title: Dyschromia related to severe combined immunodeficiency.
abstract: Severe combined immunodeficiency includes a group of diseases characterized by different inherited immunological defects. A 4-month-old girl diagnosed with Omenn syndrome, a subtype of severe combined immunodeficiency presenting with generalized erythroderma, was referred to our hospital for an allogeneic stem cell transplantation. Days before transplantation, she developed hyperpigmented macules that increased in number in the following months. As the erythroderma resolved after transplantation, diffuse hypopigmentation was simultaneously noted together with the expansion of hyperpigmented lesions. Cutaneous biopsy samples were taken at different moments, showing features of Omenn syndrome at first, and 2 months later changes consistent with hypopigmentation and repigmentation were observed. Although pigmentary disorders are rarely described in this context, these must be taken into account as a possible alternative diagnosis to graft-versus-host disease and toxicoderma in immunosuppressed patients.
authors: Departments of *Dermatology †Pathology, Hospital Universitario La Paz, Madrid, Spain.
authors: Paola
authors: P
authors: Maldonado-Cid
authors: Lucero
authors: L
authors: Noguera-Morel
authors: Ricardo
authors: R
authors: Moreno-Alonso-de-Celada
authors: Raúl
authors: R
authors: De-Lucas-Laguna
authors: Marta
authors: M
authors: Feito-Rodríguez
authors: Maria José
authors: MJ
authors: Beato-Merino
authors: Mariano
authors: M
authors: Casado-Jiménez
mesh_terms: D005260:Female; D006801:Humans; D007223:Infant; D010859:Pigmentation Disorders / Q000150:complications* / Q000473:pathology*; D016511:Severe Combined Immunodeficiency / Q000150:complications* / Q000473:pathology*; D012880:Skin Pigmentation