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Indian Journal of Dermatology
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CASE REPORT
Year : 2006  |  Volume : 51  |  Issue : 4  |  Page : 275-277
Majocchi disease in a child


From the Pathologist, Izzetpasa Mah. Mehmetcik Sk. No. 25/A, Elazig, Turkey

Correspondence Address:
Perihan Ozturk
M.D. Pathologist, Izzetpasa Mah. Mehmetcik Sk. No. 25/A, Elazig
Turkey
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/0019-5154.30294

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   Abstract 

Majocchi disease, purpuric annularis telangiectodes, is an uncommon pigmented purpuric dermatosis. It is usually observed in young adult males, but is very uncommon in childhood. In this paper, a ten-year-old female patient having very number of annular eruptions, surrounded by petechiae on her legs, diagnosed as Majocchi disease, is presented and discussed and the literature discussed.


Keywords: Child case, Majocchi disease


How to cite this article:
Ozturk P, Ataseven A, Ozturk U, Demiroren K, Dagli F. Majocchi disease in a child. Indian J Dermatol 2006;51:275-7

How to cite this URL:
Ozturk P, Ataseven A, Ozturk U, Demiroren K, Dagli F. Majocchi disease in a child. Indian J Dermatol [serial online] 2006 [cited 2020 Nov 24];51:275-7. Available from: https://www.e-ijd.org/text.asp?2006/51/4/275/30294



   Introduction Top


Pigmented purpuric dermatoses (PPD) are a group of benign dermatoses, occurring as result of lymphocytic capillaritis, increased capillary fragility and erythrocyte extravasation, characterized by asymptomatic nonpalpable purpura and petechiae on hyperpigmental base.[1],[2] Majocchi disease (purpura annularis telangiectodes), an uncommon variant of PPD, is characterized by symmetrical, annular patches, including punctuate red-brown macules, with a predilection for the lower extremities.[3],[4],[5] We present our case since Majocchi disease is uncommonly observed during childhood.


   Case Report Top


A ten-year-old female patient presented with red eruptions, the number of which gradually increasing in three years period, starting from her ankles spreading towards thighs. She had no other health problem. A history of similar lesions, not diagnosed definitely, lasting for years in her grandmother was received. Her systemic physical examination was normal. In her dermatological examination, punctuate red-brown macules coalescing to form annular patches with a range of 0.5-2 cm in diameter were present in her thighs, symmetrically [Figure - 1]. Her complete blood count, routine biochemistrical tests, erythrocyte sedimentation rate, cryoglobulins, antinuclear antibodies, complement levels, viral hepatitis markers including hepatitis B and C viruses were normal or negative.

Histopathological examination of the material extirpated from the lesion showed hyperkeratosis, parakeratosis in the epithelium, mild papillomatosis, subdermal hydropic degeneration, free phagocytic melanin pigmentations, perivascular lymphocytic infiltration and extravasation of erythrocytes [Figure - 2]. According to the clinical and histopathological examination, the patient was diagnosed as Majocchi disease.


   Discussion Top


PPD has five clinical types: Progressive pigmented purpuric dermatosis (Schamberg disease), purpura annularis telangiectodes (Majocchi disease), pigmented purpuric lichenoid dermatosis ( Gougerot-Blum disease More Details), itching purpura (eczematid like purpura of Doucas and Kapetanakis) and lichen aureus.[2] Clinical overlap may be observed between these types.[3]

Purpura annularis telangiectodes firstly has been described in 1896 by Majocchi.[3] In Majocchi disease, early purpuric lesions begin symmetrically in legs.[4],[5] Lesions may involve arms and trunk.[4] Central atrophy and telangiectasia may be present in some lesions.[5] The diameters of the lesions vary in the range of 2-20 mm. The lesions do not disappear by vitrepression. They are usually annular, occasionally linear, stellate or serpigionous-shaped.[6] The disease may last for years.[3] In our case, the lesions involving the legs were present for three years. They are annular-shaped plaques having telangiectasiae in the center, surrounded by pinpointed petechiae, not fading by vitrepression. Histopathological properties of Majocchi disease are same of the other capillaritis, but perivascular lymphocytic infiltration may be present. Extravasation of red blood cells and hemosiderin laden macrophages are found in connective tissues.[5],[7] The patient's histopathologic findings were harmonious with Majocchi disease.

Majocchi disease is usually encountered in adult males.[4] It may be observed rarely in newborn, childhood or elders.[1] Honda et al . reported a newborn with Majocchi disease.[8] We did not encounter any case diagnosed as Majocchi disease in childhood except this report in the literature. Tristani-Firouzi et al . reported three children with PPD.[1] Our case was distinctly that of a ten-year-old female.

