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2A21.1 Cutaneous mastocytosis International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01 Cutaneous mastocytosis is characterised by abnormal accumulation and proliferation of cutaneous mast cells. Most types are isolated but cutaneous mastocytosis can occur in association with systemic disease. Clinical forms include cutaneous mastocytoma, urticaria pigmentosa (the most frequent form), pseudoxanthomatous nodular cutaneous mastocytosis, telangiectasia macularis eruptiva perstans and diffuse cutaneous mastocytosis.
sections/codes in this section (2A21.1-2A21.1) postcoordination Add Stem and/or Extension codes to form a cluster code which adds detail to the condition.
Specific anatomy - multiple selections are allowed [select] XA15W6 Lower lip – XA5VD0 External lower lip – XA9TK2 Vermilion border of lower lip – XA7H02 Vermilion of lower lip XA1BP2 Inner aspect of lip – XA3141 Frenulum of lip XA3K27 External lip XA2C62 Chin XA04T9 Skin of nose XA7AA6 Neck XA4QS6 Front of neck XA1NS6 Anterior triangle of neck XA5TZ1 Submental region XA0MP5 Submandibular region XA8RA2 Suprasternal notch XA9DQ5 Supraclavicular region XA2ZF0 Side of neck XA45K8 Posterior triangle of neck XA1M78 Nape of neck XA1RS6 Head and neck XA20Q1 Head XA6CW5 Scalp XA0WK0 Frontal scalp XA0WG9 Frontal scalp margin XA9DZ0 Temporal scalp XA26C1 Temporal scalp margin XA4W34 Parietal scalp XA7JE5 Occipital scalp XA3EK3 Occipital scalp margin XA5BY6 Vertex of scalp XA6AL1 Scalp margin XA93S9 Parietal scalp margin XA86S4 Face XA6TR8 Forehead XA9SG2 Central forehead XA1UW4 Paramedian forehead XA1Z38 Lateral forehead XA90D8 Glabella XA9T94 Temple XA29E7 Orbital region XA0SB3 Periorbital region XA5WP1 Supraorbital region – XA1LZ5 Eyebrow XA6TV2 Infraorbital region XA7MK8 Cheek XA5KE9 Upper cheek XA0M67 Malar region – XA57N0 Malar eminence XA6C41 Central cheek XA3ZL3 Paranasal region XA7207 Lower cheek XA0SU2 Preauricular region XA8KA2 Mandibular region XA3H13 Nose XA0LR7 Root of nose XA5YP3 Dorsum of nose XA3057 Supratip of nose XA9JN5 Lateral side wall of nose XA56T3 Tip of nose XA3ZG3 Infratip lobule of nose XA32Q9 Ala nasi XA5ED7 Alar groove XA7LG9 Alar rim XA2TK5 Side wall of ala nasi XA1B05 Nostril XA4S17 Columella XA9YZ7 Sill of nostril XA5A87 Oral region XA1A48 Perioral region XA8JD4 Lip XA7VQ4 Upper lip – XA0K68 External upper lip –– XA5LY8 Philtrum –– XA5163 Nasolabial fold – XA8RK1 Vermilion border of upper lip – XA75S0 Vermilion of upper lip XA1EF8 Labial commissure XA3FR3 Trunk XA4QH7 Upper trunk XA5D93 Thorax XA55T2 Chest wall XA00R3 Anterior thoracic region XA8ML7 Upper anterior thoracic region – XA4MN6 Clavicular region – XA6M63 Infraclavicular region – XA5MS8 Presternal region XA7GU3 Lower anterior thoracic region XA7884 Lateral thoracic region XA9RL9 Upper lateral thoracic region – XA0XL3 Anterolateral upper thoracic region – XA5C28 Posterolateral upper thoracic region XA9MN4 Lower lateral thoracic region – XA3266 Anterolateral lower thoracic region – XA7MS4 Posterolateral lower thoracic region XA10L7 Upper back XA3PG8 Suprascapular region XA3WD7 Scapular region XA9LN5 Interscapular region XA8NK1 Infrascapular region XA6RF2 Lower thoracic paraspinal region XA8EK1 Skin of thorax XA12C1 Breast XA1NS5 Upper half of breast XA3LS6 Upper inner quadrant of breast XA2Q54 Upper outer quadrant of breast XA3PG5 Axillary tail of breast XA0US1 Central portion of breast XA2JK3 Areola XA85A1 Lactiferous ducts XA5MC5 Nipple XA3UY3 Lower half of breast XA0VX8 Lower inner quadrant of breast XA94U2 Lower outer quadrant of breast XA9CM2 Lateral half of breast XA3JH6 Medial half of breast XA0T50 Inframammary flexure XA6CY1 Lower trunk XA6GV0 Abdomen XA0U66 Upper abdomen XA8ZL8 Epigastrium XA3TD4 Hypochondrium XA1LM1 Periumbilical region XA3MT8 Umbilicus XA1DN2 Lateral lumbar region XA4TC0 Lower abdomen XA6N20 Hypogastrium XA0NH8 Iliac region XA3KX0 Abdominal wall