The article provides a current overview of the correlation between a rare dermatosis — subcorneal pustulosis (Sneddon — Wilkinson disease) — and various diseases of internal organs; features of immunological changes; the role of desmocollin 1, chemoatractants, interleukin-1β, interleukin-6, interleukin-8, interleukin-10, leukotriene B4, tumour necrosis factor-alpha and K5a complement fraction in the pathogenesis of the disease. In this work, the authors describe the features of the clinical picture of the disease, as well as diagnostic criteria, including the use of serum protein electrophoresis. Histological features of the disease are discussed in detail. In addition, modern methods for treating this disease, including the use of dapsone, acitretin, infliximab, PUVA therapy are presented.