The etiology of Majocchi disease is unknown as certain.[3] A hypothesis of the vascular pressure increase has been supposed because the lesions appear usually on the legs and buttocks.[6] Immune complex deposition and tissue destruction by the mediation of langerhans cells has been shown.[9] Because several familial cases have been described in Majocchi and Schamberg diseases, the origin of the disease may be speculated as genetic.[7] A suspicious family history was present in our case. In etiology of the PPD, viral and bacterial agents have been accused.[10],[11] Satoh et al reported five patients with PPD having odontogenic infection that their illness regressed by the treatment of the infection.[10] Erbagcı et al . reported two patients with PPD having HCV infection and suggested that HCV infection should be investigated in PPD cases which are idiopathic and resistant to treatment.[2] In our case, viral markers of hepatitis B and C were normal. Rarely, some cases which appear with a drug use and improve by leaving the responsible drugs were reported.[7] The drugs, as acetylsalicylic acid, thiamin, asetaminofen, chlordiazepoxide, meprobamat, glipizid, topical fluorouracil, pefloxacin, barbiturates, chlorthiazide, lorazepam, medroxiprogesteron acetate, tartarazin, etc may cause PPD by the toxic and allergic mechanisms.[2] In our case a history of drug intake was not present. In PPD lesions, because the increase in CD1a+ and CD3+/CD4+ T cells and expression of ICAM-1, LFA-1, ELAM-1 like cytokines was observed, contact dermatitis like delayed-type hypersensitivity reaction was responsible in the pathogenesis.[2]

Although PPD's are benign diseases, they are disturbing because they last for months. If the lesions are widespread, systemic steroid treatment may be administered for a short time. But this treatment has morbistatic effects.[5] Topical steroids, ascorbic acid, supportive socks, carbon dioxide snow, antihistaminics, stanozolol (tissue plasminogen activator) for resistant cases, griseofulvin, PUVA and cyclosporine are the therapeutic regimens.[12] Pandhi et al reported a Majocchi disease case in which 1 mg/day colchicine maintained a wellness in a period of one month.[13] We performed topical steroids and oral antihistaminic to our case and suggested monthly controls. In the follow-up of three months, her lesions were disappeared completely.

Finally, PPD is rarely seen in childhood and Majocchi disease is a rare variety of PPD. Majocchi disease in childhood was reported in a case in the literature. We report this case because of its rarity.

 
   References Top

1.Tristani-Firouzi P, Meadows KP, Vanderhooft S. Pigmented purpuric eruptions of childhood: A series of cases and review of literature. Pediatr Dermatol 2001;18:299-304.   Back to cited text no. 1  [PUBMED]  [FULLTEXT]  
2. Erbagcı Z, Tuncel A. HCV enfeksiyonlu iki olguda pigmente purpurik dermatoz gelişimi (in Turkish). Turkiye Klinikleri J Med Sci 2003;23:238-41.   Back to cited text no. 2      
3. Hale EK. Purpura annularis telangiectodes of Majocchi. Dermatol Online J 2003;9:17-8.   Back to cited text no. 3      
4. Disorders of hemostasis. In : Braun-Falco O, Plewig G, Wolff HH, Burgdorf WH, editors. Dermatology. 2nd ed. Berlin: Springer Verlag; 2000. p. 956-80.   Back to cited text no. 4      
5. Hamuryudan B, Tüzün H, Mat MC, Tüzün B. Vasküler diπer hastalıklar. In : Tüzün Y, Kotagyan A, Aydemir EH, Baransu O, editors. Dermatoloji. 2nd ed. Nobel Tıp Kitap Evleri: Istanbul; 1994. p. 400-11.   Back to cited text no. 5      
6. Kim HJ Skidmore RA, Woosley JT. Pigmented purpura over the lower extremities: Purpura annularis telangiectodes of Majocchi. Arch Dermatol 1998;134:1477.   Back to cited text no. 6      
7. Cedric C, John B, Wilkinson D. Pigmented purpuric dermatitis. Available from: http://www.emedicine.com/derm/topic327.htm   Back to cited text no. 7      
8. Honda M, Saijo S, Tagami H. Majocchi's disease in a newborn baby: A familial case. Br J Dermatol 1997;137:655-6.   Back to cited text no. 8      
9. Newton RC, Raimer SS. Pigmented purpuric eruptions. Dermatol Clin 1985;3:165-9.   Back to cited text no. 9      
10. Satoh T, Yokozeki H, Nishioka K. Chronic pigmented purpura associated with odontogenic infection. J Am Acad Dermatol 2002;46:942-4.   Back to cited text no. 10      
11. Ratnam KV, Su DW, Peters MS. Purpura simplex (inflammatory purpura without vasculitis): A clinicopathologic study of 174 cases. J Am Acad Dermatol 1991;25:642-7.   Back to cited text no. 11      
12. Smoller BR, Kamel OW. Pigmented purpuric eruptions: Immunopathologic studies supportive of a common immunophenotype. J Cutan Pathol 1991;18:423-7.   Back to cited text no. 12      
13. Pandhi R, Jain R, Radotra BD, Kumar B. Purpura annularis telangiectoides with vasculitic ulcers treated with colchicines. Int J Dermatol 2002;41:388-9.  Back to cited text no. 13      


    Figures

  [Figure - 1], [Figure - 2]



 

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    Abstract
    Introduction
    Case Report
    Discussion
    References
    Article Figures

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