A Report of Amniotic Band Syndrome and with a Review of Constricting Band Syndromes Luke Maxfield, DO,* Muneeb Shah,** Sarah Belden, DO,*** Jason Barr, DO****

*Transitional Year Intern, Sampson Regional Medical Center, Clinton, NC **Medical Student, 3rd year, Nova Southeastern University College of Osteopathic Medicine, Clearwater, FL ***Dermatology Resident, 2nd year, Affiliated Dermatology/Midwestern University OPTI, Scottsdale, AZ **** Surgical and Medical Dermatologist, Affiliated Dermatology/Midwestern University OPTI, Scottsdale, AZ

Disclosures: None Correspondence: Luke Maxfield, DO; [email protected]

Abstract Amniotic bands are intrauterine fibrous cords that adhere to the growing fetus and cause mechanical trauma or vascular compromise. Associated defects range from hand creases to amputation, or even death in severe cases. We present a patient with a history of amniotic band syndrome with right leg amputation, constriction of the right fourth digit, and new-onset keratoderma involving the left plantar foot and bilateral palmar hands. This patient’s medical presentation emphasizes the importance of understanding the relationships between constriction band syndromes and . We review previous literature documenting the association between amniotic band syndrome, pseudoainhum, and palmoplantar keratoderma. Introduction Discussion Although some authors have asserted that Constriction bands, which result in significant amniotic band lesions and pseudoainhum are morbidity and mortality, are seen in amniotic band distinct disease processes, pseudoainhum has been syndrome (ABS),1 , and pseudoainhum.2 seen in patients with amniotic band syndrome. ABS is thought to result from tears in the amnion While both diseases involve constricting cutaneous that lead to fibrous adhesions to, and constrictions lesions, their onset, causality, and relationships are of, the developing fetus. The incidence is thought dramatically different. We present a patient with a to be 1:10,000. Maternal risk factors for ABS history of amniotic band syndrome with resulting include , connective tissue right lower extremity amputation who presented disorders, and prenatal abdominal trauma, such as to our clinic with new-onset keratoderma of amniocentesis. Ainhum, which is most prevalent the bilateral palms and left plantar foot. We in continental sub-Saharan Africa, is defined as also provide a literature review of palmoplantar idiopathic auto-amputation of a digit due to a associated with amniotic band constriction band. Pseudoainhum presents as a syndrome and pseudoainhum. circumferential constriction band, usually around Figure 1. Focal hyperkeratotic plaques of the a digit, that has the potential to progress to auto- left foot with secondary fissuring. Case Presentation amputation. Unlike ainhum, pseudoainhum has A 26-year-old Caucasian female presented with no racial predilection.2 It has been described roughening over the plantar portion of her left foot as secondary, sporadic, and a component of that had been progressing over the past several years. hereditary palmoplantar keratodermas, as well The patient had attempted to soften the area using as in association with .3-5 While many foot soaks, a pumice stone, topical antifungals, and authors exclude cases of ABS auto-amputation topical clobetasol over this same period, without from the pseudoainhum category,2 others use noticeable relief. The patient denied any previous “pseudoainhum” and “ABS” interchangeably, similar episodes. She also denied a family history with the rationale that the constriction bands are of palmoplantar keratoderma or other cutaneous secondary to amniotic band syndrome.6,7 pathologies. Her medical history was positive for childhood eczema that had long since resolved, Theories on the pathogenesis of amniotic band right lower extremity amputation, and a congenital syndrome are speculative and point to both constriction band deformity of the right fourth intrinsic and extrinsic processes. The intrinsic finger. She took no prescription medications but theory states that deformities result from defective did take multivitamins. Physical exam showed germ cells within the embryo. The extrinsic theory focal hyperkeratotic plaques on the weight-bearing states that an early rupture of amnion results in surface of the left foot with secondary fissuring decreased intra-amniotic fluid, which allows (Figure 1). The hands were involved to a lesser the mesoderm to contact the fetus, resulting in degree, with thin scales over the left palm and constricting bands.7 digits bilaterally. The right hand showed a bulbous appearance of the fourth digit secondary to a focal Theories on the pathogenesis of pseudoainhum constricting band distal to the interphalangeal joint are speculative and include infectious processes, (Figure 2). Differential diagnosis included acquired fibrogenetic tendencies related to race, vascular palmoplantar keratoderma with pseudoainhum, abnormalities, and mechanical effects secondary 2 palmoplantar psoriasis, punctate palmoplantar to . Hyperkeratosis of the skin keratoderma, irritant contact , and leads to formation of a circumferential groove atopic dermatitis. The patient was sent home with around a digit. As the groove continues to Figure 2. Bulbous deformation of the distal a working diagnosis of acquired palmoplantar deepen, the underlying vasculature becomes fourth digit secondary to a focal constricting keratoderma and was prescribed topical compromised, leading to a cessation of band. Also note subtler scale over the bilateral (40%) cream to apply to the foot BID at night blood flow. The bone distal to the groove is digits and left palm. with occlusion, and 10-minute bleach bath soaks eventually separated from the body, and auto- two to three times per week. The patient failed amputation occurs. Although the presentation Palmoplantar keratodermas (PPKs) appear to return for her next appointment, and despite of pseudoainhum does not always follow this clinically as abnormal thickening of the skin of the palms and soles.8 Hyperkeratosis is present on repeated attempts to contact the patient, she was pattern, hyperkeratosis is consistently present on 5 subsequently lost to follow-up. histological exam. histological examination. PPKs can be inherited

