Description of a clinical case of bullous scleroderma in a somatically impaired patient

Abstract

Bullous scleroderma is a rare variant of localized scleroderma characterized by the formation of subepidermal tense blisters. At the moment, the etiology and pathogenesis of dermatosis is not fully understood, but a number of authors consider the pathology of the endocrine system as a trigger for the development of localized and systemic scleroderma.

Untimely or erroneous diagnosis of bullous scleroderma leads to the risk of developing a systemic process, irreversible cosmetic defects and even disability.

Treatment of bullous scleroderma should be complex and multicourse with the inclusion of systemic glucocorticoids, antibacterial and cytostatic drugs, agents that improve microcirculation, as well as topical glucocorticoids of the 3-4th class of activity, regenerating agents. Physiotherapeutic methods of treatment such as PUVA and UVA therapy are widely used; there are reports on the successful use of a combination of immunosuppressive mycophenolate mofetil and extracorporeal photopheresis, as well as intravenous infusions of N-acetylcysteine. In addition to traditional drug and physiotherapy therapies, the use of biological therapies is currently being considered.

The article presents a rare case of scleroderma bullosa on the background of pronounced chronic endocrine pathology. The interest of this clinical case lies not only in the rare form of the disease and the progressive nature of the course of dermatosis, but also in the probable association of dermatosis with the patient's endocrine gland diseases in the form of panhypopituitarism due to adenohypophysis macroadenoma with the development of secondary hypothyroidism and adrenal insufficiency. In our observation, the steady negative dynamics of the skin process — from localised plaque scleroderma with subsequent transformation into the bullous form, and further into a probable systemic process — drew attention.

Taking into account the severity of the disease course, the presence of concomitant chronic endocrinopathy, as well as positive specific serological reactions for the systemic form of scleroderma, in the presented clinical case the patient needs an interdisciplinary approach and constant dispensary observation.

Keywords

bullous scleroderma / etiology / diagnosis and treatment

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