Dermatology Online Journal

Case report: Prolonged collodion membrane causing constrictive bands of the digits and treatment
Jennifer B Roberts MD¹, David Adelson MD²
Dermatology Online Journal 16 (1): 15

1. University of Oklahoma Health Sciences Center
2. Associate Professor, Department of Dermatology, University of Oklahoma Health Sciences Center, Tulsa, Oklahoma. david-adelson@ouhsc.edu

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Abstract

Collodion babies present unique diagnostic and management challenges. We describe a 2-month-old female with lamellar ichthyosis and prolonged collodion membrane with two constrictive bands of the fingers causing significant erythema, edema, and pain. We suggest prompt release of these constrictive bands with an 11-blade and closely follow up of collodion babies until their membrane has fully desquamated.

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Case report

Figure 1	Figure 2

A 2-month-old female with a collodion membrane presented to the dermatology clinic with a two-week history of left fourth and left third digit constrictive bands at the distal interphalangeal joints causing severe erythema and edema. This patient was first seen by dermatology as an inpatient consult in the neonatal intensive care unit following an otherwise uneventful 37-week term delivery. The patient developed hypothermia on the first day of life that required treatment in an incubator and applications of hydrophilic ointment. The hypothermia resolved on day four and the patient was discharged without further complications on day ten. The patient returned to the dermatology clinic at 2 months for a follow up visit. Physical exam was notable for total ichthyosis and mild erythema that spared the genital region. The infant exhibited ectropion, eclabium, and residual collodion membrane on the scalp, as well as the extensor surface of the legs, arms and circumferentially around the fingers. The marked erythema, edema and pain of the fingers suggested substantial risk for autoamputation (Figures 1-3).

Figure 3	Figure 4

Using an 11-blade, the constrictive bands were cut on the palmar aspect. Immediately following the release of the constrictive bands, the fingers improved and exhibited less erythema and edema. At a one week follow up appointment, the left fourth digit and right third digit edema and erythema had resolved and these fingers were indistinguishable from the other fingers (Figure 4). The patient continued to have persistent total ichthyosis and mild erythema. These findings prompted a pediatric genetics consult. A hearing screen was normal.

The differential diagnosis for this patient with a prolonged collodion membrane and normal hearing screen primarily includes lamellar ichthyosis, congenital ichthyosiform erythroderma, Sjogren-Larsson Syndrome, and loricrin keratoderma, the variant form of Vohlwinkel syndrome [2]. Genetics testing for connexin 26 and connexin 30 were normal. Transglutaminase 1 (TGM1) gene analysis revealed a heterozygous state for two mutations G291D and IVS5+2 on separate alleles; both of these mutations have been previously reported in association with lamellar ichthyosis [1].

Discussion

The collodion baby is a rare phenomenon, but a common presentation of congenital ichthyoses. The entire body is covered in a parchment-like membrane. The collodion membrane usually completely peels off in 2 weeks, however, a prolonged membrane may not completely peel off until 12 weeks [2].

The classic clinical findings of lamellar ichthyosis are large, dark platelike-scales with little or no erythema, whereas patients with congenital ichthyosiform erythroderma have a fine scale with prominent erythema. However, lamellar ichthyosis and congenital ichthyosiform erythroderma exist on a continuum with many intermediate phenotypes [4, 5]. Diagnosing a collodion baby can sometimes take longer than a year because patients may change from predominantly scaly skin to predominantly erythematous skin during the first year of life [6].

Collodion babies have increased mobidity and mortality. Documented complications for collodion babies include skin infections, fissures, conjunctivitis, sepsis, hypothermia, dehydration, and constrictive bands of the extremities resulting in vascular compromise and edema [2]. Treatment of the collodion baby during the neonatal period should include a humidified incubator, hydrophilic ointment, and supportive care [2].

We report an unusual case of a prolonged collodion membrane in a patient with lamellar ichthyosis causing constrictive bands of the fingers that compromised vasculature and threatened an autoamputation. Also, we describe the promptly effective treatment of releasing these constrictive bands with an 11 blade on the palmar aspect. We suggest the importance of following infants born with collodion membranes until the membrane has fully desquamated.

References

1. Farasat J et al. Novel transglutaminase-1 mutations and genotype-phenotype investigations of 104 patients with autosomal recessive congenital ichthyosis in the USA. J Med Genet. 2009;46:103-111.

2. Van Gysel, Lijnen RLP et al. Collodion baby: a follow-up study of 17 cases. Eur Acad Dermatol Venereol. 2002;16(5):472-5. [PubMed]

3. Oji V. Traupe H. Ichthyoses: Differential diagnosis and molecular genetics. Eur J Dermatol. 2006 Jul-Aug;16(4)349-59. [PubMed]

4. Schmuth M. Gruber R. Elias PM. Williams ML. Ichthyosis update: Towards a function-driven model of pathogenesis of the disorders of cornification and the role of corneocyte proteins in these disorders. Adv Dermatol. 2007;23:231-56. [PubMed]

5. Akiyama M. Sawamura D. Shimizu H. The clinical spectrum of nonbullous congenital ichthyosiform erythroderma and lamellar ichthyosis. Clin Exp Dermatol. 2003 May;28(3):235-40. [PubMed]

6. Shwayder T. Disorders of keratinization: diagnosis and management. Am J Clin Dermatol. 2004;5(1):17-29. Review. [PubMed]

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