itis

nte C. Scully, PhD, MD, MDS, FDS, FFD, MRCPath, and J. W. Eveson, PhD, FDS, FRCPath, Bristol, England CENTRE

FOR

THE

STUDY

OF ORAL

DISEASE,

UNIVERSITY

OF BRISTOL

DENTAL

HOSPITAL

AND

SCHOOL

The pigmented purpuric dermatoses are a group of disorders in which there is chronic capillaritis, with pigmented purpuric lesions predominantly on the lower limbs. We report a case with chronic oral lesions that had histologic features most in keeping with the purpuric lichenoid dermatitis of Gougerot and Blum syndrome. (OFCAL SURC ORAL MED ORAL PATHOL

1992;74:780-2)

number of overlapping syndromes of unknown cause but characterized by capillaritis, which results in a pigmented purpuric rash mainly on the lower limbs, have been described as the pigmented purpuric dermatoses, under various eponymous titles (Majocchi’s disease, Schamberg’s disease, and GougerotBlum syndrome), or other syndromes (lichen aureus purpuricus and itching purpura) (Table I).lM5 Histologically, the superficial small vessels may show endothelial cell swelling, luminal narrowing, and a perivascular lymphocytic infiltrate mainly of T cells, some extravasation of erythrocytes with hemosiderin uptake by macrophages.’ Oral lesions with similar appearances appear not to have been recorded thus far. CASE REPORT A 4Cyear-old white man complained of blood blisters in the mouth for 15 years, which arose mainly after he ate abrasive foods. There were no other oral or systemic symptoms apart from a generalized urticarial rash during the summer months. Of note was the fact that his brother had a similar oral complaint. The remaining medical history was noncontributory. Examination revealed a fit looking, normotensive man with no obvious rash or any other extraoral lesions. Oral examination revealed nothing of note except an unusual lesion in the hard palate (Fig, 1) that consisted of lichenoid white striae with interspersed fine petechiae, and a mild desquamative gingivitis. A full hematologic work-up revealed no abnormalities, and the platelet count was nor7/14/41446

780

mal. Urinalysis was negative. A microscopic examination of the oral lesional biopsy specimen showed focal, wedge-shaped areas of parakeratosisand mild acanthosis (Fig. 2). There was a mild, predominantly lymphohistiocytic infiltrate in the superficial submucosa traversed by dilated capillaries with swollen endothelial cells. There was fibrin exudation and red blood cell extravasation into the papillary and superficial submucosa and within the lower, spongiotic layers of the epithelium (Fig. 3). In the midsubmucosal connective tissue,there were vesselswith slightly thickened walls along with hemosiderin-laden macrophages (Fig. 4). The features were those of a pigmented purpuric stomatitis similar to purpuric lichenoid dermatitis of Gougerot and Blum syndrome. DISCUSSION

The pigmented purpuric dermatoses show considerable overlap, though there are some features that have led to their subclassification in the past (Table I).‘-’ Most tend to affect men predominantly, and the lower limbs with a purpuric rash. Chronic capillaritis is seen with an early appearance of dilatation and telangiectatic puncta and purpura, followed by the appearance of hemosiderin-laden macrophages and subsequent pigmentation. When telangiectasia predominates, the term Majocchi’s disease is used *, 3,6 7and Schamberg’sdiseaseis the term given when pigmentation predominates. *s9 When there is a lichenoid element that results from patchy parakeratosis and spongiosis, the term purpuric lichenoid dermatitis (or Gougerot and Blum syndromelo) is used. This was probably the most precisediagnosis in the present case.Often the various subtypes cannot be distinguished, and other variants have been described.5,‘l-l6 The rash in pigmented purpuric dermatoses is typically

Volume Number

Pigmented purpuric

74 6

Fig. 1. Lichenoid petechiae.

white

striations

on palate

with

stomatitis

781

fine

Fig. 3. Microscopic examination shows swollen endothelial cells and hemorrhage into superficial submucosa and the lower epithelium. (Hematoxylin-eosin stain; original magnification X 120.)

