RESUMEN
We report a case in which a 43-year-old African American male with medical history of sickle cell disease (SCD) presented with a nonhealing ulcer. Biopsy revealed features of livedoid vasculopathy. Previously, livedoid vasculopathy had only been described in a patient with sickle cell trait, but never in a patient with SCD. Livedoid vasculopathy most commonly affects the distal lower extremities and is characterized by irregular, punched-out, painful ulcers that heal with stellate white scars of atrophie blanche. Histologically, it reveals segmental hyalinizing vessels, focal thrombosis, and endothelial proliferation. The etiology is currently unclear, but it has been shown to be related to procoagulant states and a diagnosis of livedoid vasculopathy should prompt a thorough hypercoagulable workup, including testing for SCD in high-risk patients.
Asunto(s)
Anemia de Células Falciformes/complicaciones , Úlcera de la Pierna/patología , Livedo Reticularis/patología , Piel/irrigación sanguínea , Adulto , Anemia de Células Falciformes/diagnóstico , Anemia de Células Falciformes/tratamiento farmacológico , Antidrepanocíticos/uso terapéutico , Biopsia , Inhibidores del Factor Xa/uso terapéutico , Humanos , Úlcera de la Pierna/etiología , Úlcera de la Pierna/terapia , Livedo Reticularis/etiología , Livedo Reticularis/terapia , Masculino , Inhibidores de Agregación Plaquetaria/uso terapéutico , Factores de Riesgo , Trasplante de Piel , Resultado del TratamientoRESUMEN
Description Alopecia of the scalp has various causes and presentations. However, linear alopecia is unusual and lupus erythematosus presenting as linear alopecia is exceedingly rare. To date, there have been 16 documented cases of linear alopecia diagnosed as chronic cutaneous lupus erythematosus occurring in a linear configuration following Blaschko's lines. We report 2 additional cases and review the clinical and histologic features along with treatment. This Blaschkoid linear variant of cutaneous lupus erythematosus has distinct clinical and histologic characteristics that set it apart from other causes of alopecia and from classic forms of cutaneous lupus. These distinct features include a linear configuration, a younger age of presentation, a disproportionate number of Asians affected, and a paucity of cases with systemic involvement. Histologically, the lesions are characterized by prominent mucin in the dermis and subcutaneous tissues. Blaschkoid linear lupus of the scalp is sufficiently distinctive to suggest the diagnosis on histology alone, in the appropriate clinical context. The most common and successful treatments included systemic and/or combination treatment with oral hydroxychloroquine, oral steroids, and/or intralesional steroids.