Severe seborrheic dermatitis with systemic infection,
diarrhea, and central nervous system deficiency as a consequence of
complement C5 deficiency (acquired deficiency in most patients).
Complement C5 Deficiency; Dermatitis Exfoliativa
Generalisata; Desquamative Erythroderma in Infants; Eczema Universale
Seborrhoeicum; Erythrodermia Desquamativa of Leiner; Erythrodermia
Desquamativa in Infants; Erythroderma Desquamativum; Erythrodermic
Seborrheic Dermatitis in the Trianon of Life; Exfoliative Dermatitis;
Seborrhoic Diathesis in Infants; Leiner-Moussous Disease.
First described in 1908 by Karl Leiner, an Austrian pediatrician.
Prevalent in infant females. Breast-fed infants are most
Not a genetic disorder in most patients. Gene
map locus of hereditary complement C5 deficiency is 9q34.1.
Leiner disease includes a heterogeneous group of
disorders. A common feature of these disorders is temporary or permanent
(hereditary forms) plasma deficiency in complement C5 activity resulting in
decrease of opsonic properties of plasma (deficiency in phagocytosis).
Clinical picture. Deficiency in complement C5 activity
Syndrome characterized by desquamation (severe
generalized seborrheic dermatitis), recurrent local and systemic infection,
severe diarrhea, marked wasting, central nervous system deficiency, and
failure to thrive. It begins as seborrheic eczematoid lesions of the scalp
and face or the gluteal region, eventually spreading to other areas.
Keratitis and corneal ulcers may occur. Caused by complement C5 deficiency
in infancy; rapid onset in second to fourth month of life.
Elective anesthesia and surgery
should be postponed until skin covering is restored in relevant areas.
Patients with Leiner disease are prone
to severe bacterial contamination as a consequence of their extensive skin
lesions and deficiency in phagocytosis. Large spectrum antibioprophylaxis
should be considered. Because of extended skin lesions, there is a danger of
systemic toxicity with topical agents (especially disinfectants and skin
Use topical agents with extreme care
because of increase systemic absorption and toxicity. Avoid any solution
containing potentially toxic components, either topical or systemic. Some
patients receive high-dose corticosteroid in the acute phase, so
intraoperative supplementation will be needed.
Other disorders with
erythroderma (some of which are included in the general term of Leiner
disease), including the following:
Atopic Eczema: Later onset, familial context of atopy.
Infantile Seborrheic Dermatitis: “Cradle cap,” usually
self-limiting, subsiding by age 6 months; no complement C5 deficiency; cured
by topical cortisone.
Ichthyosiform Erythroderma: Autosomal dominant; bullous
Netherton Syndrome: Recessive skin atopy disorder, bamboo hair,
mostly in females.
Immunodeficiency Syndromes: Group of syndromes that have in common
a dysfunction of the immune system. They are divided into T-cell system
deficiency or B-cell deficiency. For instance, Idiopathic Thrombocytopenic
Purpura (ITP), X-linked Agammaglobulinemia, Severe Combined
Immunodeficiency, Nezelof Syndrome, and Wiskott-Aldrich Syndrome are all
considered immunodeficiency syndromes.
Bykowsky MJ: Generalized seborrheic dermatitis in an immunodeficient