PMC5803949_01
Index case. Dark brown keratotic papular lesions in temporal region behind the ear (a), nail involvement showing dystrophy, erythronychia, and a V-shaped nick (b), histopathological features of index case showing focal suprabasal clefting with a thickened stratum corneum and dyskeratotic cells, with "corps ronds" and "grains" in epidermis (c and d). (Hematoxylin and eosin stain, original magnification c - x40, d - x400)
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F1
On the head and neck, lesions were present predominantly in the scalp, forehead, and temporal regions [Figure 1a], behind the ears, and nasolabial folds. Nails were dystrophic with erythronychia and V-shaped nick [Figure 1b]. Few dyskeratotic cells, with "corps ronds" and "grains" were also identified in epidermis consistent with the diagnosis of DD [Figure 1c and d].
Elder daughter. Keratotic lesions with guttate leukoderma on the abdomen
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F2
The elder daughter, aged 18 years, started developing similar keratotic papules on the neck and trunk since last 3 years with leukoderma-like macules (guttate leukoderma) on the trunk since 4 years [Figure 2].
Son. Guttate leukoderma on the back
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F3
The 12-year-old son of the index case also had brown papules on the forehead along with guttate leukoderma on back [Figure 3].
Pedigree chart
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F4
Thus, the pedigree chart [Figure 4] suggested the autosomal dominant inheritance of the illness.