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Histiocytic lymphoma occurring in a patient with dermatitis herpetiformis
Case reports Histiocytic lymphoma occurring in a patient with dermatitis herpetiformis D. Jenkins, M . D . , * C. W. Lynde, M . D . , * * and W. D. Stewart, M.D.**
Vancottver, British Columbia, Canada A patient with long-standing dermatitis herpetiformis (DH) developed histiocytic lymphoma of the small bowel. This is the twelfth reported case of lymphoma occurring in association with dermatitis herpetiformis. (J AM ACAD DERMATOL 9:252-256, 1983.) Dermatitis herpetiformis (DH) is a chronic, extremely pruritic eruption of grouped papules and vesicles. These are symmetrically distributed over preferential areas: the scalp, extensor surfaces of the limbs, elbows and knees, tips of scapulae, lumbosacral area, buttocks, and posterior portions of the thighs. 1 In almost 80% o f patients with DH, there are associated small bowel changes consistent with celiac disease (CD). 2 Malignant lymphoma, especially in the small intestine, is a recognized complication of CD.:5 Eleven previous cases have reported DH, CD, and l y m p h o m a in association. 4-~2 The patient presented here represents a further example. CASE R E P O R T
This female patient, born in 1925, had difficulty gaining weight as a child and an adult. She gave a history of moderate intermittent abdominal bloating all her life. Syntbroid replacement was begun in 1965 for hypothyroidism. The patient's daughter had celiac disease as a child and did well on a gluten-free diet. In October, 1971, our patient developed grouped papules and vesicles symmetrically distributed over the extensor surfaces of the limbs, elbows, and knees, buttocks, and posterior portions of the thighs. Direct immunofluorescence of normal buttock skin showed IgA speckling in the dermal papillae consistent with DH From the Department of Medicine, St. Paul's Hospital,* and the Division of Dermatology, VancouverGenera! Hospital.** Reprint requests to: Dr. Donald Jenkins, Departmentof Medicine, St. Paul's Hospital, 1081 Burrard St., Vancouver, B.C., Canada V6Z 1Y6. 252
(Fig. 1). After making the clinical diagnosis of classical DH, her dermatologist placed her on a therapeutic trial of dapsone (I00 rng per day). Her skin lesions almost completely cleared on dapsone over the next 2 weeks. Maintenance was then carried out with dapsone, doses varying 50 to 200 rag/day, depending on the activity of her disease over the next 8 years. Flares were noted each time the patient discontinued the medication. In August, 1981, our patient was admitted to the hospital with an acute abdominal condition. She gave a history of intermittent left lower quadrant abdominal pains over the preceding 2 months. A laparotomy was done. The jejunum was found to be perforated. The remainder of the small bowel, liver, spleen, and stomach were felt to be grossly normal. About 25 cm of the jejunum was resected on either side of the perforation. Histologic examination revealed diffuse histi0cytic lymphoma* (Rappaport classification) of the jejunum (Fig. 2). Subtotal villous atrophy consistent with CD was noted (Fig. 3). A series of examinations, including chest x-ray, liver/spleen scan, computerized tomography (CT) head and abdominal scan, upper gastrointestinal series, and bone marrow failed to show additional foci of lymphoma. The patient was tissue type HLA-A1, A31, B8, and B62. Her disease was staged as I~ (Ann Arbor classification 1971). She received three cycles of CHOP (cyclophosphamide, adriamycin, vincristine, and prednisone), followed by a course of abdominal irradiation. She tolerated this therapy quite well. Beginning in March, i982, she developed progressive symptoms of anorexia, watery diarrhea, lower abdominal pain, and spiking fevers to 39.5 ~ C. On admission in April, 1982, she was cachectic and jaundiced, with an obvious left ocular ptosis. Over the ab*lmmunoblasticlymphosarcoma,Luke's classification.
Volume 9 Number 2 August, 1983
Histiocvtic lymphoma in dermatitis heppetiformis
253
Fig. 1. Direct immunofluorescence of normal buttock skin showing IgA speckling of dermal papillae.
