INFILTRATIVE DISEASES
Langerhans cell histiocytosis
INFLAMMATORY DISEASE
Crohn’s disease
Hidradenitis suppurativa
Hirschsprung disease – perianal papulonodular dermatitis
Ann DV 123:549–551, 1996
Post-inflammatory – secondary to lip licking, contact or
photocontact dermatitis
Pyoderma vegetans
Hautarzt 35:132–137, 1984
Sarcoid
METABOLIC
Acrodermatitis enteropathica or acquired zinc deficiency –
autosomal recessive; zinc is metal moiety of enzymes such as
alkaline phosphatase (results in low serum alkaline
phosphatase), alcohol dehydrogenase, digestive enzymes
Ped Derm 19: 426–431, 2002; Ped Derm 19:180–182, 2002;
J Inorg Biochem 12:71–78, 1980; Int J Derm 17:380–387, 1978
Biotin deficiency, dietary – dermatitis around nose and mouth
J Dermatol 32:256–261, 2005; Textbook of Neonatal
Dermatology, p.254, 2001; Acta Pediatr 85:872–874, 1996
Biotinidase deficiency – autosomal recessive; pyruvate
carboxylase, propionyl-coenzyme A carboxylase, and
3-methylcrotohyl-CoA carboxylase; accumulation of
urinary organic acids; neonatal and infantile; neonatal –
holocarboxylase synthetase deficient; seen in first 6 weeks
of life; fiery red intertriginous dermatitis; infantile, juvenile, or
late onset – biotinidase deficient (biotin-responsive multiple
carboxylase deficiency); after 3 months of life, intertriginous
rash with keratoconjunctivitis, xerosis, generalized pallor,
periorificial dermatitis, alopecia, branny desquamation, and
atrophic glossitis
Ped Derm 21:231–235, 2004; Ped Derm
16:95–102, 1999; Semin Dermatol 10:296–302, 1991;
JAAD 9:97–103, 1983
Carbamoyl phosphate synthetase (urea cycle) deficiency
(arginine deficiency)
Am J Dis Child 135:437–442, 1981
Neonatal citrullinemia – autosomal recessive; deficiency of
argininosucccinic acid synthetase or ornithine transcarbamylase
or carbamoyl phosphate synthetase
; cutaneous lesions due to
arginine deficiency
JAAD 46:965–967, 2002; JAAD 14:321–326,
1986; Am J Dis Child 135:437–442, 1981
Essential amino acid deficiencies – isoleucine, valine,
methionine, threonine; same clinical picture as acrodermatitis
enteropathica
Ped Derm 19:180–182, 2002
Essential fatty acid (EFA) deficiency – unsaturated 18, 20 or
22 carbon chain lengths; n-6 series produces arachidonic acid
from linoleic acid; EFAs are precursors of eicosanoids, influence
membrane fluidity and are necessary for lamellar granule
formation in epidermis; alopecia, generalized xerotic scaling
and intertriginous erosions
AD 113:939–941, 1977; Pediatrics
31:171–192, 1963
Histidine deficiency
Am J Clin Nutr 21:367–375, 1968;
Pediatrics 31:786–801, 1963
Isoleucine deficiency – diet treatment of propionic acidemia and
arginino-succinic acidemia
JAAD 28:289, 1993; Ped Derm
9:191, 1992
Kwashiorkor – protein/amino acid deficiency – perioral
dermatitis; patient has peripheral edema yet only weighs
60–80% of expected weight; patches of hypopigmentation
with fine circumoral branny desquamation; extensor surfaces
dry, glossy and cracked; edema starts on dorsal aspects of feet
and can generalize; enamel paint spots on elbows, knees,
ankles, and intertriginous areas; flaky paint describes extensive
peeling with erosion and flexural fissuring; red tinge to hair; flag
sign
AD 137:630–636, 2001; JAAD 21:1–30, 1989;
AD 123:1674–1676, 1987
Liver disease, chronic (cirrhosis) – pseudoglucagonoma
syndrome
BJD 101:581–587, 1979
Malnutrition-associated rash of cystic fibrosis
Ped Derm
17:337–347, 2000
Maple syrup urine disease – isoleucine, leucine, and/or valine
deficiency; impaired degradation of branched chain amino
acids; elevated leucine, isoleucine, and valine in blood, urine
and tissues; erythematous scaling diaper dermatitis;
widespread erythematous dermatitis during treatment with
dietary restriction
Am J Dis Child 147:954–956, 1993;
JAAD 28:289–292, 1993
Methylmalonic acidemia – deficiency of methylmalonyl
coenzyme A mutase or its cofactors adenosylcobalamin and
methylcobalamin
Dermatol Pediatr Lat 1:46–48, 2003; Ped
Derm 16:95–102, 1999; AD 133:1563–1566, 1997; J Pediatr
124:416–420, 1994; BJD 131:93–98, 1994
Nutritional deficiency associated with anorexia nervosa
AD 140:521–524, 2004
Propionic academia (aciduria)
Ped Derm 16:95–102, 1999;
J Pediatr 124:416–420, 1994
Pyridoxine deficiency – pyridoxal phosphate is coenzyme in
tryptophan metabolism, alanine aminotransferase, apartate
aminotransferase, amino acid cocarboxylases, deaminase,
transulfurases, and desulfhydrase enzymes, amino butyric acid
synthesis and fatty acid metabolism
JAAD 15:1263–1264, 1986
Tyrosinemia type II
Ped Derm 17:337–347, 2000
Vitamin B
1
deficiency
Clinics in Derm 17:457–461, 1999
Vitamin B
2
(riboflavin) deficiency – dermatitis around nose,
eyes, ears, and genitals (oro-oculogenital syndrome)
Clinics
in Derm 17:457–461, 1999; Rook p.2657, 1998, Sixth Edition;
AD 112:70–72, 1976
Zinc deficiency, acquired – due to inadequate dietary intake –
low breast milk zinc, weaning to cow’s milk, starvation, anorexia
nervosa, prolonged hyperalimentation without supplemental
zinc
Ped Derm 19:426–431, 2002; Ped Derm 17:337–347,
2000; due to decreased gastrointestinal absorption –
gastrointestinal disorders, phytate-rich foods
Ped Derm 17:337–347, 2000; late acquired acrodermatitis
enteropathica
NEJM 352:1121, 2005
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