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Pigmentation01:19

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The color of the skin is influenced by a number of pigments, including melanin, carotene, and hemoglobin. Recall that melanin is produced by cells called melanocytes, which are found scattered throughout the stratum basale of the epidermis. The melanin is transferred to the keratinocytes via melanosomes.
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The papillary and reticular dermis are the two layers of the dermis. They are made of connective tissue with fibers of collagen extending from one to the other, making the border between the two somewhat indistinct. The dermal papillae extending into the epidermis belong to the papillary layer, whereas the dense collagen fiber bundles below belong to the reticular layer.
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Pigmented neurofibroma with hypertrichosis.

Juan A Godínez-Chaparro1, Adriana M Valencia-Herrera2, Carlos A Mena-Cedillos2

  • 1Servicio de Dermatología Pediátrica, Unidad Médica de Alta Especialidad, Hospital General Dr. Gaudencio González Garza, Centro Médico Nacional La Raza, Instituto Mexicano del Seguro Social, Mexico City.

Boletin Medico Del Hospital Infantil De Mexico
|March 3, 2023
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Summary
This summary is machine-generated.

Pigmented neurofibroma (PN) is a rare variant of neurofibroma. This case report details a rare association of PN with hypertrichosis in a patient with neurofibromatosis type 1.

Keywords:
Case reportHipertricosisHypertrichosisLatinoMelanotic neurofibromaNeurofibromaNeurofibroma melanocíticoNeurofibroma pigmentadoPigmented neurofibromaReporte de caso

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Area of Science:

  • Dermatology
  • Oncology
  • Genetics

Background:

  • Pigmented neurofibroma (PN) is a rare variant, comprising 1% of neurofibroma cases.
  • PN contains melanin-producing cells and can infrequently be associated with hypertrichosis.
  • Neurofibromatosis type 1 (NF1) is a genetic disorder associated with various skin tumors.

Observation:

  • An 8-year-old male with a diagnosed NF1 presented with a hyperpigmented plaque and hypertrichosis on his left thigh.
  • The lesion was light brown, smooth, and well-demarcated.
  • Skin biopsy revealed neurofibroma characteristics with melanin deposits in the deep portion.

Findings:

  • Histopathological analysis confirmed pigmented neurofibroma (PN) through positive staining for S100, Melan-A, and HMB45 in melanin deposits.
  • The case highlights a rare presentation of PN with hypertrichosis in an NF1 patient.
  • PN is a benign, chronically progressive tumor that can be mistaken for other pigmented skin lesions.

Implications:

  • Biopsy analysis is crucial for differentiating PN from other pigmented skin tumors like melanocytic schwannoma or dermatofibrosarcoma protuberans.
  • Surveillance is a key component of PN management.
  • Surgical resection may be considered for PN in certain cases.