Hyperpigmented lesions with acquired atrophy following Blaschko lines in a patient with diagnosed with localized scleroderma




Eduardo Marín-Hernández, Servicio de Dermatología, Hospital de Pediatría, Centro Médico Nacional Siglo XXI, Ciudad de México, México Belén Suárez-Frías, Hospital de Especialidades, Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Mexico City, Mexico Alicia G. Siordia Reyes, Servicio de Anatomía Patológica Pediátrica, Unidad Médica de Alta Especialidad Hospital de Pediatría. Centro Médico Nacional Siglo XXI, Instituto Mexicano del Seguro Social, Mexico City, Mexico


Background: Linear atrophoderma of Moulin (LAM) is a dermatosis that affects children and adolescents characterized by hyperpigmented and atrophic linear lesions following Blaschko lines. So far, less than 50 cases have been published. Therefore, it is a rare entity with unknown etiology and a chronic and self-limited course. Histologically, it is described as hyperpigmentation of the basal layer without the involvement of the dermis and subcutaneous tissue. No specific treatment exists currently. Localized scleroderma is a chronic and progressive autoimmune connective tissue disorder that affects the skin and adjacent tissues, characterized by abnormal collagen deposition and alteration in elastic fibers, blood vessels, and annexes. No reports have been published on the coexistence of localized scleroderma and LAM. Case report: We describe the case of an 11-year-old male with a clinical diagnosis of linear scleroderma since 5 years of age. Four years later, the patient developed atrophic and hyperpigmented lesions following Blaschko lines in the posterior trunk. A biopsy of both dermatoses was performed: the trunk showed epidermis with hyperpigmentation of the basal layer, and within the dermis, no alteration in the collagen bundles; the forearm biopsy corroborated scleroderma. Based on the clinical-pathological correlation, LAM coinciding with localized linear scleroderma was diagnosed. Conclusions: LAM is an infrequent entity by itself. Moreover, its coexistence with sclerodermiform syndrome has not been reported in the indexed literature.



Keywords: Atrophoderma of Moulin. Scleroderma. Linear. Blaschko.