CPC12: Eosinophilic dermatosis of haematological malignancy

Published Date: 01st July 2021

Publication Authors: Lavery MJ, Low SE, MacBeth K

 

Extract
A 73‐year old man presented with a 10‐month history of a generalized pruritic eruption. No close contacts had a rash or itch. There was no history of weight loss, loss of appetite or B symptoms. The general practitioner had given oral prednisolone to good effect, but the rash soon returned. Past history included chronic obstructive pulmonary disease and hypercholesterolaemia, for which he was taking carbocisteine (for 2 years) and atorvastatin (for 4 years), respectively. On examination there were excoriated papules and nodules along with erythematous plaques on the anterior and posterior trunk. Violaceous infiltrative nodules and plaques were evident on the upper and lower limbs. There was no lymphadenopathy or organomegaly. Skin biopsy revealed intraepidermal eosinophils along with a perivascular and interstitial dermal eosinophilic infiltrate that were felt to be secondary to a drug or allergic aetiology. No plasma cells were present. Direct immunofluorescence was negative. A peripheral eosinophilia of 1·4 × 10⁹ cells L^–1, lymphopenia of 0·8 × 10⁹ cells L^–1 and a raised lactate dehydrogenase of 337 U L^–1 were evident. IgM level was 0·41 g L^–1, with an IgG kappa paraprotein present on immunofixation. Further analysis revealed a raised kappa : lambda ratio of 1·8 and a raised beta microglobulin level of 6·3 mg L^–1. Skeletal survey did not reveal any lytic lesions and computed tomography scan was normal. Bone marrow biopsy is awaited. The culmination of peripheral and tissue eosinophilia, on a background of a plasma cell dyscrasia led to the unifying diagnosis of eosinophilic dermatosis of haematological malignancy (EDHM). Further investigations are awaited, to determine the final diagnosis of multiple myeloma (MM) or monoclonal gammopathy of undetermined significance (MGUS). EDHM is a collective term describing a skin disease with peripheral and tissue eosinophilia associated with a haematological malignancy. It is rare, with 208 reported cases. The most common associated malignancy is chronic lymphocytic lymphoma, with one reported case of multiple myeloma, and two reported cases of MM/MGUS in a separate case series. Different therapeutic modalities may be employed for managing the cutaneous features and itch, for which dupilumab has recently been reported to be efficacious. While the presence of tissue eosinophilia led to the histopathological conclusion of a drug or allergic aetiology, clinically there was suspicion of underlying malignancy. Dermatologists should remain vigilant when assessing the clinical and histological findings and investigate further if there is clinicopathological discordance. In our case the skin findings were the first presentation of a rare condition.
 

 

Lavery, M; Low, S.E; Macbeth, K. (2021). CPC12: Eosinophilic dermatosis of haematological malignancy. British Journal of Dermatology. 185(S1), p.21. [Online]. Available at: https://doi.org/10.1111/bjd.19959 [Accessed 11 April 2023]

 

 

 

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