FAMILIAL INFLAMMATORY LINEAR VERRUCOUS EPIDERMAL NEVUS (ILVEN)

Four members in three generations of a Bedouin family were affected wi th inflammatory linear verrucous epidermal nevus (ILVEN) (Fig. 1). Cas e 1 (Figure 1:IV1): A 9-year-old Bedouin boy was first seen in the Der matology outpatient clinic, Al-Sabah Hospital, Kuwait. He was a produc t of a consanguinous marriage and was born by normal vaginal delivery. The parents noticed diffuse erythema that persisted for few days all over the body at birth. At the age of 1 month, very small erythematous papules arranged in linear fashion were observed on the trunk and lim bs; they slowly increased in size and distribution. Since the age of 3 years the lesions have remained unchanged, and no improvement after v arious topical applications was noticed. The lesions were extremely pr uritic. The child had normal developmental milestones. On examination he was of average build. There were multiple (systematized) linear pso riasiform and verrucous plaques distributed bilaterally along the line s of Blaschko over both the extremities and the trunk (Fig. 2). Face, scalp, nails, and mucosae were spared. His general physical and system ic examination were within normal limits. The clinical diagnosis of IL VEN, verrucous epidermal nevus, and linear porokeratosis were consider ed. Histopathologic examination of a skin biopsy revealed psoriasiform dermatitis with mild spongiosis (Fig. 3). There were areas of orthohy perkeratosis with a broadened granular layer alternating with areas of parakeratosis with an absent granular layer. The upper dermis showed a perivascular lymphohistiocytic infiltrate with dilated blood vessels . These findings were consistent with ILVEN. Other routine investigati ons including complete blood count, serum biochemistry tests, liver an d kidney function tests, ECG, and a radiograph of the chest were withi n normal limits. Case 2 (Fig. 1:IV2): The 3-year-old male sibling of c ase 1 was noticed to have small erythematous papules arranged in linea r fashion. Slowly they increased in size and distribution. On examinat ion he had bilaterally distributed psoriasiform and verrucous plaques, like his brother, arranged along the lines of Blaschko, predominantly on the upper and lower extremities and a few on the trunk (Fig. 4). U ntil his last follow-up, new lesions were still appearing on the trunk . The parents refused a skin biopsy from this child Other routine inve stigations were normal. Case 3 (Fig. 1:III4): A 32 year-old woman (mot her of the above two cases), had linear, psoriasiform plaques on the l ateral border of the right hand at the junction of palmar and dorsal a spects since early childhood. Histopathologic examination of a skin bi opsy revealed changes classical of ILVEN (Fig. 5). The mother stated t hat her father (Fig. 1:II6) (not alive) had been also suffering from t he same disease in its systemized form.