Epidermolysis bullosa (Q81)

ICD-10 code Q81 identifies various forms of epidermolysis bullosa, a rare group of inherited skin disorders characterized by fragile skin that blisters easily. These codes clarify the specific subtype of epidermolysis bullosa, ensuring precise classification for diagnosis and treatment.

The section includes Q81.0 for epidermolysis bullosa simplex, covering types such as autosomal recessive or dominant forms, and variants with features like hypodontia or mottled pigmentation. Commonly known synonyms such as “autosomal recessive epidermolysis bullosa simplex” and “epidermolysis bullosa simplex herpetiformis” help medical coders select the exact code based on the patient’s presentation. Q81.1 covers epidermolysis bullosa letalis, including severe subtypes like generalized junctional epidermolysis bullosa, also called Herlitz type. Q81.2 classifies dystrophic epidermolysis bullosa, incorporating dominant and recessive forms with synonyms like “dominant dystrophic epidermolysis bullosa” and “generalized recessive dystrophic epidermolysis bullosa.” Q81.8 identifies other specified forms, such as junctional variants and rare syndromes including Weber-Cockayne syndrome. Finally, Q81.9 denotes unspecified epidermolysis bullosa, used when detailed subtype information is unavailable.

Understanding these codes enables clearer communication about epidermolysis bullosa’s specific type, guiding clinical care and improving coding accuracy for billing and epidemiology. For example, searching “ICD-10 code for epidermolysis bullosa dystrophica” leads directly to Q81.2, streamlining diagnosis documentation.

Clinical Terms

The following clinical terms provide additional context, helping users better understand the clinical background and common associations for each diagnosis listed in this section. Including related terms alongside ICD-10-CM codes supports coders, billers, and healthcare professionals in improving accuracy, enhancing documentation, and facilitating research or patient education.

Epidermolysis Bullosa

Group of genetically determined disorders characterized by the blistering of skin and mucosae. There are four major forms: acquired, simple, junctional, and dystrophic. Each of the latter three has several varieties.

Epidermolysis Bullosa Acquisita

Form of epidermolysis bullosa characterized by trauma-induced, subepidermal blistering with no family history of the disease. Direct immunofluorescence shows IMMUNOGLOBULIN G deposited at the dermo-epidermal junction.

Epidermolysis Bullosa Dystrophica

Form of epidermolysis bullosa characterized by atrophy of blistered areas, severe scarring, and nail changes. It is most often present at birth or in early infancy and occurs in both autosomal dominant and recessive forms. All forms of dystrophic epidermolysis bullosa result from mutations in COLLAGEN TYPE VII, a major component fibrils of BASEMENT MEMBRANE and EPIDERMIS.

Epidermolysis Bullosa Simplex

A form of epidermolysis bullosa characterized by serous bullae that heal without scarring. Mutations in the genes that encode KERATIN-5 and KERATIN-14 have been associated with several subtypes of epidermolysis bullosa simplex.

Epidermolysis Bullosa, Junctional

Form of epidermolysis bullosa having onset at birth or during the neonatal period and transmitted through autosomal recessive inheritance. It is characterized by generalized blister formation, extensive denudation, and separation and cleavage of the basal cell plasma membranes from the basement membrane.