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JAAD Grand Rounds quiz∗
JAAD
GRAND ROUNDS
JAAD Grand Rounds quiz* Daniel Schulman, PhD, Anthony C. Chu, Jason D. Michaels, MD, Robert H. Cook-Norris, MD, Julia S. Lehman, MD, Lawrence E. Gibson, MD, Whitney S. Alexander, MD, and Eric W. Hossler, MD Learning objectives: At the conclusion of this learning activity, physician participants should be able to assess their own diagnostic and patient management skills and use the results of this exercise to help determine personal learning needs. Instructions: In answering each question, refer to the specific directions provided. Because it is often necessary to provide information occurring later in a series that give away answers to earlier questions, please answer the questions in each series in sequence. ( J Am Acad Dermatol 2014;71:407-14.)
Permanent lip swelling: A precursor of systemic disease? Daniel Schulman, PhD, and Anthony C. Chu London, United Kingdom A 30-year-old woman was referred to a dermatology clinic because of pronounced permanent generalized swelling of her lower lip (Figs 1 and 2). As a 17-year-old, this patient had complained of a 4-month history of intermittent swelling of her right upper lip and had been seen by both ear, nose, and throat and plastic surgeons. At that time, both her C1 esterase level and magnetic resonance imaging scan were reported to be normal. A diagnosis of localized angioneurotic edema was suggested, and she was subsequently lost to followup. Eleven years later, she underwent colorectal surgery for a perianal fistula, followed by drainage of an ischiorectal abscess and another perianal abscess. It was reported that the histologic results of the fistula suggested a diagnosis of Crohn’s disease. A biopsy specimen of the lip was obtained (Figs 3 and 4), and this revealed a diffuse dermal lymphoplasmacytic infiltrate with several well-formed, nonnecrotizing histiocytic granulomas. No fungi or acid-fast bacilli were detected. 1. What is the most likely diagnosis? a. Angioedema b. Posttraumatic c. Infection (nonodontogenic) d. Orofacial granulomatosis e. Actinic cheilitis
The authors, editors, and peer reviewers have no relevant financial relationships. *The section editor for JAAD Grand Rounds is Erin E. Boh, MD. J AM ACAD DERMATOL
2. Granulomatous cheilitis has been shown to be associated with which causative etiology? a. MelkerssoneRosenthal syndrome b. Allergic reactions c. Crohn’s disease d. Sarcoidosis e. All of the above 3. The histologic results will typically reveal which of the following? a. Interface dermatitis b. Pautrier microabscesses c. Pagetoid spread d. Noncaseating granulomatous inflammation e. Perifollicular lymphocytic infiltrate 4. Which of the following has been described as an orofacial manifestation/association of Crohn’s disease? a. Oral ulceration b. Gingivitis c. Granulomatous cheilitis d. Lichen planus e. All of the above 5. What is the reported incidence of granulomatous cheilitis in Crohn’s disease? a. 0.1% b. 0.5% c. 1% d. 5% e. 12% 6. What is not recommended in the routine management of granulomatous cheilitis? a. Chest radiograph and serum angiotensinconverting enzyme level b. Gastrointestinal investigations to exclude Crohn’s disease c. Topical/intralesional steroids d. Contact allergy testing e. Serum radioallergosorbent tests AUGUST 2014
407
408 JAAD grand rounds
Discussion Granulomatous cheilitis (GC) is a rare chronic granulomatous inflammation of the lips of unknown etiology that rarely may be associated with peripheral facial nerve paralysis and/or lingua plicata (MelkerssoneRosenthal syndrome [MRS]). GC was first described by Miescher in 1945. GC belongs to a spectrum of diseases collectively known as orofacial granulomatosis. The term orofacial granulomatosis was introduced to encompass the broad spectrum of nonnecrotizing granulomatous inflammation in the orofacial region. The etiology of GC and the exact incidence and prevalence of the condition are all unknown. Among the numerous possibilities are genetic factors, chronic infectious odontogenic foci, autoimmune mechanisms, allergic reactions to food additives, such as cinnamaldehyde, carnosine, monosodium