symptomatic management of pain, itch, and anxiety; coordination of dressing changes; aggressive treatment of skin infections; environmental and staffing considerations; and awareness of other chronic complications that affect care, such as anemia, malnutrition [ncbi.nlm.nih.gov]

Malabsorption commonly results in a mixed anemia resulting from a lack of iron absorption, and overall malnutrition may cause failure to thrive. [raregeneticdisorder.blogspot.com]

Scarring in the mouth and esophagus can make it difficult to chew and swallow food, leading to chronic malnutrition and slow growth. [ghr.nlm.nih.gov]

Aggressive nutritional support should be employed in any child with recessive dystrophic EB who has evidence of growth retardation, since malnutrition oftentimes arises in these patients as a result of chronic loss of blood and protein through nonhealing [dermatologyadvisor.com]

Mucosal erosions of the esophagus can also be present, all these features and caloric need for wound healing can lead to malnutrition and growth retardation. [indianpediatrics.net]

small grouped hypopigmented papules on the lower back ( b ) Nail abnormalities with dystrophy or loss at birth or infancy may be an isolated finding as reported in DDEB [ 26, 27 ]. [plasticsurgerykey.com]

Abnormalities Keratosis Palmoplantaris With Arrhythmogenic Cardiomyopathy Mal De Naxos Palmoplantar Keratoderma With Arrhythmogenic Right Ventricular Cardiomyopathy And Woolly Hair Woolly Hair, Palmoplantar Keratoderma, And Cardiac Abnormalities NXD [ukgtn.nhs.uk]

The LOC syndrome is characterized by localized blistering and scarring, particularly on the face and neck, in association with upper airway disease activity and nail abnormalities [ 8, 9 ]. [ojrd.biomedcentral.com]

RESULTS: Despite multiple fractures of the acrylic resin veneers caused by severe bruxism and the small occlusal surface, this rehabilitation proved successful until the patient died at age 25, as a consequence of his hereditary disease. [ncbi.nlm.nih.gov]

Osteopenia and osteoporosis in epidermolysis bullosa. Dermatol Clin. 2010; 28 :353–5. [ PubMed : 20447502 ] Matsumoto Y, Dogru M, Tsubota K. [ncbi.nlm.nih.gov]

Patients with generalized forms of JEB and RDEB need to be monitored by serial DEXA scans for possible osteoporosis or osteopenia, and in selected EB subsets other laboratory parameters (hematological; renal) or diagnostic tests (echocardiogram) should [ojrd.biomedcentral.com]

Three patients had increased, six had maintained, and three had decreased their height centile position; the latter three all increased their height centile initially. [ncbi.nlm.nih.gov]

Immunomapping of a blister demonstrated collagen IV (basal membrane) in the blister roof and collagen VII in its floor, confirming dystrophic epidermolysis bullosa. [ncbi.nlm.nih.gov]

Epidermolysis bullosa is a group of genetic conditions that cause the skin to be very fragile and to blister easily. Blisters and skin erosions form in response to minor injury or friction, such as rubbing or scratching. [ghr.nlm.nih.gov]

[…] bullosa pseudosyndactyly (mitten-hand deformity) of the hands and feet Ectodermal dysplasia-skin fragility syndrome is a rare disorder characterized by skin erosions, skin fragility and peeling beginning at birth or infancy that may be accompanied by alopecia [raregeneticdisorder.blogspot.com]

There were patchy areas of cicatricial alopecia on the scalp and deformed blisters and erosions on the back [Figure - 1] and distal portions of the extremities. Mitten-hand deformity was observed on the hands [Figure - 2]. [indianjnephrol.org]

%) ( Benign Lichenoid Keratosis, Lichen Planus-like Keratosis, Solitary Lichen Planus, Solitary Lichenoid Keratosis,) Pachyonychia Congenita (29 %) Nevus Sebaceous (29 %) ( Nevus Sebaceous Of Jadassohn, Organoid Nevus,) Follicular Mucinosis (29 %) ( Alopecia [gulfdoctor.net]

The hallmark sign is cutaneous atrophy without scarring, present at birth, nails are absent, milia may appear on the scalp and cause alopecia. [kissesforkatie.org]

Nail dystrophy and scarring alopecia may also be present. [dermnet.com]

A 16-month-old boy was diagnosed with Hallopeau-Siemens recessive DEB on the basis of typical skin lesions composed of multiple blisters with moderately healed erosions, scarring on trauma-exposed body sites, including hands and feet, pseudosyndactyly [ncbi.nlm.nih.gov]

Phenytoin, minocycline cyclosporin and vitamin E have been used for the skin lesions. [17] However, these therapies were not helpful and have been abandoned. [indianjnephrol.org]

Skin lesions heal invariably with scars and milia. The risk for the development of squamous cell carcinomas is increased. Fig. 42.5 Generalized intermediate recessive dystrophic EB. [plasticsurgerykey.com]

Infection: Healing is diminished by secondary infection of skin lesions, usually by S. aureus or beta-hemolytic streptococci. [dermquest.com]

Histopathological examination showed hyperkeratosis, parakeratossis, and verrucous acanthosis and numerous large xanthoma cells in the papillary dermis. [ncbi.nlm.nih.gov]

We describe a 21-year-old man with recessive dystrophic epidermolysis bullosa carrying the homozygous c.6508C T (p.Gln2170X) nonsense mutation who reported an unaffected skin patch on his neck where blisters never had occurred. [ncbi.nlm.nih.gov]

The chronic inflammatory state seen in recessive dystrophic epidermolysis bullosa (RDEB) may cause Small fiber peripheral neuropathy (SFN); RDEB patients have reported the sensation of pain in line with neuropathic pain qualities. [en.wikipedia.org]

A 7-month-old boy with dense bilateral cataracts and nystagmus underwent cataract extraction, lens implantation, and limited vitrectomy with the use of specialized techniques to reduce iatrogenic skin and ocular surface complications. [ncbi.nlm.nih.gov]