Shortly after an uncomplicated preg...

Shortly after an uncomplicated pregnancy and birth, a boundary female newborn was noted to have a midline lesion in succession the posterior aspect of her scalp (Figure 1) The lesion was hairless, measuring 05 cm in diameter, with a slightly denud appearance and a revolveed edge (Figure 2). The child also had an associated "stork bite" skin change surrounding the lesion. The lesion remained unchanged at posterior well-child visits. The hair at the immediate periphery of the lesion assumeed to grow much faster than the quiescence of the child's hair, although the lesion remained hairless. The hair surrounding the lesion had a coarser, kinked web compared with the remainder of the child's hair. The child was neurodevelopmentally normal.

[FIGURES 1-2 OMITTED]

Question

Based upon the patient's history and physical examination, which undivided of the following is the greatest in quantity likely diagnosis?

[] A. Langerhans small cavity histiocytosis.

[] B Sebaceous nevus.

[] C Neonatal herpes infection.

[] D Aplasia cutis congenita.

[] E Transient bullous dermolysis of the newborn.

[] F Focal dermal hypoplasia.

Discussion

The answer is D: aplasia cutis congenita. Aplasia cutis congenita is a condition in which there is a congenital absence of skin with or without the absence of underlying conformations such as bone or dura. (12)

Aplasia cutis congenita quick in emergenciess as a well-demarcated, noninflammatory, circular or oval blister or erod region, usually at the scalp top just lateral to the midline, although other areas of the dead body also may be involved. Although aplasia cutis congenita frequently appears as a solitary lesion (70 percent) the condition may current as multiple lesions, typically 1 to 2 cm in diameter. (3) Solitary lesions range in size from 05 to 10 cm The appearance varies depending in succession when the lesion occurred in utero Lesions formed early in gestation might heal before delivery. similar a lesion may appear as an atrophic, fibrotic, alopecic scar. A more late lesion, however, would present as an gathering The depth varies: more superficial destitutions have minimal scarring and hair los whereas others penetrate the discerning dermis and subcutaneous tissue, reaching the brain-pan and dura. (1) Distorted hair vegetation surrounding a scalp lesion shows a "hair collar sign." This can be a marker for underlying wants (3) Neuroimaging with ultrasonography frequently is recommended for further evaluation of an intracranial component

greatest in quantity patients with aplasia cutis congenita have no other abnormalities, although the condition may be associated with congenital malformations involving the cardiovascular, gastrointestinal, genitourinary, and central nervous rules Larger lesions are more likely to be associated with other blemishs Lesions with findings such as vascular flushing, tethering (which may be a sign of a dural attachment), or abnormal surrounding hair may signal further abnormalities and warrant evaluation. (14)

The etiology of this disease is unclear and appears to be multifactorial; teratogens, genetic factors, trauma, and compromised skin perfusion each appear to be to play a role. (1) There are case reports of aplasia cutis congenita in infants of mothers receiving methimazole (Tapazole), although causation has not been established. Familial incidents also are reported. (5)

There is no consensus concerning the treatment of aplasia cutis congenita. Management be pendents on the size of the blemish and the physical condition of the child. Complications can include meningitis, hemorrhage, and local infection; however, small sore s typically heal with formation of a hairless scar from one side of to the other several weeks, and bony faults tend to close spontaneously within the first year. A conservative approach frequently is adopted because of the self-limited course. (1) Multiple or large gatherings require excision and surgical closure because of the risk of hemorrhage and infection. (1)

Langerhans enclosed space histiocytosis (formerly histiocytosis X) is a proliferative disorder of Langerhans small cavitys that can occur in many organ arrangements The scalp often is affected in adults and infants. The lesions usually are multiple, erythematous, scaling patches that can, at times, compare seborrheic dermatitis. (6)

Sebaceous nevus is a congenital lesion made up of excessive sebaceous glands and malformed hair follicles. Lesions typically are hairless and yellowish and repeatedly occur on the scalp or face. This lesion is rare, affecting merely 0.3 percent of newborns. Sebaceous nevus lesions carry a 20 percent lifetime risk of cancerous transformation. (3)

Neonatal herpes infection may current as a scalp ulceration or erosion. The lesions usually are erythematous vesicles before ulceration and become crusted as they progres Lesions ofttimes are multiple. There may be a history of maternal genital herpes infection, and the infant is repeatedly ill with signs of sepsis. (13)

Transient bullous dermolysis of the newborn is a rare, benign, blistering disease that not past nor futures at or near birth and analyzes during the first few month of life. (6) It is a form of dystrophic epidermolysis bullosa. Epidermolysis bullosa is a dispose of rare, inherited bullous disorders characterized through blister formation in the epidermal layer secondary to mechanical trauma. Blisters usually are precipitated from an identifiable traumatic event. (1)