Epidermolytic hyperkeratosis (EHK), also known as bullous congenital ichthyosiform erythroderma (bullous CIE), is a rare autosomal dominant genodermatosis

, although up to 50% of cases represent new mutations. EHK presents as a bullous disease in newborns, followed by a lifelong ichthyotic skin disorder. In 1902, Brocq first described it as bullous ichthyotic erythroderma to distinguish the entity from congenital ichthyotic erythroderma.

Epidermolytic hyperkeratosis (EHK, bullous congenital ichthyosiform erythroderma [bullous CIE]) results from mutations in the keratin 1 and keratin 10 genes. Many different mutation sites in the genes have been reported. Keratins are divided into 2 classes: basic type II keratins and acidic type I keratins. Keratin 1 is one of the basic type II keratins found on chromosome 12; keratin 10 is one of the acidic type I keratins found on chromosome 17. Keratins form intermediate filaments when both type I and type II keratins are present. Intermediate filaments provide structural stability to keratinocytes. Keratins 1 and 10 are coexpressed and are involved in the suprabasilar differentiation of keratinocytes. Mutations in these keratin genes lead to the formation of defective keratin proteins, which, although still able to incorporate into intermediate filaments, function poorly. This leads to skin cell collapse and clinical blistering. The thickening of the skin is thought to be compensatory to protect against blistering. United States The incidence of epidermolytic hyperkeratosis (EHK, bullous congenital ichthyosiform erythroderma [bullous CIE]) is 1 case per 200,000-300,000 persons. International The incidence of epidermolytic hyperkeratosis (EHK, bullous congenital ichthyosiform erythroderma [bullous CIE]) internationally is the same as it is in United States.

Mortality/Morbidity
Mortality and morbidities of epidermolytic hyperkeratosis (EHK, bullous congenital ichthyosiform erythroderma [bullous CIE]) include recurrent infection, sepsis, and electrolyte imbalance, which are possible during the neonatal period.

Race
No racial predilection is apparent for epidermolytic hyperkeratosis (EHK, bullous congenital ichthyosiform erythroderma [bullous CIE]).

but subtle skin thickening or scaling may be apparent during the first month of life.[1. bullous congenital ichthyosiform erythroderma [bullous CIE]) presents in neonates as widespread superficial blisters. when ruptured. Ectropion does not occur. The scale is classically described as “corrugated cardboard”–like. nails. leave raw denuded areas. and the hair. Age Epidermolytic hyperkeratosis (EHK. Moreover. However. Epidermolytic hyperkeratosis (EHK. Symptoms in some patients may ameliorate over time. Pungent body odor may often be associated. and teeth are normal. the scaling becomes thicker and the propensity to blister decreases. which. and/or peeling. bullous congenital ichthyosiform erythroderma [bullous CIE]) presents at birth or shortly thereafter with erythema.Sex No sex predilection is recognized for epidermolytic hyperkeratosis (EHK. rare autosomal recessive cases have also been reported. Hyperkeratosis appears from the third month on. . 2] Epidermolytic hyperkeratosis (EHK. as many as half the cases are a result of sporadic mutations. The ichthyosis is typically generalized and is often more prominent at flexures and overlying joints. As patients age. so patients may have a family history of a similar condition. blistering. Note the images below. bullous congenital ichthyosiform erythroderma [bullous CIE]) is a lifelong condition with an onset at birth or in the neonatal period. Palms and soles have varying degrees of hyperkeratosis. EHK is inherited in an autosomal dominant fashion. bullous congenital ichthyosiform erythroderma [bullous CIE]).

a hystrix scale. a generalized skin distribution. DiGiovanna and Bale separated the various clinical presentations of epidermolytic hyperkeratosis into 2 primary types. no digital contractures. while others are localized only. and patients may have abnormal gait. has minimal scale. The only differences are a brown scale instead of a hystrix scale and a lack of gait abnormalities. . NPS-3 epidermolytic hyperkeratosis may have associated gait abnormalities similar to NPS-1 epidermolytic hyperkeratosis. and is generalized in skin distribution. Distinctions between the 3 NPS epidermolytic hyperkeratosis subtypes are based on different clinical presentations. Palms and soles may have varying In 1994. • NPS epidermolytic hyperkeratosis subtypes do not have severe palmoplantar involvement. degrees of hyperkeratosis. the palmoplantar surface in NPS-3 epidermolytic hyperkeratosis is hyperlinear. a positive history of blistering. Some of the subtypes have general involvement. similar to NPS-1 epidermolytic hyperkeratosis and NPS-2 epidermolytic hyperkeratosis.[3] The 2 primary types were subdivided further into 3 subtypes each depending on the clinical presentations. In contrast. including NPS (without severe palm/sole hyperkeratosis) and PS (with severe palm/sole hyperkeratosis) based on the presence or absence of severe palmoplantar hyperkeratosis. o NPS-3 epidermolytic hyperkeratosis has no palmoplantar hyperkeratosis.The scale in epidermolytic hyperkeratosis is classically described as "corrugated cardboard"-like. no digital contractures. and a thin white scale is most prominent on the trunk. no history of erythroderma. o NPS-2 epidermolytic hyperkeratosis is similar to NPS-1 epidermolytic hyperkeratosis. Erythroderma is mild to moderate. o NPS-1 epidermolytic hyperkeratosis has normal palmoplantar surfaces.

