2006 ICD-9-CM Volume 1 Diagnosis Codes Home > Congenital Anomalies 740-759 > Congenital Anomalies Of The Integument 757.* > 2006 ICD-9-CM Diagnosis 757.33 Congenital pigmentary anomalies of skin View the most recent version of ICD-9-CM 757.33 An autosomal recessive syndrome occurring principally in females, characterized by the presence of reticulated, atrophic, hyperpigmented, telangiectatic cutaneous plaques, often accompanied by juvenile cataracts, saddle nose, congenital bone defects, disturbances in the growth of hair, nails, and teeth, and hypogonadism. A rare, pigmentary, and atrophic autosomal recessive disease affecting all races. It is manifested as an extreme photosensitivity to ultraviolet light as the result of a deficiency in the enzyme that permits excisional repair of ultraviolet-damaged DNA. The most common form of cutaneous mastocytosis (MASTOCYTOSIS, CUTANEOUS) that occurs primarily in children. It is characterized by the multiple small reddish-brown pigmented pruritic macules and papules. A genetic condition characterized by a sensitivity to all sources of ultraviolet radiation. An inherited skin disorder characterized by photosensitivity with severe sunburn in infancy, the development of numerous pigmented spots resembling freckles, larger atrophic lesions associated with telangiectasis, and multiple solar keratoses. Transmitted in an autosomal recessive manner, xeroderma pigmentosa involves a defect in nucleotide excision repair (NER), leading to deficient repair of DNA damaged by UV radiation and chromosome breakage. Individuals with this disease develop multiple malignant cutaneous neoplasms at an early age and may suffer from severe ophthalmic and neurologic abnormalities. --2004 The most frequent form of cutaneous mastocytosis. In children, the lesions tend to be papular, and are characterized by aggregates of elongated or spindle-shaped mast cells which fill the papillary dermis and extend into the reticular dermis. In adults, the lesions tend to have fewer mast cells compared to those in children. The lesions are located most commonly on the trunk, but they can be seen on the extremities, head and neck. (WHO, 2001) -- 2003 757.33 is a specific code that can be used to specify a diagnosis 757.33 contains 48 index entries View the ICD-9-CM Volume 1 757.* hierarchy Alternate Terminology Congenital poikiloderma Urticaria pigmentosa Xeroderma pigmentosum 757.33 Excludes albinism (270.2)  Index entries containing 757.33: Angioma (M9120/0) (see also Hemangioma, by site) 228.00 pigmentosum et atrophicum 757.33 Anomaly, anomalous (congenital) (unspecified type) 759.9 pigmentation 709.00 congenital 757.33 Asboe-Hansen's disease (incontinentia pigmenti) 757.33 Atrophoderma, atrophodermia 701.9 pigmentosum 757.33 Bloch-Siemens syndrome (incontinentia pigmenti) 757.33 Bloch-Stauffer dyshormonal dermatosis 757.33 Bloch-Sulzberger disease or syndrome (incontinentia pigmenti) (melanoblastosis) 757.33 Chloasma 709.09 eyelid 374.52 congenital 757.33 Dermatosis 709.9 pigmentary NEC 709.00 Siemens-Bloch 757.33 Disease, diseased - see also Syndrome Asboe-Hansen's (incontinentia pigmenti) 757.33 Bloch-Sulzberger (incontinentia pigmenti) 757.33 Kaposi's 757.33 lichen ruber 697.8 acuminatus 696.4 moniliformis 697.8 xeroderma pigmentosum 757.33 mast cell 757.33 systemic (M9741/3) 202.6 Nettleship's (urticaria pigmentosa) 757.33 Thomson's (congenital poikiloderma) 757.33 Incontinentia pigmenti 757.33 Kaposi's disease 757.33 lichen ruber 696.4 acuminatus 696.4 moniliformis 697.8 xeroderma pigmentosum 757.33 Lioderma essentialis (cum melanosis et telangiectasia) 757.33 Mast cell disease 757.33 systemic (M9741/3) 202.6 Mastocytosis 757.33 malignant (M9741/3) 202.6 systemic (M9741/3) 202.6 Melanoblastosis Block-Sulzberger 757.33 cutis linearis sive systematisata 757.33 Melanosis 709.09 corii degenerativa 757.33 lenticularis progressiva 757.33 Mongolian, mongolianism, mongolism, mongoloid 758.0 spot 757.33 Naegeli's syndrome (incontinentia pigmenti) 757.33 Nettleship's disease (urticaria pigmentosa) 757.33 Nevus (M8720/0) - see also Neoplasm, skin, benign comedonicus 757.33 pigmented (M8720/0) systematicus 757.33 unius lateris 757.33 verrucous 757.33 Pigmentation (abnormal) 709.00 anomalies NEC 709.00 congenital 757.33 lids (congenital) 757.33 acquired 374.52 scrotum, congenital 757.33 Poikiloderma 709.09 congenital 757.33 Rothmund (-Thomson) syndrome 757.33 Spots, spotting Mongolian (pigmented) 757.33 Syndrome - see also Disease Block-Siemens (incontinentia pigmenti) 757.33 Bloch-Sulzberger (incontinentia pigmenti) 757.33 mastocytosis 757.33 Rothmund's (congenital poikiloderma) 757.33 telangiectasis-pigmentation-cataract 757.33 Thomson's disease (congenital poikiloderma) 757.33 Urticaria 708.9 pigmentosa 757.33 Xanthelasmoidea 757.33 Xeroderma (congenital) 757.39 pigmentosum 757.33