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Rare cutaneous presentation of IgG4 related disease: A case report
P6575
P6320
Rare cutaneous presentation of IgG4 related disease: A case report Weili Yang, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Amy Chia-Ying Peng, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Chien-Hun Huang, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Hamm-Ming Sheu, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Julia Yu-Yun Lee, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Kung-Chao Chang, Department of Pathology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan
Recalcitrant seborrhoeide in an atopic female successfully treated with oral acitretin Rhonda Meys, MBBS, Department of Dermatology, University College London Hospital, London, United Kingdom; Alex Freeman, MBBS, MD, Department of Histopathology, University College London Hospital, London, United Kingdom; Emma Edmonds, MBBS, Department of Dermatology, University College London Hospital, London, United Kingdom A 30-year-old white woman with lifelong atopic eczema presented over a 1-year period with 3 episodes of debilitating, painful crural erosions. Examination revealed an exfoliative, scaly, yellow crusted poikilodermatous flexural eruption involving the axillae, groins, neck, submammary and anticubital fossae in a seborrhoeic distribution. The clinical differential diagnoses included Darier disease, streptococcal infection, pemphigus foliaceous, cutaneous T-cell lymphoma, and flexural psoriasis. Extensive blood investigations revealed a borderline low haemoglobin (11.2 g/dL), low transferrin saturation of 16% and elevated total IgE elevated (646 IU/mL). ASOT, serum zinc, skin swab, fungal scrapings, contact allergy testing, chest radiographs were normal/negative. Skin biopsies demonstrated parakeratosis, hyperkeratosis, and irregular acanthosis without spongiosis. Angiofibroplasia, vertical streaking of the collagen fibres and a mild perivascular lymphocytic infiltrate with focal pigment incontinence were seen. The histologic appearance was consistent with a chronic dermatitis. The bland histologic picture contrasted with the striking clinical appearance. Direct and indirect IMF were negative. The eruption was recalcitrant to several therapies, including super potent topical steroids, oral antibiotics, topical pimecrolimus, tacrolimus, calcipotriol/potent steroid. It was only partially responsive to high dose oral steroids (30 mg). Ciclosporin, azathioprine, and phototherapy were initially considered. In view of the marked hyperkeratosis and the patient’s increasing distress, acitretin 20 mg was commenced after careful counselling to prevent pregnancy. The eruption was highly retinoid sensitive and after 48 hours there was marked clinical improvement. Subsequent treatment with acitretin has been limited by the patient’s resultant worsening eczema. Several features of this case are noteworthy. This is an unusual presentation of a recalcitrant seborrhoeide, distinguished from usual seborrhoeic dermatitis by the rapid response to oral retinoids that has not, to our knowledge, been published previously. Acitretin, a teratogen, has a long half life (requiring a washout period of at least 3 years). We are therefore considering alitretinoin (half life 2-10 hours) as a third-line agent.
IgG4-related disease (IG4RD) comprises a recently recognized systemic syndrome characterized by mass-forming lesions mainly in exocrine tissue with lymphoplasmacytic infiltration and sclerosis, as well as IgG4-producing plasma cell expansion. The serum IgG4 level is increased in these patients. Organs involved include the salivary glands, lacrimal glands, pancreas, biliary tree, retroperitoneum, thyroid gland, and lymph nodes. Cutaneous lesions in IgG4RD have rarely been reported. A 28-year-old man presented with numerous asymptomatic brownish indurated papules and plaques over the trunk and extremities for 3 years. Physical examination showed multiple enlarged lymph nodes over bilateral neck and submental area. Hepatosplenomegaly was not noted. The patient’s serum IgG level was 3470 mg/dL, IgG4 [750 mg/dL. Skin biopsy of brownish plaques revealed dense periadnexal and perivascular lymphoplasmacytic infiltration with increased IgG4-positive plasma cells (21-74 per high power field) and increased IgG4/IgG ratio (25-48%). Lymph node biopsy showed extensive lymphoplasmacytic infiltration in patchy and interstitial patterns. The immunohistochemical stain revealed increased CD138positive and IgG4-positive plasma cells (145;388 per high power field), increased IgG4/IgG ratio ([90%), and no light chain restriction by kappa and lambda stains. The other stain showed that plasma cells were negative for HHV-8. Computed tomography (CT) showed generalized enlarged lymph nodes in the lower neck, bilateral axillary, mesentery, and bilateral iliac, and inguinal regions. Subsequently, the patient was diagnosed with IgG4RD. Methylprednisolone 50 mg/day was initiated and tapered to prednisolone 20 mg qd in 4 weeks. Serum IgG4 level decreased to 610 mg/dL and 585 mg/dL in 4 and 12 weeks, respectively. However, after 4 months, a follow-up CT did not show no significant change. Because of his suboptimal response to steroid, pulse therapy with methylprednisolone 500 mg/day for 3 days was arranged 5 months after the diagnosis. He is now under prednisolone 15 mg daily. No related malignancy was found currently. IgG4RD is a new clinicopathologic entity consistent across a wide range of organ systems. The clinical presentation of skin lesions is rarely described. Herein, we report a case of IgG4RD with significant cutaneous manifestations and lymphadenopathy. Glucocorticoids appeared to be effective for his lymphadenopathy but remained ineffective for his cutaneous lesions. Commercial support: None identified.
