Lichen Planus, Familial

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WHO-ICD-10 version:2010

Diseases of the skin and subcutaneous tissue

Papulosquamous disorders

OMIM Number

151620

Mode of Inheritance

Autosomal dominant

Description

Lichen planus is an inflammatory mucocutaneous condition characterized by violet colored lesions. These lesions are best described by the six 'P's: pruritic, polygonal, planar, purple, papules, and plaques. The lesions commonly appear on the flexor surface of the extremities, notably the wrists. They also tend to occur in areas exposed to trauma, such as lacerations, a phenomenon known as isomorphic response. Apart from cutaneous surfaces, lichen planus lesions can also appear, sometimes exclusively so, on the oral mucosa, the genital mucosa, the nails, and the scalp. The WHO, meanwhile, has classified lichen planus as a potentially malignant disorder.

The prevalence of lichen planus is unknown, but it is estimated to occur in less than 1 percent of the population, with no racial predilection. It most commonly affects middle-aged adults of both sexes, with a slight predominance in women. Diagnosis of the condition is based on the clinical appearance and a punch biopsy of the lesions. Typically, the tissue under the microscope shows a band-like infiltrate of lymphocytes at the epidermal-dermal junction and damage to the basal cell layer.

Topical steroids are the mainstay of treatment in patients with early or localized disease. For patients with generalized disease, oral corticosteroids may be recommended. In some cases where the lesions are resistant to steroids, other treatment strategies, such as topical and systemic retinoids, and oral PUVA may be used.

The exact pathogenesis of lichen planus is unknown, although many studies support an immunologic pathogenesis. Lymphocytes, particularly T-cells, play a major role. While most cases of lichen planus are idiopathic, some are linked to medication use, infection with hepatitis C virus, or hepatitis B vaccination. Studies of familial cases of lichen planus have indicated that polymorphisms in certain genes, such as TNFR2 and P53 might be associated with providing susceptibility to the disease. However, this association is not clear.

Epidemiology in the Arab World

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Other Reports

Egypt

[See also Kuwait > Nanda et al., 2001].

Iraq

[See also Kuwait > Nanda et al., 2001].

Kuwait

Nanda et al. (2001) studied 23 consecutive cases of lichen planus in children (12 boys, 11 girls) seen over a period of seven years (1992-1997) to determine the salient features, treatment, and course of the disease. The study sample included 13 Kuwaiti children. Of the remaining patients, five were Bedouins, two Egyptians, and one each was Saudi and Iraqi in origin. The most common affected sites were found to be the extensors of the lower legs and ankles. Nine patients showed oral mucosal lesions. The most common variant was the classic lichen planus with violaceous, flat topped papules and plaques (16), followed by eruptive and widespread lichen planus (3), linear lichen planus (2), lichen planus pigmentosus (1), and lichen planus actinicus (1). A history of precipitating factors was found in five patients; four of them having had URT infections, and the fifth had viral exanthema. Family history was reported for one of the patients; her father also had lichen planus. Skin biopsy revealed basal cell degeneration, with a band-like lymphocyte infiltrate in the upper dermis, along with focal hypergranulosis and irregular acanthosis. Most patients showed a significant response to topical steroids. In patients with eruptive widespread disease, UVB phototherapy was found to be safe and effective.

Mauritania

Joly et al. (1998) described three patients of African ancestry, including at least one from Mauritania, with a similar form of lichenoid erythrodermic bullous pemphigoid. All patients had non-classical lichen planus pemphigoides and exhibited a severe lichenoid erythroderma associated with bullous pemphigoid lesions involving the skin and mucosa. Immunoblot analysis of sera detected anti-BPAG1 and anti-BPAG2 antibodies and immunoelectron microscopy showed IgG deposits localized in the lamina lucida and the hemidesmosomes. All three patients were natives of an area near the Senegal River and had the common HLA-DR10 haplotype. This observation hints for the possibility that this particular type of lichenoid erythrodermic bullous pemphigoid could be determined by genetic factors.

Saudi Arabia

[See also Kuwait > Nanda et al., 2001].

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