Dermatitis exudative discoid lichenoid L98.8

Author: Prof. Dr. med. Peter Altmeyer

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Last updated on: 18.04.2024

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Synonym(s)

Dermatosis exudative discoid lichenoid; Dermatosis exudative discoid lichenoid chronic; Exudative discoid and lichenoid chronic dermatosis; Exudative discoid lichenoid chronic dermatosis; Exudative discoid lichenoid dermatitis; Oid-Oid-Disease; Sulzberger-Garbe disease; Sulzberger-Garbe Syndrome

History
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Sulzberger u. Garbe, 1937; exudative discoid lichenoid dermatitis was first described in 1937 based on observations of a group of 9 patients treated at the Skin and Cancer Clinic in New York. The name of the disease is derived from the names of two physicians: Marion B. Sulzberger and William Garbe. M.B. Sulzberger observed the first cases of the disease in 1927, when he was practicing in Zurich in a department headed by Bruno Bloch, and then in Warsaw in 1929 - in a department then headed by Joseph Jadasson . Observations of other patients with similar skin diseases were made together with W. Garbe in New York.

Definition
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Apparently very rare, highly controversial inflammatory, eczematous skin disease of unknown etiology and pathogenesis, characterized by the coexistence of eczematous, lichenoid and urticarial foci with marked pruritus. In terms of publications, < 100 casuistic articles are known, the most recent from 2013 (Tabara K et al. 2013) .

Although the dermatosis was first described 75 years ago, its existence as a distinct nosological entity is still controversial. It is evident that most cases were observed in the mid-20th century, mainly in North America. Later reports and cases from other regions are rare and have often been controversial. More extensive studies in a large patient population have not been performed. Jansen et al, in their report of the case of a 7-year-old girl, concluded that there were no clinical or histologic features to distinguish Sulzberger's scar dermatosis from nummular dermatitis. Rangioletti et al. considered this diagnosis as a transitional diagnosis in the course of distinct chronic exudative-discoid or lichenoid dermatoses.

Manifestation
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Mainly middle-aged men. Children or women are affected less frequently.

Localization
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Chest, trunk, face; typical is the affection of penis and scrotum.

Clinical features
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Intolerably severe pruritus. Rapid succession or simultaneous appearance of exudative, reddened, infiltrated, nummular plaques, reminiscent of nummular dermatitis, lichenified papules, or small urticarial papules. The skin lesions are extremely resistant to therapy.

Laboratory
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Frequent eosinophilia.

Histology
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Perivascular infiltrates from lymphocytes, granulocytes, eosinophils, plasma cells. Swelling of the vascular endothelia (unspecific image).

Differential diagnosis
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External therapy
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Medium strength glucocorticoid creams such as 0.1% betamethasone lotio R030, 0.1% triamcinolone cream R259, 0.25% prednicarbate cream (e.g. Dermatop), 0.1% methylprednisolone cream (e.g. Advantan). Caring greasing external products (e.g. Ungt. emulsif. aq., Linola Fett, Eucerin cum aq.) as well as oil baths such as Balneum Hermal plus, oil bath Cordes, Linola Fett oil bath, Eucerin Omega fatty acids oil bath, Polidocanol shower oil.

Internal therapy
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Therapy of the 1st choice are glucocorticoids, perorally, in medium dosage, initial 60-80 mg/day prednisolone equivalent, slow release to low maintenance dose over several weeks. After discontinuation the skin symptoms flare up again. The glucocorticoids can only be completely eliminated after months or years.

Cases with healing under exclusive azathioprine therapy (e.g. Imurek) are described. An attempt at therapy with antihistamines such as desloratadine (e.g. Aerius) 1-2 tbl/day or levocetirizine (e.g. Xusal) 1-2 tbl/day is possible.

Progression/forecast
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Spontaneous healing after months to years of progress.

Note(s)
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The technical term "-oid" is mainly used to describe a body, a form, a structure or the like that is comparable to something - is similar to something. In this disease, 2 types of efflorescence (e.g. lichenoid and exudative nummular plaques) should always be found that are morphologically similar.

Literature
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  1. Jansen T et al (1992) Sulzberger's scar exudative discoid and lichenoid chronic dermatosis ("Oid-Oid disease")--reality or fiction? Dermatologist 43: 426-431
  2. Rongioletti F et al (1989) Exudative discoid and lichenoid chronic dermatosis (Sulzberger's scar) a fictional disease? Int J Dermatol 28:40-43.
  3. Savill T (1891) On an epidemic skin disease. Br Med J (London) 2: 1197-1202.
  4. Stevens DM et al (1984) On the concept of distinctive exudative discoid and lichenoid chronic dermatosis (Sulzberger's scar). Am J Dermatopathol 6: 387-395
  5. Sulzberger MB, Garbe W (1937) Nine cases of a distinctive exudative discoid and lichenoid chronic dermatosis. Arch Dermatol (Chicago) 36: 247-278.
  6. Tabara K et al (2013) A 6-year-old boy with Sulzberger and Garbe dermatosis: a case report and literature review. Postepy Dermatol Alergol 30:403-8.

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Last updated on: 18.04.2024