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Acute Pityriasis Lichenoides

Acute Pityriasis Lichenoides is a skin condition of unknown cause that affects young adults and adolescents.

What are the other Names for this Condition? (Also known as/Synonyms)

  • Mucha Habermann Disease
  • Pityriasis Lichenoides Et Varioliformis Acuta (PLEVA)
  • PLEVA (Pityriasis Lichenoides Et Varioliformis Acuta)

What is Acute Pityriasis Lichenoides? (Definition/Background Information)

  • Acute Pityriasis Lichenoides is a skin condition of unknown cause that affects young adults and adolescents. In this condition, painful red papules with fluid accumulation form on the skin. Some ulcerate and bleed and form scars on healing
  • Pityriasis Lichenoides may be acute or chronic. The acute form can be itchy and painful, while the chronic form is usually without such symptoms
  • It can affect any part of the body, but it chiefly affects the chest, back, and in rare cases, the mouth and genitalia
  • The skin lesions may ‘come and go’; it may remain for a period of few months and then spontaneously disappear. They may return after a symptom-free period of many years
  • In a majority of cases, there is no treatment for Acute Pityriasis Lichenoides. In some individuals, the use of oral antibiotics and phototherapy may be beneficial. The prognosis of mild condition is typically excellent. However, chronic and severe cases are difficult to treat

Who gets Acute Pityriasis Lichenoides? (Age and Sex Distribution)

  • Acute Pityriasis Lichenoides affects a wide age group, but is more common in adolescents, teenagers, and young adults. This condition is not commonly observed in infants or during old age
  • There is a predilection for males, though both males and females are affected
  • There is no racial or ethnic preference for this condition; it is observed worldwide

What are the Risk Factors for Acute Pityriasis Lichenoides? (Predisposing Factors)

Risk factors identified for Acute Pityriasis Lichenoides include infections by the following pathogens:

  • HIV
  • Staphylococcus aureus
  • Parvovirus
  • Epstein-Barr virus (EBV)
  • Toxoplasma gondii

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases ones chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Acute Pityriasis Lichenoides? (Etiology)

  • The exact cause of Acute Pityriasis Lichenoides is not well-established or understood
  • Even though many infections can make one vulnerable to this skin condition, Acute Pityriasis Lichenoides by itself is non-infectious. It does not spread by direct physical contact, from one individual to another

What are the Signs and Symptoms of Acute Pityriasis Lichenoides?

The signs and symptoms of Acute Pityriasis Lichenoides include the following:

  • The presence of fluid-filled, red papules on the skin; these skin lesions may be painful and itchy (pruritic) in some
  • The lesions often look like chicken pox (varicella). This is not as helpful as a description, as most children are vaccinated against this childhood disease, and the public seldom sees wild chicken pox
  • With time, they may ulcerate and bleed
  • The skin may be hypopigmented or hyperpigmented, once these lesions get better
  • These lesions may be found on the chest, back, arms, palms, and soles. Rarely, the mouth and genital parts may be involved
  • There may be fever, tiredness, and headaches in some individuals
  • There may be relapse and remission of the signs and symptoms; the skin lesions may appear, subside, and then recur
  • The skin lesions may be present for a few months and then disappear spontaneously. They may then return after many years of being symptom-free

In a majority of cases, there may be no signs and symptoms.

How is Acute Pityriasis Lichenoides Diagnosed?

The diagnosis of Acute Pityriasis Lichenoides may involve the following procedures:

  • The diagnosis usually entails a good physical examination and comprehensive evaluation of one’s medical history
  • Examination of the skin by a dermatologist using a special magnified lens (dermoscopy)
  • Skin biopsy: A skin biopsy is performed and sent to a laboratory for a pathological examination, who examines the biopsy under a microscope. After putting together clinical findings, special studies on tissues (if needed) and with microscope findings, the pathologist arrives at a definitive diagnosis
  • A differential diagnosis may be undertaken to eliminate the following conditions:
    • Varicella infection
    • Guttate psoriasis
    • Lymphomatoid papulosis

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Acute Pityriasis Lichenoides?

The complications of Acute Pityriasis Lichenoides may include the following:

  • Intense scratching of the lesions can lead to secondary bacterial or fungal infections
  • When the skin lesions heal, it may result in scar formation causing cosmetic issues
  • Acute conditions may become longstanding and move into its chronic form (Pityriasis Lichenoides Chronica)
  • The longstanding lesions can cause self-image issues, emotional stress, and psychological trauma in some individuals, leading to depression

How is Acute Pityriasis Lichenoides Treated?

In many individuals, Acute Pityriasis Lichenoides can be a self-limiting skin disorder that may not require any treatment. The condition subsides and goes away on its own. The symptoms may be mild and symptomatic treatment is also not required.