[6] More recently. generalized skin involvement. and no gait abnormalities. 11.[9. 5] A case of epidermolytic hyperkeratosis with no facial involvement has also been reported. truncal sparing). there has been a report of epidermolytic hyperkeratosis and congenital platelike osteoma cutis in a child. no erythroderma. generalized skin involvement with hyperkeratosis most severe over the joints at both flexor and extensor surfaces. a tan scale. neonatal blistering. Epidermolytic hyperkeratosis has been infrequently found to be associated with other clinical findings. mild-to-moderate erythroderma. no digital contractures. a localized distribution of skin involvement (limited flexural involvement. Close-up view of ankle. and no gait abnormalities. The 3 subtypes are differentiated based on clinical presentations. no erythroderma. bullous congenital ichthyosiform erythroderma [bullous CIE]). a localized blistering. Rare cases of patients with epidermolytic hyperkeratosis and hypocalcemic rickets. 10. 12.[8] The following are clinical images of epidermolytic hyperkeratosis lesions of the ankles: Anterior ankles.• PS epidermolytic hyperkeratosis subtypes have severe palmoplantar involvement. have been reported.[4. with or without vitamin D resistance. o PS-2 epidermolytic hyperkeratosis has smooth palmoplantar hyperkeratotic surfaces but has digital contractures. Causes Defects in genes for keratin 1 (KRT1) and 10 (KRT10) are the cause of epidermolytic hyperkeratosis (EHK. 13] Usually. no digital contractures. o PS-1 epidermolytic hyperkeratosis has smooth palmoplantar hyperkeratotic surfaces. positive blistering. o PS-3 epidermolytic hyperkeratosis has cerebriform palmoplantar surfaces. these mutations are missense substitutions into the highly conserved alpha-helical rod .[7] as well as epidermolytic hyperkeratosis localized to the vulva. and may have gait abnormalities.

Palmoplantar keratoderma is usually associated with KRT1 mutations.[15] In the medical literature. X-Linked Staphylococcal Scalded Skin Syndrome Stevens-Johnson Syndrome and Toxic Epidermal Necrolysis Laboratory Studies • • • No general laboratory studies are needed for epidermolytic hyperkeratosis (EHK. with the histologic findings confirming a diagnosis of epidermolytic hyperkeratosis (EHK.[17] An epidermal nevus with histologic changes of epidermolytic hyperkeratosis is a mosaic condition in which the affected skin carries a mutation in keratin 1 or keratin 10. a girl with epidermolytic hyperkeratosis and palmoplantar keratoderma with the KRT10 mutation has been reported. Histologic Findings In epidermolytic hyperkeratosis (EHK. defects in keratin 10 are associated with the NPS epidermolytic hyperkeratosis variants. deeper . Patients with generalized EHK may be born to parents with epidermolytic epidermal nevi[16] or linear epidermolytic hyperkeratosis. coarse keratohyaline granules. new mutations in both genes continue to be reported. due to germline mutations in keratin 1 or keratin 10. bullous congenital ichthyosiform erythroderma [bullous CIE]). bullous congenital ichthyosiform erythroderma [bullous CIE]). skin biopsy can be helpful.and the nonhelical H1 domains of the keratin proteins. or fetal skin biopsies. bullous congenital ichthyosiform erythroderma [bullous CIE]). Prenatal diagnosis can be made through chorionic villus sampling.[14] Defects in keratin 1 are associated with the PS epidermolytic hyperkeratosis variants. except if necessary to follow chosen therapy or bacterial culture for suspected infection. however. Individuals with more extensive forms can have offspring with generalized epidermolytic hyperkeratosis. and vacuolar degeneration of the upper dermis. Lamellar Ichthyosis. mutation-specific testing for relatives and prenatal diagnosis is available. Once a mutation is identified in an affected individual. Keratin defect studies can be performed on buccal swabs or blood. analysis of amniotic cells. a thick granular layer. Typical findings include marked hyperkeratosis. Occasionally. Differential Diagnoses • • • • • Epidermolysis Bullosa Ichthyosis. hematoxylin and eosin findings are distinctive but not unique to epidermolytic hyperkeratosis. Procedures • • Along with clinical presentation and history.