Fig. 2. Diffuse histiocytic lymphoma of the jejunum. (Hematoxylin-eosin stain; original magnification, x 100.) domen she had multiple erythematous, infiltrated papules and nodules, the largest of which was 1.5 cm in diameter. Significant hepatomegaly was noted. There was no significant axillary or inguinal adenopathy. Biopsy of an abdominal skin nodule showed a cutaneous lymphomatous infiltrate (Fig. 4), Chest x-ray examination revealed multiple soft tissue densities in both lungs consistent with disseminated lymphoma.
She had a progressive downhill course and died in April, 1982. DISCUSSION Direct immunofluorescence of normal buttock skin was consistent with DH. Our patient had the classical eruption of DH with excoriated papules and vesicles over the elbows, knees, and sacral
254
Journal of the A m e r i c a n Academy of Dermatology
Jenkins et al
ii~: i~ i ~
Fig. 3. Subtotal mucosal villous atrophy of the jejunum consistent with celiac disease at a site distant to the lymphoma. (Hematoxylin-eosin stain; original magnification, X 100,)
4. Cutaneous lymphomatous infiltrate. (Hematoxylin-eosin stain; original magnification, x 100.)
Fig.
areas. She responded promptly to a therapeutic trial of dapsone and flared off medication. Celiac disease was suggested by the patient's history of abdominal bloating and difficulties in gaining weight. Jejunal biopsy showed mucosal changes
compatible with the diagnosis of CD. After having been diagnosed as having DH 10 years earlier, our patient was found to have histiocytic lymphoma of the jejunum. DH, a chronic pruritic vesiculobullous eruption
Volume 9 Number 2 August, 1983
Histiocytic lymphoma in dermatitis heiTet~brmis
255
Table I. Dermatitis herpetiformis, celiac disease, and lymphoma
Author (year)
Age in years
I Durationof DH priorto diagnosis of lymphoma Sex (years)
CD
Type-site Histiocytic --jejunum Histiocytic -- mesenteric Histiocytic --jej un um "Malignant lymphoma"--jejunum Histiocytic --jejunum Lymphocytic -- skin Histiocytic -- stomach Histiocytic --jejunum Undifferentiated multiple sites in small intestine Histiocytic --jejunum Histiocytic --jejunum Histiocytic --jejunum
Gjone and Nordaj (1970)4 Anderson et al (1971) '~ Goodwin and Fry (1973) '~ Connon et al (1975) 7 Tonder et al (1976) 8 Fowler and Thomas (1976) 9 Freeman et al (1977) 1~ Freeman et al (1977) l~ Freeman et al (1977) m
44 60 57 52 64 59 60 42 62
M F M M F M M M M
17 44 9 10 1/4 7 20 7/12 5
+ + + + + + + + +
10. Silk et al (1977) ll 11. Gould and Howell (1977) 12 12. Jenkins, Lynde, Stewart*
51 60 57
M M F
12 19 10
+ + +
1. 2. 3. 4. 5. 6. 7. 8. 9.
