Vijay K. Somani

Somani Skin Care Centre, India

Title: Primary localized cutaneous amyloidosis ? A review


Primary localized cutaneous amyloidosis (PLCA) refers to the deposition of amyloid in the skin without systemic involvement. It is characterized by pruritus, hyperpigmentation, and lichenification. Three types, macular, lichen/papular, and nodular are recognized. Diagnosis is primarily clinical and is confirmed by demonstration of amyloid in dermis by various techniques.  The etiopathology is not clear yet, but several factors like friction, sunlight, genetics, atopy, immune dysregulation have been implicated. Majority of cases of PLCA are sporadic but a family history may be present in up to 10% of cases.  Pathogenic mutations in oncostatin M receptor and the interleukin (IL)-31 receptor A gene (IL31RA) may lead to apoptosis of keratinocytes and resultant amyloid deposition in both familial and sporadic PLCA. Macular amyloidosis is the most frequent type seen and favours women. None of the treatment modalities, is curative or uniformly effective. The clinical presentation, and the diagnostic and therapeutic challenges posed by PLCA will be discussed.