Prurigo simplex subacuta L28.2

Authors: Prof. Dr. med. Peter Altmeyer, Pia Nagel

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Last updated on: 05.07.2021

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Synonym(s)

acne frontalis; acne necrotica; acne urticata; acne varioliformis; Chronic Prurigo; lichen urticatus; Lichen Vidal urticatus; Necrotizing lymphocytic folliculitis; Prurigo Chronicle; Prurigo simplex acuta et subacuta adultorum; Prurigo simplex chronica; Strophulus adultorum; subacute prurigo; Urticaria papulosa chronica; Urticaria perstans

History
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Hebra 1874

Definition
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Eminently chronic, polyetiological, endogenously triggered, papular, inflammatory disease of the skin, often accompanied by unbearable itching, with a typical, disseminated, symmetrical distribution pattern.

Chronic prurigo is accompanied by a characteristic, circumscribed response (spooning, boring, squeezing) to what is perceived as a "punctiform" itching (also known as prickly or painful "itching") and by the characteristic papules, plaques or nodules which are centrally ulcerated and encrusted, induced by the constantly repeated scratching process. In general, this disease is associated with a considerable impairment of the quality of life.

Etiopathogenesis
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Cause unknown. Numerous triggering factors are discussed:

Hormonal disorders, gastrointestinal disorders, liver dysfunction, gynaecological disorders, focal infections, neurological, psychiatric-psychosomatic disorders.

As the disease is often refractory to antihistamines, non-histaminergic pathomechanisms are often important.

Discussions also cover allergic diathesis and connections with atopic eczema.

Furthermore, nerve root irritations due to degenerative changes in the distribution pattern of the vertebrae in question need to be clarified.

Morphological studies could show dermal hyperplasia caused by the release of neuropeptides such as nerve growth factor, substance P and calcitonin-gene related peptides.

S.a. Prurigo diabetica, Prurigo gestationis, Prurigo hepatica, Prurigo lymphatica, Prurigo chronica multiformis.

Furthermore the following names were used in the past:

  • purple uraemica
  • Prurigo lymphatica
  • Prurigo lymphogranulomatotica(Hodgkin lymphoma).

Manifestation
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Mainly occurring in women between the ages of 20 and 30, in men mostly after the age of 60. No seasonal or regional preference.

Localization
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Usually the upper arm extensor sides, upper back, buttocks, outer side of the thighs, chest region are affected, more rarely the face. Rarely generalized. Usually free are those parts of the skin that are difficult or impossible to reach with the hands (e.g. skin between the shoulder blades). The mucous membranes, palms of the hands and soles of the feet are always free.

Clinical features
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Mostly colorful clinical picture with sharply defined, single, never confluent efflorescences of different "acuity", from freshly scratched to scarred healed.

  • Initial efflorescences (usually not detectable, possibly also non-existent!): "Urticarial", eminently itchy, 0.2-0.5 cm large, red, firm, papules, which do not fall below a certain distance from each other. In most cases, however, there is no "precursor efflorescence" but only the indication of a prickly, tortuous, punctiform itching in normal appearing skin.
  • Residual efflorescence (typical clinical findings): 0.3-0.5 cm large, centrally scratched (spooned out) papules covered with a crust or fresh granulation tissue, flatly elevated, red or reddish brown papules. In addition, depending on the duration of the symptoms, white or still reddened scars of varying age with the exact size of the pre-existing prurigopapules.
  • Typical is a punctual, unpleasantly pungent itching, which is answered with an equally targeted and typical scratching reaction (spooning), followed by sudden cessation of the itching. It is not uncommon for hard-to-break scratching automatisms to develop.
  • Notice! It is not the "disturbing efflorescences" but the localized, punctiform, tormenting itching that leads to the doctor!

Histology
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Pronounced irregular hyperplasia of the epidermis with elongation of the rectal ridges. Flat central ulcerations with fresh granulation tissue and raised epidermal margins are frequently seen. In the papillary epidermis, there is a mixed (non-specific) inflammatory infiltrate possibly with neutrophils and also eosinophils with diffuse superficial fibrosis with fibroblast proliferation. Occasionally small neuromas are found (Pautrier's neuroma).

Differential diagnosis
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  • Prurigo form of atopic eczema: Clear evidence of existing atopy (IgE, RAST). Other clinical signs of atopic eczema.
  • Dermatitis herpetiformis: Symmetrical, disseminated, very itchy to burning, 0.1-0.2 cm large, urticarial erythema, rarely vesicles. Laboratory: In some cases blood eosinophilia. Antibody detection: Anti-Gliadin-AK, Anti-Endomysium-AK, AK against tissue transglutaminase, AK against epidermal transglutaminase (most sensitive serological test for diagnosis). Immunohistology is conclusive.
  • Prurigo nodularis: Eminently chronic disease characterized by numerous, large, severely itching, large (0.6-2.0 cm in size) reddish-brown or brown nodules. Nodules are significantly larger than in Purigo simplex subacuta! The common feature is the highly chronic course and quality of the itching.
  • Polymorphic light dermatosis: Mostly urticarial non-scratched papules. Infestation of the areas exposed to light.
  • Purigoform of bullous pemphigoid: Serological detection of pemphigoid-AK. Immunohistology with detection of basement membrane AK.
  • Lymphomatoid papulose: Histology (immunohistology CD30 positive lymphocytes) is diagnostic. Disseminated occurrence rather rare.
  • Reactive perforating collagenosis: typical efflorescences with central cap-like crusts, usually no itching

General therapy
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In case of pronounced scratching automatisms, cooperation with a psychotherapist or a psychiatrist. Differentiation from the dermatocoen delusion (question of skin parasites) is necessary.

