Disease directory
Pityriasis lichenoides
Published: September 18, 2026Updated: September 18, 2026
Important: Informational content intended for healthcare professionals. It does not replace clinical evaluation or establish a doctor-patient relationship.
Overview
- Alternative names.
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Acute pityriasis lichenoides et varioliforme ( PLEVA , Mucha-Habermann disease ).
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Chronic pityriasis lichenoides ( PLC ).
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Fulminant variant: febrile ulceronecrotic Mucha-Habermann disease ( FUMHD ). Bowers 2006 , Khachemoune 2007 .
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- First descriptions.
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The acute form was described by Mucha (1916) and Habermann (1925) ; the febrile ulceronecrotic variant was described by Degos (1966) . Tsuji 1996 , Maekawa 1994 .
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- Epidemiology.
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Rare disorder; clear predominance in children and young adults ; more persistent courses in children and longer lasting PLC than PLEVA. Zang 2018 , Wahie 2007 .
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There is no clearly predominant sex in all series; the distribution by trunk and proximal limbs is typical. Bowers 2006 .
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- Codes.
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ICD-10: L41.0 (PLEVA), L41.1 (PLC), L41.9 (PL, unspecified), ICD-11: EA93 (pityriasis lichenoides).
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- Diagnostic checklist (quick)
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Compatible clinical presentation (truncus-proximal distribution, necrotizing papules in PLEVA; desquamative maculopapules in PLC). Bowers 2006 .
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Punch biopsy of 4–5 mm of early ± crusted lesion with H&E; consider a second perilesional biopsy for IFD if vasculitis is suspected. Muhlbauer 1984 .
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Immune : T panel (CD3, CD4, CD8 , CD30 ); TCR-γ/β if doubt persists with LyP/MF (lymphomatoid papulosis/mycosis fungoides). Wood 1987 , Dereure 2000 .
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Laboratory tests for differential diagnosis : VDRL/RPR, HIV, streptococcal tests according to clinical presentation. Teklehaimanot 2024 .
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