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Chronic lichenoid keratosis

What is it keratosis lichenoides chronica?

Keratosis lichenoides chronica describes violaceous keratotic papules and nodules arranged in a linear or reticulate pattern on the trunk and extremities, in association with a seborrheic dermatitis-I like it eruption on the face [1,2].

Keratosis lichenoides chronica is also called Nekam's disease, lichen verrucous and reticularis. lichenoid tri-keratosis, keratosa, lichenoides striatus, pororatosis striata, morbo moniliformis and lichen ruber moniliformis.

Who gets keratosis lichenoides chronica?

Chronic keratosis lichenoides is rare, with only around 70 cases reported in the medical literature (2019) [1]. It can occur in people of any race, age, or sex. Most cases have been described in adults aged 20 to 40 years; 24% were boys [2]. The male/female ratio is 1.73 [2]. The majority of reported cases are Caucasian.

What causes keratosis lichenoides chronica?

The cause of keratosis lichenoides chronica is not well understood. Previously thought to be a rare variant of lichen planus, it is now considered by many to be a distinct condition [1].

Some family Cases of keratosis lichenoides chronica are due to a germ line mutation in NLRP1, an inflammasome sensor gene that activates inflammatory cytokines. The aberrant activation of NLRP1 leads to located get rid of interleukin (IL)-1, secondary secretion of tumor necrosis alpha factor (TNF-α) and keratinocytes growth factor (KGF), resulting in epidermal hyperplasia and keratoses [3,4].

What are the clinical features of keratosis lichenoides chronica?

Keratosis lichenoides chronica is characterized by a thick, brownish-purple scaly papules or small nodules on the trunk and extremities. The papules are usually arranged in a linear or lattice pattern and are distributed symmetrically. They are usually asymptomatic but it can cause itching in 20% cases. Chronic lichenoid keratosis has a chronic And often progressive course [1].

In 70% cases, patients also present with facial lesions resembling seborrheic dermatitis or rosacea. The lesions are papules or plates with variable hyperkeratotic climbing that tend to be located in the convex areas of the face. The nasolabial folds are almost always spared [2].

Other features associated with keratosis lichenoides chronica include [2]:

  • Palmoplantar keratoderma (in 28% cases)
  • Mucous membrane involvement with oral and genital ulceration, presenting as recurrent aphthous ulcers (28%): These are more common in adults than children
  • Engagement of the nails with dystrophy, discoloration, longitudinal ridges, hyperkeratosis of the nail bed, or paronychia (27%)
  • Ocular involvement with blepharitis, conjunctivitis, uveitisor iridocyclitis (20%)
  • no scars alopecia of the eyebrows and scalp (7%): this most commonly affects children.

Chronic keratosis lichenoides has also been reported concurrently with renal illness, diabetes, hypothyroidism, lymphoma, hepatitisand tuberculosis [5].

What are the complications of keratosis lichenoides chronica?

Complications of keratosis lichenoides chronica include visual impairment from ocular and secondary manifestations. bacterial infection [2].

How is keratosis lichenoides chronica diagnosed?

Criteria for diagnosing keratosis lichenoides chronica have not been well established [1].

The main differential diagnosis it is lichen planus; Characteristics that distinguish keratosis lichenoides chronica from lichen planus are the absence of pruritus, a lack of response to a current or systemic corticosteroids and the presence of focal parakeratosis in histological samples

A skin biopsy It is recommended for the histological diagnosis of chronic lichenoid keratosis. Histology typically displays:

  • Variable epidermal changes, which may include hyperkeratosis, follicular Parakeratosis and alternate areas of acanthosis and atrophy.
  • A lichenoid reaction pattern with necrosis of keratinocytes and vacuole degeneration in the basal cap.
  • A mixed inflammatory infiltrate of lymphocytes, plasma cells, histiocytesand occasionally eosinophils.

Direct immunofluorescence it is typically negative [6].

What is the differential diagnosis for keratosis lichenoides chronica?

Various other conditions may present with pigmented papules and lichenoid histology.

  • Hypertrophic lichen planus – characterized by itching and follicular accentuation of hyperkeratosis, which does not occur in keratosis lichenoides chronica [2]

  • Lichen planopilaris: associated with scarring alopecia, a feature never reported in keratosis lichenoides chronica [1]

  • Discoid lupus erythematosus, also associated with scarring alopecia

Cutaneous lupus is typically photosensitive, while keratosis lichenoides chronica generally improves with sun exposure [1].

What is the treatment for keratosis lichenoides chronica?

Keratosis lichenoides chronica is highly resistant to available therapies. A recent review found that most patients experience improvement with sun exposure and that oral administration retinoids (eg, acitretin) and phototherapy, specifically photochemotherapy (PUVA), either alone or in combination, are the most effective treatments [2].

Topical agents, such as steroid creams, are generally ineffective. Systemic agents that have been reported to be helpful in some patients include:

  • Systemic corticosteroids
  • Sulfones (eg, dapsone)
  • Methotrexate
  • antimalarial agents

  • Cyclosporine

What is the result of keratosis lichenoides chronica?

Keratosis lichenoides chronica is very difficult to treat and, in most cases, remission is never achieved. Complete resolution has never been reported [2].