glutamate, cocoa, carbone, or sunset yellow (which may not be clinically relevant), sarcoidosis (although oral manifestations usually coincide with systemic signs and symptoms and consist of focal nodular elements), Crohn’s disease (CD), and infectious disorders (eg, mycobacteria, Toxoplasma gondii, Treponema pallidum, or herpes simplex virus). GC is characterized by a nontender swelling of 1 or both lips, and it primarily affects young adults. There is no sex or racial predilection. Initially the swelling is episodic, with the first episode of edema usually subsiding within hours or days. This can lead to a presumptive diagnosis of angioedema. Recurrent painless attacks are the rule, with episodes increasing in duration and eventually leading to persistent indurated swelling. Spontaneous resolution rarely occurs. GC has been recognized as an early manifestation of CD. It may precede, coincide with, or follow the onset of CD by several years. Kano et al suggested that some patients with GC are predisposed to CD. Oral lesions are well-documented clinical features in patients with CD. The spectrum of these lesions described thus far includes aphthous ulceration, labial, buccal, and gingival swellings, mucosal inflammatory hyperplasia (cobblestoning), mucosal tags and fissuring, gingivitis, granulomatous inflammation of minor salivary glands, GC, candidiasis, angular cheilitis, lichen planus, pyostomatitis vegetans, lymphadenopathy, perioral erythema, and dental caries. The prevalence of oral lesions in newly diagnosed patients has been estimated to be up to 48%. GC is found in only 0.5% of patients with CD, although most cases occur in the pediatric population. Histopathologically, GC is characterized by small, noncaseating histiocytic granulomas, often with
J AM ACAD DERMATOL
AUGUST 2014
multinucleated Langerhans giant cells. Surrounding perivascular and interstitial infiltrates of lymphocytes, plasma cells, and histiocytes are also seen. Biopsy specimens obtained during the early stages of the disease may reveal only edema and perivascular aggregations of lymphocytes. Both periodic acideSchiff and ZiehleNeelsen stains are negative for fungi and mycobacteria.
J AM ACAD DERMATOL VOLUME 71, NUMBER 2
JAAD grand rounds 409
For this series, the recommended choices are: 1, d; 2, e; 3, d; 4, e; 5, b; 6, b.
Recurrent bouts of swelling and an unknown etiology make GC a difficult condition to treat. Management is mostly empirical, and the long-term goal must include treatment of any underlying disease. A careful history will help identify any allergens causing GC; therefore, allergen testing may be necessary in cases with a high index of suspicion. Physical examination will exclude any responsible odontogenic foci. Routine investigations of the gastrointestinal tract in patients with GC or MRS with a negative history of gastrointestinal complaints is generally not recommended, nor is it necessary to inform patients with GC of the possibility that they might develop CD. The severity of symptoms often dictates the method of treatment. Benign swelling may be treated with a topical/intralesional corticosteroid (ie, triamcinolone or clobetasol). Worsening of the swelling may be treated with a short course of corticosteroids, although long-term antibiotics (ie, clofazimine, metronidazole, penicillin, and erythromycin) and antiinflammatory drugs (ie, hydroxychloroquine, ketotifen, and sulfasalazine) may be used as potential steroid-sparing alternatives. There are case reports showing the resolution of granulomatous cheilitis with use of thalidomide and infliximab. Surgical intervention may only be considered if the disease in the quiescent stage and there is pronounced disfigurement, but must be followed with several months of intralesional steroids. In summary, orofacial granulomatosis is a unifying term representing a variety of diseases in which there may be several underlying etiologic mechanisms and similar clinical and pathologic presentations, often manifesting at different locations in time, and may only enable a final diagnosis after several years. GC may be misdiagnosed because its clinical manifestations may be independent of or even precede the appearance of CD.
