Maculopapular cutaneous mastocytosis Q82.2

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

All authors of this article

Last updated on: 21.11.2021

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Synonym(s)

Brown man; Cutaneous mastocytosis; Generalized cutaneous mastocytosis; Mast cell reticulosis; Mastocytosis Syndrome; Nettleship's disease; Nettleship Syndrome; Rywlin disease; urticaria pigmentosa

History
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Nettleship, 1869; Sangster, 1878

Definition
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Maculopapular cutaneous mastocytosis (Urticaria pigmentosa of the old nomenclature) is by far the most common type of cutaneous mastocytosis. The early childhood forms of cutaneous mastocytosis often correspond to the clinical picture of solitary or multinodular mastocytoma.

The purely descriptive clinical picture of maculopapular mastocytosis is based on a clonal disease of the CD34+ hematopoietic stem cell in the bone marrow with the development of clinically distinct clinical pictures, which are characterized by mast cell proliferations in the skin (and possibly also in internal organs - especially bone marrow, lymphatic organs).

In cutaneous m astocytosis of the maculopapular cutaneous mastocytosis type, the proliferation of mast cells is restricted exclusively to the skin.

Systemic mastocytosis may occur with or without skin lesions (the skin lesions correspond to the clinical picture of maculopapular cut aneous mastocytosis or diffuse cutaneous mastocytosis).

Systemic mastocytosis often begins in adulthood. However, it may begin in childhood as monoorganic cutaneous maculopapular mastocytosis and then progress to extracutaneous manifestations in adulthood as "indolent systemic mastocytosis".

Clinical symptoms are heterogeneous depending on the type of manifestation(indolent systemic mastocytosis/advancedsystemic mastocytosis) and depend on the mast cell load. Anaphylactic reactions and other reactions triggered by mast cell mediators such as flushing symptoms, pruritus, urticaria, asthma attacks, abdominal complaints such as diarrhoea or gastrointestinal ulcers as well as joint and bone pain characterise the clinical picture.

Classification
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Maculopapular cutaneous mastocytosis = disseminated mastocytosis; 5 mastocytomas = maculopapular cutaneous mastocytosis

Occurrence/Epidemiology
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No gender preference. In childhood the incidence of cutaneous mastocytosis is 1:150,000 per year

Etiopathogenesis
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Most adult patients with this skin manifestation have an activating point mutation of the KIT gene (KIT D816V). Both the expression of KIT (CD117) on the cell surface and the mutation are not specific for mastocytosis - see below Mastocytosis (overview).

In childhood maculopapular cutaneous mastocytosis, the activating c-Kit mutation is not detectable.

Manifestation
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In childhood maculopapular cutaneous mastocytosis, the initial manifestation often occurs in the first 24 months of life, less commonly thereafter.

In the adult form (maculopapular cutaneous mastocytosis of adulthood), the peak of manifestation is found in middle adulthood (40-60 years).

Localization
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Ubiquitous; mainly on the trunk and upper and lower extremities; rarely on the face, palms of the hands and soles of the feet. Very rarely mucosal involvement.

Clinical features
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Flat, oval or round, grey-brownish or red-brownish, 0.1-0.5 cm large spots. The clinical symptomatology of the patients is due to release of the various mast cell mediators (mediator symptomatology). Besides histamine, tryptase, heparin, leukotrienes, prostaglandins as well as various cytokines like TNF-alpha, interleukins. After the lesions have been firmly coated, an urticarial reaction occurs in the foci(Dariers sign); more rarely, subepidermal blistering also occurs. Frequently, an elevated dermographism can also be triggered. Initially weak, later stronger lesional pigmentation. The most frequent accompanying symptom is pruritus, whereby interleukin-31 is attributed a special pathogenetic significance (Wagner N et al. 2018).

Regarding the clinical course, juvenile and adult formers behave differently.

Special forms (old nomenclature):

Laboratory
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In case of systemic mastocytosis, tryptase is determined according to the guidelines (standard value: <20µg/l). If the value is exceeded, systemic mastocytosis is considered possible and a bone marrow biopsy and screening for further system involvement is sought. If the values are low, extensive diagnostic procedures are usually not necessary without compelling clinical indications. In a larger study, systemic mastocytosis was confirmed in 32% of patients with cutaneous mastocytosis in bone marrow histology. The mean tryptase value of the collective with system involvement was 43.9±39.93µg/l (3.74-173µg/l), without system involvement 19.63±13.31 µg/l (2.44-54 µg/l).tryptase increases > 20µg/l were detectable in 43% of patients with pure cutaneous mastocytosis. 28% of patients with systemic mastocytosis showed normal values. Thus the laboratory value "tryptase" does not seem to be a reliable parameter for the question of systemic involvement.

In addition, the determination of N-methylhistamine or 1,4-methylimidazole acetic acid in the collective urine can be performed.

Histology
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Often not very spectacular histological image in HE-stained specimen. Discrete hyperpigmentation of the otherwise unchanged epidermis. Shattered, perivascularly accentuated round cell infiltrates in the reticular dermis. The mast cell-rich quality of the infiltrate (Giemsa staining; CD117; CD25) is only recognizable with histochemical or immunohistochemical imaging.

