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Oral lesions in pityriasis rubra pilaris
Oral lesions in pityriasis rubra pilaris DEI’ART3IEhTTS SCHOOL;
OF DER1\IATOLOGY
DEl’.\RTMENT
LABORATORY BIASShCIIUSETTS
OF THE
AND
PATHOLOGY,
OF DERMATOLOGY ,JBMES
GEKERAL
HOMER
AXD
WRIGHT
THE
HARVARD EDWIN
PATHOLOGY
MEDIC$L S. WEBSTER
LABORATORIES,
HOSPITAL
P
ityriasis ~ubra pilaris is an uncommon disorder of the-skin, characterized by follicular hyperkeratosis. Mucous membrane involvement has been reported only three times,‘3 2 and no photographs of the lesions or descriptions of the pathologic condition were presented. The purpose of this report is to describe a patient with both types of nlu~~~u membrane changes and to document the clinical and pathologic findings. CASE REPORT The patient was a 51.>-ear-old white woman with au ernptiok of t,he face which had c~lcare~l anti rrcurrecl internrit,tently orer the G years since it first”ippeared in 1958. During the fall of 1%X the eruption gradnally spread to the.,trunk and extremities and was including systemic corticosteroids. In nnre~ponsire to it variety of oral medications, E’el)ruary, 1965, the patient was admitted to the nfassachusett$ General Hospital for diagnosis and treatment. Physical examination findings were unremarkable except for the skin tlisorcler. There was an almost universal eruption which’ showed large orange-red plaques with islands of normal skin (Fig. 1). There .were distinct hyperkeratotic papules around hair follicles and a diffuse hyperkeratosis of the palms and soles, but the mucous membranes were normal. The cliniral picture was typical of pityriasis rubra pilaris. Biopsies of the skin revealed some hrperkeratoxix and a mild chronic inflammatory inIjegan to clear with the application of an ointment filtratr Uf the clcrrni~. ‘I’hr eruption i~i;nt:li;;:::g .i!!O.OOO noits of vitamirl A pr:r oltnce under occlusive rlressings. The patient :v:t* alto ei\-rr! 3!M,O11(1Itnit< of vit:lmin X per day 1)~ mouth.
This study was supported by Grants Ynitecl States Public Health Service. *Sssociate in Dermatology. **Associate in Pathology.
So. -AAT-O6X3R. CA-M9.55,
and CA-O:W~
from
the
691
Fin.
1. Photogr,zph
Fig. 8. Photograph
of cutaneous
lesions.
of oral mucous membrane
lesions.
without reappearance of these manifestations. In August, 1966, the patient complained of a mild discomfort in the mouth, and examination showed Bat erythematous lesions (‘oared with w1rit.e streaks on the bnccal mu~osa, gingivae, and soft palate (Fig. 2). Although the clinical picture resembled that of lichen planus, the biopsy did not show a bandlike infiltrate (Fig. 3). The patient was placed on a regimen of 300,000 units of vitamin zi daily for 4 weeks wit,hout improvement. Injection of the lesions with a triameino-
Fl tissue iufiltr: acantl small shows
3’. l%opathology of different a.reas of a mucous membrane lesion, The subepil 01elial ows marked edema around dilated capillaries. There is a superficial, moderately d ensr of lymphocytes with a fen- monocytes. Rare lymphocytes inx-ado the basa and 3inous layers of tltc epithclium. *\:I area of separation of the epidermis fro m thv I metibrane, with the formation of a suhepithelial vesicle, is seen. There is : no lis or downward growt,h of rete ridges. There is slight intercellular edema and )nes of vacuolar degeneration are seen in t,he basal layer. The upper epic 23rmis nne intercellular edema and focal areas of fine hasophilic keratohyalin granu Ile ‘9.
O.S., O.M. & O.P. May, 1968 lone suspension (10 mg. per milliliter) ing of the lesions.
was followed
by a decrease
in symptoms
and clear-
DISCUSSION
This articlc describes the occurrence in a patient wit,h pityriasis rubra pilaris of two distinct types of mucous membrane lesion, both of which had been noted separately by other authors.l, 2 The diffuse hyperkeratosis may not be rare but, rather, may go undetected because of the transient, subtle, and asymptomatic nature of the lesions. On the other hand, the macular lesions are striking and symptomatic and must represent an uncommon feature of pityriasis rubra pilaris. The hyperkeratotic changes may have responded to oral vitamin A, but the erythematous macular lesions did not. Injection of the lichen-planus-like lesions with a corticosteroid suspension may be an effective method of treatment. SUMMARY
A patient with pityriasis rubra pilaris had two distinct types of mucous membrane lesion. The diffuse hyperkeratosis first observed was asymptomatic and could easily have remained undetected. The lichen-planus-like lesions produced considerable discomfort and probably represent an uncommon feature. REFERENCES
1. Sutton, R. L., and V. Mosby Company, 2. Marshall, J. : Case Dermat. & Syph. 66:
Sutton, R. L.: Diseases of the Skin, ed. 10, St. Louis, 1939, The C. vol. 1, p. 329. of Pityriasis Rubra Pilaris With Lesions of Buccal Mucosa, Arch. 626, 1952.
