The amniotic band syndrome is an uncommon congenital fetal abnormality, presumably due to fetal entanglement in strands of ruptured amniotic sac. The defects caused by this syndrome vary from simple limb defects to major visceral and craniofacial defects. We cared for a newborn infant with this syndrome, who showed constriction rings of the right leg and right axilla, right club foot, thoracic scoliosis, polydactyly, absence of the right thumb and aplasia cutis of the scalp. We report this case with a brief review of the literature.
Amniotic Band Syndrome ; Axilla ; Constriction ; Ectodermal Dysplasia ; Extremities ; Foot ; Humans ; Infant, Newborn ; Leg ; Limb Deformities, Congenital ; Polydactyly ; Scalp ; Scalp Dermatoses ; Scoliosis ; Thumb

Diagnosis, Differential ; Erythema Multiforme ; diagnosis ; Erythema Nodosum ; diagnosis ; Female ; Humans ; Leg Dermatoses ; diagnosis ; Middle Aged ; Mycosis Fungoides ; diagnosis ; Skin Neoplasms ; diagnosis ; Sweet Syndrome ; diagnosis

Stasis dermatitis is an itchy, scaly, and hyperpigmented condition of the lower leg due to venous insufficiency. Hemosiderin and/or melanin have been considered responsible for the brown pigmentation. However, there are not sufficient histopathologic studies. In this retrospective study the hospital records and biopsy slides of 20 patients were reviewed to determine the pathogenetic mechanisms of brown pigmentation in stasis dermatitis. Fifteen were men (75%) and 5 were women (25%) with a mean age of 46.2+/-8.2 yr (18-76), mean age at onset of 43.4+/-18.0 yr (17-73), and a mean duration of the disease 2.8+/-2.5 yr (0.25-10). All patients had varicose vein and complained of pruritus. On histopathologic evaluation, two cases out of 20 (3 skin biopsy specimens from 25 samples) showed dermal melanocytes containing melanin, and incontinence of melanin pigment was observed in 5 cases, which indicates that melanin pigments from epidermis could contribute to cutaneous pigmentation in stasis dermatitis. However, the existence of dermal melanocytes in two cases cannot be explained because normally the dermis contains no melanocytes. Further studies concerning the role of iron or inflammatory cytokines on the development of dermal melanocytes should be conducted.
Adolescent ; Adult ; Aged ; Dermatitis/etiology/metabolism/*pathology ; Dermis/metabolism/pathology ; Female ; Humans ; Hyperpigmentation/etiology/metabolism/*pathology ; Leg Dermatoses/etiology/metabolism/pathology ; Male ; Melanins/metabolism ; Melanocytes/metabolism/*pathology ; Middle Aged ; Venous Insufficiency/complications