- Despite being a rare ailment (3 cases/100,000 births), alopecia congenita with scarring is most frequently caused by the condition known as aplasia cutis congenita (ACC).
- Treatment is not necessary. Topical petrolatum, silver sulfadiazine cream, or dressings with epidermal growth factor are examples of conservative interventions that can help to speed up healing when ulceration is present.
- ACC is a chronic illness that eventually develops a mature scar. Any bone abnormalities will typically close on their own.
Causes of Aplasia Cutis Congenita
- ACC is also part of a number of syndromes, including epidermolysis bullosa, trisomy 13, Adams-Oliver syndrome, Johanson-Blizzard syndrome, and Wolf-Hirschhorn syndrome.
- Herpes simplex and varicella-zoster virus intrauterine infections have been linked to some cases.
- ACC has also been connected to the use of a number of medicines, including misoprostol, valproic acid, methimazole, and marijuana.
- Fetus papyraceus has been linked to some cases of APC (so-called vanishing twin).
Diagnosis
The diagnosis is typically made based on clinical evidence and the appearance of localized scarring that is hairless on a newborn's vertex.Clinical Presentation
We will describe in this paragraph the clinical characteristics of ACC.Age
ACC is usually diagnosed at birth based on the characteristic appearance of the affected skin. ACC is present at birth.Number
In 70% of cases, ACC is single, but 30% of cases involve numerous lesions.Situation
About 70% of cases)involve the vertex of the scalp, which is typically immediately lateral to the midline. The back and extremities are other, less frequently affected regions.Physical Exam
- APC lesions can be 0.5 cm to more than 10 cm in size, circular, oval, linear, or stellate.
- There may be a partial or complete loss of skin. Atrophic areas typically have complete hair loss.
- There could be an ulcer, or the skin could be glossy, with a thinning or scaly surface. The surface may occasionally appear bullous.
- Some lesions may exhibit the "hair collar sign," which is characterized by a ring of longer, darker hair that surrounds the area where hair is thinning (see Figure 3). Patients with the hair collar sign are more likely to have neurologic problems or underlying bone deformities (e.g., meningomyelocele, porencephaly).
- Underlying bony or neurological abnormalities may exist in patients.
Aplasia Cutis Congenita Treatment
The size, location, and depth of the skin defect will determine how the ACC is treated. While larger flaws could necessitate surgery, smaller defects might heal on their own. In rare instances, the defect may be repaired with a skin transplant or tissue expansion.Non-drug and supportive care
No medical intervention is necessary. Topical petrolatum, silver sulfadiazine cream, or dressings with epidermal growth factor are examples of conservative interventions that can help to speed up healing when ulceration is present.Treatment Methods
Surgical intervention may be used to treat some situations. A mixed team of neurosurgeons and plastic surgeons is most suited to handle cases with underlying bone abnormalities.Prognosis
- The prognosis for ACC is generally positive, with the majority of affected people eventually regaining normal skin coverage and function.
- For larger or deeper abnormalities, there is a higher chance of infection and scarring.
- It is advised to have a dermatologist or plastic surgeon closely observe and take care of you.
Conclusion
To sum up, Aplasia Cutis Congenita (ACC) is an uncommon congenital condition marked by the absence of skin at birth. Although the exact etiology of ACC is unknown, it is believed to be related to issues with the development of the skin during embryonic development. The size, position, and depth of the skin defect will determine the course of treatment for ACC, and in some instances, the skin defect may be repaired with a skin transplant or tissue expansion. The prognosis for ACC is generally positive, with the majority of affected people eventually regaining normal skin coverage and function. However, a dermatologist or plastic surgeon is advised to provide constant supervision and treatment.Sources
- "Aplasia Cutis Congenita." MedlinePlus, U.S. National Library of Medicine, www.medlineplus.gov/ency/article/001426.htm.
- "Aplasia Cutis Congenita." Orphanet, www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=EN&Expert=2086.
- "Aplasia Cutis Congenita." The Dermatologist, www.the-dermatologist.com/content/aplasia-cutis-congenita-0.
- "Aplasia Cutis Congenita." DermNet NZ, www.dermnetnz.org/topics/aplasia-cutis-congenita/.
- "Aplasia Cutis Congenita." Genetic and Rare Diseases Information Center (GARD) - an NCATS Program, rarediseases.info.nih.gov/diseases/8274/aplasia-cutis-congenita.
- "Aplasia Cutis Congenita." eMedicine, emedicine.medscape.com/article/1105848-overview.
- Https://jeldia.com
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