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Heerfordt's syndrome presenting with an erythematous eruption of the lower legs
314
Correspondence
Journal of the American Academy of Dermatology
that cimetidine does not play a significant role in the management of herpes infections. Andrew P. Truhan, M.D., and Benjamin Raab, MD. Department of Dermatology Northwestern University Medical School Chicago, IL 60611
REFERENCES 1. Van der spuy S, Levy DW, Levin W: Cimetidine in the treatment of herpes virus infections. S Afr Med J 58: 112116, 1980. 2. Hayne ST, Mercer JB: Herpes zoster: Treatment with cimetidine. Can Med Assoc J 129:1284-1285, 1983. 3. Mavligit GM, Talpaz M: Cimetidine for herpes zoster. N Engl J Med 310:318-319, 1984. 4. Shandera R: Treatment of herpes zoster with cimetidinc. Can Med Assoc J 131:279, 1984. 5. Wagner RF Jr: Cimetidine and herpes zoster. JAM ACAD DERMATOL 10:1074, 1984. (Letter to Editor.)
Heerfordt's syndrome presenting with an erythematous eruption of the lower legs To the Editor: Sarcoidosis is an idiopathic disorder characterized by noncaseating granulomas. Due to its multiorgan involvement, it is common for this disease to present with a varied group of symptoms. Sarcoidal involvement of the parotid gland, in association with uveitis, fever, and cranial nerve palsies, has been termed "Heerfordt's Syndrome." I We report a case of this rare disorder in a black woman who presented to us because of an associated cutaneous eruption on her legs. Case report. A 32-year-old black woman presented to our clinic with a 3-wcek history of an asymptomatic erythematous eruption on her legs. She had also notcd a 3- to 4-week history of progressively increasing swelling of both of her cheeks, which recently became tender. Most recently she complained of photophobia with blurry vision and dry eyes. She denied fevers but complained of chills with occasional night sweats over the previous week. On physical examination, she was noted to have bilateral, enlarged, tender parotid and submandibular glands (Fig. 1). She was also found to have a right facial palsy. Examination of her legs showed multiple erythematous, nonblanching macules, papules, and plaques without epidennal changes (Fig. 2). Several similar plaques were present on the upper part of her left ann. An ophthalmologic examination showed bilateral anterior and posterior uveitis, with keratitis sicca. Multiple small conjunctional nodules were also noted in both eyes.
Results. Biopsy specimens were taken from her con-
Fig. 1. The patient presented with a right facial palsy
and bilateral enlarged parotid glands.
junctiva and from a plaque on her leg. Both specimens showed noncaseating granulomas, consistent with sarcoid. Special stains for fungus and acid-fast bacilli were negative. Fine needle aspirations from both parotid glands showed cytologic features of granulomatous disease. The following laboratory tests were normal or negative; purified protein deriyative (PPD), VDRL, FfAABS, prothrombin time/partial thromboplastin time (PT/PTT), antinuclear antibody (ANA), UI A, urine and throat bacterial cultures, erythrocyte sedimentation rate (ESR), hemoglobin, electrolyte profile, fasting glucose, cholesterol, uric acid, bilirubin, calcium, and immunodiffusion tests for blastomycin, coccidioidin, histoplasmin, and aspergillin. The following tests were abnormal SGOT 48 (9-33); SGPT 78 (0-40); lactic dehydrogenase (LDH) , 298 (l00-200); (CPK) , 326 (0-190); alkaline phosphatase, 195 (90-115); angiotensin-converting enzyme, 36 (1534); phosphate, 4.2 (2.5-4.0); and positive skin test to mumps. Chest x-ray examination showed prominent bilateral hilar adenopathy.
