212
Proc. roy. Soc. Med. Volume 70 March 1977
Meeting 4 June 1976
(continuedftom December 1976 'Proceedings', page 879)
Cases A Coliodion Baby J P O'Connell MB DCH (for J R Harper FRCP DCH) (General Hospital, Northampton, NNI SBD)
James L, born 12.12.75 to healthy nonconsanguinous parents. History: Normal delivery at full term. Birth weight 3.1 kg. He was the first child of this marriage, the mother having two normal male children by a previous marriage. There was no significant family history. The appearance of the baby (Fig 1) approximated the description of Perez (in Petit 1880): 'The skin of the infant was replaced by a cornified substance of uniform texture, thinner at the flexures, which gave the whole body a varnished appearance. Movement of most joints was inhibited by this 'cuirasse', this great sheath ('cet ongle immense') which covered the whole body
including the orbicularis muscles, causing the mouth and eyes to be held open and giving the facies a most disagreeable aspect'. Within twenty-four hours of birth the membrane began to fissure at sites of flexion and movement exposing red, raw granulation tissue beneath. The tightly drawn collodion mask over the face caused severe ectropion and the lips to be held apart with the infant unable to suck. He was nursed naked in an incubator and simple supportive care given: emulsifying ointment to the membrane; sodium fusidate ointment to the raw areas; and methylcellulose drops to the eyes. The baby was fed by nasogastric tube. Strict asepsis was observed, in spite of which a variety of pathogens were isolated from the skin and an enteropathic E. coli was cultured from his stools. A short course of systemic antibiotics was given on two occasions. The membrane fissured, crusted and then flaked allowing full range of movement with the exception of the orbicularis oculi muscles which, as in the case described by Perez, remained immobile and restricted causing a severe degree of ectropion. Heat regulation proved a problem at the age of two months when the membrane was dry and beginning to scale. There were several episodes of hyperpyrexia when ambient temperature was raised. This feature was noted by Read et al. (1972) who attributed it to obstruction of the sweat glands by scales. A skin biopsy taken at this time (Fig 2) showed normal sweat glands with slight plugging.
Fig 2 Skin biopsy at 8 weeks showing very definite
hyperkeratosis with somefollicular plugging. The features
Fig 1 J L, aged 12 hours
seen are nonspecific but in keeping with ichthyosis
vulgaris
Section of Pediatrics
By the age of seven months there was considerable resolution of the collodion membrane and the underlying skin presents the clinical and histological appearances of ichthyosis vulgaris. Discussion
The description by Perez in 1880 was followed by sporadic accounts of infants whose skin at birth was variously described as resembling varnished silk, paraffin paper and waxed parchment; the term collodion skin, introduced in the late nineteenth century, gave such an apt description that these more fanciful terms were discarded. With agreement on a descriptive term it was nevertheless apparent that individual cases of collodion baby showed clear differences in their natural history and prognosis. Wells & Kerr (1966) suggested that collodion skin was an early and unusual expression of several types of congenital ichthyosiform erythroderma; a bullous dominant form with a distinct histological appearance, an X-linked recessive form and, most commonly, an autosomal recessive form. This unifying concept of the condition does not account for the several reported instances of collodion baby where the skin is entirely normal once the membrane is shed (Finlay & Bound 1952). Reed et al. (1972) suggest that these represent a distinct clinical entity, lamellar ichthyosis of the newborn, distinguishable from the congenital ichthyosiform erythroderma only in its evolution. Collodion baby is also occasionally a presenting feature of the Sjorgen-Larssen syndrome, and for this reason prognosis in all cases should be guarded for the first few weeks, and the baby carefully examined for signs of spasticity (Wells 1976, personal communication). In summary, 'Collodion Baby' is not a disease entity but the common representation of several well-defined disorders which can be distinguished by reference to family history, and by observing the natural history of the condition. In view of the absence of family history, the nonspecific skin biopsy, persistence of ichythyosis and normal motor and intellectual development, we conclude that in this case the collodion appearance is an expression of an autosomal recessive nonbullous ichthyosiform erythroderma.
REFERENCES Finlay H V & Bound J P (1952) Archives of Disease in Childhood 27, 434 Petit H (1880) Progress in Medicine (Paris) 8, 524 Reed W B, Herwick R P, Harville D, Porter P S & Conant M (1972) Archives of Dermatology 105, 394 Wells R S & Kerr C B (1966) British Medical Journal i, 947
213
Two Cases of Meckel's Diverticulum shown by Technetium Scanning Jennifer Cowen MRCP (for H B Valman MRCP DCH) (Northwick Park Hospital, Harrow, Middlesex) R S, aged 8-3 and V S, aged 11 months History: Both presented with acute rectal bleeding. Neither had any physical signs apart from anemia. Each had a technetium scan showing a Meckel's diverticulum, subsequently confirmed at operation and containing gastric mucosa on histology. Barium studies on R S showed an ulcer in the diverticulum. Discussion The investigations for rectal bleeding suggested by various authors include a clotting screen, sigmoidoscopy, barium studies (Benson 1969) and angiography (Faris & Whitley 1973). Of these only barium studies will show a Meckel's diverticulum and then rarely. Benson (1969) states that unexplained repeated or massive rectal bleeding is an indication for surgery but Shandling (1965) reviewed 61 laparotomies for undiagnosed rectal bleeding in children and found that 31 were negative. Of the remainder, 24 had a Meckel's diverticulum.
