Familial lichen planus ANOTHER DISEASE OR A DISTINCT PEOPLE?

Abstract

When lichen planus strikes families, a very rare happening, it is likely to afflict younger members, to erupt more acutely, extensively, and gravely, attacking also nails and mucous membranes, and to recur. Ten patients with familial lichen planus, two each from five distinct Caucasian families, the parents of whom were unrelated by birth, were found to be carrying HLA-B7 statistically more frequently than in the normal population or in those with the characteristic forms of lichen planus. We hint that their genotype might have rendered them susceptible to a pathogen that precipitated their disease.

A retrospective and comparative study of familial and non-familial bullous lichen planus

“…This echoes the observation in FLP by others [6,7,11] . Hereditary factors may play a role in BLP, which may be helpful in a full understanding of the etiology and pathogenesis of BLP.…”

“…The other etiological factors of LP can also be psychological stress, infection, smoking, drug and hypoxia. Copeman et al [11] reported that when LP strikes families, it is more likely to afflict younger members, to erupt more acutely, extensively, and heavily, to attack nails and mucous membranes, and to recur more often. Mahood [7] described 9 members in 4 families and his results suggested that these patients appeared to differ from the non-familial form by their earlier age of onset, tendency to chronicity, increased severity, and the frequent atypical clinical presentations.…”

Lichen planus in children

“…Copeman et al found an association between familial LP and HLA-B7. [16] In a subsequent study by White and Rostom in Arab people, an association of HLA-DR1 and DR10 with lichen planus was observed. [17] The occurrence of LP in monozygotic twins indicates toward genetic linkage of LP.…”

Frontal fibrosing alopecia and lichen planopilaris in HLA-identical mother and daughter

“…[12] Copeman et al [13] found an association of HLA-B7 in familial cases but not in sporadic cases. Other associations include HLA-DR1, which seems to be correlated with peptide anchorage and presentation of specific T helper lymphocytes.…”