There may be a family history of the disorder with an autosomal recessive pattern of inheritance. Skin Early - Recurrent vesicles, bullae and hemorrhagic crusts occur around mucous membranes and on the skin, particularly the face and distal extremities. These heal with ice pick scarring and can continue into the late teens. Late - The skin becomes waxy, thickened and yellow. Papules, plaques and nodules develop on the face, axillae and scrotum, coinciding with deposits in the dermis.
Verrucous lesions can occur on the elbows, knees and sites of trauma.
The skin may develop a generalized hyperkeratosis. Scalp - Patchy areas of alopecia may develop where hyaline deposits occur. Oral cavity - A cobblestone appearance may develop with multiple papules on the tongue, lips and gingiva. The tongue may feel hard, with a woody induration and ulceration. There may be transient swelling of the lips and tongue.