XA4SN6 Anterior abdominal wall XA25R8 Lumbosacral region XA6ZR2 Mid back XA7ZW8 Lumbar paraspinal region XA8FK6 Posterior lumbar region XA9ET2 Lower back XA6DS1 Coccygeal area XA2UC8 Sacral region XA4L23 Sacrococcygeal region XA2P90 Back XA8HA7 Anogenital region XA5FG3 Genital region XA9PG6 Female external genitalia XA1AK8 Male external genitalia XA8MT4 Scrotum XA9PX3 Perigenital region XA0LF4 Suprapubic area XA10Z0 Mons pubis XA2XG2 Inguinocrural fold XA00B4 Inguinal canal XA4B34 Perianal region XA53N9 Perineum XA2F27 Intergluteal cleft XA9Y82 Lateral border of foot XA3WM8 Medial border of foot XA3T29 Arch of foot XA4LC9 Toes XA2RP7 Great toe XA4774 Perionychium of great toe – XA8L19 Proximal nail fold of great toe – XA7WP9 Eponychium of great toe – XA7GD8 Lateral nail fold of great toe – XA2F64 Hyponychium of great toe XA1RE3 Great toenail – XA64R9 Lunula of great toe – XA0HX8 Nail bed of great toe – XA47T1 Nail plate of great toe XA6VJ2 Pad of great toe XA8ZZ3 Second toe XA5446 Perionychium of second toe – XA1ED1 Proximal nail fold of second toe – XA0SL7 Eponychium of second toe – XA7003 Lateral nail fold of second toe – XA2ZJ7 Hyponychium of second toe XA7GG3 Second toenail – XA9439 Lunula of second toe – XA7B22 Nail bed of second toe – XA1WQ6 Nail plate of second toe XA3626 Pad of second toe XA0SP3 Third toe XA3UC8 Perionychium of third toe – XA2484 Proximal nail fold of third toe – XA8DQ2 Eponychium of third toe – XA1MM3 Lateral nail fold of third toe – XA2H72 Hyponychium of third toe XA5JP9 Pad of third toe XA3D73 Third toenail – XA9UL1 Lunula of third toe – XA6189 Nail bed of third toe – XA3LW9 Nail plate of third toe XA4KK7 Fourth toe XA40R3 Perionychium of fourth toe – XA2Y79 Proximal nail fold of fourth toe – XA0XZ8 Eponychium of fourth toe – XA0PV4 Lateral nail fold of fourth toe – XA4ZB3 Hyponychium of fourth toe XA9316 Pad of fourth toe XA6TS5 Fourth toenail – XA2PD3 Lunula of fourth toe – XA65U3 Nail bed of fourth toe – XA8F87 Nail plate of fourth toe XA42W4 Fifth toe XA1AV3 Perionychium of fifth toe – XA43K6 Proximal nail fold of fifth toe – XA2W24 Eponychium of fifth toe – XA38J0 Lateral nail fold of fifth toe – XA0DD8 Hyponychium of fifth toe XA3C43 Pad of fifth toe XA3VM6 Fifth toenail – XA1PK7 Lunula of fifth toe – XA9L52 Nail bed of fifth toe – XA4U10 Nail plate of fifth toe XA9LJ5 Plantar surface of toe XA7J49 Dorsal surface of toe XA14Y9 Side of toe XA9E36 Toenail XA6AS2 Extremities XA4BA8 Upper extremity XA2ND5 Shoulder XA3PZ3 Anterior surface of shoulder XA5BU5 Apex of shoulder XA34G7 Posterior surface of shoulder XA17J1 Axilla XA41A1 Anterior axillary fold XA86E8 Apex of axilla XA2RY5 Posterior axillary fold XA6809 Upper arm XA22Q1 Anterior surface of upper arm XA2W33 Lateral surface of upper arm XA5TK8 Posterior surface of upper arm XA3J41 Medial surface of upper arm XA9FF8 Elbow XA9NE6 Antecubital fossa XA6599 Lateral condylar surface of elbow XA3RT8 Elbow tip XA4983 Medial condylar surface of elbow XA7WB0 Forearm XA8ZL6 Volar surface of forearm XA1VA2 Lateral surface of forearm XA8WH0 Dorsal surface of forearm XA2Q46 Medial surface of forearm XA2J63 Wrist XA6AR5 Volar surface of wrist XA3LK1 Radial border of wrist XA0SH5 Dorsal surface of wrist XA0J47 Ulnar border of wrist XA5R12 Hand XA30Z6 Dorsum of hand XA3T43 Knuckles XA65Z3 Interdigital web space of hand XA1BR6 First interdigital web space of hand XA5PY9 Second interdigital web space of hand XA4012 Third interdigital web space of hand XA3WG2 Fourth interdigital web space of hand XA3NY8 Palm of hand XA3FJ0 Proximal palm XA2JN4 Thenar eminence XA5TQ4 Hypothenar eminence XA50E4 Central palm XA00D7 Distal palm XA2593 Fingers and thumb XA8DJ6 Thumb XA0RL8 Perionychium of thumb – XA13E9 Proximal nailfold of thumb – XA20L7 Eponychium of thumb – XA4GD7 Lateral nailfold of thumb – XA63L0 Hyponychium of thumb XA5PD5 Thumbnail – XA6DM1 Lunula of thumb – XA9N39 Nail bed of thumb – XA5V24 Nail plate of