BIBLIOGRAPHY Carr D. Granulomatous cheilitis in Crohn’s disease. Br Med J 1974;4: 636. Carr RD. Is Melkersson-Rosenthal syndrome hereditary? Arch Dermatol 1996;93:426-7. Dupuy A, Cosnes J, Revuz J, Delchier JC, Gendre JP, Cosnes A. Oral Crohn disease: clinical characteristics and long-term follow-up of 9 cases. Arch Dermatol 1999;135:439-42. Field EA, Tyldesley WR. Oral Crohn’s disease revisited—a 10-year review. Br J Oral Maxillofac Surg 1989;27:114-23. Greene RM, Rogers RS 3rd. Melkersson-Rosenthal syndrome: a review of 36 patients. J Am Acad Dermatol 1989;21: 1263-70. Halme L, Meurman JH, Laine P, von Smitten K, Syrjanen S, Lindqvist C, et al. Oral findings in patients with active or inactive Crohn’s disease. Oral Surg Oral Med Oral Pathol 1993; 76:175-81. Hornstein OP. Melkersson-Rosenthal syndrome: a neuromucocutaneous disease of complex origin. Curr Probl Dermatol 1973;5:117-56. Kano Y, Shiohara T, Yagita A, Nagashima M. Granulomatous cheilitis and Crohn’s disease. Br J Dermatol 1990;123:409-41. Kano Y, Shiohara T, Yagita A, Nagashima M. Association between cheilitis granulomatosa and Crohn’s disease. J Am Acad Dermatol 1993;28:801. € Miescher G. Uber essentielle granulomat€ ose Makrocheilie (Cheilitis granulomatosa). Dermatologica 1945;91:57-85. Plauth M, Jenss H, Meyle J. Oral manifestations of Crohn’s disease. An analysis of 79 cases. J Clin Gastroenterol 1991;13:29-37. Rogers RS 3rd. Granulomatous cheilitis, Melkersson-Rosenthal syndrome, and orofacial granulomatosis. Arch Dermatol 2000;136:1557-8. Talbot T, Jewell L, Schloss E, Yakimets W, Thomson AB. Cheilitis antedating Crohn’s disease: case report and literature update. J Clin Gastroenterol 1984;6:349-54. Tan O, Atik B, Calka O. Plastic surgical solutions for MelkerssonRosenthal syndrome facial liposuction and cheiloplasty procedures. Ann Plast Surg 2006;56:268-73. Targan SR, Hanauer SB, van Deventer SJ, Mayer L, Present DH, Braakman T, et al. A short term study of chimeric monoclonal antibody cA2 to tumor necrosis factor alpha for Crohn’s disease. Crohn’s Disease cA2 Study Group. N Engl J Med 1997; 337:1029-35. Thomas P, Walchner M, Ghoreschi K, R€ ocken M. Successful treatment of granulomatous cheilitis with thalidomide. Arch Dermatol 2003;139:136-8. van der Waal RI, Schulten EA, van der Meij EH, van de Scheur MR, Starink TM, van der Waal I. Cheilitis granulomatosa: overview of 13 patients with long-term follow-up—results of management. Int J Dermatol 2002;41:225-9. van der Waal RI, Schulten EA, van de Scheur MR, Wauters IM, Starink TM, van der Waal I. Cheilitis granulomatosa. Eur Acad Dermatol Venereol 2001;15:519-23. Wiesenfeld D, Ferguson MM, Mitchell DN, MacDonald DG, Scully C, Cochran K, et al. Oro-facial granulomatosis: a clinical and pathological analysis. Q J Med 1985;54:101-13. Worsaae N, Christensen KC, Schmidt M, Reibel J. MelkerssonRosenthal syndrome and cheilitis granulomatosa: a clinicopathological study of thirty-three patients with special reference to their oral lesions. Oral Surg Oral Med Oral Pathol 1982;54:404-13.
