Background and Aims

Common variable immunodeficiency (CVID) is a primary immunodeficiency disorder of the humoral immunity which causes hypogammaglobulinemia. It is not a single disease and therefore a predisposition to infection and inflammatory conditions including systemic autoimmunity and lymphoproliferative complications are also commonly associated.

Morphea, also known as localized scleroderma, is an idiopathic inflammatory disorder that causes sclerotic changes only in the skin.

Methods

We present the co-existence of morphea and CVID.

Results

Here we present a very rare case of a middle-aged male patient diagnosed as CVID under regular IVIG treatment. No infectious complications developed during the five year follow up period. However, six months ago brown-white colored plaques on the skin on periumbilical, femoral and gluteal areas consequently occured. The findings in the biopsy of the plaque were compatible with the typical morphology of morphea and sclerosan dermatitits. Methotrexate 25 mg per week and low dose corticosteroid were initiated following the spreading of the lesions.

Conclusions

In our knowledge, this is the first case report presenting the co-existence of CVID and morphea. To clarify whether there is an association between these diseases, larger case series have to be revised. Dermatologists and immunologists may keep in mind this co-existence in PID.