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Proteins left behind from the bite may induce immune mimicry leading to development of antibodies directed against the skin The investigative findings (H&E, DIF, IIF and ELISA) are consistent with pempihigus foliaceous, showing antibodies against Dsg 1 PARANEOPLASTIC PEMPHIGUS Paraneoplastic pemphigus is a rare but severe form of pemphigus with a poor prognosis It is due to an abnormal B or T cell immne respoonse to an underlying malignancy It is distinguished by antibodies to a wide range of epitopes, including desmoglein 3, desmoglein 1, envoplakin, periplakin, and desmoplakins , among others Cytotoxic CD8 T cells also play a role which can lead to an interface dermatitis seen alongside acantholysis This broad range of antibody targets and the cytotoxic T cell role contributes to the polymorphic clinical presentation we often see Clinical features: Patients often unwell Typically begins with painful oral erosions, which can progress to severe and recalcitrant ulceration with pan-stomatitis and hyperplastic mucosa Haemorrhagic cheilitis (crusted, bleeding lips) and severe ulceration in the oropharynx and genitalia are characteristic and often out of proportion to cutaneous involvement The rash is often polymorphic with a variety of lesions and it can mimic other conditions like erythema multiforme, lichen planus or GVHD Other organs can be affected: Lung involvement can lead to bronchiolitis obliterans (which can be severe) GI tract can be involved Associated cancers: Lymphoma ≈ 40% of cases CLL ≈ 30% Castleman’s disease ≈ 10% Castleman disease is a rare disorder that involves an overgrowth of cells in body’s lymph nodes Most common form of the disorder affects a single lymph node (unicentric Castleman disease) usually in chest or abdomen Multicentric castleman disease affects multiple lymph nodes throughout the body and has been associated with HHV 8 and HIV Thymoma ≈ 6% Spindle cell neoplasms ≈ 6% Waldenstrom’s macroglobulinaemia ≈ 6% Investigations Management To do…

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