*present report. of immune origin, is associated with celiac disease, albeit usually a mild form in almost 80% of cases3 There is a difference in the degree of the severity of the gastrointestinal lesions and symptoms in the two conditions. The intestinal lesion in DH may be minimal when the patient is ingesting a normal gluten load. Symptomatic malabsorption is seen much less frequently in DH, in contrast to CD. The gastrointestinal lesions in DH and CD are fundamentally similar, but there is a difference in the degree of severity of the gastrointestinal lesions and their clinical manifestations in the two conditions." Several studies have shown that adherence to a strict gluten-free diet results in a disappearance of the rash in some cases. In the majority of cases the dose of the dapsone can be markedly reduced on such a diet. The rash relapses on gluten challenge.'-' Family studies and the high incidence of HLA-B8 suggest a genetic relationship between these two disorders. 'a Both DH and CD show a striking increase in the incidence of HLA-B8 antigens. The incidence of HLA-B8 in DH approaches 90%, ,a and in CD, 88%.'4 Our patient was HLA-B8 type. The association of CD with gastrointestinal malignancy has been well described. ,5-,7 Harris et a115 observed an overall incidence of intestinal lymphoma of 6.9% in 202 cases of CD. Lym-
phoma complicating CD has been observed to occur more commonly in the jejunum. Lymphoma in association with other malabsorptive syndromes has usually been found in the ileum.18 Our case, in conjunction with the eleven previously reported cases (Table I), suggests that patients with long-standing DH can also develop a lymphoma as a complication of their disease. Nine of the twelve patients were male. The age range was 42 to 62 years; mean age was 55.7 years. The mean interval between the appearance of DH and the diagnosis of lymphoma of the reported cases is 12.8 years. Harris et a115 reported that the mean interval between the onset of CD and the diagnosis of lymphoma was 21.2 years. Pathologically, histiocytic lymphoma was found in nine of twelve cases. The jejunum was the site of the lymphoma in eight cases. All patients had clinical or histologic evidence of gluten-sensitive enteropathy. It has been suggested that DH may be part of a wider spectrum of disease. 1"~ Genetic linkage and the formation of immune complexes following exposure to a dietary antigen such as gluten may explain the association of DH with malignancy.'4 DH has been associated with many other malignancies, including leukemia, 2~ multiple myeloma, ~ carcinoma of the upper respiratory tract, 21 larynx,'-" lung, ''2 thyroid, <'-'3 pancreas, "a'24 kid-
256
Jenkhls et al
ney,4 o v a r y , 21.211 vulva," ~ prostate, "'~ and stomach. A gluten-free diet has been s h o w n to improve the C D associated with D H . It does not consistently ameliorate the eruption o f D H , although it m a y allow reduction o f d a p s o n e used for maintenance in DH. It has b e e n suggested but not proved that strict gluten-free diet decreases the possibility o f m a l i g n a n c y in C D . 8"~5 Hopefully such a diet could d e c r e a s e the risk of m a l i g n a n t complications of DH. D H , CD, and l y m p h o m a are associated. It w o u l d be prudent to have an increased index of suspicion in patients with DH, particularly in those with a b d o m i n a l complaints.
REFERENCES 1. Katz SI, Hall RP, Lawley TJ, Strober W: Dermatitis herpetiformis: The skin and the gut. Ann Intern Med 93:857-874, 1980. 2. Katz S1, Strober W: The pathogenesis of dermatitis herpetiformis. J Invest Dermatol 70:63-75, 1978. 3. Cooper BT, Holmes GKT, Ferguson R: Celiac disease and malignancy. Medicine 59:249-261, 1980. 4. Gjone E, Nordog A: Dermatitis herpetiformis, steatorrhoea and malignancy. Br Med J 1:610, 1970. 5. Anderson H, Dotevall G, Mobacken H: Malignant mesenteric lymphoma in a patient with dermatitis herpetiformis, hypochlorhydria and small-bowel abnormalities. Scand J Gastroenterol 6:397-399, 1971. 6. Goodwin P, Fry L: Reticulum cell sarcoma complicating dermatitis herpetiformia. Proc R Soc Med 66:625-626, 1973. 7. Connon JJ, McFarland J, Kelly A, et al: Acute abdominal complications of coeliac disease. Scand J Gastroenterol 10"843-846, 1975. 8. Tonder M, Sorlie D, Keamey MS: Adult celiac disease: A case with ulceration, dermatitis herpetiformis and reticulosarcoma. Scand J Gastroenterol 11:107-111, 1976. 9. Fowler JM, Thomas DJB: Lymphoma in dermatitis herpetiformis. Br Med J 2:757, 1976.
