Sneddon-Wilkinson subcorneal pustulosis or IgA pemphigus?

Background. Sneddon-Wilkinson’s subcorneal pustulosis is a rare chronic relapsing disease; single descriptions of this pathology in pediatric practice make any such case an important source of information. Over the past 5 years we followed-up 4 patients who required differential diagnosis with this dermatosis. This article presents the most difficult case. Description of the clinical case. A 17-year-old girl with severe protein-caloric deficiency against the background of organic lesions of the central nervous system and lifelong antiepileptic therapy was admitted with progressive lesions of the skin; trigger for the occurrence of skin lesion was the Mycoplasma pneumoniae respiratory infection. Clinical signs and histopathological characteristics of skin lesions were consistent with those of Sneddon-Wilkinson subcorneal pustulosis, but the detection of IgA antibodies in the epidermis during immunohistochemical study favored IgA pemphigus, serological detection of which is not widely available. Also, the choice of management tactics was not easy, since systemic glucocorticosteroids are recommended for patients with pemphigus, but in the treatment of neutrophilic dermatoses, preference is given to the sulfone preparations. Conclusion. The presented case allows to discuss the difficulties of differential diagnosis and the need to revise the approaches to classification and therapy.

subcorneal pustular dermatosis, Sneddon-Wilkinson pustulosis, neutrophilic dermatoses, intraepidermal neutrophilic IgA dermatosis, pustular psoriasis, IgA pemphigus