MAXFIELD, SHAH, BELDEN, BARR Page 44 or occur sporadically and, in contrast to ABS, have References often been seen in association with pseudoainhum 1. Dyer JA, Chamlin S. Acquired raised bands of and various inherited syndromes such as mal 4,5 infancy: association with amniotic bands. Pediatr de Meleda and Vohwinkel syndrome. Mal de Dermatol. 2005;22(4):346-9. Meleda is an autosomal-recessive disease that appears early in life and is characterized by a triad 2. Rashid RM, Cowan E, Abassi SA, Brieva J, of acral hyperkeratosis, malodorous hyperhidrosis, 4 Alam M. Destructive deformation of the digits and nail abnormalities. Vohwinkel syndrome is with auto-amputation: a review of pseudo-ainhum. an autosomal-dominant disease characterized by J Eur Acad Dermatol Venereol. 2007;21(6):732-7. diffuse honeycombed palmoplantar keratoderma with associated sensorineural deafness, mental 5 3. Kumar P, Gandhi V. Pseudoainhum in Psoriasis. retardation, and alopecia. Vitamin A analogs, Indian J Dermatol. 2012;57(3):238-9. such as etretinate, have been shown to be effective in treating hereditary PPKs, as have urea-based 4. Bergman R, Bitterman-Deutsch O, Fartasch M, 2 topical formulations. Our patient’s case does not Gershoni-Baruch R, Friedman-Birnbaurm R. Mal fit into any of the known hereditary PPKs. de Meleda keratoderma with pseudoainhum. Brit J Dermatol. 1993;128:207-12. The diagnosis of ABS is primarily clinical and does not routinely require workup beyond prenatal 5. Rani Z, Ahmad TJ, Hussain I. Vohwinkel’s ultrasonography showing amniotic bands and syndrome: Case report and review of literature. J 6 restricted motion of the fetus. Similarly, in most Pak Assoc Dermatol. 2003;13:92-6. instances, pseudoainhum is a clinical diagnosis. Plain radiograph of the affected digit should be 6. Verma A, Mohan S, Kumar S. Late Presentation performed to assess the integrity of the bone distal of Amniotic Band Syndrome: A Case Report. J to the constricting band, as complete resorption Clin Diagn Res. 2007;1(2):65-8. of the digit has been observed in some cases.9 A thorough workup must be initiated to determine 7. Kibria R. Pseudo ainhum - a prospective study. if the pseudoainhum is an isolated occurrence, Professional Med J. 2014;21(1):044-8. part of a larger hereditary syndrome, or secondary rd to another disease process. A family history is 8. Patel S, Zirwas M, English JC 3 . Acquired necessary to rule out hereditary PPKs. In equivocal palmoplantar keratoderma. Am J Clin Dermatol. cases, a biopsy can be performed. 2007;8(1):1-11.

When pseudoainhum arises from psoriasis, treating 9. Arkush L, De Silva B, Gordon D. Hanging on the underlying psoriasis, with oral retinoids and by a thread: a rare case of secondary pseudoainhum. topical corticosteroids, has led to resolution in some BMJ Case Rep [Internet]. 2016 Feb 2 [cited 2016 cases.10 A combination of a topical calcineurin- April 17]. Available from: http://casereports.bmj. inhibitor and UVB phototherapy was successful com/content/2016/bcr-2015-213854.long. in a single case.11 Other reports show success with surgical release of the fibrotic band under local 10. Anwar MI, Iftikhar N, Hasnai SH, Ishaq BM. anesthesia.3 Amputation can be considered when Pseudoainhum in Acute Psoriasis. J Coll Phys Surg all other treatment modalities are exhausted, as Pak. 2012;22(12):786-8. auto-amputation is inevitable and can be painful in some cases. 11. Ahn SJ, Oh SH, Chang SE, Choi JH, Koh JK. A case of infantile psoriasis with pseudoainhum Conclusion successfully treated with topical pimecrolimus and In summary, our patient, with a previous history low-dose narrowband UVB phototherapy. J Eur of ABS, presented with new-onset PPK. While Acad Dermatol Venereol. 2006;20:1332-4. the co-occurrence of these distinct pathologies is likely coincidental, this presentation provided an opportunity to review the existing literature on ABS and constriction band syndromes. Additionally, it prompted the investigation of a seldom-explored relationship between ABS and PPK. Unfortunately, our patient, with no personal or family history of constriction band lesions or PPK, was lost to follow-up and further in-depth workup that may have been beneficial, including histology and genetic testing.

Page 45 A REPORT OF AMNIOTIC BAND SYNDROME AND KERATODERMA WITH A REVIEW OF CONSTRICTING BAND SYNDROMES