P6338 Recalcitrant interstitial granulomatous-like variant of scleromyxedema responding to intravenous immunoglobulin therapy Wei Sheng Tan, MBBS, National Skin Centre, Singapore; Steven Thng, MBBS, National Skin Centre, Singapore Background: Scleromyxedema is the generalized papular and sclerodermoid form of idiopathic cutaneous mucinosis. It may be associated with a monoclonal gammopathy and neurologic symptoms. Typical histologic features include a diffuse deposition of mucin in the papillary and mid reticular dermis, increased collagen deposition, and a proliferation of irregularly arranged fibroblasts. Case report: A 39-year-old man initially presented with a firm, yellowish, waxy papular eruption over his limbs. The clinical impression was that of granuloma annulare, with corresponding supportive skin biopsies showing marked dermal mucin deposition and dermal histiocytic aggregates between collagen bundles and occasional foci of necrobiosis. He failed to respond to potent topical corticosteroids, 3 months of oral isotretinoin 20 mg/day, 6 weeks of narrowband UVB treatment, oral pentoxyphylline, and a 3-month course of oral methotrexate (10-15 mg/week). His condition continued to progress with almost total body surface involvement within 2 years of disease onset. A repeat biopsy subsequently showed typical features of scleromyxedema and CD68 staining was also positive for scattered spindle shaped cells in the dermis. A systemic workup was positive for a monoclonal IgG l gammopathy on serum electrophoresis with no evidence of a plasma cell dyscrasia. The patient failed to respond to 39 sessions of oral psoralen UVA therapy, and was unable to tolerate thalidomide therapy because of the side effects. His skin lesions had near complete clearance after 6 monthly infusions of IVIG (2 g/kg over 4 days) without any adverse effects. Discussion: This case highlights the difficulties in diagnosing and managing this rare variant of scleromyxedema and is the third case reported to date. The condition may mimic interstitial granuloma annulare clinically and histologically. Interstitial drug eruption and interstitial granulomatous dermatitis can also have similar histology findings. Because of the rarity of the condition, no randomized trials have been carried out to compare the efficacy of the various treatments. The same treatments used for granuloma annulare that were attempted in our patient have also been reported to be effective in treating scleromyxedema. However, our patient failed to respond to all the various treatment modalities except for IVIG. Our experience in treating this patient suggests that this variant of scleromyxedema may be more recalcitrant to therapy. Commercial support: None identified.
AB60
J AM ACAD DERMATOL
Commercial support: None identified.
P6898 Recurrence of lepromatous leprosy in a patient with intolerance of dapsone Fred Bernardes Filho, MD, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Gisele Reis Cunha Silva, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Karina de Almeida Pinto Fernandes, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Lorena Branco de Azevedo Cariello, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Talita Fonseca Medeiros Silva, MD, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Thaıs de Barros Castro Alves, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Ver^ onica Vilas-B^ oas Ferrari, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil Background: Even though there is no doubt about the efficacy of preconized schemes, the cure of multibacillary types of lepromatous leprosy by using polychemotherapy is still controversial. It is questioned by positive results of bacilloscopy tests at the moment of therapeutic discharge as shown in many clinical cases. According to the World Health Organization, the estimated risk for recurrence after polychemotherapy is 1.1% for paucibacillary, and 0.8% for multibacillary taking into account 9 years after treatment. The adverse effects of the drugs along with their administration were not considered at the moment of the polychemotherapy. Such fact contributed for the absence of the patients and the adoption of either monotherapeutical schemes or amount of drugs that are not sufficient for the cure. This procedure might contribute for recurrences. Dapsone is considered the leading cause of adverse effects. Case report: Male patient, 64 years old, 1 week ago had underlying bilateral erythematous patches in the jaws and also in the upper left eyelid and hypochromic stain with sensitivity changes in the left axillary line, and also paresthesia in both hands and feet. There was previous treatment with multibacillary polychemotherapy, but then the administration of dapsone was interrupted because of the adverse effects. The treatment continued solely with clofazimine, and the the patient stopped treatment after being cured. 40 mg/day of prednisone started. Positive bacilloscopy. Recurrence with lepromatous leprosy reactions. Alternative scheme was started for lepromatous leprosy without dapsone and gradual reduction of dosage of corticoids. Discussion: Health professionals must be well prepared for both the diagnosis and the management of adverse effects of the drugs in the multidrug therapy. Because of the scarcity of therapeutic measures, the training for early detection of recurrences is fundamental. Research must be encouraged to find new drugs with minor side effects and/or resistance in order to extinguish lepromatous leprosy. Commercial support: None identified.