OF DER1\IATOLOGY
DEl’.\RTMENT
LABORATORY BIASShCIIUSETTS
OF THE
AND
PATHOLOGY,
OF DERMATOLOGY ,JBMES
GEKERAL
HOMER
AXD
WRIGHT
THE
HARVARD EDWIN
PATHOLOGY
MEDIC$L S. WEBSTER
LABORATORIES,
HOSPITAL
P
ityriasis ~ubra pilaris is an uncommon disorder of the-skin, characterized by follicular hyperkeratosis. Mucous membrane involvement has been reported only three times,‘3 2 and no photographs of the lesions or descriptions of the pathologic condition were presented. The purpose of this report is to describe a patient with both types of nlu~~~u membrane changes and to document the clinical and pathologic findings. CASE REPORT The patient was a 51.>-ear-old white woman with au ernptiok of t,he face which had c~lcare~l anti rrcurrecl internrit,tently orer the G years since it first”ippeared in 1958. During the fall of 1%X the eruption gradnally spread to the.,trunk and extremities and was including systemic corticosteroids. In nnre~ponsire to it variety of oral medications, E’el)ruary, 1965, the patient was admitted to the nfassachusett$ General Hospital for diagnosis and treatment. Physical examination findings were unremarkable except for the skin tlisorcler. There was an almost universal eruption which’ showed large orange-red plaques with islands of normal skin (Fig. 1). There .were distinct hyperkeratotic papules around hair follicles and a diffuse hyperkeratosis of the palms and soles, but the mucous membranes were normal. The cliniral picture was typical of pityriasis rubra pilaris. Biopsies of the skin revealed some hrperkeratoxix and a mild chronic inflammatory inIjegan to clear with the application of an ointment filtratr Uf the clcrrni~. ‘I’hr eruption i~i;nt:li;;:::g .i!!O.OOO noits of vitamirl A pr:r oltnce under occlusive rlressings. The patient :v:t* alto ei\-rr! 3!M,O11(1Itnit< of vit:lmin X per day 1)~ mouth.
This study was supported by Grants Ynitecl States Public Health Service. *Sssociate in Dermatology. **Associate in Pathology.
So. -AAT-O6X3R. CA-M9.55,
and CA-O:W~
from
the
691
Fin.
1. Photogr,zph
Fig. 8. Photograph
of cutaneous
lesions.
of oral mucous membrane
lesions.
without reappearance of these manifestations. In August, 1966, the patient complained of a mild discomfort in the mouth, and examination showed Bat erythematous lesions (‘oared with w1rit.e streaks on the bnccal mu~osa, gingivae, and soft palate (Fig. 2). Although the clinical picture resembled that of lichen planus, the biopsy did not show a bandlike infiltrate (Fig. 3). The patient was placed on a regimen of 300,000 units of vitamin zi daily for 4 weeks wit,hout improvement. Injection of the lesions with a triameino-
Fl tissue iufiltr: acantl small shows
3’. l%opathology of different a.reas of a mucous membrane lesion, The subepil 01elial ows marked edema around dilated capillaries. There is a superficial, moderately d ensr of lymphocytes with a fen- monocytes. Rare lymphocytes inx-ado the basa and 3inous layers of tltc epithclium. *\:I area of separation of the epidermis fro m thv I metibrane, with the formation of a suhepithelial vesicle, is seen. There is : no lis or downward growt,h of rete ridges. There is slight intercellular edema and )nes of vacuolar degeneration are seen in t,he basal layer. The upper epic 23rmis nne intercellular edema and focal areas of fine hasophilic keratohyalin granu Ile ‘9.
O.S., O.M. & O.P. May, 1968 lone suspension (10 mg. per milliliter) ing of the lesions.
was followed
by a decrease
in symptoms
and clear-
DISCUSSION
This articlc describes the occurrence in a patient wit,h pityriasis rubra pilaris of two distinct types of mucous membrane lesion, both of which had been noted separately by other authors.l, 2 The diffuse hyperkeratosis may not be rare but, rather, may go undetected because of the transient, subtle, and asymptomatic nature of the lesions. On the other hand, the macular lesions are striking and symptomatic and must represent an uncommon feature of pityriasis rubra pilaris. The hyperkeratotic changes may have responded to oral vitamin A, but the erythematous macular lesions did not. Injection of the lichen-planus-like lesions with a corticosteroid suspension may be an effective method of treatment. SUMMARY
A patient with pityriasis rubra pilaris had two distinct types of mucous membrane lesion. The diffuse hyperkeratosis first observed was asymptomatic and could easily have remained undetected. The lichen-planus-like lesions produced considerable discomfort and probably represent an uncommon feature. REFERENCES
1. Sutton, R. L., and V. Mosby Company, 2. Marshall, J. : Case Dermat. & Syph. 66:
Sutton, R. L.: Diseases of the Skin, ed. 10, St. Louis, 1939, The C. vol. 1, p. 329. of Pityriasis Rubra Pilaris With Lesions of Buccal Mucosa, Arch. 626, 1952.