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Dyschromia Related to Severe Combined Immunodeficiency

Maldonado-Cid, Paola MD*; Noguera-Morel, Lucero MD*; Moreno-Alonso-de-Celada, Ricardo MD*; De-Lucas-Laguna, Raúl MD*; Feito-Rodríguez, Marta MD*; Beato-Merino, Maria José MD; Casado-Jiménez, Mariano PhD*

The American Journal of Dermatopathology: December 2013 - Volume 35 - Issue 8 - p e131–e134
doi: 10.1097/DAD.0b013e31827a23eb
Extraordinary Case Report

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.

Departments of *Dermatology

Pathology, Hospital Universitario La Paz, Madrid, Spain.

Reprints: Dr Paola Maldonado-Cid, MD, Department of Dermatology, Hospital Universitario La Paz, Pº de la Castellana 261, CP 28046, Madrid, Spain (e-mail:

The authors declare no conflicts of interest.

© 2013 by Lippincott Williams & Wilkins.