Journal of the American Academyof Dermatology
I0. Freeman HJ, Weinstein WM, Shnitka TK, et al: Primary abdominal lymphoma presenting manifestation of celiac sprue or complicating dermatitis herpetiformis. Am J Med 63:585-594, 1977. 11. Silk DBA, Mowat NAG, Riddell RH, Kirby JD: Intestinal lymphoma complicating dermatitis herpetiformis. Br J Dermatol 96:555-560, 1977. 12. Gould DJ, Howell R: Dermatitis herpetiformis and reticulum cell sarcoma, a rate complication. Br J Dermat01 96:561-562, 1977. 13. Grenier R, Rostas A, Wilkinson RD: Dermatitis herpetiformis and leiomyomas with HLA-B8, a marker of immune diseases. Can Med Assoc J 115:882-884, 1976. 14. Kutz SI, Falclmk M, Dahl MV, et al: HL-A8 a genetic link between dermatitis herpetifollnis and gluten-sensitive enteropathy. J Clin Invest 51:297%2980, 1972. 15. Harris OD, Cooke WY, Thompson H, Waterhouse JAM: Malignancy in adult celiac disease and idiopathic steatorrhea. Am J Med 42:899-912, 1967. 16. Selby WS, Gallagher ND: Malignancy in a 19 year experience of adult celiac disease. Dig Dis Sci 24:684-688, 1979. 17. Cooper BT, Holmes GKT, Ferguson R, Cooke WT: Celiac disease and malignancy. Medicine 59:249-261, 1980. 18. Alexander JO: Dermatitis herpetil'ormis. Philadelphia, 1975, W. B. Sounders Co., pp. 266-279. 19. Davies MG, Marks R, Nuki G: Dermatitis herpetiformis--a skin manifestation of a generalized disturbance in immunity. Q J Med 47:221-248, 1978. 20. Francois A, Carli-Basset C, Ginies G, et al: Maladie de Duhring-Brocq, signal symptbme d'une leuc~mie lympho'ide chronique. Ball Soc Fr Dermato178:60-62, 1971. 21. Mansson T: Malignant disease in dermatitis herpetiformis. Acta Derm Venereol (Stockh) 51:379-382, 1971. 22. Michel P-J, Corrgard R: Maladie de Duhring-Bmcq chez un sujet atteint de cancer du poumon. Bull Soc Fr Dermatol 77:289-290, 1971. 23. Curth HO: Scientific exhibits; dermatoses and malignant internal tumors. Arch Dermatol 71:95-107, 1955. 24. Traub EF: Dermatitis herpetiformis: Cancer of the pancreas. Arch Dermatol 38:974, 1938.
Vancottver, British Columbia, Canada A patient with long-standing dermatitis herpetiformis (DH) developed histiocytic lymphoma of the small bowel. This is the twelfth reported case of lymphoma occurring in association with dermatitis herpetiformis. (J AM ACAD DERMATOL 9:252-256, 1983.) Dermatitis herpetiformis (DH) is a chronic, extremely pruritic eruption of grouped papules and vesicles. These are symmetrically distributed over preferential areas: the scalp, extensor surfaces of the limbs, elbows and knees, tips of scapulae, lumbosacral area, buttocks, and posterior portions of the thighs. 1 In almost 80% o f patients with DH, there are associated small bowel changes consistent with celiac disease (CD). 2 Malignant lymphoma, especially in the small intestine, is a recognized complication of CD.:5 Eleven previous cases have reported DH, CD, and l y m p h o m a in association. 4-~2 The patient presented here represents a further example. CASE R E P O R T
This female patient, born in 1925, had difficulty gaining weight as a child and an adult. She gave a history of moderate intermittent abdominal bloating all her life. Syntbroid replacement was begun in 1965 for hypothyroidism. The patient's daughter had celiac disease as a child and did well on a gluten-free diet. In October, 1971, our patient developed grouped papules and vesicles symmetrically distributed over the extensor surfaces of the limbs, elbows, and knees, buttocks, and posterior portions of the thighs. Direct immunofluorescence of normal buttock skin showed IgA speckling in the dermal papillae consistent with DH From the Department of Medicine, St. Paul's Hospital,* and the Division of Dermatology, VancouverGenera! Hospital.** Reprint requests to: Dr. Donald Jenkins, Departmentof Medicine, St. Paul's Hospital, 1081 Burrard St., Vancouver, B.C., Canada V6Z 1Y6. 252
