Schimmelpenning Syndrome

A full-term newborn delivered vaginally with birth weight of 3240 g presented to us with linear verrucous plaques involving face, scalp, upper back and upper limbs. Few plaques were erythematous to skin-colored while some were hyperpigmented (Fig. 1). Whole left ear was covered with plaques. Histopathological examination showed hyperkeratosis, focal parakeratosis, follicular plugging, irregular acanthosis, papillomatosis and focal fusion of rete ridges. The patient was diagnosed as Schimmelpenning syndrome. Echocardiography revealed patent ductus arteriorus (PDA) for which the patient was reffered to Pediatric Cardiology services.

Fig. 1 Erythematous skin-coloured linear verrucous plaques present over face, scalp and ear.

Schimmelpenning syndrome (SS) is a congenital neurocutaneous disorder encompassing the cutaneous nevi (nevus sebaceous of Jadassohn) in conjunction with extracutaneous (neurologic, skeletal, cardiovascular, ophthalmic, and urologic) anomalies. It is a type of epidermal nevus syndrome with nevus sebaceous in blashkoid distribution as the hallmark lesion and cerebral abnormalities as the most common systemic findings (66%). Vascular anomalies are found in 12.6 to 33% of patients with SS. Nevus syringocystadenomatosus papilliferus, which is a sweat gland adenoma, can be differentiated from SS as lesions are pink and nodular. Surgical excision of nevus is the mainstay of treatment.