Discoid Lupus Erythematosus Presenting With Disfiguring Acneiform Plaques: A Diagnostic Challenge

Abstract

Hypertrophic and acneiform forms are very rare variants of discoid lupus erythematosus (DLE), which can suppose a diagnostic and therapeutic challenge. We present a South American woman with facial disfiguring lesions of 7 years of evolution with clinical and histopathological characteristic of both hypertrophic and acneiform DLE. No criteria for systemic lupus erythematosus were present in the patient. To the best of our knowledge, no patients with concomitant hypertrophic and acneiform DLE have been previously reported in the literature.

Letter re: Lupus erythematosus masquerading as acneiform lesions: two cases and review of the literature

“…5 The exact underlying pathogenesis remains unknown, but it is plausible that it is secondary to destruction of the pilosebaceous unit mediated by the dense inflammatory infiltrate. Immunostaining may show clusters of CD123-positive plasmacytoid dendritic cells, 2 as in our case. DIF might also be useful, showing deposits at the dermoepidermal junction.…”

“…One case showed a combination of comedogenic and hypertrophic LE. 2 The mean time until diagnosis was 3.5 years. Lesions favour mainly the facial area (77%), followed by scalp (23%), and are pruritic in 42% of the cases.…”

Plasmacytoid Dendritic Cell Dermatosis Associated to Myeloproliferative/Myelodysplastic Neoplasms

“…22 This has been shown particularly for histiocytoid Sweet's syndrome, palisaded neutrophilic and granulomatous dermatitis, and in rare instances for erythema nodosum and pyoderma gangrenosum. [22][23][24][25][26][27][28][29] Moreover, this relationship has also been established for the L-Group histiocytosis or histiocytic sarcoma. [30][31][32] The reason for MDS/MPN presenting with this broad spectrum of clinical and histologic cutaneous manifestations is not known.…”

“…Interestingly, there are more and more papers coming out based on FISH or next-generation sequencing studies suggesting that the skin infiltrate of neutrophilic dermatoses that arise in the context of myeloid malignancies carry the same molecular alterations than the malignant clone 22. This has been shown particularly for histiocytoid Sweet’s syndrome, palisaded neutrophilic and granulomatous dermatitis, and in rare instances for erythema nodosum and pyoderma gangrenosum 22–29…”