dyskeratosis is frequently present to varying degrees. Patients may benefit from the use of mild antibacterial soaps or chlorhexidine-containing . secondary sepsis.[19] On electron microscopy. Genetic counseling and prenatal diagnosis also can be offered. Pathology of epidermolytic hyperkeratosis (hematoxylin and eosin stain). round-to-oval. Wound care for blistering and moisturization/emollients are important in the newborn period.[20] Newborns with epidermolytic hyperkeratosis who have denuded skin are at increased risk for infection. large. In addition. bullous congenital ichthyosiform erythroderma [bullous CIE]) is important in order to properly inform and counsel parents. Medical Care Accurate diagnosis of epidermolytic hyperkeratosis (EHK. These newborns should be transferred to the neonatal ICU to be monitored and treated as needed. topical emollients continue to be the mainstay of treatment. Hematoxylin and eosin staining of epidermolytic hyperkeratosis is shown in the images below. and an odor. The accumulation of scale predisposes to overgrowth of bacteria. and the shaped masses appear to be keratohyaline granules. and electrolyte imbalance. In older children. those with focal involvement revealing skip areas of normal epidermis are more likely to have a sporadic. enlarged. They should be handled gently to avoid further trauma to the skin. Pathology of epidermolytic hyperkeratosis (hematoxylin and eosin stain).granular cells become dense. dense clumps of keratin tonofilaments can be seen in the lower epidermal layers. in particular with Staphylococcus aureus. and irregular. mosaic form of epidermolytic hyperkeratosis.[18] Patients whose pathologic slides demonstrate continuous involvement of the entire horizontal epidermis with these distinctive findings are more likely to have generalized disease.

Consultations Refer patients who are considering conceiving children to a geneticist for reproductive concerns and assistance. normal fetal keratinization does not begin until the 24th week. To date. however. alpha-hydroxy acid. Diet No special diet is needed for patients with epidermolytic hyperkeratosis (EHK. Prenatal diagnosis can be made by ultrastructural analysis and by direct gene sequencing. The earliest documented diagnosis by this method is at the 15th week of gestation. Surgical Care No surgical care is needed or recommended for epidermolytic hyperkeratosis (EHK. 22. lactic acid. topical retinoids. • • Prenatal diagnosis of epidermolytic hyperkeratosis can be performed by ultrastructural analysis of fetal skin biopsy specimens and amniotic fluid cells. 23] . and urea. bullous congenital ichthyosiform erythroderma [bullous CIE]). Epidermolytic hyperkeratosis tends to improve with increasing age.[21. but the chorionic villus sampling theoretically can be tested as early as the eighth week of gestation. reports of improvement have been noted with high-dose beta-carotene. keratin filament aggregates have been detected for diagnostic purposes in the 19th week of gestation. resulting in sloughing of large plates of scale. bullous congenital ichthyosiform erythroderma [bullous CIE]).cleansers. systemic retinoids. calcipotriol. Keratolytic agents are poorly tolerated by these patients. antibacterial soap. Chorionic villus sampling can diagnose epidermolytic hyperkeratosis earlier by direct gene sequencing if the familial mutation is known. Keratin 1 and keratin 10 are expressed suprabasally as early as week 14 of gestation. 10% glycerin. Activity Activity restrictions are not necessary for patients with epidermolytic hyperkeratosis (EHK. bullous congenital ichthyosiform erythroderma [bullous CIE]) Medication Summary No reported cure or specific therapy is available for epidermolytic hyperkeratosis (EHK. bullous congenital ichthyosiform erythroderma [bullous CIE]).