410 JAAD grand rounds
Zervou F, Gikas A, Merikas E, Peros G, Sklavaina M, Loukopoulos J, et al. Oral lesions in patients with inflammatory bowel disease. Ann Gastroenterol 2004;17:395-401.
Adult with papular eruption on the central aspect of the face Jason D. Michaels, MD, Robert H. Cook-Norris, MD, Julia S. Lehman, MD, and Lawrence E. Gibson, MD Rochester, Minnesota An otherwise healthy 38-year-old man presented with a 5-week history of multiple slightly erythematous to brown papules that began on the right upper eyelid and progressed over 5 weeks to encompass both periocular and perioral distribution. Despite 1 month of treatment with oral erythromycin and topical metronidazole, his condition progressed (Fig 5). Microscopic examination of a representative lesion on the left infraorbital rim revealed palisaded granulomata with prominent central caseation (Fig 6). Gomori methenamine silver and acid-fast bacilli stains were negative for microorganisms. 7. What is the most likely diagnosis? a. Lupus vulgaris b. Acne rosacea c. Perioral dermatitis d. Lupus miliaris disseminatus faciei e. Sarcoidosis 8. Which of the following is a histopathologic finding that can be used to differentiate this patient’s condition from classic granulomatous rosacea? a. Perivascular lymphohistiocytic infiltrate, interspersed with occasional multinucleated cells, neutrophils, plasma cells, and eosinophils b. Caseation necrosis with a ruptured follicular wall involving $ 1 hair follicles c. Myriad small ducts in the dermis with comma-like tails d. Grouped fibroblasts resembling abortive follicular papillae e. There is no distinct histopathologic feature for this condition 9. This condition is observed most commonly in which geographic region or ethnic group? a. Southern America, specifically the Amerindian population of Peru b. The Mediterranean region c. Light-skinned individuals, commonly of Nordic descent d. Dark-skinned individuals, commonly of Japanese descent e. There is no geographic or ethnic predilection for the disease in question
J AM ACAD DERMATOL
AUGUST 2014
10. Which of the following is regarded as an appropriate treatment option for this condition or the associated scarring? a. 1450-nm diode laser b. Isotretinoin c. Minocycline d. Dapsone e. All of the above 11. If left untreated, what is the natural history of this condition? a. Spontaneous resolution within 3 years, with residual scarring b. Overgrowth of convex facial structures because of sebaceous gland hypertrophy c. Persistent growth of cutaneous lesions and rare development of aggressive sarcomatous tumors d. The cutaneous lesions will persist indefinitely without treatment e. None of the above Discussion Lupus miliaris disseminatus faciei (LMDF) is an uncommon chronic inflammatory dermatosis characterized by 1- to 3-mm red to yellow to brown papules affecting primarily the central aspect of the face—namely, the periocular area. Occasionally, lesions may extend beyond the face to the axillae, where they may be mistaken for granulomatous disease caused by aluminum zirconiumecontaining antiperspirants. LMDF is believed to occur more frequently in dark-skinned individuals, especially in the Japanese population. The histopathologic examination varies according to the stage of disease evolution at the time of biopsy. Early lesions reveal superficial perivascular and periappendageal infiltrates composed of lymphocytes, histiocytes, and, rarely, neutrophils. Established lesions are associated with a ruptured follicular wall and have variable features of sarcoidal granuloma (ie, epithelioid cell tubercles), sarcoidal granuloma with abscess (ie, numerous neutrophils surrounding ruptured hair follicle), caseation necrosis (ie, lymphocytes and Langerhans giant cells surrounding a central area of necrosis), and tuberculid granuloma (ie, a dense lymphocytic infiltrate surrounding epithelioid cell tubercles). Late lesions are characterized by mild epidermal thinning and extensive fibrosis with scattered lymphocytes, neutrophils, and histiocytes. Initially believed to be a cutaneous tuberculid because of its distinct histopathologic features, LMDF is now considered a severe variant of granulomatous rosacea. Others argue that LMDF is distinct from granulomatous rosacea because it appears occasionally in areas that are not affected by rosacea
GRAND ROUNDS
JAAD Grand Rounds quiz* Daniel Schulman, PhD, Anthony C. Chu, Jason D. Michaels, MD, Robert H. Cook-Norris, MD, Julia S. Lehman, MD, Lawrence E. Gibson, MD, Whitney S. Alexander, MD, and Eric W. Hossler, MD Learning objectives: At the conclusion of this learning activity, physician participants should be able to assess their own diagnostic and patient management skills and use the results of this exercise to help determine personal learning needs. Instructions: In answering each question, refer to the specific directions provided. Because it is often necessary to provide information occurring later in a series that give away answers to earlier questions, please answer the questions in each series in sequence. ( J Am Acad Dermatol 2014;71:407-14.)