A subtle examination with clarification of an underlying internal disease is important.

Notice! Always exclude internal diseases with Prurigo simplex subacuta!

External therapy
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UVB; balneo-phototherapy, saline baths or oil baths with added polidocanol, e.g. Balneum Hermal plus; PUVA therapy systemic or balneo-photochemotherapy ( PUVA bath therapy) are indicated.

Nursing measures with hydrophilic creams or lotions, possibly with a 1-3% polidocanol addition (e.g. Optiderm lotion/cream) are necessary accompanying measures, in case of less itching the application of a 1% polidocanol shaking mixture R200 is sufficient.

In the case of persistent itching, foil dressings containing glucocorticoids can be applied temporarily (1-2 hrs/day; use of a household cling film fixed with an adhesive strip), e.g. with 0.1% triamcinolone acetonide cream (Triamgalen, Volon A) or 0.05-0.1% betamethasone (Betagalen, Betnesol, R030, R029 ).

Stubbornly therapy-resistant prurigo papules should be consistently and repeatedly (intervals of 3-4 weeks) injected with a glucocorticoid-containing crystal suspension (10 mg triamcinolone acetonide, e.g. Volon A, diluted 1:1-1:3 with 1% scandicain, applied intrafocally with a thin needle, not sublesionally!)

Capsaicin: local therapy with a 0.025/0.05/0.1% "hydrophilic capsaicin cream" (apply thinly 4-6 times daily)

Internal therapy
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Short-term glucocorticoids such as prednisone (e.g. Decortin) initial 40-60 mg, gradual reduction within 14 days. This therapeutic approach is generally only morbostatic and not permanently successful.

Alternatively: Oral, low or non-sedating antihistamines such as desloratadine (e.g. Aerius) 1-2 tbl/day or levocetirizine (e.g. Xusal) 1-2 tbl/day. In cases of considerable itching, also sedative antihistamines such as dimetinden (e.g. Fenistil) 3 times/day 1 mg or anxiolytic antihistamines such as Hydroxyzin 25-75 mg/day (e.g. Atarax).

Alternative: Gabapentin 900mg/day

Alternative: Pregabalin 72-225mg/day.

Alternative: Successes have been described with the psychopharmacological drug olanzapine (initial 5 mg; as long-term therapy 10 mg/day p.o.).

Experimental: Neurokinin-1 receptor antagonists(aprepitant): the results of a randomized study 80mg aprepitant/day over 4 weeks) are still pending.

Experimental: Anti-IL-31 receptor A antibody (nemolizumab): the results remain to be seen.

Not effective: Chloroquine: The use of chloroquine (e.g. Resochin) has not proved effective

Remember! Due to the chronicity of the clinical picture, intensive and close patient guidance is necessary!

Progression/forecast
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Chronic, often years-long course.

Naturopathy
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Good results are achieved with dermatological climatic therapy (especially stimulating climate at the North Sea and in the high mountains).

Cooling rubbing with vinegar water or a 2% menthol spirit.

Supplementary: Menthol: Local therapy with a 1-5% menthol cream

Note(s)
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The subdivision into different Subtypes of the Prurigo simplex subacuta do not follow a clearly recognizable etiopathogenetic principle. Therefore, it rather leads to confusion (Ständer S 2018) and is reproduced here only for historical reasons.

The "Acne urticata" described by M. Kaposi in 1893 is a variant of the prurigo simplex subacuta, which is restricted exclusively to the face and is mainly used in young (mentally unstable) women.

Literature
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  1. Akar HH et al (2014) Prurigo simplex subacuta or prurigo simplex acuta? Eur Ann Allergy Clin Immunol 46:152-153
  2. Balakirski G et al (2014) Bullous pemphigoid: a new look at a well-known disease. dermatologist 65:1013-1016
  3. Bergner T et al (1990) Prurigo simplex subacuta. Act Dermatol 16: 221-225
  4. Boyd K et al (2014) The role of capsaicin in dermatology.
    Prog Drug Res. 2014;68:293-306.
  5. Dauden E, Garcia-Diez A (2003) Severe resistant subacute prurigo successfully controlled by long-term cyclosporin. J Dermatolog Treat 14: 48-50
  6. Friday M, by Kobyletzki G, Pieck C, Altmeyer P (1999) Balneologic photochemotherapy of prurigo simplex subacuta. dermatologist 50: 344-349
  7. Huyn J et al (2006) Olanzapine therapy for subacute prurigo. Clin Exp Dermatol 31: 1-2
  8. Pereira MP et al (2018) Chronic prurigo. Dermatologist 69:321-328
  9. Schmidt E et al (2002) Subacute prurigo variant of bullous pemphigoid: autoantibodies show the same specificity compared with classic bullous pemphigoid. J Am Acad Dermatol 47: 133-136
  10. Stand S (2018) Pruritus, Prurigo. In: Braun-Falco`s Dermatology, Venerology Allergology G. Plewig et al (Hrsg) Springer Verlag S 591-592

Disclaimer

Please ask your physician for a reliable diagnosis. This website is only meant as a reference.

Authors

Last updated on: 05.07.2021