2026 ICD-10-CM Diagnosis Code Q82.4Ectodermal dysplasia (anhidrotic)

AHRQ’s CCSR groups this code into broader clinical categories.

• Ectodermal Dysplasia

  a group of hereditary disorders involving tissues and structures derived from the embryonic ectoderm. they are characterized by the presence of abnormalities at birth and involvement of both the epidermis and skin appendages. they are generally nonprogressive and diffuse. various forms exist, including anhidrotic and hidrotic dysplasias, focal dermal hypoplasia, and aplasia cutis congenita.

• Ectodermal Dysplasia 1, Anhidrotic

  an x-linked form of ectodermal dysplasia which results from mutations of the gene encoding ectodysplasin.

• Ectodermal Dysplasia 3, Anhidrotic

  an autosomal dominant form of ectodermal dysplasia which is due to mutations in the gene for the edar receptor.

• Ectodermal Dysplasia, Hypohidrotic, Autosomal Recessive

  an autosomal recessive form of ectodermal dysplasia which is due to mutations in the gene for the edar receptor or edar-associated death domain protein.

• Focal Facial Dermal Dysplasias

  a heterogenous group of genetic disorders characterized by scar-like atrophic lesions on the temple region of the head including preauricular area. location of skin defects is likely related to defects in fusion of embryonic facial prominences during development of the face. focal facial dermal dysplasia (ffdd) is generally divided into four subtypes according to the location of the lesions and inheritance pattern: ffdd1 (brauer syndrome); ffdd2 (brauer-setleis syndrome); ffdd3 (setleis syndrome); and ffdd4. mutations in twist2 protein and/or cyp26c1 (see cyp26 family) are associated with ffdd3, and 4.

• Acanthosis Nigricans

  a circumscribed melanosis consisting of a brown-pigmented, velvety verrucosity or fine papillomatosis appearing in the axillae and other body folds. it occurs in association with endocrine disorders, underlying malignancy, administration of certain drugs, or as in inherited disorder.

• Hypohidrosis

  abnormally diminished or absent perspiration. both generalized and segmented (reduced or absent sweating in circumscribed locations) forms of the disease are usually associated with other underlying conditions.

• Focal Dermal Hypoplasia

  a genetic skin disease characterized by hypoplasia of the dermis, herniations of fat, and hand anomalies. it is found exclusively in females and transmitted as an x-linked dominant trait.

• Edar Receptor

  a ectodysplasin receptor subtype that is specific for ectodysplasin a1. it signals via the specific signaling adaptor edar-associated death domain protein. loss of function of the edar receptor is associated with autosomal recessive anhidrotic ectodermal dysplasia and ectodermal dysplasia 3, anhidrotic.

• Anhidrotic Ectodermal Dysplasia 1

  a rare genetic disorder characterized by mutations in the gene encoding ectodysplasin a. it results in abnormalities in the morphogenesis of the structures originating from the ectoderm.

• Autosomal Recessive Hypohidrotic Ectodermal Dysplasia

  a rare autosomal recessive disorder characterized by developmental abnormalities of the skin, sweat glands, hair and nails. patients have a reduced ability to sweat. other signs and symptoms include hypotrichosis and teeth malformations.

• Cleft Lip/Palate-Ectodermal Dysplasia Syndrome|CLPED1

  a very rare genetic disorder characterized by cleft lip and palate, sparse scalp hair, and partial syndactyly of the fingers and toes.

• Cranioectodermal Dysplasia|Sensenbrenner Syndrome

  an autosomal recessive disorder associated with mutation(s) in at least one of four genes (wdr35, ift122, wdr19, or ift43). it is characterized by distinctive abnormalities of the face and skull, in association with developmental abnormalities of the structures derived from ectodermal tissues.

• Ectodermal Dysplasia

  a group of inherited disorders characterized by malformations of the structures that derive from the ectoderm, such as hair, teeth, nails and sweat glands.

• Ectodermal Dysplasia and Immunodeficiency 1|EDAID1|IKBKG/NEMO|NEMO Deficiency Syndrome

  an x-linked recessive condition caused by mutation(s) in the ikbkg gene, encoding nf-kappa-b essential modulator (nemo). it is characterized by the onset of recurrent severe infections due to immunodeficiency in early infancy or in the first years of life. affected individuals may present with ectodermal dysplasia, including conical incisors, hypo/anhidrosis, and thin skin or hair.

