Abstract
Paraneoplastic pemphigus (PNP) is a rare autoimmune disorder associated with underlying benign or malignant neoplasia. Its signs and symptoms may be the first presentation of a concealed malignancy. Due to late diagnosis, prognosis of PNP is not good, so early diagnosis and treatment is of paramount importance. We present a case of 22 years old female who presented to our out patient department (OPD) with history of recurrent, severe, recalcitrant, painful oral ulcers; lichenoid lesions over the hands and feet and widespread blistering and erosions involving the soles. Histopathological examination was consistent with paraneoplastic pemphigus and showed features of lichenoid dermatitis. CT scan revealed retroperitoneal mass suggestive of Castleman disease. The patient was referred to surgical team for further management.
Highlights
Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease associated with underlying benign or malignant neoplasia.[1]
PNP can be associated with Castleman disease (CD)
We report a case of 22 years old unmarried female who presented with painful oral ulcers for 3-4 years, rashes over both hands and feet for 4-5 months, and non-healing ulcers over feet for 2-3 months
Summary
Paraneoplastic pemphigus (PNP) is a rare autoimmune blistering disease associated with underlying benign or malignant neoplasia.[1]. Dermatological examination revealed multiple ulcers and erosions over the tongue (Figure 1), buccal mucosa, and palate She had mild scaly plaques with violaceous pigmentation over her lips. Adhikari H, et al A sinister disease hides behind recalcitrant mucocutaneous disorder metacarpophalangeal joints (Figure 2). She had welldefined erythematous plaques with whitish adherent scales distributed symmetrically over the dorsal aspect of the fingers and toes, mostly involving the proximal interphalangeal joints and extending distally (Figure 3). There were erythematous plaques on bilateral soles, with multiple ulcers and extensive erosions with overlying yellowish slough and crusts (Figure 4).
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