Erythrokeratodermia variabilis et progressiva in a Filipino Girl: A case report

ABSTRACT

@#Introduction: Erythrokeratodermia variabilis et progressiva (EKVP) is a rare, usually autosomal dominant disorder characterized by co-existence of erythematous to hyperpigmented scaly plaques and stationary or migratory patches symmetrically distributed on the face, buttocks and extremities. Case summary: This report describes an 8-month-old Filipino female infant who initially presented at 13 days old with erythematous scaly patches on the scalp, periorbital, perioral, and genital areas. These patches progressed to multiple erythematous plaques with well-defined erythematous to hyperpigmented border, some topped with hemorrhagic and yellowish crusts and scales on the scalp, face, neck, upper and lower extremities, genital and gluteal area. Histologic examination of the skin showed dense aggregates of neutrophils in the stratum corneum, parakeratosis, mild spongiosis, and irregular acanthosis. There were focal areas of hypogranulosis underneath the areas of parakeratosis; however, other areas showed normal granular cell layer. The dermis showed moderate superficial perivascular lymphohistiocytic cell infiltrates with melanophages. Histopathologic changes were nonspecific but may be consistent with EKVP. Patient was initially treated with IV antibiotics; however lesions continued to progress. She was then started on oral acitretin, resulting to improvement of lesions. Conclusion: A Filipino girl with erythrokeratodermia variabilis et progressiva is reported. The knowledge of this disease and its treatment will allow clinicians to promptly initiate treatment and monitor treatment side effects