• Ectodermal Dysplasia and Immunodeficiency 2|Autosomal Dominant Anhidrotic Ectodermal Dysplasia|EDAID2|Ectodermal Dysplasia, Anhidrotic, with Immunodeficiency 2|Ectodermal Dysplasia, Anhidrotic, with T-Cell Immunodeficiency, Autosomal Dominant|Ectodermal Dysplasia, Hypohidrotic, with Immunodeficiency 2

  a rare disorder caused by mutations in the nfkbia gene resulting in an autosomal dominant inheritance pattern. it is characterized by abnormal development of ectodermal tissues including the skin, hair, teeth, and sweat glands and immune system deficiency. it results in dry and wrinkled skin, sparse scalp and body hair, missing teeth, and reduced ability to sweat. patients have abnormally low levels of antibodies causing inability to fight infections.

• Ectodermal Dysplasia and Immunodeficiency|Anhidrotic Ectodermal Dysplasia with Immune Deficiency|EDA-ID|Hypohidrotic Ectodermal Dysplasia with Immune Deficiency

  a rare disorder caused by mutations either in the ikbkg gene resulting in an x-linked recessive inheritance pattern or in the nfkbia gene resulting in an autosomal dominant inheritance pattern. it is characterized by abnormal development of ectodermal tissues including the skin, hair, teeth, and sweat glands and immune system deficiency. it results in dry and wrinkled skin, sparse scalp and body hair, missing teeth, and reduced ability to sweat. patients have abnormally low levels of antibodies causing inability to fight infections.

• Ectodysplasin-A1|ED1-A1|EDA Protein|EDA-A1|EDA1|Ectodermal Dysplasia Protein 4|Ectodysplasin A|Ectodysplasin A Isoform 1|Ectodysplasin A1|Ectodysplasin A1 Isoform|Ectodysplasin-A|Ectodysplasin-A Isoform 1|Tumor Necrosis Factor Ligand 7C|X-Linked Anhidroitic Ectodermal Dysplasia Protein

  ectodysplasin-a1 (391 aa, ~41 kda) is encoded by the human eda gene. this protein plays a role in the positive regulation of signaling mediated by tumor necrosis factor receptor superfamily member edar.

• Ectrodactyly-Ectodermal Dysplasia-Cleft Syndrome|EEC Syndrome

  a rare form of ectodermal dysplasia, inherited in an autosomal dominant fashion, manifesting with varying degrees of severity, ectrodactyly and cleft lip/palate.

• EDA wt Allele|ECTD1|ED1|ED1-A1|ED1-A2|EDA|EDA-A1|EDA-A2|EDA1|EDA2|Ectodermal Dysplasia 1, Anhidrotic Gene|Ectodysplasin A wt Allele|Ectodysplasin Gene|HED|HED1|ODT1|Oligodontia 1 Gene|STHAGX1|TNLG7C|XHED|XLHED

  human eda wild-type allele is located in the vicinity of xq13.1 and is approximately 423 kb in length. this allele, which encodes ectodysplasin-a protein, is involved in the morphogenesis of ectodermally derived tissues. mutation of the gene is associated with x-linked hypohidrotic ectodermal dysplasia type 1 and x-linked, selective tooth agenesis type 1.

• Ellis-Van Creveld Syndrome|Chondroectodermal Dysplasia|Chondroectodermal Dysplasia

  a rare autosomal recessive syndrome caused by mutations in the evc gene. it is characterized by dwarfism, small chest, ectodermal dysplasia, and postaxial polydactyly. there is an increased frequency of congenital heart malformations.

• NECTIN1 wt Allele|CD111|CLPED1|ED4|Ectodermal Dysplasia 4 (Margarita Island Type) Gene|HIgR|HV1S|HVEC|Herpes Simplex Virus Type 1 Sensitivity Gene|Nectin Cell Adhesion Molecule 1 wt Allele|OFC7|PRR|PRR1|PVRL1|PVRR|PVRR1|Poliovirus Receptor-Related 1 (Herpesvirus Entry Mediator C) Gene|SK-12

  human nectin1 wild-type allele is located in the vicinity of 11q23.3 and is approximately 106 kb in length. this allele, which encodes nectin-1 protein, is involved in both viral entry and the promotion of cell-cell adhesion. mutation of the gene is associated with both cleft lip/palate-ectodermal dysplasia syndrome and orofacial cleft 7.