APRIL 2013
P6320
Rare cutaneous presentation of IgG4 related disease: A case report Weili Yang, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Amy Chia-Ying Peng, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Chien-Hun Huang, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Hamm-Ming Sheu, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Julia Yu-Yun Lee, Department of Dermatology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan; Kung-Chao Chang, Department of Pathology, National Cheng Kung University Medical College and Hospital, Tainan, Taiwan
Recalcitrant seborrhoeide in an atopic female successfully treated with oral acitretin Rhonda Meys, MBBS, Department of Dermatology, University College London Hospital, London, United Kingdom; Alex Freeman, MBBS, MD, Department of Histopathology, University College London Hospital, London, United Kingdom; Emma Edmonds, MBBS, Department of Dermatology, University College London Hospital, London, United Kingdom A 30-year-old white woman with lifelong atopic eczema presented over a 1-year period with 3 episodes of debilitating, painful crural erosions. Examination revealed an exfoliative, scaly, yellow crusted poikilodermatous flexural eruption involving the axillae, groins, neck, submammary and anticubital fossae in a seborrhoeic distribution. The clinical differential diagnoses included Darier disease, streptococcal infection, pemphigus foliaceous, cutaneous T-cell lymphoma, and flexural psoriasis. Extensive blood investigations revealed a borderline low haemoglobin (11.2 g/dL), low transferrin saturation of 16% and elevated total IgE elevated (646 IU/mL). ASOT, serum zinc, skin swab, fungal scrapings, contact allergy testing, chest radiographs were normal/negative. Skin biopsies demonstrated parakeratosis, hyperkeratosis, and irregular acanthosis without spongiosis. Angiofibroplasia, vertical streaking of the collagen fibres and a mild perivascular lymphocytic infiltrate with focal pigment incontinence were seen. The histologic appearance was consistent with a chronic dermatitis. The bland histologic picture contrasted with the striking clinical appearance. Direct and indirect IMF were negative. The eruption was recalcitrant to several therapies, including super potent topical steroids, oral antibiotics, topical pimecrolimus, tacrolimus, calcipotriol/potent steroid. It was only partially responsive to high dose oral steroids (30 mg). Ciclosporin, azathioprine, and phototherapy were initially considered. In view of the marked hyperkeratosis and the patient’s increasing distress, acitretin 20 mg was commenced after careful counselling to prevent pregnancy. The eruption was highly retinoid sensitive and after 48 hours there was marked clinical improvement. Subsequent treatment with acitretin has been limited by the patient’s resultant worsening eczema. Several features of this case are noteworthy. This is an unusual presentation of a recalcitrant seborrhoeide, distinguished from usual seborrhoeic dermatitis by the rapid response to oral retinoids that has not, to our knowledge, been published previously. Acitretin, a teratogen, has a long half life (requiring a washout period of at least 3 years). We are therefore considering alitretinoin (half life 2-10 hours) as a third-line agent.