Volume 13 Number 2, Part 1 August, 1985
Fig. 2. The legs showed multiple erythematous nonblanching macules, papules, and plaques. The patient refused any further investigation of her condition. She was discharged with a diagnosis of sarcoidosis with Heerfordt's syndrome. Prior to discharge she was begun on oral prednisone, 80 mg every day. Comment. Heerfordt's syndrome (febris uvea-parotidica subchronica, uveoparotid fever) is a rare symptom complex of sarcoidosis. Since its initial description by Heerfordt in 1909, there have been less than 140 cases described worldwide. 2 The majority of patients with Heerfordt's syndrome are in their second or third decade of life. 3 Afflicted women outnumber men almost 2: 1. 2.3 There is one reported case of Heerfordt's syndrome occurring in two sisters,4 but no other familial association has been noted. Heerfordt, in his original description, suggested that this syndrome consisted of the following symptoms: (1) prolonged low-grade fever, (2) the disease process localized to the parotid gland and the uvea (of the eye), and (3) frequent occurrence of paresis of the cranial nerves. l
Correspondence
315
The complete syndrome, consisting of uveitis, parotid gland enlargement, and fever and cranial nerve palsy, make upless than 10% of all reported cases,2-8 Most authors have defined the syndrome as sarcoidosis with uveitis and parotid gland enlargement. The additional clinical features of fever and cranial nerve palsy are present much less frequently and are not considered necessary to establish the diagnosis. Indeed, several reviews of patients with Heerfordt's syndrome have shown that less than 50% of patients had fever or cranial nerve palsy.3,5 The parotid gland enlargement and uveitis are usually bilatera1. 1,2.6 Of those patients with cranial nerve palsies, approximately 50% have bilateral involvement. 2-6 The facial nerve is by far the most commonly affected cranial nerve. The course of Heerfordt's syndrome is characteristically prolonged over months to years. Involvement of other organs, including lungs, lymph nodes, spleen, and skin, is frequent. 7 Over 80% of the patients showed bilateral hilar adenopathy on chest x-ray examination at the time of their initial presentation. 2,5-7 Several authors' have suggested that Heerfordt's syndrome represents an extensive and active form of satcoidosis. 5.B The uveitis occurs early in the disease, is severe as a rule, and often il,i chronic orrecurrent. 5 Crick et aP reported that 38% of his patients with Beerfordt's syndrome went on to develop progressive pulmonary sarcoidosis. Only 3% of his other sarcoid patients developed similar lung involvement. 5 Associated cutaneous findings have been reported in from 17% to 43% of patients with Heerfordt's syndrome. These 'have included erythema nodosum and maculop\lpular eruptions. This correlates well with the percentage of patients with systemic sarcoidosis who have cutaneous lesions as noted by Hanno and Callen, 9 Then~ are no reports of histopathologic examinations of any of the cutaneous lesion In previous patients with Heerfordt;s syndrome. The biopsy specimen from our patient's leg showed multiple noncaseating granulomas , in the dennis and subcutaneous tissue. This would be classified as a ., specific" cutaneous lesion of sarcoidosis, ~ccording to Hanno and Callen. 9 This type of lesion is commonly associated with chronic sarcoidosis. Indeed, previous reports of patients with Heerfordt's syndrome show that the disease often follows a chronic course. ' A "nonspecific" cutaneous lesion of sarcoidosis, such as erythema nodosum, is more commonly associated with the acute transient type of sarcoidosis. 9 Unfortunately, our patient was lost to follow-up im,
316
Journal of the American Academy of Dermatology
Correspondence
mediately after her diagnosis was established, so we have been unable to observe her clinical course.
Mark G. Rubin, M.D., Joseph W. McGoey, M.D. lJepartment o/Dermatology, Henry Ford Hospital 2799 W. Grand Blvd. Detroit, MI48202 REFERENCES 1. Heerfordt CF: Uber eine "Febris uveo-parotidea subchronica" an der Glandula parotis und der Uvea des Auges lokalisiert und haufig mit Paresen cerebrospinalea Neruen kompliziert. Albrecht V. Groefos. Arch Ophthalmol 70:254-273, 1909. . 2. Fujmori I, Gujimori I, Honda K, et al: A case report of Heerfordt's syndrome. Proceedings of the VI International Congress on Sarcoidosis 1972. Tokyo, 1974, University of Tokyo Press, pp. 327-330.
