Extensive Angiolymphoid Hyperplasia with Eosinophilia with Severe Anemia and Uncontrolled Diabetes Mellitus

Angiolymphoid hyperplasia with eosinophilia (ALHE), a rare benign vascular proliferation, was initially thought to be a late stage of Kimura’s disease, is now considered a separate entity. We report a case of ALHE with extensive (>50) lesions, severe anemia (Haemoglobin = 5.7gm%)and severe uncontrolled diabetes mellitus (Fasting Blood Sugar = 430mg%). These associations have not been reported previously. Our patient also had lymphadenopathy, a feature of Kimura’s disease.


Introduction
Angiolymphoid hyperplasia with eosinophilia (ALHE), a rare benign vascular proliferation, was initially thought to be a late stage of Kimura's disease, is now considered a separate entity. We report a case of ALHE with extensive (>50) lesions, severe anemia (Haemoglobin = 5.7gm%)and severe uncontrolled diabetes mellitus (Fasting Blood Sugar = 430mg%). These associations have not been reported previously. Our patient also had lymphadenopathy, a feature of Kimura's disease.  A57-year-old male, uncontrolled diabetic presented with complaints of multiple itchy purple raised lesions on left side of face for three years, with a history of bleeding on trauma. Lesions started as few pink-purple nodules over left preauricular region and gradually increased in size and numbers since then. No history of pain, similar lesions on the other side of face or elsewhere on body was noted. One examination multiple violaceous papules and nodules, ranging in size from 0.5 centimeter to one centimeter, limited to left side of scalp and face were noted. Lesions were soft to firm in consistency and slightly tender. Few excoriations were present. No lymphadenopathy or salivary gland enlargement was noted (Figures 1 & 2). Volume=59.7 femtoliters, Mean Corpuscular Haemoglobin=15.5picograms, Mean Corpuscular Haemoglobin Concentration=26.0grams/deciliter, Packed Cell Volume = 21.9%), increased red cell distribution width (15.9%), leucocytosis (11,900percubicmillimeter) and elevated fasting blood sugar levels(430milligram percent).Eosinophils (4%), liver and renal function tests, serum IgE levels were normal. The serology was negative for antibodies to Human Immuno deficiency Virus (HIV)and Hepatitis-C virus. High frequency ultrasonography of skin revealed multiple nodular hypo echoic mass lesions in sub epidermal region (suggestive of lesions) and few well defined hypo echoic masses with an echogenic center (suggestive of enlarged lymphnodes) in preauricular region ( Figure 3).   Angiolymphoid hyperplasia with eosinophilia (ALHE) is a rare benign but potentially disfiguring vascular proliferation. It was first described by Wells and Whimster in 1969 [1]. They considered it to be a late stage of Kimura's disease, adisorder described in the Japanese literature twenty years earlier. Currently, however, most authors believe that ALHE and Kimura's disease are two separate entities [2,3]. Our patient had most features of ALHE with lymphadenopathy, which is a feature of Kimura's disease. The epidemiological, clinical and histopathological differences between the two entities along with features present in our patient are summarized in the table (Table1).

Discussion
Were port and emphasize this case considering these unusual features:

Figure 6
Extensive lesions (>50) associated with severe uncontrolled diabetes mellitus (DM): The association of DM with ALHE has not been reported in literature so far. Also, there is anyone case report of a patient (non-diabetic) with extensive lesions [1]. The association of DM with various macro and microvascular abnormalities has long been known and the major cells affected are endothelial cells and vascular smooth muscle cells as depicted in the Figure  6 [4,5]. Whether this endothelial and vascular smooth muscle cell dysfunction has any role to play in vascular proliferation seen in ALHE needs to be proved with further reports and studies.
Severe unexplained anaemia (Hb 5.7gm%): After thorough evaluation by an expert haematologist, no underlying cause for severe anomie was found except bleeding from the lesions. Hence chronic blood loss (even though very little) from the lesions may lead to severe anaemia and hence haemoglobin in should be monitored. Our patient was treated with three injections of ferritin carboxymaltose (two on day one followed by one on day seven) following which Hb was noted 9.40gm%.
With lymphadenopathy which is a feature of Kimura's disease.

Conclusion
ALHE is a rare entity and such extensive involvement with severe uncontrolled diabetes mellitus and with severe anemia has not been reported previously. Monitoring of haemoglobin and blood sugars may be advisable inpatients with extensive lesions.

Key Messages
a) Extensive lesions of Angiolymphatic Hyperplasia with Eosinophilia may cause severe anaemia, hence haemoglobin in must be monitored in such cases. b) Uncontrolled diabetes mellitus may be associated with increased severity of the disease.