune response to infections, medications, insect bites, or other stimuli. It may also occur without a known trigger, making it a complex dermatological entity. While generally benign, it is essential to differentiate it from cutaneous lymphoma, which can have a similar clinical presentation.

Clinical Presentation

The symptoms of cutaneous lymphoid hyperplasia vary based on the subtype but typically include:

• Skin lesions – Red to purple papules, nodules, or plaques that may be asymptomatic or slightly itchy.
• Localized or widespread involvement – Lesions may be solitary or clustered, occurring anywhere on the body but commonly affecting the face, ears, upper chest, and extremities.
• Chronic or recurrent course – Symptoms may persist or reappear over time.
• Secondary infection risk – Due to persistent skin lesions, there is a risk of bacterial superinfection.

Types of Cutaneous Lymphoid Hyperplasia

Cutaneous lymphoid hyperplasia is classified into different patterns based on histological findings:

1. Nodular Pattern This form is characterized by localized clusters of erythematous to violaceous papules or nodules. These lesions are painless and may resemble cutaneous lymphoma or inflammatory skin disorders.

2. Bandlike and Perivascular Patterns In these cases, dense lymphocytic infiltrates form a bandlike pattern in the dermis. This pattern closely resembles mycosis fungoides, a type of cutaneous T-cell lymphoma. The lesions may present as patches, plaques, or nodules.

Jessner’s Lymphocytic Infiltrate

A closely related condition, Jessner’s lymphocytic infiltrate of the skin, is a benign and chronic skin disorder presenting as:

• Papular and plaque-like skin eruptions affecting the face, neck, and back.
• Lesions that persist or recur over time.
• An unclear cause, though some cases may be linked to genetic predisposition or immune system dysfunction.

Jessner’s lymphocytic infiltrate may be considered a form of lupus erythematosus tumidus, an autoimmune condition affecting the skin.

Causes and Risk Factors

Cutaneous lymphoid hyperplasia can develop due to various factors:

• Infections – Particularly tick-borne diseases such as Lyme disease, which can trigger borrelial lymphocytoma.
• Immune system reactions – Chronic antigenic stimulation from unknown triggers.
• Medications – Certain drugs, including ACE inhibitors and multiple sclerosis treatments like glatiramer acetate, may cause lymphocytic skin reactions.
• Insect bites – Bites from ticks, mosquitoes, and other arthropods may lead to reactive lymphoid hyperplasia.
• Tattoo reactions – Pigment particles in tattoos have been linked to pseudolymphoma-like reactions in the skin.

Diagnosis

Proper diagnosis is crucial to differentiate CLH from malignant lymphoma. Diagnostic methods include:

• Clinical examination – Evaluating the appearance, distribution, and persistence of lesions.
• Skin biopsy – Histopathological analysis shows dense lymphocytic infiltrates without atypical malignant cells.
• Immunohistochemistry – Helps distinguish between reactive lymphoid hyperplasia and cutaneous lymphoma.
• Polymerase Chain Reaction (PCR) testing – Used to detect Borrelia burgdorferi DNA in suspected Lyme disease-related cases.

Differential Diagnosis

Cutaneous lymphoid hyperplasia can resemble several other skin conditions, including:

• Cutaneous lymphoma – Mycosis fungoides, B-cell lymphoma, Sézary syndrome.
• Lupus erythematosus – Chronic autoimmune skin disease.
• Sarcoidosis – A granulomatous inflammatory disorder affecting multiple organs.
• Drug-induced eruptions – Skin reactions caused by medications.

Treatment and Management

The treatment of cutaneous lymphoid hyperplasia depends on the underlying cause and the severity of symptoms.

1. Observation and Monitoring

• In cases where lesions are mild and asymptomatic, a watch-and-wait approach may be sufficient.

2. Medications

• Topical and intralesional corticosteroids – Reduce inflammation and improve lesion appearance.
• Systemic corticosteroids – Used for more extensive or persistent cases.
• Antibiotics – If Lyme disease or bacterial infection is suspected.
• Immunosuppressants – Occasionally prescribed for refractory cases.

3. Physical Therapies

• Cryotherapy – Freezing lesions with liquid nitrogen.
• Laser therapy – Used for persistent or cosmetically concerning lesions.
• Surgical excision – Rarely needed but may be considered for large, disfiguring, or resistant nodules.

Prognosis

Cutaneous lymphoid hyperplasia is generally benign and does not progress to cancer. However, because it closely resembles cutaneous lymphoma, ongoing monitoring is recommended, especially in cases where lesions persist or evolve.

• Most cases resolve with treatment or spontaneously regress.
• Recurrence is possible, particularly in Jessner’s lymphocytic infiltrate.
• Complications are rare but may include secondary infections or scarring in chronic cases.

Prevention

While cutaneous lymphoid hyperplasia cannot always be prevented, some measures can help reduce risk:

• Avoiding tick bites – Wearing protective clothing and using insect repellent in tick-prone areas.
• Managing infections early – Prompt treatment of bacterial or viral infections that may trigger immune responses.
• Monitoring medication effects – Being aware of potential drug-induced reactions.

See Also

• Lymphoma
• Lupus erythematosus
• Cutaneous sarcoidosis
• Skin lesion
• Lyme disease
• Tick bite

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