Permanent lip swelling: A precursor of systemic disease? Daniel Schulman, PhD, and Anthony C. Chu London, United Kingdom A 30-year-old woman was referred to a dermatology clinic because of pronounced permanent generalized swelling of her lower lip (Figs 1 and 2). As a 17-year-old, this patient had complained of a 4-month history of intermittent swelling of her right upper lip and had been seen by both ear, nose, and throat and plastic surgeons. At that time, both her C1 esterase level and magnetic resonance imaging scan were reported to be normal. A diagnosis of localized angioneurotic edema was suggested, and she was subsequently lost to followup. Eleven years later, she underwent colorectal surgery for a perianal fistula, followed by drainage of an ischiorectal abscess and another perianal abscess. It was reported that the histologic results of the fistula suggested a diagnosis of Crohn’s disease. A biopsy specimen of the lip was obtained (Figs 3 and 4), and this revealed a diffuse dermal lymphoplasmacytic infiltrate with several well-formed, nonnecrotizing histiocytic granulomas. No fungi or acid-fast bacilli were detected. 1. What is the most likely diagnosis? a. Angioedema b. Posttraumatic c. Infection (nonodontogenic) d. Orofacial granulomatosis e. Actinic cheilitis
The authors, editors, and peer reviewers have no relevant financial relationships. *The section editor for JAAD Grand Rounds is Erin E. Boh, MD. J AM ACAD DERMATOL
2. Granulomatous cheilitis has been shown to be associated with which causative etiology? a. MelkerssoneRosenthal syndrome b. Allergic reactions c. Crohn’s disease d. Sarcoidosis e. All of the above 3. The histologic results will typically reveal which of the following? a. Interface dermatitis b. Pautrier microabscesses c. Pagetoid spread d. Noncaseating granulomatous inflammation e. Perifollicular lymphocytic infiltrate 4. Which of the following has been described as an orofacial manifestation/association of Crohn’s disease? a. Oral ulceration b. Gingivitis c. Granulomatous cheilitis d. Lichen planus e. All of the above 5. What is the reported incidence of granulomatous cheilitis in Crohn’s disease? a. 0.1% b. 0.5% c. 1% d. 5% e. 12% 6. What is not recommended in the routine management of granulomatous cheilitis? a. Chest radiograph and serum angiotensinconverting enzyme level b. Gastrointestinal investigations to exclude Crohn’s disease c. Topical/intralesional steroids d. Contact allergy testing e. Serum radioallergosorbent tests AUGUST 2014
407
408 JAAD grand rounds
Discussion Granulomatous cheilitis (GC) is a rare chronic granulomatous inflammation of the lips of unknown etiology that rarely may be associated with peripheral facial nerve paralysis and/or lingua plicata (MelkerssoneRosenthal syndrome [MRS]). GC was first described by Miescher in 1945. GC belongs to a spectrum of diseases collectively known as orofacial granulomatosis. The term orofacial granulomatosis was introduced to encompass the broad spectrum of nonnecrotizing granulomatous inflammation in the orofacial region. The etiology of GC and the exact incidence and prevalence of the condition are all unknown. Among the numerous possibilities are genetic factors, chronic infectious odontogenic foci, autoimmune mechanisms, allergic reactions to food additives, such as cinnamaldehyde, carnosine, monosodium glutamate, cocoa, carbone, or sunset yellow (which may not be clinically relevant), sarcoidosis (although oral manifestations usually coincide with systemic signs and symptoms and consist of focal nodular elements), Crohn’s disease (CD), and infectious disorders (eg, mycobacteria, Toxoplasma gondii, Treponema pallidum, or herpes simplex virus). GC is characterized by a nontender swelling of 1 or both lips, and it primarily affects