What is pityriasis lichenoides?


According to MedScape, pityriasis lichenoides is “a rare cutaneous disorder of unknown etiology,” characterized by “a spectrum of clinical presentations ranging from acute papular lesions that rapidly evolve into pseudovesicles and central necrosis (pityriasis lichenoides et varioliformis acuta or PLEVA) to small, scaling, benign-appearing papules (pityriasis lichenoides chronica or PLC).”

According to a 2007 article published in the American Journal of Clinical Dermatology, pityriasis lichenoides is “an uncommon, acquired spectrum of skin conditions that poses various challenges to patients as well as clinicians.”

All this is a fancy way of saying that PL/PLC/PLEVA represent a family of inflammatory skin rashes without a clear-cut cause or cure; sometimes it goes away on its own, and sometimes it doesn’t.

Pityriasis Lichenoides Fast Facts

  • Characterized by the acute onset of red, raised papules on the torso and proximal extremeties (arms, legs) that are otherwise asymptomatic in the case of PLC, and that may itch and ulcerate in the case of PLEVA
  • Can affect both genders but slightly more prevalent in males
  • Most often affects adolescents and young adults, usually appearing before the age of 30
  • Generally not harmful to general health (with the notable exception ofof febrile ultranecrotic mucha-haberman disease), although rare cases of malignant transformation have been reported
  • Not directly contagious and usually self-limiting, although it may persist over the course of months and years


Pityriasis lichenoides chronica (PLC) tends to present as a more mild, less symptomatic rash (smaller papules, little to no itching) that continues for a longer period of time, from months to years.

Distinguishing criteria of PLC: 

  • Small papules that spotaneously appear, flatten and regress over the course of weeks, leaving behind hyperpigmented spots
  • Usally present on torso and proximal extremities
  • Papules tend not to itch
  • Papules tend not to ulcerate (turn into an open sore)

On the other hand, pityriasis lichenoides et varioliformis acuta (PLEVA) usually presents with angrier, redder lesions that are 5-15mm in diameter and that may exhibit other symptoms like ulceration and itching:

Distinguishing criteria of PLEVA: 

  • Somewhat larger papules of around 5-15mm
  • Present on torso and proximal extremetires
  • Itching may be present
  • Ulceration may be present

Both types of lesions can exist on the same patient at the same time; a biopsy is required to conclusively distinguish these subtypes, as they exhibit different microscopic structures, although debate still exists as to whether they are truly distinct conditions or exist on a unified spectrum.

A third, extremely rare condition called febrile ultranecrotic Mucha-Haberman disease (FUMHD) exists within the pityriasis lichenoides family and is characterized by large, highly irritated papules that rapidly transform into painful blood blisters and pustules. This condition is considered a medical emergency and anyone who exhibits a severe worsening of their PL condition should seek urgent care immediately.

Underlying Mechanism Behind PLC/PLEVA

I’ll discuss this in greater depth in a future post, but there are three generally accepted hypotheses on the mechanism behind the chronic skin inflammation that characterizes pityriasis lichenoides:

  • Inflammation caused by an infection: An inflammatory reaction caused by infectious agents; suspects include members of the herpes family like the Epstein-Barr virus (EBV) and cytomegalovirus (CMV), toxoplasma gondii, parovirus and staph.
  • Benign T-cell lymphoproliferative disorder: Biopsies of patients with PLC and PLEVA have shown an excessive number of immune T-cells present in the lesions, leading some studies to conclude that the condition is on the benign end of a spectrum that includes cutaneous T-cell lymphomas like mycosis fungoides.
  • Hypersensitivity vasculitis: Circulating immune complexes may deposit themselves in the skin and encourage lesion formation.

How I was diagnosed with pityriasis lichenoides: symptoms and signs

It took three months and three missed diagnoses from the moment my symptoms first appeared to the point when my “mysterious red spots” were biopsied and conclusively identified as pityriasis lichenoides et varioliformis acuta (PLEVA). Take a read below: Does any of this sound familiar?

I remember the exact day that my journey with pityriasis lichenoides began. In mid-December of 2012, I woke up, went to the bathroom to brush my teeth and noticed that I had broken out in a strange rash, apparently overnight — a few dozen red bumps dotted my lower torso, clustering around my navel and extending around my torso to my flanks. This is how you’d describe the event in more precise medical parlance: PLC and PLEVA usually “present with the sudden appearance of multiple red papules on the trunk, buttocks, and proximal extremities. It can, however, occur anywhere on the body.”

I didn’t feel itchy or otherwise unwell, so as a relatively healthy guy in my mid-twenties, I chalked it up to some kind of allergic reaction and continued about my day.

The next morning, I woke up and realized that the rash had spread; I could see the raised red spots on my arms and the tops of my legs. At breakfast, one of my friends noticed the spots and jokingly asked if I had contracted chicken pox (the thought had occurred to me, but I knew I had already had it as a child). This friend’s observation prompted me to visit an urgent care clinic, where a group of puzzled nurse practitioners pronounced the rash as a “viral exanthem,” or a reaction caused by my body fighting some kind of virus.

Misdiagnosis #1: Viral exanthem

Prescribed treatment: Wait it out

The nurses said that the rash would probably go away in a week or two, but Christmas came and went and the red spots were still present; the original spots had started to flake and fade into dull brown, but a new crop appeared to take their place. There were still no aggravating symptoms — no itchiness, no fatigue, just the spots.

At my parents’ suggestion, I visited my old pediatrician for a second opinion. After inquiring about systemic symptoms (still none — I felt healthy as ever), the good doctor said that he suspected folliculitis — often called the “hot tub disease,” it is the result of a bacterial or fungal infection that inflames the hair follicles. He prescribed a course of the antibiotic Augmentin.

Misdiagnosis #2: Folliculitis

Prescribed treatment: Augmentin

I completed the antibiotic course but there was zero improvement in the spots. At this point, more than a month had passed since the symptoms appeared. I got a referral to a dermatologist who took a quick look and immediately pronounced the spots to be allergic dermatitis. She advised me to change out my laundry detergent and soaps and prescribed triamcinolone steroid cream to reduce the inflammation. You guessed it: no effect.

Misdiagnosis #3: Allergic dermatitis

Prescribed treatment: Triamcinolone cream

After another month, I finally got a referral to an esteemed dermatologist in my area. He did a complete and careful examination and took a biopsy of one of the lesions; within a day, he had called me with a diagnosis of pityriasis lichenoides.

The moral of this story is simple: if mysterious red spots appear on your body and you can’t identify a proximate cause, get to a dermatologist and ask for a biopsy! Obtaining an accurate diagnosis is the first step to understanding pityriasis lichenoides and determining the steps to take in order to manage this condition.