Nettleship's syndrome I

A chronic skin disease that usually begins during early childhood. The infantile form is characterized by large pigmented macules or nodules. When stroked or otherwise irritated the lesions produce urticaration (Darier’s sign) and itching. The lesions contain large numbers of mast cells. The juvenile form usually lasts until puberty, then disappears spontaneously. The spinal marrow and internal organs may be affected.

In adults it is characterized by efflorescences the size of a lens. Vesiculation is not constantly present and is absent in patients with later onset.

Etiology unknown; both sporadic and inherited cases have been reported. Transmission may be congenital with simple autosomal dominance, with reduced penetrance, or recessive inheritance. In affected family there is a frequent occurrence among twins.

In 1869, while employed as assistant to Jonathan Hutchinson at the London Hospital, Nettleship published a brief report concerning a child with an unusual form of urticaria. He gave a graphic account of the skin lesions of Emile P, aged two years, living at Blackheath Hill, who was admitted to the hospital on July 27, 1879, with a chronic urticarial skin eruption, which had appeared when she was aged three months. Nettleship stated "A remarkable feature is that the wheals have brown stains. The appearance produced is very singular as the child, being of beautifully fair skin, the brown patches are the more conspicuous. At first sight the diagnosis of chloasma would occur to most, but the entire absence of branniness and the early age of the patient are fatal to this suspicion. It has also been set at rest by the microscope. The urticarious irritability of the child's skin is proved at once on scratching it." Nettleship noted that there was no history of urticaria in other members of the family."

• E. Nettleship:
  Chronic urticaria leaving brown stains: nearly two years’ duration.
  British Medical Journal, London, 1869, 2: 435.
  
  Rare forms of urticaria.
  British Medical Journal, London, 1869, 2: 323-324.