• Tumor Necrosis Factor Receptor Superfamily Member EDAR|Anhidrotic Ectodysplasin Receptor 1|Downless Homolog|EDA-A1 Receptor|EDAR|Ectodermal Dysplasia Receptor|Ectodysplasin A Receptor|Ectodysplasin A1 Isoform Receptor|Ectodysplasin-A Receptor

  tumor necrosis factor receptor superfamily member edar (448 aa, ~49 kda) is encoded by the human edar gene. this protein is involved in signaling that modulates the development of ectodermal structures.

• Marshall Syndrome

  an autosomal dominant condition caused by mutation(s) in the col11a1 gene, encoding collagen alpha-1(xi) chain. the syndrome may be characterized by facial dysmorphism, cataracts, myopia, hearing loss, and short stature. mutation(s) in the col11a1 gene are causative in stickler syndrome, but the phenotype of marshall syndrome is more mild.

• Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis Syndrome|Marshall Syndrome|Marshall Syndrome|PFAPA Syndrome|PFAPA Syndrome

  an autoinflammatory syndrome of childhood which often resolves in adolescence, and is characterized by periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis. the fever cycle generally occurs every three to five weeks, and during the interim periods the child appears healthy; diagnosis is exclusionary.

• Acanthosis Nigricans

  a melanotic cutaneous lesion that develops in the axilla and other body folds. it may be idiopathic, drug-induced, or it may be associated with the presence of an endocrine disorder or malignancy.

• Hyperandrogenism, Insulin Resistance, Acanthosis Nigricans Syndrome|HAIR-AN Syndrome

  a condition characterized by hyperandrogenism, insulin resistance, and acanthosis nigricans, typically associated with obesity in teenage girls. it is considered to be a subtype of polycystic ovarian syndrome, but may occur in male individuals. etiology is unclear, but some cases may be associated with mutations affecting the tyrosine kinase domain of the insulin receptor.

• Insulin Resistant Diabetes Mellitus with Acanthosis Nigricans and Hyperandrogenism|Type A Insulin Resistance Syndrome

  a syndrome of insulin resistance caused by mutation(s) in the insr gene, encoding the insulin receptor. this condition is characterized by a clinical triad of hyperinsulinemia, acanthosis nigricans, and hyperandrogenism without lipodystrophy. this is the least severe of a spectrum of disorders; the other two conditions are rabson-mendenhall syndrome and donohoe syndrome.

• Grade 2 Hypohidrosis, CTCAE|Grade 2 Hypohidrosis

  symptomatic; limiting instrumental adl

• Grade 3 Hypohidrosis, CTCAE|Grade 3 Hypohidrosis

  increase in body temperature; limiting self care adl

• Grade 4 Hypohidrosis, CTCAE|Grade 4 Hypohidrosis

  heat stroke

• Grade 5 Hypohidrosis, CTCAE|Grade 5 Hypohidrosis

  death

• Hypohidrosis

  reduced sweating. causes include burns, dehydration, radiation, and leprosy.

• Hypohidrosis, CTCAE|Hypohidrosis|Hypohidrosis

  a disorder characterized by reduced sweating.

• Grade 2 Hypohidrosis, CTCAE|Grade 2 Hypohidrosis

  symptomatic; limiting instrumental adl or mild/moderate impact on age-appropriate normal daily activity (pediatric)

• Grade 3 Hypohidrosis, CTCAE|Grade 3 Hypohidrosis

  increase in body temperature; limiting self-care adl or severe impact on age-appropriate normal daily activity (pediatric)

• Grade 4 Hypohidrosis, CTCAE|Grade 4 Hypohidrosis

  life-threatening consequences; urgent intervention indicated

Skin Conditions

Your skin is your body's largest organ. It covers the entire outside of your body. There are many ways that your skin protects your body and helps keep you healthy. For example, it:

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