Congenital onychodysplasia of the index fingers. An abnormal grip theory

Congenital onychodysplasia of the index fingers, a condition characterized by hypoplasias and dysplasias of the nails peculiarly restricted to the index fingers, is assumed to be related to a peculiar grip of the thumbs leading to ischemia of the index fingers while the baby is in utero. This is based on our observation of a case two days after birth. Major characteristics of this condition, including index finger involvement and radial-prone involvement of the nails, can be well explained by this hypothesis, as can exceptional cases of middle finger involvement. A case of acquired polyonychia is presented which also appears to favor the ischemia theory for this condition.     

Congenital onychodysplasia of the index fingers - Iso-Kikuchi syndrome. A case involving the second toenail

We report a case of congenital onychodysplasia of the index fingers (Iso-Kikuchi syndrome) in a 2-year-old boy who had nail deformities on both index fingers and the left second toe. He had a micronychia of (he left index fingernail, malalignmcnt and abnormal lunula of the right index fingernail and micronychia and misalignment of the left second toenail. Congenital onychodysplasia of the index fingers (COIF) is a rare condition characterized by various forms of nail dysplasia commonly involving the index fingers, but not infrequently also the neighbouring fingers such as the middle fingers and thumbs.^1,2 The five criteria characterizing COIF include the following:) congenital occurrence; (ii) unilateral or bilateral index finger involvement; (Hi) variability in nail appearance; (iv) possible hereditary involvement; and (v) frequent) associated bone abnormalities.³ The nails of COIF include the full spectrum of nail dysplasia, from an irregular lunula, malalignmeni, micronychia (hypoplastic and rudimental), polyonychia (split rudimental), and anonychia, specifically affecting the index fingers.⁴ Our patient represents various forms of nail dysplasia of the both index fingers and left second toe such as micronychia, malalignment and abnormal lunula. To our knowledge, the association with second toenail dysplasia in COIF has not previously been reported     

Congenital onychodysplasia: Iso-Kikuchi syndrome

We report the first German case of Iso-Kikuchi syndrome, which is characterized by a congenital onychodysplasia commonly involving the index fingers, but not infrequently also the neighbouring fingers, and typical bony alterations of the corresponding terminal phalanges. This is also the first report of associated great toe anomalies.     

Epidermolysis bullosa acquisita in identical twins

Identical twin sisters developed generalized erythema and bullae on skin and mucous membranes at 18 and 19 years of age. Atrophic scars and milia were formed later. Indirect immunofluorescence (IF) study of the separated skin by incubation in 1.0M NaCl showed antibasement membrane zone (BMZ) antibodies bound to the dermal side. The HLA-DR typing demonstrated DR2/DW11. Pulse therapy resulted in marked clinical improvement. This is the first report of epidermolysis bullosa acquisita (EBA) in identical twins and suggests the possibility that the disease may have a genetic component.