Erythroderma Desquamativum (Leiner's Disease)

The Pattern of Endogenous Eczema in the Northern Frontier, Kingdom of Saudi Arabia

“…In adults, blepharitis was seen in a few patients. A case of severe exfoliative dermatitis (Leiner disease) 8 was seen in a male patient (foreign resident). There was good response of the associated dandruff to shampoo containing salicylic acid and tar.…”

Leiner's disease (erythroderma desquamativum): A review and approach to therapy

“…The changes within the intertriginous areas are more exudative and edematous without scales, and have a tendency for accumulating greasy debris 5,27 . At this stage, there is no fever, chills, pruritus, Nikolsky sign, nor changes in the oral mucosa, although there may be generalized soft nontender lymphadenopathy 5,12,14 . Within 2 to 6 days of scaling, the extensive exfoliation in the infant can lead to critical complications, such as infections, impaired thermoregulation, and severe fluid loss 19…”

“…By combining the infant's medical history with histological and immunohistochemical studies, the most common causes of infantile erythroderma can be differentiated from LD (Table 4). 12,21,25,41,42 …”

“…Moreover, with ever‐increasing evidence of biotin deficiency causing the dermatological and gastrointestinal symptoms in LD, biotin level measurement and treatment response may be included for the diagnosis of LD 4,23 . Thus, the dramatic clinical response to fresh plasma containing active complement as well as biotin replacement validates the importance of this finding in the diagnosis of LD 12,15,23,27 . Laboratory studies frequently show anemia, dysgammaglobulinemia with low IgG and high IgM, and leukocytosis, with normal humoral and cellular immunity 4,10,11,17,27,38 .…”

“…27 Secondary anemia, dysgammaglobulinemia, hypoproteinemia, as well as carbohydrate, protein and fat malabsorption and poor metabolism, are frequently found. 12,15,23,25 The associated pyogenic and fungal infections are recurrent and vary from local to systemic. Most commonly, S. aureus, Candida and gram-negative bacteria cause these secondary infections.…”