2011.3-9.Granulosis

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Our Dermatol Online. 2011; 2(3): 144-146
Date of submission: 21.02.2011 / acceptance: 21.03.2011
Conflicts of interest: None
 

GRANULOSIS RUBRA NASI – A CASE REPORT. A LITERATURE REVIEW

GRANULOSIS RUBRA NASI – OPIS PRZYPADKU. PRZEGLĄD LITERATURY

Brzeziński Piotr1, Poklękowska Katarzyna2

16th Military Support Unit, Ustka, Poland,
2Mazowiecki Branch of the National Health Fund, Warsaw, Poland
 

Corresponding author: Dr. Piotr Brzeziński    e-mail: brzezoo77@yahoo.com


 

Abstract
Granulosis Rubra Nasi (GRN), a rare childhood dermatitis that was also defined as "Acne papulo-rosacea of the nose". Is an inflammatory dermatosis which consists on erythema, papules and itch. The hyperhidrosis the most conspicuous features of the disease and small beads of sweat and erythema on the tip of the nose may often occur. Sometimes small red papules and comedo-like lesions may be present. Etiology is unknown. lt's uncommon, chronic process and benign course. Usually subsides spontaneously at adolescence. The treatment is symptomatic and cosmetic. An 14-year-old boy presented with erythematous lesions over the nose of childhood duration without sensitivity to sunlight. Physical examination showed also excessive sweating of the nose, and erythema of the nose covered by small pustules, papules ang macules. The patient responded well to low doses of oral Isotretinoine. Should remember GRN could be a complication of hyperhidrosis.
 
Streszczenie
Granulosis Rubra Nasi (GRN), rzadkie zapalenie skóry w dzieciństwie, które zostało równie? określony jako "trądzik grudkoworó?owaty nosa". Jest to zapalna choroba skóry, która polega występowaniu rumienia, grudek i świądu. Nadmierne pocenie się jest najbardziej wyrazistą cechą choroby, a małe krople potu i rumień na czubku nosa często mogą wystąpić. Czasami mogą być obecna małe czerwone grudki i zmiany zaskórnikopodobne. Etiologia jest nieznana. Często ma przewlekły i łagodny przebieg. Zwykle ustępuje samoistnie w wieku dojrzewania. Stosuje się leczenie objawowe i kosmetyczne. 14-letni chłopiec zgłosił się z rumieniowymi zmianami na nosie trwającymi od dzieciństwa, bez wra?liwość na światło słoneczne. Badanie lekarskie wykazało równie? nadmierne pocenie się na nosie, rumień w nosie w obrębie drobnych krostek, grudek i plamek. Pacjent dobrze zareagował na małe dawki doustnej izotretinoiny. Nale?y pamiętać, ?e GRN mo?e być powikłaniem nadmiernej potliwości.
 
Key words:  granulosis rubra nasi; dermatosis; hyperhydrosis; izotretinoin
Słowa klucze:  ziarnistość czerwona nosa; choroby skóry; nadpotliwość; izotretinoina

 

Introduction
The first case Granulosis Rubra Nasi (GRN) was described in 1901 by German dermatologist Josef Jadasson as "Nasi hyperhidrotic Erythematosa micropapules Dermatosis Infantum” [1]. Some footnotes Italian treat of the first case described by Luthen in 1900 [2]. The present accepted name we owe to Jadassohn. The malady is usually limited to the nose, to the front and sides, in addition to involving this part, it has been observed to affect also the upper lip, cheek, and eyebrow.
 
Case Report
14 years old boy was adopted in the Dermatology surgery of changes in the nasal skin under way since childhood, with periodic remissions and exacerbations. So far, no treatment (Fig. 1). Nose leather on the front and sides were inflamed. Redness gradually disappeared to the sides. On the skin of nose could be seen a small red specs and papules. The papules gradually develop into pustules lesions. On the sides can often be seen drops of sweat, which gave the appearance of moist, slightly shiny lesions. Lesions without sensitivity to sunlight. In addition, generally healthy. The patient was treated initially with liquid zinc, has no effect. After application of 20mg oral Isotretinoine, after months skin lesions regressed, erythema decreased. Also decreased sweating in the area. Due to side effects (excessive drying and cracking of the lips) and the price of the drug the boy's mother decided to leave after 2 months of medication. This resulted in recurrence of symptoms and to obtain a definitive starting point.
 
