Abstract:

Intraepidermal particulate pattern IgG direct immunofluorescence (DIF) staining, although rarely reported in the literature, has been found to be highly associated with Ro/La antibody positivity in patients with subacute lupus erythematosus (SCLE). We report a case of a patient with preexisting Sjogren’s syndrome who developed cutaneous SCLE with this distinctive particulate pattern on DIF. A 71-year-old female with Sjogren’s syndrome and diffuse large B-cell lymphoma on chemotherapy complicated by a recent admission for neutropenic fever presented with a new, progressive rash on her face and arms, characterized by erythematous papules and small plaques. Skin biopsy demonstrated interface dermatitis with prominent follicular involvement characterized by CD123 positive cells along the epidermal basal layer. DIF showed particulate intracytoplasmic epidermal IgG staining, felt to be consistent with SCLE. Laboratory evaluation revealed high titer antinuclear antibody (1:5120) with strongly positive Ro and La antibodies, confirming the diagnosis. Treatment with hydroxychloroquine lead to resolution of her cutaneous symptoms. This case highlights a rare and characteristic pattern of DIF that is correlated with a specific clinical subset of cutaneous lupus erythematosus. Thought not all reports support the strength of the association of particulate pattern DIF in SCLE, this pattern may allow the dermatopathologist to identify patients who have signs of SCLE in conjunction with other connective tissue disorders such as Sjogren’s.

Financial Disclosure:
No current or relevant financial relationships exist.

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Josiah Hanson, MD