thumb XA5ZV0 Pad of thumb XA76N2 Dorsum of thumb XA6NZ0 Index finger XA6YH1 Perionychium of index finger – XA90K8 Eponychium of index finger – XA2UG0 Hyponychium of index finger XA40D9 Index fingernail – XA1GS3 Lunula of index finger – XA1SB3 Nail bed of index finger – XA2XE0 Nail plate of index finger XA6TA9 Pad of index finger XA0Y38 Middle finger XA1FY2 Perionychium of middle finger – XA8YE5 Proximal nail fold of middle finger – XA13L6 Eponychium of middle finger – XA2N38 Lateral nail fold of middle finger – XA8KX8 Hyponychium of middle finger XA9YZ9 Middle fingernail – XA8VS0 Lunula of middle finger – XA2A53 Nail bed of middle finger – XA10T8 Nail plate of middle finger XA79X0 Pad of middle finger XA06X8 Ring finger XA7K11 Perionychium of ring finger – XA1F61 Proximal nail fold of ring finger – XA8L06 Eponychium of ring finger – XA3HG9 Lateral nail fold of ring finger – XA1W89 Hyponychium of ring finger XA6Y59 Ring fingernail – XA4P58 Lunula of ring finger – XA3MW5 Nail bed of ring finger – XA3PS0 Nail plate of ring finger XA6C72 Pad of ring finger XA5EN3 Little finger XA89P0 Perionychium of little finger – XA4KU5 Proximal nail fold of little finger – XA2AV8 Eponychium of little finger – XA3LC5 Lateral nail fold of little finger – XA1C10 Hyponychium of little finger XA4WN3 Pad of little finger XA29K9 Little fingernail – XA3R66 Lunula of little finger – XA6HB9 Nail bed of little finger – XA4A79 Nail plate of little finger XA4HZ3 Side of finger XA7GT9 Tips of fingers XA41X5 Tip of index finger XA9Y99 Tip of middle finger XA91S7 Tip of ring finger XA8QW7 Tip of little finger XA0EH9 Fingernails XA66R9 Skin of elbow XA45A6 Lower extremity XA3VA7 Buttock XA5UE3 Gluteal fold XA5S78 Thigh XA98B3 Anterior surface of thigh XA8RH9 Lateral surface of thigh XA4TQ2 Trochanteric region XA0183 Posterior surface of thigh XA1YQ6 Medial surface of thigh XA9ZB4 Upper medial surface of thigh XA8KL5 Knee XA9L17 Patellar region XA77E9 Lateral surface of knee XA9S09 Medial surface of knee XA4DM3 Popliteal fossa XA3YG1 Lower leg XA33X4 Anterior surface of lower leg XA4K86 Calf XA4RR4 Lateral surface of lower leg XA0LQ2 Posterior surface of lower leg XA15P0 Medial surface of lower leg XA5U49 Distal lower leg XA90X0 Proximal lower leg XA67V4 Ankle XA2V14 Anterior surface of ankle XA7AM4 Lateral surface of ankle XA1D83 Lateral supramalleolar region XA41K4 Lateral retromalleolar region XA7P78 Medial surface of ankle XA87M9 Medial supramalleolar region XA1SM7 Medial retromalleolar region XA6AP4 Posterior surface of ankle XA47V8 Foot XA99M7 Hindfoot XA5HK0 Heel XA5ZE2 Posterior surface of heel XA1QH8 Medial surface of heel XA3R99 Lateral surface of heel XA2N02 Plantar surface of heel XA5151 Midfoot XA02P2 Dorsal surface of midfoot XA5YL1 Forefoot XA81Z3 Dorsal surface of forefoot XA1FL5 Interdigital web space of foot XA81N1 First interdigital web space of foot XA8HC5 Second interdigital web space of foot XA9LB9 Third interdigital web space of foot XA2A07 Fourth interdigital web space of foot XA6KE9 Plantar surface of forefoot XA6V29 First metatarsal head region XA2P22 Second metatarsal head region XA0HX4 Third metatarsal head region XA86J0 Fourth metatarsal head region XA05N7 Fifth metatarsal head region XA8BE2 Dorsum of foot XA1XM4 Sole of foot Histopathology - only one may be selected [select] XH6A72 Mast cell sarcoma XH2992 Malignant mastocytosis XH10N1 Aggressive systemic mastocytosis XH1H01 Systemic mastocytosis with AHNMD XH5191 Systemic mastocytosis with associated haematological clonal non-mast cell disorder XH5ER6 Mast cell leukaemia XH1VJ3 Erdheim-Chester disease XH1J17 Mastocytoma, NOS XH74F2 Cutaneous mastocytosis XH2RG8 Diffuse cutaneous mastocytosis XH8VS0 Urticaria pigmentosa XH2Y59 Indolent systemic mastocytosis XH2RL8 Solitary mastocytoma of skin Has manifestation - multiple selections are allowed [select] MG30.10 Chronic cancer pain
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