ICD-10-CM Code Q82.4

Ectodermal dysplasia (anhidrotic)

Version 2020 Billable Code POA Exempt

Valid for Submission

Q82.4 is a billable code used to specify a medical diagnosis of ectodermal dysplasia (anhidrotic). The code is valid for the year 2020 for the submission of HIPAA-covered transactions. The ICD-10-CM code Q82.4 might also be used to specify conditions or terms like acanthosis nigricans, ackerman syndrome, acrorenal field defect, ectodermal dysplasia, and lipoatrophic diabetes, alopecia, onychodysplasia, hypohidrosis, deafness ectodermal dysplasia, amelo-onycho-hypohidrotic syndrome, anhidrotic ectodermal dysplasia with immune deficiency, etc The code is exempt from present on admission (POA) reporting for inpatient admissions to general acute care hospitals.

Short Description:Ectodermal dysplasia (anhidrotic)
Long Description:Ectodermal dysplasia (anhidrotic)

Tabular List of Diseases and Injuries

The Tabular List of Diseases and Injuries is a list of ICD-10 codes, organized "head to toe" into chapters and sections with guidance for inclusions, exclusions, descriptions and more. The following references are applicable to the code Q82.4:

Type 1 Excludes

Type 1 Excludes
A type 1 excludes note is a pure excludes note. It means "NOT CODED HERE!" An Excludes1 note indicates that the code excluded should never be used at the same time as the code above the Excludes1 note. An Excludes1 is used when two conditions cannot occur together, such as a congenital form versus an acquired form of the same condition.
  • Ellis-van Creveld syndrome Q77.6

Index to Diseases and Injuries

The Index to Diseases and Injuries is an alphabetical listing of medical terms, with each term mapped to one or more ICD-10 code(s). The following references for the code Q82.4 are found in the index:


The following clinical terms are approximate synonyms or lay terms that might be used to identify the correct diagnosis code:

  • Acanthosis nigricans
  • Ackerman syndrome
  • Acrorenal field defect, ectodermal dysplasia, and lipoatrophic diabetes
  • Alopecia, onychodysplasia, hypohidrosis, deafness ectodermal dysplasia
  • Amelo-onycho-hypohidrotic syndrome
  • Anhidrotic ectodermal dysplasia with immune deficiency
  • Anhidrotic ectodermal dysplasia, immunodeficiency, osteopetrosis, lymphedema syndrome
  • Anonychia
  • Anonychia with bizarre flexural pigmentation
  • Autosomal dominant hypohidrotic ectodermal dysplasia syndrome
  • Basan syndrome
  • Berlin syndrome
  • Blepharocheilodontic syndrome
  • Book syndrome
  • BRESEK syndrome
  • Cerebellar ataxia and ectodermal dysplasia
  • Congenital anomaly of macula
  • Congenital anomaly of oral mucosa
  • Congenital ectodermal dysplasia of face
  • Congenital hypoplasia of breast
  • Contracture with ectodermal dysplasia and orofacial cleft syndrome
  • Cranioectodermal dysplasia
  • Curly hair, ankyloblepharon, nail dysplasia syndrome
  • Curry-Hall syndrome
  • Cutaneous syndrome with ichthyosis
  • Dento-oculocutaneous syndrome
  • Dermodental dysplasia
  • Dermo-odonto dysplasia
  • Ectodermal dysplasia
  • Ectodermal dysplasia and sensorineural deafness syndrome
  • Ectodermal dysplasia syndactyly syndrome
  • Ectodermal dysplasia trichoodontoonychial type
  • Ectodermal dysplasia with blindness syndrome
  • Ectodermal dysplasia with ectrodactyly and macular dystrophy syndrome
  • Ectodermal dysplasia with hair-nail defect
  • Ectodermal dysplasia with hair-tooth defects
  • Ectodermal dysplasia with hair-tooth-nail defects
  • Ectodermal dysplasia with hair-tooth-nail-sweating defect
  • Ectodermal dysplasia with nail defect
  • Ectodermal dysplasia with natal teeth Turnpenny type
  • Ectodermal dysplasia with sweating defect
  • Ectodermal dysplasia with tooth-nail defects
  • Ectodermal dysplasia with tooth-nail-sweating defect
  • Ectodermal dysplasia with tooth-sweating defect
  • Ectodermal dysplasia, intellectual disability, central nervous system malformation syndrome
  • Ectodermal dysplasia, syndactyly and pili torti
  • Ectodermal dysplasia-ocular malformation syndrome
  • Ectodermal syndrome with hair-sweating defects
  • Ectodermal syndrome with hair-tooth-sweating defects
  • Familial focal facial dermal dysplasia
  • Fried's tooth and nail syndrome
  • Greither type of ectodermal dysplasia
  • Hay-Wells syndrome of ectodermal dysplasia
  • Hypohidrosis-diabetes insipidus syndrome
  • Hypohidrotic X-linked ectodermal dysplasia
  • Johanson-Blizzard syndrome
  • Keratitis ichthyosis and deafness syndrome
  • Kirman syndrome
  • Lelis syndrome
  • Limb mammary syndrome
  • Marshall syndrome
  • Melanin pigmentation of oral mucosa
  • Melanosis of mucosa of body orifice
  • Moynahan's syndrome
  • Nail and tooth abnormalities, marginal palmoplantar keratoderma, oral hyperpigmentation syndrome
  • Oculoosteocutaneous syndrome
  • Odonto onycho dysplasia with alopecia syndrome
  • Odontomicronychial ectodermal dysplasia
  • Odonto-onychial dysplasia with alopecia
  • Odonto-onycho-dermal dysplasia
  • Odonto-tricho-ungual-digito-palmar syndrome
  • Pilodental dysplasia, refractive errors syndrome
  • Robinson nail dystrophy-deafness syndrome
  • Roselli-Gulienetti ectodermal dysplasia
  • Salamon's syndrome
  • Sandman-Andra syndrome
  • Schinzel-Giedion syndrome
  • Schoepf-Schulz-Passage syndrome
  • Senter syndrome
  • Tricho-dento-osseous syndrome
  • Trichodermodysplasia and dental alterations syndrome
  • Trichodysplasia with amelogenesis imperfecta syndrome
  • Tricho-oculodermovertebral syndrome
  • Trichoodontoonychial dysplasia
  • Tricho-onychodental dysplasia
  • Zlotogora Ogur syndrome