young adults. There is no sex or racial predilection. Initially the swelling is episodic, with the first episode of edema usually subsiding within hours or days. This can lead to a presumptive diagnosis of angioedema. Recurrent painless attacks are the rule, with episodes increasing in duration and eventually leading to persistent indurated swelling. Spontaneous resolution rarely occurs. GC has been recognized as an early manifestation of CD. It may precede, coincide with, or follow the onset of CD by several years. Kano et al suggested that some patients with GC are predisposed to CD. Oral lesions are well-documented clinical features in patients with CD. The spectrum of these lesions described thus far includes aphthous ulceration, labial, buccal, and gingival swellings, mucosal inflammatory hyperplasia (cobblestoning), mucosal tags and fissuring, gingivitis, granulomatous inflammation of minor salivary glands, GC, candidiasis, angular cheilitis, lichen planus, pyostomatitis vegetans, lymphadenopathy, perioral erythema, and dental caries. The prevalence of oral lesions in newly diagnosed patients has been estimated to be up to 48%. GC is found in only 0.5% of patients with CD, although most cases occur in the pediatric population. Histopathologically, GC is characterized by small, noncaseating histiocytic granulomas, often with
J AM ACAD DERMATOL
AUGUST 2014
multinucleated Langerhans giant cells. Surrounding perivascular and interstitial infiltrates of lymphocytes, plasma cells, and histiocytes are also seen. Biopsy specimens obtained during the early stages of the disease may reveal only edema and perivascular aggregations of lymphocytes. Both periodic acideSchiff and ZiehleNeelsen stains are negative for fungi and mycobacteria.
J AM ACAD DERMATOL VOLUME 71, NUMBER 2
JAAD grand rounds 409
For this series, the recommended choices are: 1, d; 2, e; 3, d; 4, e; 5, b; 6, b.
Recurrent bouts of swelling and an unknown etiology make GC a difficult condition to treat. Management is mostly empirical, and the long-term goal must include treatment of any underlying disease. A careful history will help identify any allergens causing GC; therefore, allergen testing may be necessary in cases with a high index of suspicion. Physical examination will exclude any responsible odontogenic foci. Routine investigations of the gastrointestinal tract in patients with GC or MRS with a negative history of gastrointestinal complaints is generally not recommended, nor is it necessary to inform patients with GC of the possibility that they might develop CD. The severity of symptoms often dictates the method of treatment. Benign swelling may be treated with a topical/intralesional corticosteroid (ie, triamcinolone or clobetasol). Worsening of the swelling may be treated with a short course of corticosteroids, although long-term antibiotics (ie, clofazimine, metronidazole, penicillin, and erythromycin) and antiinflammatory drugs (ie, hydroxychloroquine, ketotifen, and sulfasalazine) may be used as potential steroid-sparing alternatives. There are case reports showing the resolution of granulomatous cheilitis with use of thalidomide and infliximab. Surgical intervention may only be considered if the disease in the quiescent stage and there is pronounced disfigurement, but must be followed with several months of intralesional steroids. In summary, orofacial granulomatosis is a unifying term representing a variety of diseases in which there may be several underlying etiologic mechanisms and similar clinical and pathologic presentations, often manifesting at different locations in time, and may only enable a final diagnosis after several years. GC may be misdiagnosed because its clinical manifestations may be independent of or even precede the appearance of CD.