One patient had significant improvement in clinical disease on a combination therapy of acitretin 40 mg/d and erythromycin 500 mg twice daily. sepsis. Complications Patients with epidermolytic hyperkeratosis (EHK. bullous congenital ichthyosiform erythroderma [bullous CIE]) to the neonatal ICU to monitor for sepsis and electrolyte imbalance may be necessary.[27] Further Inpatient Care Neonatal epidermolytic hyperkeratosis (EHK. and administration of intravenous fluids or antibiotics. sepsis. Inpatient & Outpatient Medications Inpatient medications for epidermolytic hyperkeratosis (EHK. then tapered down to a dose of acitretin 30 mg/d and erythromycin 500 mg/d. 0. bullous congenital ichthyosiform erythroderma [bullous CIE]) are determined by clinical need to treat secondary effects of this condition (eg. bullous congenital ichthyosiform erythroderma [bullous CIE]).[24] Another patient had complete clearing of her lesions after 2 months of acitretin (35 mg/day. bullous congenital ichthyosiform erythroderma [bullous CIE]) patients may need to be transferred to the neonatal ICU for monitoring of infection. and a pungent smell can be noted. although widespread application should be avoided because of the risk of systemic absorption. . a vitamin D3 analogue. bullous congenital ichthyosiform erythroderma [bullous CIE]) are at an increased risk for recurrent infections. Transfer Transfer of infants with epidermolytic hyperkeratosis (EHK.[25] Acitretin at 25 mg/day may significantly improve a patient’s quality of life and skin condition. but the lesions recurred 3 months after discontinuation of acitretin. Further Outpatient Care Schedule routine follow-up visits as needed for symptomatic relief or to follow laboratory studies during systemic therapies for epidermolytic hyperkeratosis (EHK. electrolyte imbalance. Outpatient medications for epidermolytic hyperkeratosis are determined by what works best with each individual patient (see Medication). electrolyte imbalance).5 mg/kg/day).Improvement has been reported with topical retinoid therapy.[26] A patient with the mosaic-type disease was successfully treated with topical maxacalcitol.

bullous congenital ichthyosiform erythroderma [bullous CIE]) is a lifelong condition. MD is a member of the following medical societies: American Academy of Dermatology. MD is a member of the following medical societies: American Academy of Dermatology. Diseases & Prosedures Epidermolytic Hyperkeratosis (Bullous Congenital Ichthyosiform Erythroderma) • Author: Tina S Chen. Chief of Pediatric Dermatology. and Women's Dermatologic Society . California Society of Dermatology and Dermatologic Surgery. MD Pediatric Dermatology Fellow.Prognosis Epidermolytic hyperkeratosis (EHK. Some patients may experience amelioration of symptoms as they age. Medscapa REFERENCE Drugs. Irvine. and Society for Pediatric Dermatology Disclosure: Nothing to disclose. University of California. MD. University of California. MD embryology gut ganglion formation-hirschsprung disease Contributor Information and Disclosures Author Tina S Chen. Rady Children's Hospital. Society for Pediatric Dermatology. San Diego Tina S Chen. School of Medicine Brandie J Metz. bullous congenital ichthyosiform erythroderma [bullous CIE]) about the potential of passing the chromosomal defect on to offspring. Department of Dermatology. Patient Education Educate patients with epidermolytic hyperkeratosis (EHK. MD Assistant Clinical Professor of Dermatology and Pediatrics. Coauthor(s) Brandie J Metz. Chief Editor: Dirk M Elston.

Department of Dermatology. Pfizer Grant/research funds investigator.Disclosure: Nothing to disclose. MD. Katholieke Universiteit Leuven. MD Assistant Clinical Professor. Jeffrey J Miller. Specialty Editor Board Marjan Garmyn. Texas Dermatological Society. MSCE is a member of the following medical societies: Society for Investigative Dermatology Disclosure: AMGEN Consulting fee Consulting. AMGEN Grant/research funds Investigator. MD Director. MD. Paul L Foster School of Medicine. Associate Scholar. Texas Tech University Health Sciences Center. MSCE Medical Director. Pennsylvania State University College of Medicine. MD is a member of the following medical societies: American Academy of Dermatology. MD Associate Professor of Dermatology. Celgene Consulting fee DMC Chair. Chair and Adjunct Head. Center for Clinical Epidemiology and Biostatistics. Pennsylvania State Milton S Hershey Medical Center Jeffrey J Miller. Faculty of Medicine. University of Leuven. Department of Dermatology. Mountain View Dermatology. Joel M Gelfand. PhD Professor. Abbott Grant/research funds investigator. Association of Military Dermatologists. Staff Dermatologist. Richard P Vinson. MD. Belgium Disclosure: Nothing to disclose. and Society for Investigative Dermatology Disclosure: Nothing to disclose. Genentech Grant/research funds investigator. MD is a member of the following medical societies: Alpha Omega Alpha. New York . Association of Professors of Dermatology. Consulting Staff. Department of Dermatology. University of Pennsylvania Joel M Gelfand. Abbott Consulting fee Consulting. Centocor Consulting fee Consulting. Belgium. NIAMS and NHLBI Grant/research funds investigator Chief Editor Dirk M Elston. and Texas Medical Association Disclosure: Nothing to disclose. North American Hair Research Society. Clinical Studies Unit. Novartis investigator. PA Richard P Vinson. American Academy of Dermatology. Assistant Professor. Ackerman Academy of Dermatopathology.

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