The Schimmelpenning-Feuerstein-Mims (SFM) syndrome is a rare phakomatosis which comprises a nevus sebaceous of Jadassohn, seizures and developmental delay associated with a wide spectrum of extracutaneous abnormalities including neurological, skeletal, ocular, cardiovascular and urogenital defects. We are presenting a case of an 18-year-old patient with systemic features of the SFM syndrome and...

In 1906 Jadassohn and Lewandowsky described a rare genodermatoses named it pachyonychia congenita. Pachy means thick. It is group of involving keratin mutation with thickened nails variable associated findings. Pachyonychia congenita disease having four types. However, type 1 2 can be considered common among them. often mimic fungal infection as nail hyperkeratosis occur in both.

Dermoscopy disclosed a sharply demarcated lesion corresponding to the jelly-sign (red arrows). Additionally, multiple small and loosely arranged brown globules (blue circles) resembling the so-called dermoscopic “concentric structures,” were seen (Figure 2). A punch biopsy of the lesion was performed. Histopathological examination revealed multiple heavily pigmented intra-epidermal nests of bas...

El nevo sebáceo de Jadassohn se presenta como una lesión hamartomatosa, extensión variable. Puede estar presente al nacimiento y generalmente localiza en cuero cabelludo. La zona alopecia. En raras ocasiones puede malignizarse la edad adulta. Se un caso neonatal hace revisión literatura.
 Conflicto interés: Los autores declaran no poseer conflicto interés
 Recibido: 01/12/2020 Aceptad...

PC is commonly described as a rare genodermatosis characteristically manifesting as massive subungual hyperkeratosis with nail thickening, focal palmoplantar keratoderma alongwith deep f issur ing and blistering, oral leukokeratosis and discrete follicular hyperkeratosis [1,2]. Other features including abnormalities of teeth, hairs and larynx can also be seen depending on the clinical types. On...