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Type VI Aplasia Cutis Congenita: Bart's Syndrome.

Kulalı F, Bas AY, Kale Y, Celik IH, Demirel N, Apaydın S - Case Rep Dermatol Med (2015)

Bottom Line: Bart's syndrome (BS) is diagnosed clinically based on the disorder's unique signs and symptoms but histologic evaluation of the skin can help to confirm the final diagnosis.The patient was managed conservatively with topical antibacterial ointment and wet gauze dressing.Periodic follow-up examinations showed complete healing.

View Article: PubMed Central - PubMed

Affiliation: Division of Neonatology, Etlik Zübeyde Hanim Women's Health Teaching and Research Hospital, Ankara, Turkey.

ABSTRACT
Bart's syndrome is characterized by aplasia cutis congenita and epidermolysis bullosa. We present the case of a newborn male who developed blisters on the mucous membranes and the skin following congenital localized absence of skin. Bart's syndrome (BS) is diagnosed clinically based on the disorder's unique signs and symptoms but histologic evaluation of the skin can help to confirm the final diagnosis. The patient was managed conservatively with topical antibacterial ointment and wet gauze dressing. Periodic follow-up examinations showed complete healing. We emphasized that it is important to use relatively simple methods for optimal healing without the need for complex surgical interventions.

No MeSH data available.


Related in: MedlinePlus

Sharply demarcated lesion margin.
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fig1: Sharply demarcated lesion margin.

Mentions: He had normal weight, length, head circumference, and vital signs. On physical examination, he was found to have absence of skin over the anteromedial aspect of both lower legs, starting from the knees and extending to dorsal and medial plantar aspect of the feet. The lesions have sharply demarcated borders covered by a red ultrathin translucent membrane (Figure 1). On the second day, he developed blisters on the upper lip mucous membranes and on the left wrist (Figure 2). Results of his complete blood count, liver and renal function tests, electrolytes, ionized calcium, and magnesium were within normal limits. Serologic tests for TORCH were negative. Ophthalmological examination, abdominal and cranial ultrasound screening, and echocardiography revealed normal findings. Histopathological examination of the left wrist lesion demonstrated full or partial developed subepithelial vesicle formation (Figure 3). Direct immunofluorescent staining showed weak focal perivascular deposition of IgM, IgA, C3, and Fibrinogen but did not demonstrate IgG, C4, and C1q depositions that have suggested dystrophic epidermolysis bullosa. The combination of aplasia cutis congenital (ACC) and EB led to the diagnosis of Bart's Syndrome.


Type VI Aplasia Cutis Congenita: Bart's Syndrome.

Kulalı F, Bas AY, Kale Y, Celik IH, Demirel N, Apaydın S - Case Rep Dermatol Med (2015)

Sharply demarcated lesion margin.
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4644546&req=5

fig1: Sharply demarcated lesion margin.
Mentions: He had normal weight, length, head circumference, and vital signs. On physical examination, he was found to have absence of skin over the anteromedial aspect of both lower legs, starting from the knees and extending to dorsal and medial plantar aspect of the feet. The lesions have sharply demarcated borders covered by a red ultrathin translucent membrane (Figure 1). On the second day, he developed blisters on the upper lip mucous membranes and on the left wrist (Figure 2). Results of his complete blood count, liver and renal function tests, electrolytes, ionized calcium, and magnesium were within normal limits. Serologic tests for TORCH were negative. Ophthalmological examination, abdominal and cranial ultrasound screening, and echocardiography revealed normal findings. Histopathological examination of the left wrist lesion demonstrated full or partial developed subepithelial vesicle formation (Figure 3). Direct immunofluorescent staining showed weak focal perivascular deposition of IgM, IgA, C3, and Fibrinogen but did not demonstrate IgG, C4, and C1q depositions that have suggested dystrophic epidermolysis bullosa. The combination of aplasia cutis congenital (ACC) and EB led to the diagnosis of Bart's Syndrome.

Bottom Line: Bart's syndrome (BS) is diagnosed clinically based on the disorder's unique signs and symptoms but histologic evaluation of the skin can help to confirm the final diagnosis.The patient was managed conservatively with topical antibacterial ointment and wet gauze dressing.Periodic follow-up examinations showed complete healing.

View Article: PubMed Central - PubMed

Affiliation: Division of Neonatology, Etlik Zübeyde Hanim Women's Health Teaching and Research Hospital, Ankara, Turkey.

ABSTRACT
Bart's syndrome is characterized by aplasia cutis congenita and epidermolysis bullosa. We present the case of a newborn male who developed blisters on the mucous membranes and the skin following congenital localized absence of skin. Bart's syndrome (BS) is diagnosed clinically based on the disorder's unique signs and symptoms but histologic evaluation of the skin can help to confirm the final diagnosis. The patient was managed conservatively with topical antibacterial ointment and wet gauze dressing. Periodic follow-up examinations showed complete healing. We emphasized that it is important to use relatively simple methods for optimal healing without the need for complex surgical interventions.

No MeSH data available.


Related in: MedlinePlus