(Fig. 1). After making the clinical diagnosis of classical DH, her dermatologist placed her on a therapeutic trial of dapsone (I00 rng per day). Her skin lesions almost completely cleared on dapsone over the next 2 weeks. Maintenance was then carried out with dapsone, doses varying 50 to 200 rag/day, depending on the activity of her disease over the next 8 years. Flares were noted each time the patient discontinued the medication. In August, 1981, our patient was admitted to the hospital with an acute abdominal condition. She gave a history of intermittent left lower quadrant abdominal pains over the preceding 2 months. A laparotomy was done. The jejunum was found to be perforated. The remainder of the small bowel, liver, spleen, and stomach were felt to be grossly normal. About 25 cm of the jejunum was resected on either side of the perforation. Histologic examination revealed diffuse histi0cytic lymphoma* (Rappaport classification) of the jejunum (Fig. 2). Subtotal villous atrophy consistent with CD was noted (Fig. 3). A series of examinations, including chest x-ray, liver/spleen scan, computerized tomography (CT) head and abdominal scan, upper gastrointestinal series, and bone marrow failed to show additional foci of lymphoma. The patient was tissue type HLA-A1, A31, B8, and B62. Her disease was staged as I~ (Ann Arbor classification 1971). She received three cycles of CHOP (cyclophosphamide, adriamycin, vincristine, and prednisone), followed by a course of abdominal irradiation. She tolerated this therapy quite well. Beginning in March, i982, she developed progressive symptoms of anorexia, watery diarrhea, lower abdominal pain, and spiking fevers to 39.5 ~ C. On admission in April, 1982, she was cachectic and jaundiced, with an obvious left ocular ptosis. Over the ab*lmmunoblasticlymphosarcoma,Luke's classification.
Volume 9 Number 2 August, 1983
Histiocvtic lymphoma in dermatitis heppetiformis
253
Fig. 1. Direct immunofluorescence of normal buttock skin showing IgA speckling of dermal papillae.
Fig. 2. Diffuse histiocytic lymphoma of the jejunum. (Hematoxylin-eosin stain; original magnification, x 100.) domen she had multiple erythematous, infiltrated papules and nodules, the largest of which was 1.5 cm in diameter. Significant hepatomegaly was noted. There was no significant axillary or inguinal adenopathy. Biopsy of an abdominal skin nodule showed a cutaneous lymphomatous infiltrate (Fig. 4), Chest x-ray examination revealed multiple soft tissue densities in both lungs consistent with disseminated lymphoma.
She had a progressive downhill course and died in April, 1982. DISCUSSION Direct immunofluorescence of normal buttock skin was consistent with DH. Our patient had the classical eruption of DH with excoriated papules and vesicles over the elbows, knees, and sacral
254
Journal of the A m e r i c a n Academy of Dermatology
Jenkins et al
ii~: i~ i ~
Fig. 3. Subtotal mucosal villous atrophy of the jejunum consistent with celiac disease at a site distant to the lymphoma. (Hematoxylin-eosin stain; original magnification, X 100,)
4. Cutaneous lymphomatous infiltrate. (Hematoxylin-eosin stain; original magnification, x 100.)
Fig.
areas. She responded promptly to a therapeutic trial of dapsone and flared off medication. Celiac disease was suggested by the patient's history of abdominal bloating and difficulties in gaining weight. Jejunal biopsy showed mucosal changes
compatible with the diagnosis of CD. After having been diagnosed as having DH 10 years earlier, our patient was found to have histiocytic lymphoma of the jejunum. DH, a chronic pruritic vesiculobullous eruption
Volume 9 Number 2 August, 1983
Histiocytic lymphoma in dermatitis heiTet~brmis
255
Table I. Dermatitis herpetiformis, celiac disease, and lymphoma
Author (year)
Age in years
I Durationof DH priorto diagnosis of lymphoma Sex (years)
CD
Type-site Histiocytic --jejunum Histiocytic -- mesenteric Histiocytic --jej un um "Malignant lymphoma"--jejunum Histiocytic --jejunum Lymphocytic -- skin Histiocytic -- stomach Histiocytic --jejunum Undifferentiated multiple sites in small intestine Histiocytic --jejunum Histiocytic --jejunum Histiocytic --jejunum
Gjone and Nordaj (1970)4 Anderson et al (1971) '~ Goodwin and Fry (1973) '~ Connon et al (1975) 7 Tonder et al (1976) 8 Fowler and Thomas (1976) 9 Freeman et al (1977) 1~ Freeman et al (1977) l~ Freeman et al (1977) m
44 60 57 52 64 59 60 42 62
M F M M F M M M M
17 44 9 10 1/4 7 20 7/12 5
+ + + + + + + + +
10. Silk et al (1977) ll 11. Gould and Howell (1977) 12 12. Jenkins, Lynde, Stewart*
51 60 57
M M F
12 19 10
+ + +
1. 2. 3. 4. 5. 6. 7. 8. 9.