BIBLIOGRAPHY Carr D. Granulomatous cheilitis in Crohn’s disease. Br Med J 1974;4: 636. Carr RD. Is Melkersson-Rosenthal syndrome hereditary? Arch Dermatol 1996;93:426-7. Dupuy A, Cosnes J, Revuz J, Delchier JC, Gendre JP, Cosnes A. Oral Crohn disease: clinical characteristics and long-term follow-up of 9 cases. Arch Dermatol 1999;135:439-42. Field EA, Tyldesley WR. Oral Crohn’s disease revisited—a 10-year review. Br J Oral Maxillofac Surg 1989;27:114-23. Greene RM, Rogers RS 3rd. Melkersson-Rosenthal syndrome: a review of 36 patients. J Am Acad Dermatol 1989;21: 1263-70. Halme L, Meurman JH, Laine P, von Smitten K, Syrjanen S, Lindqvist C, et al. Oral findings in patients with active or inactive Crohn’s disease. Oral Surg Oral Med Oral Pathol 1993; 76:175-81. Hornstein OP. Melkersson-Rosenthal syndrome: a neuromucocutaneous disease of complex origin. Curr Probl Dermatol 1973;5:117-56. Kano Y, Shiohara T, Yagita A, Nagashima M. Granulomatous cheilitis and Crohn’s disease. Br J Dermatol 1990;123:409-41. Kano Y, Shiohara T, Yagita A, Nagashima M. Association between cheilitis granulomatosa and Crohn’s disease. J Am Acad Dermatol 1993;28:801. € Miescher G. Uber essentielle granulomat€ ose Makrocheilie (Cheilitis granulomatosa). Dermatologica 1945;91:57-85. Plauth M, Jenss H, Meyle J. Oral manifestations of Crohn’s disease. An analysis of 79 cases. J Clin Gastroenterol 1991;13:29-37. Rogers RS 3rd. Granulomatous cheilitis, Melkersson-Rosenthal syndrome, and orofacial granulomatosis. Arch Dermatol 2000;136:1557-8. Talbot T, Jewell L, Schloss E, Yakimets W, Thomson AB. Cheilitis antedating Crohn’s disease: case report and literature update. J Clin Gastroenterol 1984;6:349-54. Tan O, Atik B, Calka O. Plastic surgical solutions for MelkerssonRosenthal syndrome facial liposuction and cheiloplasty procedures. Ann Plast Surg 2006;56:268-73. Targan SR, Hanauer SB, van Deventer SJ, Mayer L, Present DH, Braakman T, et al. A short term study of chimeric monoclonal antibody cA2 to tumor necrosis factor alpha for Crohn’s disease. Crohn’s Disease cA2 Study Group. N Engl J Med 1997; 337:1029-35. Thomas P, Walchner M, Ghoreschi K, R€ ocken M. Successful treatment of granulomatous cheilitis with thalidomide. Arch Dermatol 2003;139:136-8. van der Waal RI, Schulten EA, van der Meij EH, van de Scheur MR, Starink TM, van der Waal I. Cheilitis granulomatosa: overview of 13 patients with long-term follow-up—results of management. Int J Dermatol 2002;41:225-9. van der Waal RI, Schulten EA, van de Scheur MR, Wauters IM, Starink TM, van der Waal I. Cheilitis granulomatosa. Eur Acad Dermatol Venereol 2001;15:519-23. Wiesenfeld D, Ferguson MM, Mitchell DN, MacDonald DG, Scully C, Cochran K, et al. Oro-facial granulomatosis: a clinical and pathological analysis. Q J Med 1985;54:101-13. Worsaae N, Christensen KC, Schmidt M, Reibel J. MelkerssonRosenthal syndrome and cheilitis granulomatosa: a clinicopathological study of thirty-three patients with special reference to their oral lesions. Oral Surg Oral Med Oral Pathol 1982;54:404-13.
410 JAAD grand rounds
Zervou F, Gikas A, Merikas E, Peros G, Sklavaina M, Loukopoulos J, et al. Oral lesions in patients with inflammatory bowel disease. Ann Gastroenterol 2004;17:395-401.
