Hemorrhagic Darier's Disease

Haemorrhagic macules on the palms and soles

“…These macules, typically preceded by trauma, arise on palmar and plantar surfaces, and usually present without initial blistering . Early lesions are reddish, while older lesions take on a black colouration, resembling India ink or silver nitrate stains, before resolving in 3 weeks . The pigmentation in these lesions is a result of erythrocytes that reside within the epidermis …”

“…Haemorrhagic lesions show the same suprabasal acantholysis as nonhaemorrhagic lesions of DD. However, intraepidermal collections of erythrocytes are prominently seen . In early lesions (clinically red), the erythrocytes are found within the suprabasal lacunar space, while in later lesions (clinically black), the red blood cells are found at higher levels of the epidermis, and become amorphous in the stratum corneum before undergoing transepidermal elimination .…”

“…1,2 These acral changes are quite common, being seen in 96% of patients. 2 By contrast, the haemorrhagic lesions in DD, first described in 1964 Jones et al, 3 are uncommon, being seen in only 6% of patients. 2 The pathognomonic haemorrhagic lesions of DD classically appear as irregularly shaped, jagged, red to black macules 2-8 mm in diameter.…”

“…4 Early lesions are reddish, while older lesions take on a black colouration, resembling India ink or silver nitrate stains, before resolving in 3 weeks. 3,4 The pigmentation in these lesions is a result of erythrocytes that reside within the epidermis. 3 The pathogenesis of DD is related to malfunction of the SERCA2 sarcoplasmic/endoplasmic reticulum calcium transport pump, secondary to various mutation in the ATP2A2 gene encoding for this pump.…”

“…3,4 The pigmentation in these lesions is a result of erythrocytes that reside within the epidermis. 3 The pathogenesis of DD is related to malfunction of the SERCA2 sarcoplasmic/endoplasmic reticulum calcium transport pump, secondary to various mutation in the ATP2A2 gene encoding for this pump. 5 SERCA2 is responsible for maintaining cellular calcium homeostasis.…”