3. Garland HG, Thompson JG: Uveo-parotid tuberculosis. Q J Med 2:157-180, 1933. 4. Snell NI, Karlish A: Heerfordt's syndrome in two sisters. Br Med J 4:731-732,1975. 5. Crick R, Hoyle C, Smellie H: The eyes in sarcoidosis. Br J OphthalmoI45:461-481, 1961. 6. Stjernberg N, Wiman L: Uvea-parotid fever (Heerfordt's syndrome) or sarcoid affection of the eyes and parotid glands. Proceedings of the VI International Congress on Sarcoidosis 1972. Tokyo, 1974, University of Tokyo Press, pp. 331-337. 7. Greenberg G, Anderson R, Sharpstone P, et al: Enlargement of parotid gland due to sarcoidosis. Br Med J 2:861862, 1964. 8. Scaddihg IG: Sarcoidosis. London, 1967, Eyre & Spottiswciode, pp. 200-201. . 9. Hanno R, Callen J: Sarcoidosis. Med Clin North Am 4:847-866, 1980.
ABSTRACTS Nevus of Spitz (Gennan text) Gartman H, Ganser M: Z Hautkr 60:22-49, 1985 The authors analyzed 652 nevi of Spitz that were examined clinically and histologically at four German dermatologic university clinics during the last 35 years. This nevus type was found in 61 % of female patients . The lower extremity was most frequently affected in 30.1% of' patients, followed by head, upper extremity, trunk, neck, and genital area. Histologically, spindle cells were found 'in 44.9% of patients, spindle and epithelioid cells in 34%, and epithelioid cells only in 21 %. Since this nevus appears in a high percentage of adults, the term benign juvenile melanoma should be replaced by Ackerman's term, nevus of large spindle and/or epithelioid cells or McGovern's practical term, nevus of Spitz. . Alfred Hollander, M.D. Preliminary results of Re-PUVA.therapy in cutaneous T-cell lymphoma (Gennan text) Thiele B, Rasokat H, Mahrle G: Z Hautkr 60:116119, 1985 Eight patients with cutaneous T cell lymphoma (6 mycosis fungoides and 2 parapsoriasis variegata) were treated with etretinate, 50 to 100 mg, daily, combined with PUVA (RePUVA). Three patients showed complete regression and five, partial regres~ion. The only ~ide effect was a bullous dermatitis In two patients. It healed after the etretinate dosage was reduced. Alfred Hollander, M.D.
Differential roentgen therapy of keratoacanthoma (German text) Koster W, Nasemann T, Reimlinger S, Wiskemann A: Z Hautkr 60:215-218, 1985 Roentgen therapy is recommended as an alternative for surgical treatment of keratoacanthoma, particularly for old patients with a large tumor of over 5 cm in diameter. After performance of a punch biopsy to exclude basal cell carcinoma, roentgen therapy with a dose of 2,500 r is given. If there is no tumor regression after 2 weeks, squamous cell carcinoma is suspected and a dose of 2,500 r is added. Alfred Hollander, MD. Light microscopic examinations of spermatogenesis of rats after high dose application of 13·cis·retinoic acid (German text) Kuhlwein A, Schulte B: Z Hautkr 60:245-248, 1985 The influence of a toxic dose of 13-cis-retinoic acid (isotretinoin), 40 rng/kg/daily, on the spermatogenesis of thirty Wistar rats was examined. After eight weeks' application, all rats showed normal spermatogenesis in all parts of the testes, as did twenty control rats. In examinations of humans no negative influence on the spermatogenesis was observed with a daily therapeutic dose of 0.5 to 1 mg/kg. Alfred Hollander, M.D.