*present report. of immune origin, is associated with celiac disease, albeit usually a mild form in almost 80% of cases3 There is a difference in the degree of the severity of the gastrointestinal lesions and symptoms in the two conditions. The intestinal lesion in DH may be minimal when the patient is ingesting a normal gluten load. Symptomatic malabsorption is seen much less frequently in DH, in contrast to CD. The gastrointestinal lesions in DH and CD are fundamentally similar, but there is a difference in the degree of severity of the gastrointestinal lesions and their clinical manifestations in the two conditions." Several studies have shown that adherence to a strict gluten-free diet results in a disappearance of the rash in some cases. In the majority of cases the dose of the dapsone can be markedly reduced on such a diet. The rash relapses on gluten challenge.'-' Family studies and the high incidence of HLA-B8 suggest a genetic relationship between these two disorders. 'a Both DH and CD show a striking increase in the incidence of HLA-B8 antigens. The incidence of HLA-B8 in DH approaches 90%, ,a and in CD, 88%.'4 Our patient was HLA-B8 type. The association of CD with gastrointestinal malignancy has been well described. ,5-,7 Harris et a115 observed an overall incidence of intestinal lymphoma of 6.9% in 202 cases of CD. Lym-
phoma complicating CD has been observed to occur more commonly in the jejunum. Lymphoma in association with other malabsorptive syndromes has usually been found in the ileum.18 Our case, in conjunction with the eleven previously reported cases (Table I), suggests that patients with long-standing DH can also develop a lymphoma as a complication of their disease. Nine of the twelve patients were male. The age range was 42 to 62 years; mean age was 55.7 years. The mean interval between the appearance of DH and the diagnosis of lymphoma of the reported cases is 12.8 years. Harris et a115 reported that the mean interval between the onset of CD and the diagnosis of lymphoma was 21.2 years. Pathologically, histiocytic lymphoma was found in nine of twelve cases. The jejunum was the site of the lymphoma in eight cases. All patients had clinical or histologic evidence of gluten-sensitive enteropathy. It has been suggested that DH may be part of a wider spectrum of disease. 1"~ Genetic linkage and the formation of immune complexes following exposure to a dietary antigen such as gluten may explain the association of DH with malignancy.'4 DH has been associated with many other malignancies, including leukemia, 2~ multiple myeloma, ~ carcinoma of the upper respiratory tract, 21 larynx,'-" lung, ''2 thyroid, <'-'3 pancreas, "a'24 kid-
256
Jenkhls et al
ney,4 o v a r y , 21.211 vulva," ~ prostate, "'~ and stomach. A gluten-free diet has been s h o w n to improve the C D associated with D H . It does not consistently ameliorate the eruption o f D H , although it m a y allow reduction o f d a p s o n e used for maintenance in DH. It has b e e n suggested but not proved that strict gluten-free diet decreases the possibility o f m a l i g n a n c y in C D . 8"~5 Hopefully such a diet could d e c r e a s e the risk of m a l i g n a n t complications of DH. D H , CD, and l y m p h o m a are associated. It w o u l d be prudent to have an increased index of suspicion in patients with DH, particularly in those with a b d o m i n a l complaints.
