Title of Case(s):

Lymphadenopathy in an eczematous infant

Background:

Severe or infected eczema may be associated with widespread lymphadenopathy. We present a case where significant lymphadenopathy in a baby with ezcema was the sentinel feature of a rare condition.

Case Presentation Summary:

A 4 month old boy presented with one month of increasing left axillary swelling. He was the 5^th child of non-consanginous Bangladeshi parents. He was born at term and received IV antibiotics for culture-negative neonatal sepsis. He had developed a severe generalised scaly skin rash at 2 weeks of age which had responded to topical clobetasone, emollients and an hydrolysed formula after dermatology review. Skin swabs had grown methicillin-sensitive staphylococcus aureus and he had received 2 courses of oral antibiotics. He was vaccinated, including BCG vaccination at 1 month of age. He was gaining weight (2^nd centile).

Examination found a 5cm x 5cm non-tender mass with mild overlying erythema (confirmed to be a conglomerate of enlarged abnormal left axillary lymph nodes with ultrasound scan). The BCG site appeared erythematous, without discharge. Examination was otherwise normal apart from some improving eczematous skin.

Investigations revealed T-B-NK+ severe combined immunodeficiency with a RAG1 mutation. Further chest, abdominal and brain imaging showed no features of disseminated BCG.

He was commenced on BCG treatment (isoniazid, rifampicin, clarithromycin, amikacin) and continued on this throughout a matched sibling bone marrow transplant one month later. He is now doing well, off BCG therapy with good donor marrow engraftment.

Key Learning Points:

The combination of neonatal lymphopenia (present in this patient), neonatal eczematous rash (maternal T cell engraftment) and atypical infection requires immunodeficiency investigation. All neonatal lymphopenia should be followed up.

BCG dissemination is more common in immunodeficiency and is a major complicating factor in transplant. Neonatal SCID screening was been recently induced in London and may prevent BCG administration in children with primary immunodeficiency.

Title of Case(s):

Lymphadenopathy in an eczematous infant

Background:

Severe or infected eczema may be associated with widespread lymphadenopathy. We present a case where significant lymphadenopathy in a baby with ezcema was the sentinel feature of a rare condition.

Case Presentation Summary:

A 4 month old boy presented with one month of increasing left axillary swelling. He was the 5^th child of non-consanginous Bangladeshi parents. He was born at term and received IV antibiotics for culture-negative neonatal sepsis. He had developed a severe generalised scaly skin rash at 2 weeks of age which had responded to topical clobetasone, emollients and an hydrolysed formula after dermatology review. Skin swabs had grown methicillin-sensitive staphylococcus aureus and he had received 2 courses of oral antibiotics. He was vaccinated, including BCG vaccination at 1 month of age. He was gaining weight (2^nd centile).

Examination found a 5cm x 5cm non-tender mass with mild overlying erythema (confirmed to be a conglomerate of enlarged abnormal left axillary lymph nodes with ultrasound scan). The BCG site appeared erythematous, without discharge. Examination was otherwise normal apart from some improving eczematous skin.

Investigations revealed T-B-NK+ severe combined immunodeficiency with a RAG1 mutation. Further chest, abdominal and brain imaging showed no features of disseminated BCG.

He was commenced on BCG treatment (isoniazid, rifampicin, clarithromycin, amikacin) and continued on this throughout a matched sibling bone marrow transplant one month later. He is now doing well, off BCG therapy with good donor marrow engraftment.

Key Learning Points:

The combination of neonatal lymphopenia (present in this patient), neonatal eczematous rash (maternal T cell engraftment) and atypical infection requires immunodeficiency investigation. All neonatal lymphopenia should be followed up.

BCG dissemination is more common in immunodeficiency and is a major complicating factor in transplant. Neonatal SCID screening was been recently induced in London and may prevent BCG administration in children with primary immunodeficiency.