Figure 1. Granulosis Rubra Nasi to 14-year-old boy
 
Discussion
GRN is rare, and the precise frequency is unknown. Most occurrences of granulosis rubra nasi are genetically determined, with an autosomal dominant or autosomal recessive pattern. [3]. The gene locus has not been identified. Just as in the analyzed case in GRN usually starts in early childhood in patients aged 6 months to 10 years [4]. Men suffer more. 7of 6 patients described by Jadassohna were boys [1], Mendoza described case of 10-year-old boy [5] and Akhdari case of a 18-year-old boy [6]. Initially, hyperhidrosis is the most conspicuous feature of the disease. Small beads of sweat can be seen on the tip of the nose. With persistent hyperhidrosis, diffuse erythema develops on the tip of the nose. Erythema gradually extends and may involve cheeks, upper lip, and chin. Erythema is covered by small beads of sweat. Can be seen small erythematous macules, erythematous papules, and vesicles or pustules lesions [6-9]. Many affected patients have poor peripheral circulation and hyperhidrosis of palms and soles [5,10]. GRN usually resolves spontaneously at puberty, but sometimes it lasts longer. Pathogenesis is unknown. Granulosis rubra nasi is a disorder of the eccrine glands, possibly representing a unique form of sweat retention [6,9]. The occurrence of a significant increase in the production of sweat on his nose and hyperhidrosis is present on the central face, palms, and soles; appears to be responsible for the secondary changes of erythema and erythematous papules. In differental diagnosis be taken into account: erythematous papulopustular dermatosis of the face, particularly acne vulgaris and rosacea, which do not have excessive sweating, photodematosis, which are more extensive with the absence of hyperhidrosis, otherwise lupus erythematosus, lupus vulgaris and leishmaniasis, actinic keratosis or skin cancer. However, evidence of chronic skin damage in childhood are rare (excluding specific genodermatosis) [5,9,11]. Pinkus and Lebet and give a possibility of coexistence GRN with hydrocystoma, also in one of case reports by Jadassohna been a few changes hydrocystoma [12,13]. MacLeod describes the symptoms of the disease in hot weather. Heid describes a case of 19-year-old woman with hyperhidrosis, GRN, tachycardia and pheochromocytoma [12]. After surgical removal of pheochromocytoma was sweating involution and regression of the GRN. The lesions usually disappear during puberty. Treatment is symptomatic and cosmetic. Brazilian authors have used in the treatment of botulinum toxin type A [2].
 
Conclusion
Granulosis Rubra Nasi is a rare disorder. Should remember GRN could be a complication of hyperhidrosis. In the treatment of low doses Isotretinoine can be used with good effect.
 
REFERENCES
1. Jadasson J. Granulosis Rubra Nasi. Archiv Dermatol und Syph 1901: 58:145-58. 2. Grazziotin TC, Buffon RB, da Silva Manzoni AP, Libis AS, Weber MB: Treatment of granulosis rubra nasi with botulinum toxin type A. Dermatol Surg. 2009; 35: 1298- 1299. 3. Grinoni F. Contributo clinico allo studio dell'etiopatogenesi della Granulosis Rubra Nasi. G Dermatol Sif. 1955; 96: 227. 4. Zuccati G, Filippeschi C, Mastrolorenzo A, Rapaccini AL, Tiradritti L, Staderini C: Granulosis rubra nasi. G Ital Dermatol Venereol. 1990; 125: 275-276. 5. Mendoza JP, Saldaña LS, Yokota ARRPL, Sialer MC, Anduaga ES: Granulosis rubra nasi. Dermatol. Peru. 2003; 13: 125-127. 6. Akhdari N. Granulosis rubra nasi. Int J Dermatol. 2007; 46: 396. 7. Williams DI: Granulosis rubra nasi. Proc R Soc Med. 1947; 499: 37 8. Goldsmith WM: Granulosis rubra nasi (Jadassohn). Proc R Soc Med. 390: 20 9. Bocian M, Bettina P, García R, Laterza A y col. Granulosis Rubra Nasi. A propósito de dos casos. Arch Arg Dermatol 2003: 53: 171-3. 10. Raymond GP, Delgrange-Delcourt T, Tétrault C: Granulosis rubra nasi: report of a new case. Union Med Can. 1978; 107: 800-801. 11. Brody M: Granulosis rubra nasi. Br J Dermatol Syph. 1947; 59: 380. 12. Heid E, Samain F, Jelen G, Boivin S: Granulosis rubra nasi and pheochromocytoma. Ann Dermatol Venereol. 1996; 123: 106-108. 13. Winkelried W: Case of granulosis rubra nasi. Proc R Soc Med. 1915: 20


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