Clinical and histopathological characteristics of primary cutaneous amyloidosis in 794 Chinese patients.

BACKGROUND

Primary cutaneous amyloidosis (PCA) is not uncommon in Chinese patients. The disease is usually persistent and quite pruritic. Patients who suffer from this disease usually respond poorly to conventional treatment. We thus reviewed our cases of PCA to discuss the clinical and pathological characteristics.

METHODS

Seven hundred and ninety-four Chinese patients with PCA who visited the Department of Dermatology, Taipei Veterans General Hospital during the last 26-year period were examined and retrospectively studied. The diagnosis in these patients was confirmed by histopathological studies.

RESULTS

Among the many types of PCA, lichen amyloidosis was the most common clinical variant (67%). Pure cases of macular amyloidosis accounted only 8% and were often associated with lichenoid lesions to form biphasic amyloidosis, which was composed of 25% in our series. Other rare types of PCA, such as nodular, anosacral, and vitiliginous amyloidosis, always required a careful differential diagnosis clinically from other similar skin disorders. In addition, 56 familial cases were found. Histopathologically, the most common epidermal findings of PCA were hyperkeratosis, irregular acanthosis with thinning of rete ridges, and expansion of dermal papillae by amyloid deposition. Special histochemical stains were helpful for confirming the existence of amyloid.

CONCLUSIONS

Our study represents the largest number of cases of PCA collected to date. Based on the data, most cases are sporadic, except 56 familial cases which may suggest the possible genetic role. Rare types of PCA, such as anosacral and vitiliginous amyloidosis which need special attention, compose a diagnostic challenge to a dermatologist. Histochemically, H&E stain can give a primary clue for the diagnosis of amyloidosis and crystal violet stain is a very simple and sensitive method to detect the existence of amyloid.