Search

Hypereosinophilic syndrome (HES) is a rare haematologic disorder characterised by unexplained, persistent eosinophilia with organ involvement. We report the case of a 74-year-old patient who presented with a pemphigoid-like bullous rash as the only manifestation of an HES. This report highlights the importance of the dermatological manifestations of this potentially life-threating condition that may initially present as a skin-limited disease. Bullous pemphigoid (BP) is a very common autoimmune blistering dermatosis that may share common clinical and histological features or associate with an HES. HES should be considered when patients diagnosed with BP demonstrate atypical course under corticosteroids and/or recalcitrant blood eosinophilia., Competing Interests: Competing interests: None declared., (© BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ.)

Competing Interests: Competing interests: None declared.

Competing Interests: Competing interests: None declared.

Competing Interests: None disclosed.

Competing Interests: The authors declare that there is no conflict of interest.

Background: Hypereosinophilic syndrome (HES) encompasses a group of diseases with blood hypereosinophilia and eosinophil-mediated organ dysfunction. HES-associated skin abnormalities, termed cutaneous HES (cHES) here, may influence diagnosis of HES. We sought to better define clinical and histopathological features of cHES., Methods: We retrospectively reviewed clinical records and cutaneous histopathology of adult patients with HES evaluated at our institution from 2007 to 2018., Results: Forty-one percent (61/150) patients with HES had cHES. The most common clinical morphologies were urticarial (30%) and eczematous (26%). Skin specimens most often showed a spongiotic pattern (31%) with abundant inflammation (50%) including eosinophils (85%). Two specimens (8%) showed interstitial granulomatous dermatitis, and two specimens showed eosinophilic fasciitis (8%). Vasculitis was not identified in any specimen. Eighty-four percent of patients with cHES had ≥1 other organ system involved: pulmonary 41%, ENT 26%, and nervous 23%. Sixty percent (53/89) of non-cHES patients had at least two organ systems involved. Cardiac or gastrointestinal involvement was more common in non-cHES than cHES (p < 0.05)., Conclusion: Our review confirms that there are no specific clinical or histopathological cHES patterns, but HES should be considered in patients who have eczematous or urticarial reactions of unknown etiology and persistent peripheral hypereosinophilia., (© 2023 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)