A mutation in the KDSR gene has been reported to be associated with this condition. This gene encodes 3-ketodihydrosphingosine reductase, an enzyme in the ceramide synthesis pathway. The authors also reported that the use of systemic isotretinoin resulted in almost complete resolution of the lesions in two cases. Two other reports suggest that isotretinoin may be of use.
Progressive symmetric erythrokeratodermia
Congenital erosive and vesicular dermatosis
Subcutaneous granuloma annulare
Atrophia maculosa varioliformis cutis
Dermal dendrocyte hamartoma
Erythrokeratodermia variabilis
Relapsing linear acantholytic dermatosis
Nodular lichen myxedematosus