Korean J Dermatol.  2020 Jul;58(6):423-426.

A Case of ‘Salt and Pepper Appearance of the Skin’ in Mixed Connective Tissue Disease

  • 1Department of Dermatology, Pusan National University School of Medicine, Yangsan, Korea
  • 2Biomedical Research Institute, Pusan National University Hospital, Busan, Korea


The salt and pepper appearance of the skin is composed of salt (vitiligo-like depigmentation) and pepper (perifollicular pigmentation). Mixed connective tissue disease (MCTD) is a distinct disease entity with mixed features of systemic lupus erythematosus, systemic sclerosis, myositis, and rheumatoid arthritis with high titers of antibodies to U1 ribonucleoprotein (U1RNP). There are a few reports of pigmentary changes in MCTD. A 51-year-old woman presented with variably sized hypopigmented macules along with relative hyperpigmentation of the perifollicular areas on the face and neck; she was diagnosed with MCTD in a rheumatology clinic. Histopathology revealed dermal sclerosis in both hypo- and hyperpigmented areas, loss of melanocyte and pigment only in the hypopigmented area, and normal number of melanocyte and pigment in the hyperpigmented area. To the best of our knowledge, ‘salt and pepper appearance of the skin’ has rarely been reported in MCTD.


Hyperpigmentation; Hypopigmentation; Mixed connective tissue disease; Salt and pepper appearance
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