A Case of Erythroderma Secondary to Hypereosinophilia

Abstract

A 67-year-old female was admitted to our emergency in altered sensorium with generalized erythroderma and patchy hair loss. She underwent splenectomy for lymphangioma [Table/ Fig-1] six months before. Clinical examination showed erythroderma, sacral edema, mucosal dark pigmented lesions, patchy hair loss and madarosis. Basic laboratory investigations showed anaemia (Haemoglobin-7.3 gm/dl), leucocytosis (White blood cell count-19,000/c.mm) with hypereosinophilia (Absolute eosinophil count : 11,768/c.mm) and thrombocytosis (5,08,000/c.mm).She had severe hypoalbuminemia (albumin -1.9 gm/dl) probably secondary to loss of protein through skin. Anti-nuclear antibody (ANA), anti-double stranded DNA (anti-Ds DNA) and antineutrophil cytoplasmic antibody (ANCA) were negative. In the absence of any significant history of atopy, allergic disorders, or parasites to explain her high eosinophil counts she was submitted to a work up for primary hypereosinophilia and any associated end organ damage. In view of high Vitamin B12 levels (16,680ng/L) and hypereosinophilia, there was a strong suspicion of myeloproliferative disorders. Molecular genetic work up showed absence of Fip1-like-1 fused with platelet derived growth factor receptor alpha (FIP1L1-PDGFRα) and BCR-ABL mutation. Bone marrow biopsy showed scanty marrow with eosinophilia [Table/ Fig-2].T-cell receptor rearrangement studies were done and primary cutaneous T cell lymphoma was ruled out. A skin biopsy showed subcorneal eosinophilic collection with spongiotic dermatitis [ Hypereosinophilic syndrome (HES) is a myeloproliferative disorder characterised by marked peripheral eosinophilia and end organ damage attributable to eosinophilia without secondary cause. Early recognition and treatment are essential to prevent morbidity and mortality. Cytoreduction with Steroids, Hydroxyurea and Imatinib are the main stay of treatment. Molecular studies like Fip1-like-1 fused with platelet derived growth factor receptor alpha (FIP1L1-PDGFRα) etc., are recommended in view of therapeutic implication. In this paper we report a rare case of HES developing in a lady 6 months after surgical removal of lymphangioma of spleen, which in itself is rare.

Diffuse Exfoliative Rash with Sepsis and Eosinophilia: A Case of Erythroderma?

“…High eosinophils in the setting of positive ANA and scleroderma antibodies may suggest hypereosinophilia compounded by scleroderma [6,7]. Classically, hypereosinophilic syndrome (HES) is a myeloproliferative disorder defined as peripheral eosinophilia (>1500 cells/mm 3 ) with end-organ damage due to tissue eosinophilia and absence of a secondary cause [8]. The main organs involved are skin (erythroderma), lungs, intestine, heart (myocardial fibrosis, chronic heart failure), and kidneys [8].…”

“…Classically, hypereosinophilic syndrome (HES) is a myeloproliferative disorder defined as peripheral eosinophilia (>1500 cells/mm 3 ) with end-organ damage due to tissue eosinophilia and absence of a secondary cause [8]. The main organs involved are skin (erythroderma), lungs, intestine, heart (myocardial fibrosis, chronic heart failure), and kidneys [8]. Although his renal function and lung imaging were unremarkable, cardiac echocardiography showed a reduced left ventricular ejection fraction (EF 35%) with diastolic dysfunction.…”

Case for diagnosis. Erythroderma as manifestation of hypereosinophilic syndrome

“…Histopathologic examination of the skin lesion is usually nonspecific, with viable eosinophilic infiltration. 4 - 7 …”

“…It is thus crucial that in all cases of erythroderma, HES should be considered as a differential diagnosis. 6 , 7 …”

An Approach to Hypereosinophilic Syndrome Presenting With Cutaneous Features

“… 39,40 Involvement of the limbs and trunk is most commonly reported as presenting sites, but lesions have been reported on the face, neck, and extremities. Hypereosinophilic syndrome should also be considered with the presentation of erythroderma, as it has been reported in several cases 41–43 . Other cutaneous signs of internal organ dysfunction may be evident on physical examination.…”