Correspondence
Journal of the American Academy of Dermatology
that cimetidine does not play a significant role in the management of herpes infections. Andrew P. Truhan, M.D., and Benjamin Raab, MD. Department of Dermatology Northwestern University Medical School Chicago, IL 60611
REFERENCES 1. Van der spuy S, Levy DW, Levin W: Cimetidine in the treatment of herpes virus infections. S Afr Med J 58: 112116, 1980. 2. Hayne ST, Mercer JB: Herpes zoster: Treatment with cimetidine. Can Med Assoc J 129:1284-1285, 1983. 3. Mavligit GM, Talpaz M: Cimetidine for herpes zoster. N Engl J Med 310:318-319, 1984. 4. Shandera R: Treatment of herpes zoster with cimetidinc. Can Med Assoc J 131:279, 1984. 5. Wagner RF Jr: Cimetidine and herpes zoster. JAM ACAD DERMATOL 10:1074, 1984. (Letter to Editor.)
Heerfordt's syndrome presenting with an erythematous eruption of the lower legs To the Editor: Sarcoidosis is an idiopathic disorder characterized by noncaseating granulomas. Due to its multiorgan involvement, it is common for this disease to present with a varied group of symptoms. Sarcoidal involvement of the parotid gland, in association with uveitis, fever, and cranial nerve palsies, has been termed "Heerfordt's Syndrome." I We report a case of this rare disorder in a black woman who presented to us because of an associated cutaneous eruption on her legs. Case report. A 32-year-old black woman presented to our clinic with a 3-wcek history of an asymptomatic erythematous eruption on her legs. She had also notcd a 3- to 4-week history of progressively increasing swelling of both of her cheeks, which recently became tender. Most recently she complained of photophobia with blurry vision and dry eyes. She denied fevers but complained of chills with occasional night sweats over the previous week. On physical examination, she was noted to have bilateral, enlarged, tender parotid and submandibular glands (Fig. 1). She was also found to have a right facial palsy. Examination of her legs showed multiple erythematous, nonblanching macules, papules, and plaques without epidennal changes (Fig. 2). Several similar plaques were present on the upper part of her left ann. An ophthalmologic examination showed bilateral anterior and posterior uveitis, with keratitis sicca. Multiple small conjunctional nodules were also noted in both eyes.
Results. Biopsy specimens were taken from her con-
Fig. 1. The patient presented with a right facial palsy
and bilateral enlarged parotid glands.
junctiva and from a plaque on her leg. Both specimens showed noncaseating granulomas, consistent with sarcoid. Special stains for fungus and acid-fast bacilli were negative. Fine needle aspirations from both parotid glands showed cytologic features of granulomatous disease. The following laboratory tests were normal or negative; purified protein deriyative (PPD), VDRL, FfAABS, prothrombin time/partial thromboplastin time (PT/PTT), antinuclear antibody (ANA), UI A, urine and throat bacterial cultures, erythrocyte sedimentation rate (ESR), hemoglobin, electrolyte profile, fasting glucose, cholesterol, uric acid, bilirubin, calcium, and immunodiffusion tests for blastomycin, coccidioidin, histoplasmin, and aspergillin. The following tests were abnormal SGOT 48 (9-33); SGPT 78 (0-40); lactic dehydrogenase (LDH) , 298 (l00-200); (CPK) , 326 (0-190); alkaline phosphatase, 195 (90-115); angiotensin-converting enzyme, 36 (1534); phosphate, 4.2 (2.5-4.0); and positive skin test to mumps. Chest x-ray examination showed prominent bilateral hilar adenopathy.
