Progressive Nodular Histiocytosis Associated with Eale's Disease

Area of Science:

• Dermatology
• Ophthalmology
• Pathology

Background:

• Progressive nodular histiocytosis (PNH) is a rare normolipemic macrophage disorder.
• It is a subgroup of non-Langerhans cell histiocytosis (LCH) with a progressive course and no spontaneous resolution.
• PNH typically presents without systemic involvement.

Purpose of the Study:

• To report a rare case of progressive nodular histiocytosis (PNH).
• To describe the clinical presentation, diagnostic findings, and treatment challenges.
• To discuss the association with Eale's disease.

Main Methods:

• Case report of a 30-year-old male with PNH.
• Clinical examination including dermatological and ophthalmic assessments.
• Skin biopsy with immunohistochemical analysis (CD68+, S-100-, CD34-, CD21-, CD35-, HMB45-).

Main Results:

• Patient presented with widespread skin lesions and gradual vision loss.
• Skin biopsy confirmed a spindle cell histiocytic tumor.
• Ophthalmic examination showed arteriolar attenuation in both eyes.
• Systemic treatments (Imatinib, Pazopanib) showed no response.

Conclusions:

• PNH is a rare condition with a characteristic presentation.
• The association with Eale's disease is noted.
• Optimal systemic treatment for PNH remains undetermined; surgical excision is primary for symptomatic lesions.