A relationship between Kimura disease (KD) and angiolymphoid hyperplasia with eosinophilia (ALHE) has been debated. Given substantial clinical and histological overlap, these entities were once considered to represent a disease spectrum; however, they are now widely considered to be nosologically distinct. A diagnosis of either condition is further complicated by resemblance to various malignancies, which must be carefully excluded. Coexistence of ALHE and KD in a patient is extremely rare, with only four cases reported in the English literature. We report what is to our knowledge the first case of ALHE and KD overlap with evidence of diffuse visceral involvement.
A 56-year-old previously healthy man presented to the dermatology clinic with a 2-year history of an expanding, violaceous, infiltrated plaque on the right flank. Biopsy revealed a diffuse dermal vascular proliferation of bland, capillary-sized vessels admixed with conspicuous fibrohistiocytic cells including scattered multinucleated floret cells. Further workup revealed a monoclonal gammopathy, an osteolytic chest wall plasmacytoma underlying the plaque, and regional lymphadenopathy leading to a diagnosis of adenopathy and extensive skin patch overlying a plasmacytoma (AESOP).Biopsy of an enlarged lymph node revealed Castleman disease. The patient subsequently developed polyneuropathy and peripheral edema, which supported an additional diagnosis of polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes (POEMS) syndrome. Herein, we discuss the unique findings of our patient, the potential pathogenesis of AESOP, and the link between these three rare paraneoplastic entities along with review of the literature. K E Y W O R D S adenopathy and extensive skin patch overlying a plasmacytoma, AESOP, Castleman disease, POEMS, polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes
Most dermatologists remain unaware of melanoma reporting requirements. Resultant underestimates of the true incidence of melanoma could have resource allocation implications. Interventions aimed at improving knowledge of the mandate should focus on younger clinicians and those with lower melanoma case volumes.
A 62-year-old man with a long-standing history (>40 years) of Hailey-Hailey disease was admitted from an outside hospital due to anemia (hemoglobin, 8.6 g/dL [reference range, 14.0-17.5 g/dL]), thrombocytopenia (platelets, 7×10 3 /µL [reference range, 150-350×10 3 /µL]), and worsening skin rash. The patient reported that his Hailey-Hailey disease worsened abruptly 1 month prior to admission and had progressed steadily since then. He described the rash as painful, especially with movement. Over the preceding month, he had been treated with topical triamcinolone, topical diphenhydramine, oral prednisone, fluconazole, and oral clindamycin, all without improvement. The skin lesions continued to worsen and persistently bled; he then presented to our institution for further care. Physical examination demonstrated widespread shallow erosions with hemorrhagic drainage and crusting located on the lower back, chest, abdomen (top), axillae (bottom), groin, arms, and legs. No vesicles or pustules were noted. The patient had no cognitive dysfunction or focal neurologic deficits. A punch biopsy was performed. WHAT'S THE DIAGNOSIS? a. Hailey-Hailey disease flare b. impetigo c. Kaposi varicelliform eruption (eczema herpeticum) d. multiple myeloma e. tinea corporis
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