Acquired Perforating Dermatosis in Systemic Diseases

Summary

Acquired perforating dermatosis (APD) encompasses a group of skin disorders characterised by transepidermal elimination of altered dermal materials, most commonly collagen or elastic fibres. It typically manifests in adulthood and is strongly associated with systemic conditions such as chronic kidney disease, diabetes mellitus and other microvascular or metabolic disorders. Clinically, patients present with intensely pruritic, umbilicated papules or nodules bearing a central keratotic plug. Histopathological examination reveals cup-shaped depressions in the epidermis filled with necrotic debris and dermal elements. The pathogenesis remains incompletely understood but is thought to involve a combination of microcirculatory disturbances, deposition of metabolic by-products (for example advanced glycation end-products), mechanical trauma and immune dysregulation, including type 2 inflammatory pathways. The condition imposes a considerable burden on quality of life owing to persistent itch and potential for secondary infection. Management strategies focus on control of the underlying systemic disease, symptomatic relief with topical agents or phototherapy and, increasingly, targeted immunomodulatory therapies. Recent advances have begun to elucidate the molecular drivers of dermal extrusion and to explore novel treatments that address both skin manifestations and systemic contributors.

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Acquired Perforating Dermatosis in Systemic Diseases publication trend

The graph below shows the total number of articles in acquired perforating dermatosis in systemic diseases across all publications each year (not limited to Nature Index journals).

Technical terms

Transepidermal elimination: Process by which altered dermal substances are extruded through the epidermis.

Acquired perforating dermatosis (APD): A group of adult-onset perforating skin disorders associated with systemic disease.

Keratotic plug: Central crusted core in a skin lesion composed of necrotic debris and dermal fibres.

Type 2 inflammation: Immune response driven by cytokines such as interleukin-4 and interleukin-13, often linked to allergic and pruritic conditions.

Janus kinase inhibitor: Small-molecule drug that blocks JAK enzymes involved in cytokine signalling pathways.

References

  1. Dupilumab improve acquired reactive perforating collagenosis characterized by type 2 inflammation. Frontiers in Immunology (2023).
  2. Effectiveness of baricitinib in acquired reactive perforating collagenosis: a case report. Frontiers in Immunology (2024).
  3. A Case of Acquired Reactive Perforating Dermatosis with Complete Resolution of Eruptions on Upper and Lower Limbs During the Treatment of Diabetes Mellitus and Peripheral Artery Disease. Medicina (2024).

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