Volume 13 Number 2, Part 1 August, 1985
Fig. 2. The legs showed multiple erythematous nonblanching macules, papules, and plaques. The patient refused any further investigation of her condition. She was discharged with a diagnosis of sarcoidosis with Heerfordt's syndrome. Prior to discharge she was begun on oral prednisone, 80 mg every day. Comment. Heerfordt's syndrome (febris uvea-parotidica subchronica, uveoparotid fever) is a rare symptom complex of sarcoidosis. Since its initial description by Heerfordt in 1909, there have been less than 140 cases described worldwide. 2 The majority of patients with Heerfordt's syndrome are in their second or third decade of life. 3 Afflicted women outnumber men almost 2: 1. 2.3 There is one reported case of Heerfordt's syndrome occurring in two sisters,4 but no other familial association has been noted. Heerfordt, in his original description, suggested that this syndrome consisted of the following symptoms: (1) prolonged low-grade fever, (2) the disease process localized to the parotid gland and the uvea (of the eye), and (3) frequent occurrence of paresis of the cranial nerves. l
Correspondence
315
The complete syndrome, consisting of uveitis, parotid gland enlargement, and fever and cranial nerve palsy, make upless than 10% of all reported cases,2-8 Most authors have defined the syndrome as sarcoidosis with uveitis and parotid gland enlargement. The additional clinical features of fever and cranial nerve palsy are present much less frequently and are not considered necessary to establish the diagnosis. Indeed, several reviews of patients with Heerfordt's syndrome have shown that less than 50% of patients had fever or cranial nerve palsy.3,5 The parotid gland enlargement and uveitis are usually bilatera1. 1,2.6 Of those patients with cranial nerve palsies, approximately 50% have bilateral involvement. 2-6 The facial nerve is by far the most commonly affected cranial nerve. The course of Heerfordt's syndrome is characteristically prolonged over months to years. Involvement of other organs, including lungs, lymph nodes, spleen, and skin, is frequent. 7 Over 80% of the patients showed bilateral hilar adenopathy on chest x-ray examination at the time of their initial presentation. 2,5-7 Several authors' have suggested that Heerfordt's syndrome represents an extensive and active form of satcoidosis. 5.B The uveitis occurs early in the disease, is severe as a rule, and often il,i chronic orrecurrent. 5 Crick et aP reported that 38% of his patients with Beerfordt's syndrome went on to develop progressive pulmonary sarcoidosis. Only 3% of his other sarcoid patients developed similar lung involvement. 5 Associated cutaneous findings have been reported in from 17% to 43% of patients with Heerfordt's syndrome. These 'have included erythema nodosum and maculop\lpular eruptions. This correlates well with the percentage of patients with systemic sarcoidosis who have cutaneous lesions as noted by Hanno and Callen, 9 Then~ are no reports of histopathologic examinations of any of the cutaneous lesion In previous patients with Heerfordt;s syndrome. The biopsy specimen from our patient's leg showed multiple noncaseating granulomas , in the dennis and subcutaneous tissue. This would be classified as a ., specific" cutaneous lesion of sarcoidosis, ~ccording to Hanno and Callen. 9 This type of lesion is commonly associated with chronic sarcoidosis. Indeed, previous reports of patients with Heerfordt's syndrome show that the disease often follows a chronic course. ' A "nonspecific" cutaneous lesion of sarcoidosis, such as erythema nodosum, is more commonly associated with the acute transient type of sarcoidosis. 9 Unfortunately, our patient was lost to follow-up im,
316
Journal of the American Academy of Dermatology
Correspondence
mediately after her diagnosis was established, so we have been unable to observe her clinical course.
Mark G. Rubin, M.D., Joseph W. McGoey, M.D. lJepartment o/Dermatology, Henry Ford Hospital 2799 W. Grand Blvd. Detroit, MI48202 REFERENCES 1. Heerfordt CF: Uber eine "Febris uveo-parotidea subchronica" an der Glandula parotis und der Uvea des Auges lokalisiert und haufig mit Paresen cerebrospinalea Neruen kompliziert. Albrecht V. Groefos. Arch Ophthalmol 70:254-273, 1909. . 2. Fujmori I, Gujimori I, Honda K, et al: A case report of Heerfordt's syndrome. Proceedings of the VI International Congress on Sarcoidosis 1972. Tokyo, 1974, University of Tokyo Press, pp. 327-330.
