Pityriasis lichenoides

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Pityriasis lichenoides et varioliformis acuta
PLEVA.jpg
Photo of the Pityriasis lichenoides et varioliformis acuta or Mucha Habermann's Disease, showing the right leg
Specialty Dermatology   OOjs UI icon edit-ltr-progressive.svg

Pityriasis lichenoides represents a distinct subset of inflammatory skin disorders that includes pityriasis lichenoides chronica, febrile ulceronecrotic Mucha-Habermann disease, and pityriasis lichenoides et varioliformis acuta (PLEVA). [1]

Contents

PLEVA typically manifests as an acute to subacute skin eruption of several tiny red papules that grow into polymorphic lesions. It may also leave behind varicella-like scars and hyper- or hypopigmentation sequelae. [2]

Pityriasis lichenoides chronica (PLC) has very small reddish-brown flat maculopapules with a mica-like scale that appear more gradually; it also has long remission intervals between episodes of relapse. [2]

Febrile ulceronecrotic Mucha-Habermann disease (FUMHD) is best treated as a dermatological emergency because it is an acute, severe, widespread eruption of purpuric and ulceronecrotic plaques that can have a 25% fatality rate and accompanying systemic involvement. [2]

Signs and symptoms

The characteristic feature of PLEVA is the rapid evolution of 2- to 3-mm-diameter erythematous macules into papules with a fine micaceous scale. The thicker the scale, the more frequently it breaks free at the edges while staying attached in the middle. The central punctum of the papule frequently develops into a vesiculopustular structure, experiences hemorrhagic necrosis, ulcerates, and is covered in reddish-brown crusts. There may be postinflammatory hyper- and hypopigmentation as well as varioliform scars. Pruritus and burning are among the symptoms. Although diffuse and widespread patterns can also occur, the trunk, extremities, and flexural areas are the most common sites for PLEVA to occur. Lesions can occur at any stage of development, making the eruption polymorphous. Subsequent crops of lesions can persist for weeks, months, or even years. [1]

The rapid progression of necrotic papules to large coalescent ulcers with necrotic crusts, hemorrhagic bullae, and pustules distinguishes Febrile ulceronecrotic Mucha-Habermann disease from PLEVA. Both a secondary infection of the ulcers and a large, painful necrosis of the skin are possible. The mucosa of the mouth and genitalia may also be impacted. Atrophic scars and hypopigmentation are common after ulcer healing. [1]

PLC presents with a far slower clinical course than both febrile ulceronecrotic Mucha-Habermann disease and PLEVA. Similar to PLC, the lesion begins as an erythematous papule that turns reddish-brown and is easily detached to reveal a shiny, pinkish-brown surface. The lesion also has a centrally adherent micaceous scale. In contrast to PLEVA and febrile ulceronecrotic Mucha-Habermann disease, the papule in question regresses and flattens on its own over a few weeks. Frequently, a hyper- or hypopigmented macule is left behind. [1]

Diagnosis

The clinical examination of the skin is used to diagnose and distinguish between the various forms of pityriasis lichenoides. For pityriasis lichenoides, a skin biopsy followed by a histopathologic examination is a confirmatory procedure. Histologically, interface dermatitis with a noticeable lymphocytic infiltrate is the hallmark of pityriasis lichenoides. [2]

Related Research Articles

<span class="mw-page-title-main">Pityriasis lichenoides et varioliformis acuta</span> Medical condition

Pityriasis lichenoides et varioliformis acuta is a disease of the immune system. It is the more severe version of pityriasis lichenoides chronica. The disease is characterized by rashes and small lesions on the skin. The disease is more common in males and usually occurs in young adulthood, although it has been seen in every age group and every race. It is possible for the disease to go into remission for short periods of time or forever.

<span class="mw-page-title-main">Tinea versicolor</span> Skin disease

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<span class="mw-page-title-main">Skin condition</span> Any medical condition that affects the integumentary system

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<span class="mw-page-title-main">Lichen planus</span> Human chronic inflammatory disease

Lichen planus (LP) is a chronic inflammatory and autoimmune disease that affects the skin, nails, hair, and mucous membranes. It is not an actual lichen, but is named for its appearance. It is characterized by polygonal, flat-topped, violaceous papules and plaques with overlying, reticulated, fine white scale, commonly affecting dorsal hands, flexural wrists and forearms, trunk, anterior lower legs and oral mucosa. The hue may be gray-brown in people with darker skin. Although there is a broad clinical range of LP manifestations, the skin and oral cavity remain as the major sites of involvement. The cause is unknown, but it is thought to be the result of an autoimmune process with an unknown initial trigger. There is no cure, but many different medications and procedures have been used in efforts to control the symptoms.

