Loose anagen hair syndrome L73.8

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 29.10.2020

Synonym(s)

loose anagen hair; Loose anagen hair; Loose anagen-hair of childhood; Loose anagen hair syndrome; Loose anagen syndromes; Noonan-like syndrome with loose anagen hair; Phenomenon of easily pullable hair

History
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Nödl, 1986

Definition
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Rare growth disorder of the scalp hairs occurring in children with greatly increased depilatability of the scalp hairs.

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Etiopathogenesis
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Unsolved. In some families autosomal dominant inheritance with variable expression of a mutation of the K6HF gene is discussed, which leads to a disturbed attachment of hair shaft and root sheath.

Manifestation
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Small and schoolchild age.

Clinical features
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Already with a light pull the hairs can be depilated in tufts and painlessly. Often several, sometimes larger bald patches. Prompt regrowth of new hairs. The hair appears clinically inconspicuous.

Syndromal occurrence: The "loose anagen hair syndrome" can occur in the context of RASopathies, such as Noonan-like syndrome (Mazzanti syndrome), with cardiological defects, growth retardation, hyperpigmented skin.

Histology
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The hair shaft cross-sections are irregularly oval or drop-shaped. Gap formation between inner and outer root sheath. Merging of the individual layers of the inner root sheath to form a homogeneous envelope and crumbly disintegration often already below the middle of the follicle.

Diagnosis
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In the trichogram almost exclusively thin anagen hairs without root sheaths.

Therapy
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No causal therapy known. Avoid traction. Wearing a hair net at night.

Progression/forecast
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Not exactly known, possibly it is a temporary maturation disorder with regression during puberty. This course distinguishes the clinical picture from the Short Anagen Hair Syndrome, which apparently shows no regression tendencies.

Literature
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Gripp KW et al (20143) Expanding the SHOC2 mutation associated phenotype of Noonan syndrome with loose anagen hair: structural brain anomalies and myelofibrosis. Am J Med Genet A 161A: 2420-1430
Mazzanti L et al (2003) Noonan-like syndrome with loose anagen hair: A new syndrome? On J Med Genet 118A: 279-286
Tosti A et al (2002) Loose anagen hair syndrome and loose anagen hair. Arch Dermatol 138: 521-522
Chapalain V et al (2002) Is the loose anagen hair syndrome a keratin disorder? A clinical and molecular study. Arch Dermatol 138: 501-506
Mazzanti L et al (2003) Noonan-like syndrome with loose anagen hair: a new syndrome? On J Med Genet 118A: 279-286
Nödl F, Zaun H, Zinn KH (1986) Increased epilatability of anagen hairs in children as a result of a maturation defect of the follicles with disturbed attachment of hair shaft and root sheath. The phenomenon of easily pullable hairs. Nude Dermatol 12: 55-57
Fence H (1984) Differential diagnosis of alopecia in children. In: Happle R, Grosshans E (Eds). Pediatric dermatology. Springer, Berlin, pp. 157-166