Diffuse cutaneous mastocytosis Q82.2

Author: Prof. Dr. med. Peter Altmeyer

Last updated on: 07.03.2023

Synonym(s)

Diffuse cutaneous mastocytosis; Diffuse mastocytosis of the skin; Diffuse skin mastocytosis

Definition
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Very rare cutaneous mastocytosis with extensive, massive skin involvement and dense mast cell infiltration of the entire corium. Frequently, clinical symptoms of histamine liveration are detected (up to shock symptomatology).

A special form is erythrodermic mastocytosis (type II) with progressive course and frequent development of mast cell leukemia.

Manifestation
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Especially occurring in infants (also congenital) and small children.

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Clinical features
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The clinical description of this form is not clear. The following clinical types can be subsumed under it:

Disseminated, large, yellowish, flat, lichenified spots and plaques. Fuzzy hyperpigmentation. Severe itching, urticarial dermographism, large blistered eruptions after scratching and rubbing.
The diffuse form, often without any pigmentation of the skin, presents clinically, peculiarly diffuse doughy leathery consistency (generalized peau d'orange), usually with a strong tendency to (artificial) blistering (see figure). This variant can be considered a clinical entity.
Erythrodermic mastocytosis with frequent systemic involvement and signs of histamine liberation such as: flush, diarrhea, spastic bronchitis, gastric ulcer, drop in blood pressure, intense itching.

Histology
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S.u. Urticaria pigmentosa.

General therapy
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No causal therapy possible. It is important to educate the patient: avoid any mechanical, thermal or chemical irritation of the skin that could lead to the release of mediators (see Table 1). Low histamine diet (avoid fish, cheese, perishable food, red wine, caffeine, etc.). Natural sunlight can initially provoke degranulation, but in some cases leads to a long-term improvement.

External therapy
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Depending on the degree of clinical manifestation, an initial therapy trial with local antihistamines (e.g. Tavegil Gel) or a short-term therapy trial with weakly to moderately effective glucocorticoids such as 1% hydrocortisone lotio R123 or triamcinolone cream R259 can be carried out.

Internal therapy
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Antihistamines, possibly as a combination of H₁ antagonists e.g. levocetirizine (1 x Xusal tablet 1 time/day) and H₂ antagonists e.g. cimetidine (Tagamet 200-800 mg/day), have a positive effect on itching and flushing symptoms; the choice of mildly sedating antihistamines such as clemastine (e.g. Tavegil) may be useful in some cases, especially in children. If not successful, try therapy with ketotifen (e.g. Zaditen 2 times/day 1 mg). Disodium cromoglicic acid (e.g. Colimune 4 times/day 200 mg p.o.) especially in cases of gastrointestinal symptoms.

Glucocorticoids are not a permanent solution because of their side effects and are reserved for severe cases (e.g. bullous forms in children). If necessary, prednisolone (e.g. Decortin H) in medium dosage for 2-3 weeks, then reduce to the lowest possible maintenance dose. See also Mastocytosis, systemic.

Progression/forecast
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Cheap. Spontaneous regression within the first 5-10 years of life.

Tables
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Non-specific mast cell granulators

Trigger	Examples
Physical stimuli	heat, cold, mechanical stimuli
Food	alcohol, polypeptides in jellyfish, lobster, shrimps, various fish, fermented vegetables (sauerkraut), spinach, tomatoes, green beans, strawberries, bananas, oranges, nuts
Drugs	Acetylsalicylic acid, morphine, codeine, dextranes, chymotrypsin, polymyxin B, X-ray contrast media, non-steroidal anti-inflammatory drugs, muscle relaxants (curare)
Bacterial toxins
insect venoms, snake venoms
Phopholipase A and C
Complement Anaphylatoxins	_C3a, C5a

Literature
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Di Raimondo C et al (2021) Cutaneous mastocytosis: A dermatological perspective. Australas J Dermatol 62:e1-e7.
Golitz LE et al (1984) Bullous mastocytosis: diffuse cutaneous mastocytosis with extensive blisters mimicking scalded skin syndrome or erythema multiforme. Pediatr Dermatol 1:288-294
Haustein UF, Bedri M (1997) Bullous mastocytosis in a child. Dermatologist 48: 127-129
Heide R et al (2001) Clinical scoring of cutaneous mastocytosis. Acta Derm Venereol 81: 273-276
Lange M et al (2012) Diffuse cutaneous mastocytosis: analysis of 10 cases and a brief review of the literature. J Eur Acad Dermatol Venereol 26: 1565-1571.
Ludolph-Hauser D et al (2001) Occult cutaneous mastocytosis. Dermatologist 52: 390-393
Middelkamp Hup MA et al (2002) Comparison of mastocytosis with onset in children and adults. J Eur Acad Dermatol Venereol 16: 115-120.
Rajesh J et al (2002) Diffuse cutaneous mastocytosis: pseudoxanthomatous variant. J Dermatol 29: 354-356
Wagner N et al (2018) Mastocytosis - pathogenesis, clinical manifestation and treatment.J Dtsch Dermatol Ges 16:42-57.