A 28 year old woman gave birth to a small for age newborn at 38 weeks of pregnancy. On examination, the newborn was found to have rhinitis, distended abdomen; wrinkled skin, and palmoplantar blisters. The woman had an earlier history of abortion and stillbirth. The most likely diagnosis is:

**Core Concept:**
The given set of symptoms and history suggest a diagnosis related to congenital disorders of glycosaminoglycan (GAG) synthesis or storage. GAGs are long chains of sugar molecules that provide structure and function to various tissues and fluids in the body. Disorders affecting GAG synthesis or storage can lead to various clinical manifestations.

**Why the Correct Answer is Right:**
The most likely diagnosis in this case is **Hereditary Spherocytosis (HS)**. Hereditary spherocytosis is an autosomal dominant disorder that affects the synthesis or stability of the membrane of red blood cells (RBCs). This leads to the production of spherocytes, which are oval or round in shape, rather than the normal biconcave disc-shaped RBCs. The clinical manifestations include jaundice, hepatosplenomegaly, and hemolysis, as seen in this case.

**Why Each Wrong Option is Incorrect:**

A. **Erythrokeratoderma variabilis (EV)** is an autosomal dominant disorder characterized by intermittent hyperkeratotic plaques, which are not present in this case.

B. **Hereditary elliptocytosis (HE)** is a disorder of RBC membrane stability, not synthesis. It is characterized by elliptocytes, which are oval in shape, not spherocytes.

C. **Hereditary elliptocytosis (HE)** is a disorder of RBC membrane stability, not synthesis. It is characterized by elliptocytes, which are oval in shape, not spherocytes.

D. **Hereditary spherocytosis (HS)** is a disorder affecting RBC synthesis or stability, leading to spherocytes, as explained above. This option is the correct answer.

**Clinical Pearl:**
Hereditary spherocytosis is a relatively common disorder, affecting around 1 in 5000 individuals worldwide. It is often diagnosed incidentally due to jaundice, hepatosplenomegaly, and hemolysis. Early diagnosis and treatment can help prevent severe complications and hemolysis-induced anemia.

✓ Correct Answer: C. Congenital syphilis