11 year old boy with alopecia & madarosis with loss of all teeth except upper two as shown in pic with dry skin all over the body since birth. all features especially facial features resembling senility. kindly suggest diagnosis/investigation needed/management?




Congenital ectodermal dysplasia. 1.. Hair changes including sparse and thick hair. No eye brows hair and no eye lashes. 2. Peg shaped teeth and other teeth deformities. 3. Nails are thick with ridges. 4. Hyperpigmented skin These features are suggestive of ectodermal dysplasia

ectosermal dysplasia but hot water bath on head may improove rich blood supply over to scalp nutrition of hair will be improove

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Congenital hypohidrotic ECTODERMAL DYSPLASIA Inherited condition 2 or more ectodermally derived anatomic structures fail to develop.. CLINICAL FEATURES 1. Affected individuals typically display heat intolerance because of a reduced number of eccrine sweat glands. 2. fever of undetermined origin 3. fine, sparse hair, including a reduced density of eyebrow and eyelash hair 4. periocular skin may show a fine wrinkling with hyperpigmentation. 5. salivary glands may be hypoplastic or absent 6. patients may exhibit varying degrees of xerostomia. 7. The nails may also appear dystrophic and brittle 8. The teeth are usually markedly reduced in number Oligodontia or hypodontia and their crown shapes are characteristically abnormal 9. The incisor crowns usually appear tapered, conical, or pointed, and the molar crowns are reduced in diameter. TREATMENT The dental problems are best managed by prosthetic replacement of the dentition with complete dentures, overdentures, or fixed appliances, depending on the number and location of the remaining teeth. With careful site selection, endosseous dental implants may be considered

Congenital unhidrotic Ectodermal dysplasia.

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Anhidrotic (hypohidrotic) Ectodermal Dysplasia

Hypohidrotic ectodermal dysplasia (also known as "anhidrotic ectodermal dysplasia", and "Christ-Siemens-Touraine syndrome"is one of about 150 types of ectodermal dysplasia in humans. Before birth, these disorders result in the abnormal development of structures including the skin, hair, nails, teeth, and sweat gland

Anhydrotic ectodermal dysplasia. In this condition there will be no sweat glands eye lashes are absent. Sparse hair and hot dry skin chlid always prefers to lie in water or bath frequently. Frequently child will get hyper thermia can be diagnosed by sweat test. And skin biopsy

Can you please tell me about the child’s habits is he prefers cold ?is he sweats on exercion? Is he having peg shaped teeth?

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Congenital anhydrotic ectodermal dysplasia Rare. Please rule out secondary syphilis. This is a major differential diagnosis. And often missed. Do a simple VDRL. TPHA. Test. Management changes if diagnosis changes.

Congenital ectodermal dysplasia

Congenital ectodermal dysplasia / Hypohidrotic dysplasia

Congenital ectodermal dysplasia.

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