Adult with papular eruption on the central aspect of the face Jason D. Michaels, MD, Robert H. Cook-Norris, MD, Julia S. Lehman, MD, and Lawrence E. Gibson, MD Rochester, Minnesota An otherwise healthy 38-year-old man presented with a 5-week history of multiple slightly erythematous to brown papules that began on the right upper eyelid and progressed over 5 weeks to encompass both periocular and perioral distribution. Despite 1 month of treatment with oral erythromycin and topical metronidazole, his condition progressed (Fig 5). Microscopic examination of a representative lesion on the left infraorbital rim revealed palisaded granulomata with prominent central caseation (Fig 6). Gomori methenamine silver and acid-fast bacilli stains were negative for microorganisms. 7. What is the most likely diagnosis? a. Lupus vulgaris b. Acne rosacea c. Perioral dermatitis d. Lupus miliaris disseminatus faciei e. Sarcoidosis 8. Which of the following is a histopathologic finding that can be used to differentiate this patient’s condition from classic granulomatous rosacea? a. Perivascular lymphohistiocytic infiltrate, interspersed with occasional multinucleated cells, neutrophils, plasma cells, and eosinophils b. Caseation necrosis with a ruptured follicular wall involving $ 1 hair follicles c. Myriad small ducts in the dermis with comma-like tails d. Grouped fibroblasts resembling abortive follicular papillae e. There is no distinct histopathologic feature for this condition 9. This condition is observed most commonly in which geographic region or ethnic group? a. Southern America, specifically the Amerindian population of Peru b. The Mediterranean region c. Light-skinned individuals, commonly of Nordic descent d. Dark-skinned individuals, commonly of Japanese descent e. There is no geographic or ethnic predilection for the disease in question
J AM ACAD DERMATOL
AUGUST 2014
10. Which of the following is regarded as an appropriate treatment option for this condition or the associated scarring? a. 1450-nm diode laser b. Isotretinoin c. Minocycline d. Dapsone e. All of the above 11. If left untreated, what is the natural history of this condition? a. Spontaneous resolution within 3 years, with residual scarring b. Overgrowth of convex facial structures because of sebaceous gland hypertrophy c. Persistent growth of cutaneous lesions and rare development of aggressive sarcomatous tumors d. The cutaneous lesions will persist indefinitely without treatment e. None of the above Discussion Lupus miliaris disseminatus faciei (LMDF) is an uncommon chronic inflammatory dermatosis characterized by 1- to 3-mm red to yellow to brown papules affecting primarily the central aspect of the face—namely, the periocular area. Occasionally, lesions may extend beyond the face to the axillae, where they may be mistaken for granulomatous disease caused by aluminum zirconiumecontaining antiperspirants. LMDF is believed to occur more frequently in dark-skinned individuals, especially in the Japanese population. The histopathologic examination varies according to the stage of disease evolution at the time of biopsy. Early lesions reveal superficial perivascular and periappendageal infiltrates composed of lymphocytes, histiocytes, and, rarely, neutrophils. Established lesions are associated with a ruptured follicular wall and have variable features of sarcoidal granuloma (ie, epithelioid cell tubercles), sarcoidal granuloma with abscess (ie, numerous neutrophils surrounding ruptured hair follicle), caseation necrosis (ie, lymphocytes and Langerhans giant cells surrounding a central area of necrosis), and tuberculid granuloma (ie, a dense lymphocytic infiltrate surrounding epithelioid cell tubercles). Late lesions are characterized by mild epidermal thinning and extensive fibrosis with scattered lymphocytes, neutrophils, and histiocytes. Initially believed to be a cutaneous tuberculid because of its distinct histopathologic features, LMDF is now considered a severe variant of granulomatous rosacea. Others argue that LMDF is distinct from granulomatous rosacea because it appears occasionally in areas that are not affected by rosacea