3. Garland HG, Thompson JG: Uveo-parotid tuberculosis. Q J Med 2:157-180, 1933. 4. Snell NI, Karlish A: Heerfordt's syndrome in two sisters. Br Med J 4:731-732,1975. 5. Crick R, Hoyle C, Smellie H: The eyes in sarcoidosis. Br J OphthalmoI45:461-481, 1961. 6. Stjernberg N, Wiman L: Uvea-parotid fever (Heerfordt's syndrome) or sarcoid affection of the eyes and parotid glands. Proceedings of the VI International Congress on Sarcoidosis 1972. Tokyo, 1974, University of Tokyo Press, pp. 331-337. 7. Greenberg G, Anderson R, Sharpstone P, et al: Enlargement of parotid gland due to sarcoidosis. Br Med J 2:861862, 1964. 8. Scaddihg IG: Sarcoidosis. London, 1967, Eyre & Spottiswciode, pp. 200-201. . 9. Hanno R, Callen J: Sarcoidosis. Med Clin North Am 4:847-866, 1980.
ABSTRACTS Nevus of Spitz (Gennan text) Gartman H, Ganser M: Z Hautkr 60:22-49, 1985 The authors analyzed 652 nevi of Spitz that were examined clinically and histologically at four German dermatologic university clinics during the last 35 years. This nevus type was found in 61 % of female patients . The lower extremity was most frequently affected in 30.1% of' patients, followed by head, upper extremity, trunk, neck, and genital area. Histologically, spindle cells were found 'in 44.9% of patients, spindle and epithelioid cells in 34%, and epithelioid cells only in 21 %. Since this nevus appears in a high percentage of adults, the term benign juvenile melanoma should be replaced by Ackerman's term, nevus of large spindle and/or epithelioid cells or McGovern's practical term, nevus of Spitz. . Alfred Hollander, M.D. Preliminary results of Re-PUVA.therapy in cutaneous T-cell lymphoma (Gennan text) Thiele B, Rasokat H, Mahrle G: Z Hautkr 60:116119, 1985 Eight patients with cutaneous T cell lymphoma (6 mycosis fungoides and 2 parapsoriasis variegata) were treated with etretinate, 50 to 100 mg, daily, combined with PUVA (RePUVA). Three patients showed complete regression and five, partial regres~ion. The only ~ide effect was a bullous dermatitis In two patients. It healed after the etretinate dosage was reduced. Alfred Hollander, M.D.
Differential roentgen therapy of keratoacanthoma (German text) Koster W, Nasemann T, Reimlinger S, Wiskemann A: Z Hautkr 60:215-218, 1985 Roentgen therapy is recommended as an alternative for surgical treatment of keratoacanthoma, particularly for old patients with a large tumor of over 5 cm in diameter. After performance of a punch biopsy to exclude basal cell carcinoma, roentgen therapy with a dose of 2,500 r is given. If there is no tumor regression after 2 weeks, squamous cell carcinoma is suspected and a dose of 2,500 r is added. Alfred Hollander, MD. Light microscopic examinations of spermatogenesis of rats after high dose application of 13·cis·retinoic acid (German text) Kuhlwein A, Schulte B: Z Hautkr 60:245-248, 1985 The influence of a toxic dose of 13-cis-retinoic acid (isotretinoin), 40 rng/kg/daily, on the spermatogenesis of thirty Wistar rats was examined. After eight weeks' application, all rats showed normal spermatogenesis in all parts of the testes, as did twenty control rats. In examinations of humans no negative influence on the spermatogenesis was observed with a daily therapeutic dose of 0.5 to 1 mg/kg. Alfred Hollander, M.D.