A six year-old female child presented with excessively thickened skin over her palms and soles. The child was born of a normal vaginal delivery with redness on both her soles; after a few months, the skin over the soles got thickened and started peeling off. Similar thickening began on the palms at the age of two years and warty spots on the sides of the mouth and nose were also noticed. Later, the parents noted that the child's hair was short, not dense and was very slow to grow unlike the children of her age. Her developmental milestones were however, appropriate for her age. The ugly keratoderma had restricted her mobility; hence, the child could not mingle with her peers. There was no history of hyperhidrosis, consanguinity or any skin disease in the family.
Examination revealed an active girl with apparently normal intelligence. Her physical parameters were within normal limits for her age. Cutaneous examination revealed thick, hyperkeratotic plaques with underlying erythema on the thenar eminence of both palms and fingers (). The keratotic plaques were hard, well defined, 3 cm × 4 cm in size, thick in places and thin in others and extended to the dorsum of the distal phalanges. The lesions on the soles had formed ‘Keratotic Sandals’ () with underlying erythema and thick adherent scales. Keratoderma on the soles had extended to involve the dorsum of the feet along the borders. There were some flexion deformities on both soles. Verrucous plaques were seen on the angles of the mouth and around the nares (). Nails of both hands corresponding to skin involvement on the fingers were lusterless and ridged. Similarly, all toenails were lusterless and rough. The nails on last two toes of the right foot were spared, as was the skin. Hair over the scalp, eyebrows and eyelashes was thin, sparse and light brown in color. Oral, genital and ocular mucosa, joints and teeth were found to be normal.
Keratotic plaques on palms
Keratotic plaques on the angles of the mouth and the nares
Routine hemogram, peripheral smear, liver and renal function tests, urine and stool examination did not reveal any abnormality. Blood for venereal diseases research laboratory test was non-reactive. Potassium hydroxide mount of the skin scrapings did not show the presence of any fungus. Serum zinc estimation was not available.
Histopathology examination of the lesions showed thick layer of keratosis, parakeratosis, thickened granular cell layer, acanthosis and irregular elongation of rete ridges. Sparse perivascular mononuclear infiltrate was visible in the papillary dermis. A few sweat ducts were also seen. Hair appeared to have less pigment and hair shafts were thin when observed under a light microscope.
Therapy with oral zinc did not improve the condition at all. Salicylic acid with Clobetasol propionate combination in ointment base did not affect the tough scales. Paring was done and a Urea and Lactic acid combination used, which resulted in some relief.