Granuloma multiforme
Other namesMkar disease and Granuloma multiforme (Leiker)[1]
SpecialtyDermatology Edit this on Wikidata

Granuloma multiforme is a cutaneous condition most commonly seen in central Africa, and rarely elsewhere, characterized by skin lesions that are on the upper trunk and arms in sun-exposed areas.[2]:707 It may be confused with tuberculoid leprosy, with which it has clinical similarities. The condition was first noted by Gosset in the 1940s, but it was not until 1964 that Leiker coined the term to describe "a disease resembling leprosy" in his study in Nigeria.[3]

Signs and symptoms

The upper trunk and arms are the main areas affected. The clinical picture differs greatly. Typically, the first lesions are papules, which quickly develop into polycyclic and annular lesions with papular or nodular margins. There might also be plaques that are elevated. Lesions typically leave behind residual hypopigmented macules and extend with central healing over months or years. It irritates and is itchy, particularly when new lesions are developing. The lesions differ from leprosy in that they do not exhibit nerve enlargement, local sensation impairment, or impaired sweating. Also, the lesions never become ulcerated. The illness is a cause of cosmetic disfigurement, but it does not affect the patient's overall health.[4]

Causes

Granuloma multiforme is primarily thought to be caused by cumulative photodamage to the dermal collagen, with the lesions in this condition almost exclusively occurring in sun-exposed areas.[3]

Diagnosis

The best way to understand the histologic features is to look at a radial biopsy with a raised border and central zone. The absence of elastic fibers, the disease's hallmark, is best shown in the central zone using an elastic tissue stain like Verhoeff-Van Gieson's. A zone of transition with granulomatous infiltration is visible on the elevated border. Both a perivascular lymphocytic infiltrate and elastic tissue phagocytosis are seen within the giant cells.[5]

Other conditions that should be taken into account in the differential diagnosis of granuloma multiforme include sarcoidosis, necrobiosis lipoidica diabeticorum, granuloma annulare, and the typical mimic of tuberculoid leprosy,[3] which has sensory impairment and thickening of the nerve trunk but no degenerated collagen.[6] In younger age groups, granuloma annulare is characterized by asymptomatic lesions that histopathologically show increased mucin deposition encircled by a granulomatous zone with a scarcity of giant cells with fewer nuclei.[3] Necrobiosis lipoidica is characterized by a yellow-colored appearance of the plaques, telangiectasia, involvement of the lower limbs, as well as the presence of fibrosis mixed with necrosis, vessel wall changes, and fat deposition in the deep reticular dermis.[6]

Treatment

Although topical steroids have been tried, there is currently no effective treatment for granuloma multiforme.[5]

See also

References

  1. Meyers, Wayne; Connor, DH; Shannon, R (January 1970). "Histologic characteristics of granuloma multiforme (Mkar disease). Including a comparison with leprosy and granuloma annulare. Report of first case from Congo (Kinshasa)". International Journal of Leprosy and Other Mycobacterial Diseases. 38 (3): 241–249. PMID 5533688.
  2. James, William D.; Berger, Timothy G.; et al. (2006). Andrews' Diseases of the Skin: clinical Dermatology. Saunders Elsevier. ISBN 978-0-7216-2921-6.
  3. 1 2 3 4 Kumari, Rashmi; Devinder Mohan, Thappa; Chougule, Abhijit; Adityan, Balaji (2009). "Granuloma multiforme: A report from India". Indian Journal of Dermatology, Venereology and Leprology. 75 (3): 296–299. doi:10.4103/0378-6323.51259. hdl:1807/48406. PMID 19439886.
  4. Sandhu, Kamaldeep; Saraswat, Abir; Gupta, Somesh; Shukla, Rajeev; Handa, Sanjeev (2004). "Granuloma multiforme". International Journal of Dermatology. 43 (6): 441–443. doi:10.1111/j.1365-4632.2004.01979.x. ISSN 0011-9059. PMID 15186227.
  5. 1 2 Bhatia, Shibani; Shenoi, Shrutakirthi D; Pai, Kanthilatha; Ps, Srilatha (2019). "Granuloma multiforme: an uncommon differential for leprosy". Tropical Doctor. 49 (1): 55–58. doi:10.1177/0049475518803191. ISSN 0049-4755. PMID 30286700. S2CID 52920727.
  6. 1 2 Cherian, S (December 1990). "Granuloma multiforme in India". International Journal of Leprosy and Other Mycobacterial Diseases. 58 (4): 719–721. PMID 2280121.

Further reading

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