Differential diagnosis
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malignant cutaneous lymphoma;

urticaria

Drug exanthema

Therapy
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General therapy
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Patient education about the nature of the disease and provoking factors. Avoidance of triggering drugs such as non-steroidal anti-inflammatory drugs, acetylsalicylic acid, codeine, procaine, polymyxin B, muscle relaxants, X-ray contrast media. No mechanical irritation such as friction (dry rub) or sudden temperature changes (jumping into cold water). Avoid insect bites. Caution. Triggering by i.v. applied, short-acting narcotics is possible!

Diet: A low-histamine diet, possibly also a low-salicylate diet, is recommended, see below. Urticaria, chronic. Histamine relievers should be avoided.

External therapy
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Cooling lotions with addition of polidocanol 5% R200. Alternatively gels containing antihistamines (e.g. Fenistil Gel, Tavegil Gel, Soventol Gel). Creams containing glucocorticoids like 0.5% hydrocortisone cream R120 help in the short term but are not a permanent solution. They should therefore not be used in therapy.

Radiation therapy
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Therapy with UVA1 irradiation in medium to high doses or PUVA therapy leads to an improvement of clinical symptoms in some patients.

Internal therapy
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Antihistamines: Combinations of a non-sedating H 1 -antagonist such as levocetirizine (Xusal) 1 time/day 5 mg p.o. or a sedating H 1 -antagonist such as dimetindene (Fenistil) 3 times/day 1-2 mg p.o. with an H 2 -antagonist such as cimetidine (e.g. Tagamet) 400-800 mg/day p.o. are used. S.a.u. Mastocytosis, systemic.

Mast cell stabilizers (e.g. ketotifen (e.g. Zaditen Kps./syrup): Adults: 2 times/day 1-2 mg p.o., children over 3 years: 2 times/day 1 Kps., children from 6 months to 3 years: 2 times/day 2.5 ml syrup.

In individual cases, improvement is achieved with disodium cromoglicic acid (e.g. Colimune) 4 times/day 100-200 mg.

Glucocorticoids: In severe forms, including bullous forms, glucocorticoids in medium doses such as prednisone (e.g. Decortin) 40-60 mg/day may be considered, with gradual dose reduction to the maintenance dose according to the clinic.

Experimental approaches with positive results exist for omalizumab (Xolair), an IgE antibody.

Progression/forecast
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Favorable. > 95% of patients have a normal life expectancy.

In>50% of juvenile cutaneous mastocytoses, spontaneous remission occurs by adolescence. Systemic involvement can be detected in 10% of children. Especially in children with a later first manifestation (>years). Clinical manifestations of systemic involvement include diarrhea, flushing, headache and bone pain.

In adult cuntaneous mastocytosis, the disease is usually chronic and insidiously progressive. Only a small proportion show remissions. Systemic involvement is observed in about half of the patients. Systemic involvement (mast cell infiltration in bone marrow, liver, spleen and/or lymph nodes) is observed in a variety of organ diseases such as gastritis, ventricular or duodenal ulcer, flush, malabsorption syndrome.

Tables
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Antihistamines for Urticaria pigmentosa

Active substance

Example preparation

Age

Dosage/day

Application

Non-sedating

Cetirizine

Zyrtec

2-12 J.

½-1 Mßl.

Syrup

> 12 J.

10 mg

fimtbl.

Levocetirizine

Xusal

> 6 J.

5 mg

Filmtbl.

Loratadine

Lisino

2-12 J.

½-1 Mßl.

Syrup

> 12 J.

10 mg

Tbl.

Desloratadine

Aerius

2-5 J.

2.5 ml (1.25 mg)

Syrup

6-12 J.

5 ml (2.5 mg)

Syrup

> 12 J.

10 mg

Filmtbl.

Doxylamine Succinate

Mereprine

½-5 J.

1-2 times 1 teaspoon.

Syrup

6-12 J.

2-3 times 1 tsp.

Syrup

> 12 J.

2-4 times 2 teas.

Syrup

Sedating

Clemastine

Tavegil

1-6 J.

2 times 1-2 teas.

Syrup

6-12 J.

2 times 1 tbs.

Syrup

> 12 J.

2 times 1 mg

Tbl.

Dimetinden

Fenistil

1-8 J.

3 times 1 teaspoon.

Syrup

Literature
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  1. Arber DA et al (2016) The 2016 revision to the World Health Organization classification of myeloidneoplasms
    and acute leukemia. Blood 127:2391-405.
  2. Czarnetzki BM et al (1985) Phototherapy of urticaria pigmentosa; clinical response and changes of cutaneous reactivity, histamies and chemotactic leukotrienes. Arch Dermatol Res 277: 105-113
  3. Comte C et al (2003) Urticaria pigmentosa localized on radiation field. Eur J Dermatol 13: 408-409
  4. Gobello T et al (2003) Medium versus high-dose ultraviolet A1 therapy for urticaria pigmentosa: a pilot study. J Am Acad Dermatol 49: 679-684
  5. Guler E et al (2001) Urticaria pigmentosa associated with Wilms tumor. Pediatric Dermatol 18: 313-315
  6. Ludolph-Hauser D et al (2001) Occult cutaneous mastocytosis. dermatologist 52: 390-393
  7. Nettleship E (1869) Rare forms of urticaria. Br Med J 2: 323
  8. Nettleship E (1869) Chronic urticaria leaving brown stains: nearly two years' duration. BMJ 2: 435
  9. Requena L (1992) Erythrodermic mastocytosis. Cutis 49: 189-192
  10. Sangster A (1878) Urticaria Pigmentosa. Lancet I: 683

Disclaimer

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

Authors

Last updated on: 21.11.2021