What are the other Names for this Condition? (Also known as/Synonyms)

• Cutaneous Lymphoid Hyperplasia
• Lymphadenosis Benigna Cutis
• Pseudolymphoma of Spiegler-Fendt

What is Lymphocytoma Cutis? (Definition/Background Information)

• Lymphocytoma Cutis is a benign inflammatory condition characterized by an abnormal accumulation of lymphocytes (type of white blood cells) on the skin, causing it to resemble a cutaneous lymphoma (a malignant condition). Thus, it is described as a type of “pseudolymphoma”
• Lymphocytoma Cutis is a rare condition that is usually observed in young adult Caucasian males. The cause of development of the condition is unknown. It is reportedly a reactive process, caused by triggering factors such as trauma, tattoo inks, insect bites and stings, and certain infections. Nevertheless, in many individuals, no triggers are observed. A diagnosis of Lymphocytoma Cutis involves skin biopsy
• Following a diagnosis, the healthcare provider may choose to undertake a watchful waiting or treat the condition via topical and systemic steroids, phototherapy, or surgical excision. The prognosis is generally good since the condition is not progressive, although some individuals are at an increased risk for malignancy (development of lymphoma of skin)

Who gets Lymphocytoma Cutis? (Age and Sex Distribution)

• Lymphocytoma Cutis is a rare skin condition that is mostly seen in young adults below the age of 40 (median age 34 years). It is generally uncommon in children and adults over 40 years of age
• Even though both males and females are affected, a male predominance is observed (male-female ratio is 3:1)
• Worldwide, all racial or ethnic groups are at risk. However, a vast majority of the cases are noted in Caucasians (fair-skinned individuals)

What are the Risk Factors for Lymphocytoma Cutis? (Predisposing Factors)

In some cases, the triggering factors for Lymphocytoma Cutis may include:

• Acupuncture
• Earrings and body piercings
• Tattoo inks and dyes
• Insect stings and spider bites
• Infections caused by the following:
  • Human immunodeficiency virus (HIV)
  • Herpes simplex virus (HSV)
  • Herpes zoster virus (shingles)
  • Varicella zoster (chickenpox)
  • Borrelia burgdorferi bacterium (Lyme disease)
• Desensitization injections
• Certain medications such as antihypertensives, anticonvulsants, antidepressants, and lipid-lowering agents
• Scabies
• Trauma
• Vaccinations

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Lymphocytoma Cutis? (Etiology)

Currently, the exact cause of formation of Lymphocytoma Cutis is unknown. It is classified as a pseudolymphoma since it presents features of lymphoma of skin.

• Lymphocytoma Cutis is also known as Cutaneous Lymphoid Hyperplasia since there is an accumulation (infiltration) of lymphocytes beneath the skin layers. This abnormal accumulation (hyperplasia) may be moderate or severe, and can be present as soft or firm nodules under the skin
• It is believed to be a hyperplastic reaction due to certain triggering agents such as infections, vaccinations, gold piercings, and insect bites. However, in many cases, no precipitating factors may be established for the condition, when it is termed idiopathic Lymphocytoma Cutis

According to medical research, pseudolymphomas are categorized as either B-cell type or T-cell type. Lymphocytoma Cutis is described as a B-cell form of pseudolymphoma.

What are the Signs and Symptoms of Lymphocytoma Cutis?

The signs and symptoms of Lymphocytoma Cutis may include:

• Presence of shiny papules or nodules below the skin
• In nearly three-fourths of the cases, the lesions may be solitary or few in numbers (localized form); a large number of lesions (disseminated form) are not generally observed
• These lesions may be red, purple, violet, brown, or even skin-colored
• They may present dry scaly or crusted appearance, and may be soft or firm
• Itching or soreness is not usually observed
• Many individuals develop the lesions on the face (over 70% of the cases); other body sites affected include the chest and arms

How is Lymphocytoma Cutis Diagnosed?

A diagnosis of Lymphocytoma Cutis is made using the following tools:

• Physical examination and evaluation of medical history - often, a healthcare provider may suspect the condition based on the attributes and behavior of the lesion. However, a skin tissue biopsy is needed to conform a diagnosis of Lymphocytoma Cutis
• Dermoscopy: It is a diagnostic tool where a dermatologist examines the skin using a special magnified lens
• Wood’s lamp examination: In this procedure, the healthcare provider examines the skin using ultraviolet light. It is performed to examine the change in skin pigmentation
• Imaging studies, such as CT or MRI scans, if necessary
• Positron emission tomography (PET) scan can detect small collection of fast-growing cells, which may not be seen on a CT scan
• Skin tissue biopsy: A tissue biopsy is performed and sent to a laboratory for a pathological examination. The pathologist examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis

A differential diagnosis to eliminate other skin conditions may be needed before arriving at a definitive diagnosis. These may include:

• Angiolymphoid hyperplasia
• Cutaneous lupus erythematosus
• Cutaneous lymphoma
• Jessner lymphocytic infiltrate 
• Polymorphous light eruption, plaque form
• Rosacea
• Sarcoidosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Lymphocytoma Cutis?

The complications of Lymphocytoma Cutis may include:

• Emotional stress from cosmetic concerns
• Individuals with Lymphocytoma Cutis have an elevated risk for developing lymphoma of skin, a cancerous growth of the lymphoid tissue
• Treatment-associated complications

How is Lymphocytoma Cutis Treated?

The treatment of Lymphocytoma Cutis mainly involves identifying and removing any offending agent or triggering factor. Further, based on the healthcare provider’s evaluation, a symptomatic treatment of the skin signs and symptoms may be undertaken via the following measures:

• Wait and watch approach
• Use of topical steroid creams
• Intralesional corticosteroid injections
• Oral and systemic steroids, if necessary
• Phototherapy (PUVA) and photodynamic therapy (delta ALA)
• Superficial or localized radiation therapy
• Administration of medications such as hydroxychloroquine
• Excisional surgery and cryosurgery, where possible

Regular follow up visits with the healthcare provider is important and recommended.

How can Lymphocytoma Cutis be Prevented?

• Currently, there are no available preventative measures or guidelines for Lymphocytoma Cutis
• Avoiding the triggering factors may help reduce one’s risk for the same

What is the Prognosis of Lymphocytoma Cutis? (Outcomes/Resolutions)

• Lymphocytoma Cutis is typically a benign and frequently non-progressive condition. even though a spontaneous resolution of the condition is not known to occur, it may be managed with appropriate treatments
• Individuals with Lymphocytoma Cutis have an increased risk for transformation of the condition to a malignancy (cutaneous lymphoma), and thus long-term follow-up is necessary

Additional and Relevant Useful Information for Lymphocytoma Cutis:

The following DoveMed website link is a useful resource for additional information:

https://www.dovemed.com/healthy-living/skin-disorders/