Fig. 2. Microscopic examination shows focal parakeratosis and hemorrhage into the superficial submucosa and epithelium together with a lymphohistocytic infiltrate. (Hematoxylin-eosin stain; original magnification X60.)

Fig. 4. Microscopic examination shows mild inflammatory infiltration, thicker vessel, and hemosiderin deposition in the deeper connective tissues. (Hematoxylin-eosin stain; X 120.) original magnification

782

Scully and Eveson

ORAL

SURG

ORAL

MELI ORAL

PATHOL

December

1992

Table 1. Main features of the pigmented purpuric dermatoses Syndrome

affected

Majocchi’s disease* (purpura annularis telangiectoides) Schamberg’s disease* (progressive pigmentary dermatosis) Purpuric lichenoid dermatitis of Gougerot and Blum syndrome Itching purpura Lichen aureus purpuricus

M=F

Any

M

Lower

limbs

M

Lower

limbs

M M

Wide distribution Lower limbs unilaterally

*Occasionally

Main

site

Characteristics Chronic discrete annular lesions and telangiectasia Chronic irregular macular Chronic purpura and lichenoid lesions Short-lived organ pruritic macules Chronic yellow or brown macules and/or papules

familial.

purpuric and chronic, and lasts for years. It may be symptomless or cause mild pruritus. There is no specific treatment. Oral lesions appear not to have previously been described. In this case, the main differential diagnosis was from lichen planus and related lesions. Unfortunately, the patient was lost to follow-up but the long history (15 years) suggeststhat the oral lesions are, like the rash, chronic, but clearly may be seen in the absence of frank skin lesions. REFERENCES 1. Aiba S, Tagami II. Immunohistologic studies in Schamberg’s disease. Arch Dermatol 1988;124:1058-62. 2. Farrokhzad S, Champion RH. Pigmented purpuric dermatoses. Dermatologica 1970;140:45-53. A. Le purpura annulaire telangiectasique de Majoc3. Touraine chi et ses parentes. Presse Med 1949;57:934-6. 4. Randall SJ, Kierland, RR, Montgomery H. Pigmented purpuric eruptions. Arch Dermatol Syphil 1951;64:177-82. 5. Mosto SJ, Casala AM. Disseminated pruriginous angiodermatitis (itching purpura). Arch Dermatol 1965;91:351-6. 6. Mackee GM. Purpura annularis telangiectodes. J Cutan Genitourin Dis 1915;33:129-41. I. Majocchi D. Sopra una dermatosi telangiectode non ancora descritta; purpura annularis. G Ital Ma1 Vener Pelle 1896; 31:263-4.

8. Schamberg JF. A peculiar progressive pigmentary disease of the skin. Br J Dermatol 1901;13:1-5. 9. Schamberg JF. Report of three cases of progressive pigmentary dermatosis with particular reference to the blood cholesterol. Br J Dermatol 1927;39:389-93. 10. Gougerot H, Blum P. Purpura angiosclertux prurigineux aux elements lichenoides. Bull Sot Fr Dermatol Syphiligr 1925; 32:161-3. 11. Doucas C, Kapetanakis J. Eczematid-like purpura dermatoses. Dermatologica 1970;140:45-53. 12. Loewenthal LJA. Itching purpura. Br J Dermatol 1954;66:95103. 13. Kanitakis C, Tositis G. Lichen purpurique. Ann Dermatol Venereol 1982;109:445-52. 14. Price ML, Wilson-Jones E, Calnan CD, et al. Lichen aureus: a localised persistent form of pigmented purpuric dermatosis. Br J Dermatol 1984;112:307-14. 15. Baden HP. Familial Schambera’s disease. Arch Dermatol 1964;90:400. 16. Gould WM, Farber EM. A familial pigmented purpuric eruption. Dermatologica 1966;132:400-8. Reprint requests: J. W. Eveson, PhD, FDS, FRCPath Centre for the Study of Oral Disease University of Bristol Dental Hospital Lower Maudlin Street Bristol BSl 2LY England

and School

Pigmented purpuric stomatitis.

The pigmented purpuric dermatoses are a group of disorders in which there is chronic capillaritis, with pigmented purpuric lesions predominantly on th...
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