Fig 1 Technetium scan showing stomach, right kidney, bladder and Meckel's diverticulum (arrowed)
Proc. roy. Soc. Med. Volume 70 March 1977
Meeting 4 June 1976
(continuedftom December 1976 'Proceedings', page 879)
Cases A Coliodion Baby J P O'Connell MB DCH (for J R Harper FRCP DCH) (General Hospital, Northampton, NNI SBD)
James L, born 12.12.75 to healthy nonconsanguinous parents. History: Normal delivery at full term. Birth weight 3.1 kg. He was the first child of this marriage, the mother having two normal male children by a previous marriage. There was no significant family history. The appearance of the baby (Fig 1) approximated the description of Perez (in Petit 1880): 'The skin of the infant was replaced by a cornified substance of uniform texture, thinner at the flexures, which gave the whole body a varnished appearance. Movement of most joints was inhibited by this 'cuirasse', this great sheath ('cet ongle immense') which covered the whole body
including the orbicularis muscles, causing the mouth and eyes to be held open and giving the facies a most disagreeable aspect'. Within twenty-four hours of birth the membrane began to fissure at sites of flexion and movement exposing red, raw granulation tissue beneath. The tightly drawn collodion mask over the face caused severe ectropion and the lips to be held apart with the infant unable to suck. He was nursed naked in an incubator and simple supportive care given: emulsifying ointment to the membrane; sodium fusidate ointment to the raw areas; and methylcellulose drops to the eyes. The baby was fed by nasogastric tube. Strict asepsis was observed, in spite of which a variety of pathogens were isolated from the skin and an enteropathic E. coli was cultured from his stools. A short course of systemic antibiotics was given on two occasions. The membrane fissured, crusted and then flaked allowing full range of movement with the exception of the orbicularis oculi muscles which, as in the case described by Perez, remained immobile and restricted causing a severe degree of ectropion. Heat regulation proved a problem at the age of two months when the membrane was dry and beginning to scale. There were several episodes of hyperpyrexia when ambient temperature was raised. This feature was noted by Read et al. (1972) who attributed it to obstruction of the sweat glands by scales. A skin biopsy taken at this time (Fig 2) showed normal sweat glands with slight plugging.
Fig 2 Skin biopsy at 8 weeks showing very definite
hyperkeratosis with somefollicular plugging. The features
Fig 1 J L, aged 12 hours
seen are nonspecific but in keeping with ichthyosis
vulgaris
Section of Pediatrics
By the age of seven months there was considerable resolution of the collodion membrane and the underlying skin presents the clinical and histological appearances of ichthyosis vulgaris. Discussion
The description by Perez in 1880 was followed by sporadic accounts of infants whose skin at birth was variously described as resembling varnished silk, paraffin paper and waxed parchment; the term collodion skin, introduced in the late nineteenth century, gave such an apt description that these more fanciful terms were discarded. With agreement on a descriptive term it was nevertheless apparent that individual cases of collodion baby showed clear differences in their natural history and prognosis. Wells & Kerr (1966) suggested that collodion skin was an early and unusual expression of several types of congenital ichthyosiform erythroderma; a bullous dominant form with a distinct histological appearance, an X-linked recessive form and, most commonly, an autosomal recessive form. This unifying concept of the condition does not account for the several reported instances of collodion baby where the skin is entirely normal once the membrane is shed (Finlay & Bound 1952). Reed et al. (1972) suggest that these represent a distinct clinical entity, lamellar ichthyosis of the newborn, distinguishable from the congenital ichthyosiform erythroderma only in its evolution. Collodion baby is also occasionally a presenting feature of the Sjorgen-Larssen syndrome, and for this reason prognosis in all cases should be guarded for the first few weeks, and the baby carefully examined for signs of spasticity (Wells 1976, personal communication). In summary, 'Collodion Baby' is not a disease entity but the common representation of several well-defined disorders which can be distinguished by reference to family history, and by observing the natural history of the condition. In view of the absence of family history, the nonspecific skin biopsy, persistence of ichythyosis and normal motor and intellectual development, we conclude that in this case the collodion appearance is an expression of an autosomal recessive nonbullous ichthyosiform erythroderma.
REFERENCES Finlay H V & Bound J P (1952) Archives of Disease in Childhood 27, 434 Petit H (1880) Progress in Medicine (Paris) 8, 524 Reed W B, Herwick R P, Harville D, Porter P S & Conant M (1972) Archives of Dermatology 105, 394 Wells R S & Kerr C B (1966) British Medical Journal i, 947
213
Two Cases of Meckel's Diverticulum shown by Technetium Scanning Jennifer Cowen MRCP (for H B Valman MRCP DCH) (Northwick Park Hospital, Harrow, Middlesex) R S, aged 8-3 and V S, aged 11 months History: Both presented with acute rectal bleeding. Neither had any physical signs apart from anemia. Each had a technetium scan showing a Meckel's diverticulum, subsequently confirmed at operation and containing gastric mucosa on histology. Barium studies on R S showed an ulcer in the diverticulum. Discussion The investigations for rectal bleeding suggested by various authors include a clotting screen, sigmoidoscopy, barium studies (Benson 1969) and angiography (Faris & Whitley 1973). Of these only barium studies will show a Meckel's diverticulum and then rarely. Benson (1969) states that unexplained repeated or massive rectal bleeding is an indication for surgery but Shandling (1965) reviewed 61 laparotomies for undiagnosed rectal bleeding in children and found that 31 were negative. Of the remainder, 24 had a Meckel's diverticulum.
Fig 1 Technetium scan showing stomach, right kidney, bladder and Meckel's diverticulum (arrowed)