Clinical Information

  • 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.

Diagnostic Related Groups

The ICD-10 code Q82.4 is grouped in the following groups for version MS-DRG V37.0 What are Diagnostic Related Groups?
The Diagnostic Related Groups (DRGs) are a patient classification scheme which provides a means of relating the type of patients a hospital treats. The DRGs divides all possible principal diagnoses into mutually exclusive principal diagnosis areas referred to as Major Diagnostic Categories (MDC).
applicable from 10/01/2019 through 09/30/2020.


Present on Admission (POA)

Q82.4 is exempt from POA reporting - The Present on Admission (POA) indicator is used for diagnosis codes included in claims involving inpatient admissions to general acute care hospitals. POA indicators must be reported to CMS on each claim to facilitate the grouping of diagnoses codes into the proper Diagnostic Related Groups (DRG). CMS publishes a listing of specific diagnosis codes that are exempt from the POA reporting requirement. Review other POA exempt codes here .

CMS POA Indicator Options and Definitions
POA Indicator CodePOA Reason for CodeCMS will pay the CC/MCC DRG?
YDiagnosis was present at time of inpatient admission.YES
NDiagnosis was not present at time of inpatient admission.NO
UDocumentation insufficient to determine if the condition was present at the time of inpatient admission.NO
WClinically undetermined - unable to clinically determine whether the condition was present at the time of inpatient admission.YES
1Unreported/Not used - Exempt from POA reporting. NO

Convert Q82.4 to ICD-9

  • 757.31 - Cong ectodermal dysplas

Code Classification

  • Congenital malformations, deformations and chromosomal abnormalities (Q00-Q99)
    • Other congenital malformations (Q80-Q89)
      • Other congenital malformations of skin (Q82)

Code History

  • FY 2016 - New Code, effective from 10/1/2015 through 9/30/2016
    (First year ICD-10-CM implemented into the HIPAA code set)
  • FY 2017 - No Change, effective from 10/1/2016 through 9/30/2017
  • FY 2018 - No Change, effective from 10/1/2017 through 9/30/2018
  • FY 2019 - No Change, effective from 10/1/2018 through 9/30/2019
  • FY 2020 - No Change, effective from 10/1/2019 through 9/30/2020

Information for Patients

Skin Conditions

Your skin is your body's largest organ. It covers and protects your body. Your skin

  • Holds body fluids in, preventing dehydration
  • Keeps harmful microbes out, preventing infections
  • Helps you feel things like heat, cold, and pain
  • Keeps your body temperature even
  • Makes vitamin D when the sun shines on it

Anything that irritates, clogs, or inflames your skin can cause symptoms such as redness, swelling, burning, and itching. Allergies, irritants, your genetic makeup, and certain diseases and immune system problems can cause rashes, hives, and other skin conditions. Many skin problems, such as acne, also affect your appearance.