In those with significant symptoms, there is no definitive cure for Acute Pityriasis Lichenoides. The treatment strategies that may be adopted may help some individuals more than others, and these may include:

  • If the skin rashes do not cause any signs and symptoms, then observation may be the best treatment option
  • Oral antibiotics
  • Application of topical steroidal creams and topical tacrolimus
  • Phototherapy, if oral antibiotics are not helpful
  • The presence of chronic skin lesions may cause psychological discomfort; individuals may require reassurance to help with stress and anxiety
  • Follow-up care with regular screening and check-ups are important

Despite therapy, the lesions persist for a long period of time, resulting in a chronic skin disorder.

How can Acute Pityriasis Lichenoides be Prevented?

There is currently no effective means to prevent Acute Pityriasis Lichenoides, because the cause of this disorder is unknown. Nevertheless, the following measures may help mitigate risks and avoid the condition from becoming worse:

  • Maintaining good personal hygiene and care
  • Avoiding scratching of the ‘itchy’ lesions
  • Avoiding or minimizing sun exposure

What is the Prognosis of Acute Pityriasis Lichenoides? (Outcomes/Resolutions)

  • The skin lesions in Acute Pityriasis Lichenoides are not life-threatening, but the prognosis depends upon the severity of the condition; severe disorders are difficult to treat and they may become chronic (lasting for a very long period of time)
  • In a majority of cases, the condition is asymptomatic or mild, which subsides on its own without the requirement for any treatment
  • Since Pityriasis Lichenoides can be a longstanding condition, some individuals may have both acute and chronic form of the condition side-by-side
  • Close observation and follow-up visits may be effective

Additional and Relevant Useful Information for Acute Pityriasis Lichenoides:

  • It has not been observed that dietary factors contribute to Pityriasis Lichenoides development
  • Cleaning the skin too hard with strong chemicals or soaps may aggravate the condition. Care must be taken avoid strong soaps and chemicals that could potentially worsen the condition

What are some Useful Resources for Additional Information?

American Academy of Dermatology
930 E. Woodfield Road Schaumburg, IL 60173
Phone: (866) 503-SKIN (7546)
Fax: (847) 240-1859
Website: http://www.aad.org

References and Information Sources used for the Article:

Wolff, K., & Johnson, R. A. (2009). Fitzpatrick's color atlas and synopsis of clinical dermatology. McGraw-Hill Medical.

Thiers, B. H. (1989). Year Book of Dermatology 1988. Archives of Dermatology, 125(8), 1150.

Burns, T., & Breathnach, S. (1992). Rook's Textbook of dermatology Vol 4. London: Blackwell Scientific Publications, 1992.

Bolognia, J. L., Schaffer, J. V., Duncan, K. O., & Ko, C. J. (2014). Dermatology Essentials E-Book. Elsevier Health Sciences.

Helpful Peer-Reviewed Medical Articles:

Bowers, S., & Warshaw, E. M. (2006). Pityriasis lichenoides and its subtypes. Journal of the American Academy of Dermatology, 55(4), 557-572.

Khachemoune, A., & Blyumin, M. L. (2007). Pityriasis lichenoides. American journal of clinical dermatology, 8(1), 29-36.

Magro, C., Crowson, A. N., Kovatich, A., & Burns, F. (2002). Pityriasis lichenoides: a clonal T-cell lymphoproliferative disorder. Human pathology, 33(8), 788-795.

Weinberg, J. M., Kristal, L., Chooback, L., Honig, P. J., Kramer, E. M., & Lessin, S. R. (2002). The clonal nature of pityriasis lichenoides. Archives of dermatology, 138(8), 1063-1067.

Dereure, O., Levi, E., & Kadin, M. E. (2000). T-Cell clonality in pityriasis lichenoides et varioliformis acuta: a heteroduplex analysis of 20 cases. Archives of dermatology, 136(12), 1483-1486.

Tsianakas, A., & Hoeger, P. H. (2005). Transition of pityriasis lichenoides et varioliformis acuta to febrile ulceronecrotic Mucha–Habermann disease is associated with elevated serum tumour necrosis factor‐α. British Journal of Dermatology, 152(4), 794-799.

Klein, P. A., Jones, E. C., Nelson, J. L., & Clark, R. A. (2003). Infectious causes of pityriasis lichenoides: a case of fulminant infectious mononucleosis1. Journal of the American Academy of Dermatology, 49(2), 151-153.

Tomasini, D., Tomasini, C. F., Cerri, A., Sangalli, G., Palmedo, G., Hantschke, M., & Kutzner, H. (2004). Pityriasis lichenoides: a cytotoxic T‐cell‐mediated skin disorder. Evidence of human parvovirus B19 DNA in nine cases. Journal of cutaneous pathology, 31(8), 531-538.