Hailey–Hailey disease: A review of clinical features in 26 cases with special reference to the secondary infections and their control

Adalimumab therapy used successfully for recalcitrant Hailey-Hailey disease

“…Clinically, this process correlates with the exacerbation of the symptoms of HHD and Darier disease following secondary infections. 7 , 8 Although further investigation is needed, these findings offer a possible explanation for how TNF-α inhibition contributed to sustained remission of HHD symptoms in our patient, in whom multiple therapies had failed, by potentially decreasing the susceptibility to this infection.…”

A Case Series of Hailey Hailey Disease in Male and Female Patients

“…Hailey-Hailey disease [HHD] also known as familial benign chronic pemphigus it is a rare blistering skin disease first described by an Howard and Hugh Hailey in 1939. [1] Erythema, Papulovesicular and blisters developed especially where skin folds and other regions exposed to increased friction such as retroarticular region, lateral parts of the neck, axillae, flex or sides of the elbows, navel, inguinal region, genital and perianal region in women and also in sub mammary region.fig- 1 2 .It caused by heterozygous mutations in the ATP2C1 gene that encodes the secretory pathway Ca +2 /Mn 2+ ATP-ase Protein of the Golgi apparatus, leading to alteration in Ca 2+ -dependent intracellular signaling and resulting in the loss of cellular adhesion in the epidermis. [2]The clinical diagnosis is made based on monomorphic eruption of dome-shaped blisters and pustules.…”

Hailey Hailey-a blooming disease

“…2 The first onset of HHD generally occurs between 20 and 40 years of age but is uncommon in India(1:50,000). 3,4 It is clinically characterized by recurrent vesicles which rupture, leaving painful fissures and scaly erythematous plaques in the flexures. 5 This condition with intertriginous area involvement could be easily missed because of its rarity.…”