Intravascular histiocytosis of the skin

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Abstract

Intravascular histiocytosis is a rare dermatologic condition characterized by pathological vascular dilation and accumulation of histiocytes within the lumen of blood vessels. Despite its rarity, challenges in diagnosis and treatment persist due to the absence of standardized protocols and a limited understanding of the disease pathogenesis.

A key initiating factor is damage to the vascular wall, promoting histiocyte migration and proliferation within vessels. This process may be triggered by infections, cardiovascular dysfunction, hepatic or renal pathology. However, some cases occur in individuals without comorbidities, suggesting the involvement of unidentified triggers or genetic predispositions.

Clinical manifestations vary and include ill-defined erythematous to violaceous patches, plaques, nodules, and papules, most commonly affecting the trunk and extremities. The lesions may also present as hemorrhagic nodules and papules, complicating the differential diagnosis with other dermatoses. The condition’s rarity and non-specific presentation often result in misdiagnosis and inappropriate treatment, worsening the clinical course.

Currently, there is no universal treatment protocol for intravascular histiocytosis. Corticosteroids are frequently used, but their efficacy and long-term outcomes remain poorly defined.

This article describes a clinical case of a female patient presenting with papular eruptions on the neck and scalp. The diagnosis was confirmed by dermatoscopy and histological examination of a skin biopsy. The case highlights the need for further research to establish standardized diagnostic and therapeutic strategies for intravascular histiocytosis.

About the authors

Dmitriy V. Bulanov

The Russian National Research Medical University named after N.I. Pirogov

Email: dbulanov81@gmail.com
ORCID iD: 0009-0005-3772-6643
SPIN-code: 2641-6658

MD, Cand. Sci. (Medicine), Associate Professor

Russian Federation, Moscow

Julia S. Podverbnaya

The Russian National Research Medical University named after N.I. Pirogov

Author for correspondence.
Email: julia_123_julia_123@mail.ru
ORCID iD: 0009-0002-9236-6714
SPIN-code: 1653-2617
Russian Federation, Moscow

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2. Fig. 1. Dermatoscopic picture of intravascular skin histiocytosis: erythematous-papular element of the skin of the anterior surface of the neck. [Photo from the archive of the Russian National Research Medical University named after N.I. Pirogov. Published for the first time with the permission of the administration of the institution].

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3. Fig. 2. Microscopic examination (staining with hematoxylin and eosin, ×200): in the papillary layer of the dermis, ectatized, full-blooded capillary-type vessels are detected with the presence of groups of closely spaced histiocytes with basophilic nuclei and eosinophilic cytoplasm in the lumen (arrows). [Photo from the archive of the Russian National Research Medical University named after N.I. Pirogov. Published for the first time with the permission of the administration of the institution].

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4. Fig. 3. Microscopic examination (staining with hematoxylin and eosin, x200): periadnexal and perivascular lymphocytic infiltrates are detected in the reticular layer of the dermis (arrows). [Photo from the archive of the Russian National Research Medical University named after N.I. Pirogov. Published for the first time with the permission of the administration of the institution].

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