NIH: National Institute of Arthritis and Musculoskeletal and Skin Diseases

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Anhidrotic ectodermal dysplasia with immune deficiency Anhidrotic ectodermal dysplasia with immune deficiency (EDA-ID) is a form of ectodermal dysplasia, which is a group of conditions characterized by abnormal development of ectodermal tissues including the skin, hair, teeth, and sweat glands. In addition, immune system function is reduced in people with EDA-ID. The signs and symptoms of EDA-ID are evident soon after birth, and due to the severity of the immune system problems, most people with this condition survive only into childhood.Skin abnormalities in children with EDA-ID include areas that are dry, wrinkled, or darker in color than the surrounding skin. Affected individuals tend to have sparse scalp and body hair (hypotrichosis). EDA-ID is also characterized by missing teeth (hypodontia) or teeth that are small and pointed. Most children with EDA-ID have a reduced ability to sweat (hypohidrosis) because they have fewer sweat glands than normal or their sweat glands do not function properly. An inability to sweat (anhidrosis) can lead to a dangerously high body temperature (hyperthermia), particularly in hot weather and during exercise, because the body cannot cool itself by evaporating sweat.The immune deficiency in EDA-ID varies among individuals with this condition. Children with EDA-ID often produce abnormally low levels of proteins called antibodies or immunoglobulins. Antibodies help protect the body against infection by attaching to specific foreign particles and germs, marking them for destruction. A reduction in antibodies makes it difficult for children with this disorder to fight off infections. In EDA-ID, immune system cells called T cells and B cells have a decreased ability to recognize and respond to foreign invaders (such as bacteria, viruses, and yeast) that have sugar molecules attached to their surface (glycan antigens). Other key aspects of the immune system may also be impaired, leading to recurrent infections.Children with EDA-ID commonly get infections in the lungs (pneumonia), ears (otitis media), sinuses (sinusitis), lymph nodes (lymphadenitis), skin, bones, and gastrointestinal tract. Approximately one quarter of individuals with EDA-ID have disorders involving abnormal inflammation, such as inflammatory bowel disease or rheumatoid arthritis.There are two forms of EDA-ID that have similar signs and symptoms and are distinguished by the modes of inheritance: X-linked recessive or autosomal dominant.
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Hypohidrotic ectodermal dysplasia Hypohidrotic ectodermal dysplasia is one of more than 100 types of ectodermal dysplasia. Starting before birth, these disorders result in the abnormal development of ectodermal tissues, particularly the skin, hair, nails, teeth, and sweat glands.Most people with hypohidrotic ectodermal dysplasia have a reduced ability to sweat (hypohidrosis) because they have fewer sweat glands than normal or their sweat glands do not function properly. Sweating is a major way that the body controls its temperature; as sweat evaporates from the skin, it cools the body. Reduced sweating can lead to a dangerously high body temperature (hyperthermia), particularly in hot weather. In some cases, hyperthermia can cause life-threatening health problems.Affected individuals tend to have sparse scalp and body hair (hypotrichosis). The hair is often light-colored, brittle, and slow-growing. Hypohidrotic ectodermal dysplasia is also characterized by several missing teeth (hypodontia) or teeth that are malformed. The teeth that are present erupt from the gums later than usual and are frequently small and pointed.Some people with hypohidrotic ectodermal dysplasia have distinctive facial features, including a prominent forehead, thick lips, and a flattened bridge of the nose. Additional features of this condition can include thin, wrinkled, and dark-colored skin around the eyes; chronic skin problems such as eczema; and a bad-smelling discharge from the nostrils (ozena).Intellectual ability and growth are typically normal in people with hypohidrotic ectodermal dysplasia.
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