Topical trametinib for epidermal and sebaceous nevi in a child with Schimmelpenning-Feuerstein-Mims syndrome.

We present the case of a 20-month-old girl with Schimmelpenning-Feuerstein-Mims (SFM) syndrome with extensive head, neck, and torso skin involvement successfully managed with topical trametinib. Trametinib interferes downstream of KRAS and HRAS in the MAPK signaling pathway, of which KRAS was implicated in our child's pathogenic variant. Although other dermatologic conditions have shown benefit from oral trametinib, its topical use has not been well reported. Our patient showed benefit from the use of twice-daily topical trametinib, applied to the epidermal and sebaceous nevi over a 16-month period, leading to decreased pruritus and thinning of the plaques.

A case of inflammatory linear verrucous epidermal nevus successfully treated with Brodalumab.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a rare type of epidermal nevus, commonly arising in childhood. We present a case of a 13-year-old female with Blaschkoid psoriasiform plaques extending from her left foot to the scalp, sparing the right side of the body. While treatment options historically show variable success, we trialed an IL-17a receptor inhibitor as studies have shown increased levels of IL-17 receptor expression in ILVEN keratinocytes. At both 3 and 6 months after treatment initiation there was found to be significant improvement. We propose brodalumab as an effective treatment option for widespread ILVEN.

Bilateral Systematised Epidermolytic Epidermal Nevus: A case report.

Verrucous epidermal nevi (VEN) are benign congenital hamartomas consisting of keratinocytes. Histological examination mostly exhibits hyperkeratosis, acanthosis, papillomatosis and, rarely, the features of epidermolytic hyperkeratosis (EHK). We report a case of a 6-year-old boy who presented at Aga Khan University Hospital, Karachi, Pakistan with bilaterally symmetrical linear epidermal nevi following Blaschko's lines and showing epidermolytic hyperkeratosis on histology. The patient was treated with topical keratolytics and emolients which led to considerable improvement. To the best of the authors' knowledge, this is the first report of VEN from Pakistan.

Inflammatory linear verrucous epidermal nevus treated successfully with crisaborole ointment in a 5-year-old boy.

Inflammatory linear verrucous epidermal nevus (ILVEN) is a chronic, a linear, or whorled array of inflammatory, following the lines of Blaschko. Treatment of ILVEN is challenging with numerous therapies of varying degrees of success reported. We present a case of ILVEN in a 5-year-old-boy, treated successfully with crisaborole 2% ointment. This brief report suggests that there may be additional cellular immunologic pathways responsible for the presentation of ILVEN that may be explained by management with crisaborole use.

Inflammatory linear verrucous epidermal nevus associated with erosive monoarthritis.

Inflammatory linear verrucous epidermal nevus and linear psoriasis are different entities with clinical and histopathologic similarities. Isolated reports of inflammatory linear verrucous epidermal nevus with concomitant psoriasis or a positive family history of psoriasis have been described, and the possibility that inflammatory linear verrucous epidermal nevus may be a mosaic form of cutaneous psoriasis has been postulated. We report a 17-year-old boy with a congenital, linear, erythematous, keratotic plaque on the dorsum of the fifth finger of the left hand with ipsilateral nail dystrophy. Histopathologic examination showed epidermal hyperplasia with alternating orthokeratosis and parakeratosis. During follow-up, he developed erosive monoarthritis of the distal interphalangeal joint. This case seems to confirm the association between inflammatory linear verrucous epidermal nevus and arthritis and supports a possible relationship between inflammatory linear verrucous epidermal nevus and psoriasis.

An inflammatory verrucous epidermal nevus concomitant with psoriasis: treatment with adalimumab.

A 42-year-old-man presented with generalized inflammatory linear verrucous epidermal nevus with concomitant psoriasis vulgaris. Because of unresponsiveness of the conventional treatment, we considered treatment with adalimumab. Adalimumab was well tolerated and no side effects were observed. Adalimumab has a limited effect in treating ILVEN lesions. However, it was effective in treating the psoriasis and pruritus related to ILVEN. For these reasons adalimumab can be used in treatment of ILVEN concomitant with psoriasis.

Topical photodynamic therapy for nevus sebaceous on the face.

Recently, topical photodynamic therapy (PDT) has been tried to treat sebaceous gland disorders. However, only one case of nevus sebaceous treated with PDT has been reported. Our aim was to investigate the outcomes of PDT on nevus sebaceus on the face. A total of 12 patients were treated with topical 20% ALA or methyl aminolevulinate (MAL) after CO(2) laser ablasion. The lesions were irradiated with light emitting diodes (LED) device. The regimen was delivered repeatedly at 1 to 4 week intervals to each patient. Clinical improvement was visually assessed at 1 month after treatment by the lesional responses; No response was defined as less than 25%, mild improvement as 25-50%, moderate improvement as 51-75% and marked improvement as more than 75% decrease of lesional volume. All 12 patients showed mild (3 patients, 25%), moderate (7 patients, 58%), and marked (2 patients, 17%) improvement of the lesions. However, 2 patients showed partial recurrences after completion of treatment. There was no significant side effect.These results suggest that topical PDT may be considered as an effective alternative treatment modality of nevus sebaceous on the face.