Schneiderman Prelims Vol-I. qxd

INFILTRATIVE DISORDERS
Amyloidosis – the separation of epidermis and dermis occurs
within the dermal deposits of amyloid; protein AL consists
of a polypeptide of light chain immunoglobulin composed of
fragments of the variable (amino-terminal) region; clinical
lesions include petechiae, purpura, ecchymoses, plaques,
tumefactive lesions, pigmentary changes, scleroderma-like
infiltration, bullae, alopecia, cord-like blood vessel thickening,
nail dystrophy, and cutis laxa 
Eur J Dermatol 10:139–142,
2000; Medicine 24:124–128, 1994; Cutis 43:346–352, 1989;
AD 124:1683–1686, 1988; BJD 113:85–95, 1985; AD
117:782–784, 1981; oral hemorrhagic bullae Rook p.3056,
1998, Sixth Edition
Langerhans cell histiocytosis – purpuric vesicles 
JAAD
37:314–317, 1997; JAAD 13:481–496, 1985
Mastocytosis – bullous mastocytosis presents before age of
9 months; occurs in absence of pigmentary changes and
without lesions of urticaria pigmentosa; if present in infancy,
extracutaneous involvement is common 
Ped Derm 19:375–381,
2002; AD 127:1049–1054, 1991; AD 101:547–562, 1970
100
A CLINICIAN
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S GUIDE TO DERMATOLOGIC DIFFERENTIAL DIAGNOSIS, VOLUME 1
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BULLAE, HEMORRHAGIC
101
INFLAMMATORY DISORDERS
Erythema multiforme with epidermal necrosis
Pyoderma gangrenosum, bullous 
BJD 102:235–237, 1980;
Trans St John’s Hosp Dermatol Soc 60:142–151, 1974;
association with polycythemia vera 
Clin Exp Dermatol
12:375–377, 1987; leukemia JAAD 9:751–758, 1983; CML Proc
R Soc Med 67:1239–1240, 1974; hairy cell leukemia JAAD
11:300–302, 1985
METABOLIC
Acrodermatitis enteropathica
Cryoglobulinemia – mixed cryoglobulinemia secondary to
hepatitis C infection 
Cutis 72:290,295, 2003
Diabetic bulla – intraepidermal or subepidermal separation
without acantholysis 
JAAD 7:427–455, 1982
Disseminated intravascular coagulation – obstetric
complications, extensive tissue damage, gram-negative
septicemias, immune reactions, malignancy, snake bites,
angiomas, protein S or protein C deficiency 
Br Med J
312:683–687, 1996; BJD 88:221–229, 1973
Hyperhomocysteinemia and antiphospholipid antibodies 
JAAD
49:S161–163, 2003
Neonatal purpura fulminans – ecchymoses of limbs at sites of
pressure in first day of life; enlarge rapidly, hemorrhagic bullae
with central necrosis; homozygous protein C or protein S
deficiency 
Semin Thromb Hemost 16:299–309, 1990
Paroxysmal nocturnal hemoglobinuria – petechiae, ecchymoses,
red plaques which become hemorrhagic bullae with necrosis;
lesions occur on legs, abdomen, chest, nose, and ears;
deficiency of enzymes – decay-accelerating factor (DAF) and
membrane inhibitor of reactive lysis (MIRL); acquired
intravascular hemolytic anemia; due to a drop in pH of serum
during sleep; Ham test (acid hemolysis); sucrose lysis test, low
leukocyte alkaline phosphatase; anemia, hemoglobinuria (dark
urine), increased serum hemoglobin, hemosiderinuria; abdominal
pain, recurrent infections, headache, venous thrombosis,
progressive bone marrow failure, and ultimately lymphoreticular
malignancy (especially, leukemia) 
AD 138:831–836, 2002;
AD 122:1325–1330, 1986; AD 114:560–563, 1978
Pellagra 
Cutis 69:96–98, 2002; Semin Dermatol 10:282,1991
Porphyria cutanea tarda (PCT)
Pseudo-PCT
Renal failure with or without furosemide
Scurvy 
JAAD 41:895–906, 1999
Wilson’s disease 
JAAD 21:1030, 1989