<span class="mw-page-title-main">Hypopigmentation</span> Area of skin becoming lighter than the baseline skin color

Hypopigmentation is characterized specifically as an area of skin becoming lighter than the baseline skin color, but not completely devoid of pigment. This is not to be confused with depigmentation, which is characterized as the absence of all pigment. It is caused by melanocyte or melanin depletion, or a decrease in the amino acid tyrosine, which is used by melanocytes to make melanin. Some common genetic causes include mutations in the tyrosinase gene or OCA2 gene. As melanin pigments tend to be in the skin, eye, and hair, these are the commonly affected areas in those with hypopigmentation.

<span class="mw-page-title-main">Pityriasis rosea</span> Skin disease

Pityriasis rosea is a type of skin rash. Classically, it begins with a single red and slightly scaly area known as a "herald patch". This is then followed, days to weeks later, by an eruption of many smaller scaly spots; pinkish with a red edge in people with light skin and greyish in darker skin. About 20% of cases show atypical deviations from this pattern. It usually lasts less than three months and goes away without treatment. Sometimes malaise or a fever may occur before the start of the rash or itchiness, but often there are few other symptoms.

Pityriasis commonly refers to flaking of the skin. The word comes from the Greek πίτυρον 'bran'.

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<span class="mw-page-title-main">Febrile neutrophilic dermatosis</span> Medical condition

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<span class="mw-page-title-main">Ecthyma gangrenosum</span> Medical condition

Ecthyma gangrenosum is a type of skin lesion characterized by vesicles or blisters which rapidly evolve into pustules and necrotic ulcers with undermined tender erythematous border. "Ecthyma" means a pus forming infection of the skin with an ulcer, "gangrenosum" refers to the accompanying gangrene or necrosis. It is classically associated with Pseudomonas aeruginosa bacteremia, but it is not pathognomonic. Pseudomonas aeruginosa is a gram negative, aerobic bacillus.

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Tumid lupus erythematosus is a rare, but distinctive entity in which patients present with edematous erythematous plaques, usually on the trunk.

<span class="mw-page-title-main">Annular erythema of infancy</span> Medical condition

Annular erythema of infancy(AEI) consists of self-limited eruptions of erythematous, annular to polycyclic patches and plaques. It is an idiopathic figurate erythema. Over several days, a single lesion disappears without leaving behind any scale or hyperpigmentation. Mostly affecting the trunk, face, and extremities, this rash has no symptoms. The diagnosis of AEI is made through a combination of histopathologic and clinical examinations. The disease first manifests in infancy, and if treatment is not received, the periodic eruptions usually stop after the first year of life.

Adrenergic urticaria is a skin condition characterized by an eruption consisting of small (1-5mm) red macules and papules with a pale halo, appearing within 10 to 15 min after emotional upset. There have been 10 cases described in medical literature, and involve a trigger followed by a rise in catecholamine and IgE. Treatment involves propranolol and trigger avoidance.

Doucas and Kapetanakis pigmented purpura is a skin condition characterized by scaly and eczematous patches, which also have petechiae and hemosiderin staining.

Postinflammatory hypopigmentation is a cutaneous condition characterized by decreased pigment in the skin following inflammation of the skin.

References

  1. 1 2 3 4 Bowers, Sacharitha; Warshaw, Erin M. (2006). "Pityriasis lichenoides and its subtypes". Journal of the American Academy of Dermatology. 55 (4): 557–572. doi:10.1016/j.jaad.2005.07.058. PMID   17010734.
  2. 1 2 3 4 Khachemoune, Amor; Blyumin, Marianna L (2007). "Pityriasis Lichenoides: Pathophysiology, Classification, and Treatment". American Journal of Clinical Dermatology. 8 (1): 29–36. doi:10.2165/00128071-200708010-00004. ISSN   1175-0561. PMID   17298104. S2CID   261387177.

Further reading