Unusual presentation of angiokeratoma in a cirrhotic patient.

Sir,

Angiokeratomas are vascular malformations, which are clinically characterized by solitary or multiple papules or plaques. The lesion histologically defined as dilated blood vessels with sub epidermal position and it has epidermal proliferative reaction. In all form of angiokeratoma, the epidermal changes occur in the secondary stage. There are several clinical types of angiokeratoma depending on the multiplicity and location of the lesions.[1] Angiokeratoma corporis diffusum is the systemic form, which frequently link to some inborn error of metabolism, primarily Fabry's disease and fucosidosis.

The localized forms are; (1) solitary papular angiokeratomas; (2) scrotal or vulval angiokeratomas; (3) multiple congenital angiokeratomas; and (4) bilateral angiokeratomas in the dorsum of hands and feet (Mibelli type).[23]

Now we will describe an unusual form of angiokeratoma, which was acquired and manifested as aggregated papules and exophytic nodules bilaterally in the lower extremities. This presentation had acquired in a cirrhotic patient suffering from ascites and edema of the lower extremities.

A 53-year-old male patient presented with the complaint of abdominal distention. He had a history of recurrent epistaxis from 5 years and lower leg edema since 10 years ago. On examination, he was icteric and pale. Abdominal distention and shifting dullness were found. He had bilateral pitting edema of the lower extremities.

He was admitted to the hospital. Laboratory investigations revealed anemia and thrombocytopenia. We found indirect hyperbilirubinemia and the aspartate aminotransfrase (AST) was lower than the normal range. He also had hypoproteinemia with hypoalbominemia. The serum potassium level was lower than the normal range and lactate dehydrogenase (LDH) was vice-versa. The viral markers were negative. Endoscopic examination was normal and patient had no esophageal varicose veins. Abdominal sonography showed the liver's span of 147 mm and coarse parenchymal echo with nodularity. The portal vein diameter was 16 mm that was larger than the normal limit. There was splenomegaly and ascitic fluids were seen in the abdominopelvic cavity. These findings were compatible with cirrhosis.

Multiple black and blue papules and nodules had appeared on the back of his both knees since one-year ago and gradually increased in number and size. The papules were hyperkeratotic and angiomatous in appearance [Figure 1].

Figure 1

Multiple black and blue papules and exophytic nodules

Biopsy specimen was taken from a papule and the pathology report was consistent with angiokeratoma [Figure 2].

Figure 2

Dilated capillary of papillary dermis with hyperkeratotic overlying epidermis

Our patient had coagulopathy. So the liver biopsy was contraindicated.

Ultimately, after several evaluations, no reason was found for the cause of cirrhosis. Therefore, with the diagnosis of cryptogenic cirrhosis, the patient was treated symptomatically.

Cirrhosis of liver is defined as a chronic and progressive process, which is characterized by fibrosis and structurally abnormal nodules in liver. Cryptogenic cirrhosis means cirrhosis of liver with undetermined etiology.[4]

There are some cutaneous findings related to cirrhosis such as palmar erythema, dupuytren contracture, and Bier spots.[5]

There are also some vascular lesions related to cirrhosis such as spider angioma. However, based on our knowledge until now, there is not any association reported between cirrhosis and angiokeratoma and our case is the first case of cirrhosis with angiokeratoma, in which the association of these two diseases was found. Angiokeratoma may be related to cirrhosis with the same pathogenesis of spider angiomas or perhaps because our patient had longstanding lower extremities edema based on his cirrhosis this maybe the vascular stasis due to the edema, which had some role in the dilatation of dermal papillary capillaries and development of angiokeratoma.

These lesions had a typical pathology for angiokeratoma and there is not any doubt for us about this term, which is applied for them. These lesions were of new onset and were aggregated to the popliteal fossa bilaterally, so we did not find similar presentation of angiokeratoma yet and this presentation might be a new presentation of angiokeratoma.