REFERENCES 1. Katz SI, Hall RP, Lawley TJ, Strober W: Dermatitis herpetiformis: The skin and the gut. Ann Intern Med 93:857-874, 1980. 2. Katz S1, Strober W: The pathogenesis of dermatitis herpetiformis. J Invest Dermatol 70:63-75, 1978. 3. Cooper BT, Holmes GKT, Ferguson R: Celiac disease and malignancy. Medicine 59:249-261, 1980. 4. Gjone E, Nordog A: Dermatitis herpetiformis, steatorrhoea and malignancy. Br Med J 1:610, 1970. 5. Anderson H, Dotevall G, Mobacken H: Malignant mesenteric lymphoma in a patient with dermatitis herpetiformis, hypochlorhydria and small-bowel abnormalities. Scand J Gastroenterol 6:397-399, 1971. 6. Goodwin P, Fry L: Reticulum cell sarcoma complicating dermatitis herpetiformia. Proc R Soc Med 66:625-626, 1973. 7. Connon JJ, McFarland J, Kelly A, et al: Acute abdominal complications of coeliac disease. Scand J Gastroenterol 10"843-846, 1975. 8. Tonder M, Sorlie D, Keamey MS: Adult celiac disease: A case with ulceration, dermatitis herpetiformis and reticulosarcoma. Scand J Gastroenterol 11:107-111, 1976. 9. Fowler JM, Thomas DJB: Lymphoma in dermatitis herpetiformis. Br Med J 2:757, 1976.
Journal of the American Academyof Dermatology
I0. Freeman HJ, Weinstein WM, Shnitka TK, et al: Primary abdominal lymphoma presenting manifestation of celiac sprue or complicating dermatitis herpetiformis. Am J Med 63:585-594, 1977. 11. Silk DBA, Mowat NAG, Riddell RH, Kirby JD: Intestinal lymphoma complicating dermatitis herpetiformis. Br J Dermatol 96:555-560, 1977. 12. Gould DJ, Howell R: Dermatitis herpetiformis and reticulum cell sarcoma, a rate complication. Br J Dermat01 96:561-562, 1977. 13. Grenier R, Rostas A, Wilkinson RD: Dermatitis herpetiformis and leiomyomas with HLA-B8, a marker of immune diseases. Can Med Assoc J 115:882-884, 1976. 14. Kutz SI, Falclmk M, Dahl MV, et al: HL-A8 a genetic link between dermatitis herpetifollnis and gluten-sensitive enteropathy. J Clin Invest 51:297%2980, 1972. 15. Harris OD, Cooke WY, Thompson H, Waterhouse JAM: Malignancy in adult celiac disease and idiopathic steatorrhea. Am J Med 42:899-912, 1967. 16. Selby WS, Gallagher ND: Malignancy in a 19 year experience of adult celiac disease. Dig Dis Sci 24:684-688, 1979. 17. Cooper BT, Holmes GKT, Ferguson R, Cooke WT: Celiac disease and malignancy. Medicine 59:249-261, 1980. 18. Alexander JO: Dermatitis herpetil'ormis. Philadelphia, 1975, W. B. Sounders Co., pp. 266-279. 19. Davies MG, Marks R, Nuki G: Dermatitis herpetiformis--a skin manifestation of a generalized disturbance in immunity. Q J Med 47:221-248, 1978. 20. Francois A, Carli-Basset C, Ginies G, et al: Maladie de Duhring-Brocq, signal symptbme d'une leuc~mie lympho'ide chronique. Ball Soc Fr Dermato178:60-62, 1971. 21. Mansson T: Malignant disease in dermatitis herpetiformis. Acta Derm Venereol (Stockh) 51:379-382, 1971. 22. Michel P-J, Corrgard R: Maladie de Duhring-Bmcq chez un sujet atteint de cancer du poumon. Bull Soc Fr Dermatol 77:289-290, 1971. 23. Curth HO: Scientific exhibits; dermatoses and malignant internal tumors. Arch Dermatol 71:95-107, 1955. 24. Traub EF: Dermatitis herpetiformis: Cancer of the pancreas. Arch Dermatol 38:974, 1938.