IgG4-related disease (IG4RD) comprises a recently recognized systemic syndrome characterized by mass-forming lesions mainly in exocrine tissue with lymphoplasmacytic infiltration and sclerosis, as well as IgG4-producing plasma cell expansion. The serum IgG4 level is increased in these patients. Organs involved include the salivary glands, lacrimal glands, pancreas, biliary tree, retroperitoneum, thyroid gland, and lymph nodes. Cutaneous lesions in IgG4RD have rarely been reported. A 28-year-old man presented with numerous asymptomatic brownish indurated papules and plaques over the trunk and extremities for 3 years. Physical examination showed multiple enlarged lymph nodes over bilateral neck and submental area. Hepatosplenomegaly was not noted. The patient’s serum IgG level was 3470 mg/dL, IgG4 [750 mg/dL. Skin biopsy of brownish plaques revealed dense periadnexal and perivascular lymphoplasmacytic infiltration with increased IgG4-positive plasma cells (21-74 per high power field) and increased IgG4/IgG ratio (25-48%). Lymph node biopsy showed extensive lymphoplasmacytic infiltration in patchy and interstitial patterns. The immunohistochemical stain revealed increased CD138positive and IgG4-positive plasma cells (145;388 per high power field), increased IgG4/IgG ratio ([90%), and no light chain restriction by kappa and lambda stains. The other stain showed that plasma cells were negative for HHV-8. Computed tomography (CT) showed generalized enlarged lymph nodes in the lower neck, bilateral axillary, mesentery, and bilateral iliac, and inguinal regions. Subsequently, the patient was diagnosed with IgG4RD. Methylprednisolone 50 mg/day was initiated and tapered to prednisolone 20 mg qd in 4 weeks. Serum IgG4 level decreased to 610 mg/dL and 585 mg/dL in 4 and 12 weeks, respectively. However, after 4 months, a follow-up CT did not show no significant change. Because of his suboptimal response to steroid, pulse therapy with methylprednisolone 500 mg/day for 3 days was arranged 5 months after the diagnosis. He is now under prednisolone 15 mg daily. No related malignancy was found currently. IgG4RD is a new clinicopathologic entity consistent across a wide range of organ systems. The clinical presentation of skin lesions is rarely described. Herein, we report a case of IgG4RD with significant cutaneous manifestations and lymphadenopathy. Glucocorticoids appeared to be effective for his lymphadenopathy but remained ineffective for his cutaneous lesions. Commercial support: None identified.
P6338 Recalcitrant interstitial granulomatous-like variant of scleromyxedema responding to intravenous immunoglobulin therapy Wei Sheng Tan, MBBS, National Skin Centre, Singapore; Steven Thng, MBBS, National Skin Centre, Singapore Background: Scleromyxedema is the generalized papular and sclerodermoid form of idiopathic cutaneous mucinosis. It may be associated with a monoclonal gammopathy and neurologic symptoms. Typical histologic features include a diffuse deposition of mucin in the papillary and mid reticular dermis, increased collagen deposition, and a proliferation of irregularly arranged fibroblasts. Case report: A 39-year-old man initially presented with a firm, yellowish, waxy papular eruption over his limbs. The clinical impression was that of granuloma annulare, with corresponding supportive skin biopsies showing marked dermal mucin deposition and dermal histiocytic aggregates between collagen bundles and occasional foci of necrobiosis. He failed to respond to potent topical corticosteroids, 3 months of oral isotretinoin 20 mg/day, 6 weeks of narrowband UVB treatment, oral pentoxyphylline, and a 3-month course of oral methotrexate (10-15 mg/week). His condition continued to progress with almost total body surface involvement within 2 years of disease onset. A repeat biopsy subsequently showed typical features of scleromyxedema and CD68 staining was also positive for scattered spindle shaped cells in the dermis. A systemic workup was positive for a monoclonal IgG l gammopathy on serum electrophoresis with no evidence of a plasma cell dyscrasia. The patient failed to respond to 39 sessions of oral psoralen UVA therapy, and was unable to tolerate thalidomide therapy because of the side effects. His skin lesions had near complete clearance after 6 monthly infusions of IVIG (2 g/kg over 4 days) without any adverse effects. Discussion: This case highlights the difficulties in diagnosing and managing this rare variant of scleromyxedema and is the third case reported to date. The condition may mimic interstitial granuloma annulare clinically and histologically. Interstitial drug eruption and interstitial granulomatous dermatitis can also have similar histology findings. Because of the rarity of the condition, no randomized trials have been carried out to compare the efficacy of the various treatments. The same treatments used for granuloma annulare that were attempted in our patient have also been reported to be effective in treating scleromyxedema. However, our patient failed to respond to all the various treatment modalities except for IVIG. Our experience in treating this patient suggests that this variant of scleromyxedema may be more recalcitrant to therapy. Commercial support: None identified.
AB60
J AM ACAD DERMATOL
Commercial support: None identified.
P6898 Recurrence of lepromatous leprosy in a patient with intolerance of dapsone Fred Bernardes Filho, MD, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Gisele Reis Cunha Silva, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Karina de Almeida Pinto Fernandes, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Lorena Branco de Azevedo Cariello, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Talita Fonseca Medeiros Silva, MD, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Thaıs de Barros Castro Alves, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil; Ver^ onica Vilas-B^ oas Ferrari, Instituto de Dermatologia Prof. Rubem David Azulay da Santa Casa da Miseric ordia do Rio de Janeiro (IDPRDA/SCMRJ), Rio de Janeiro, Brazil Background: Even though there is no doubt about the efficacy of preconized schemes, the cure of multibacillary types of lepromatous leprosy by using polychemotherapy is still controversial. It is questioned by positive results of bacilloscopy tests at the moment of therapeutic discharge as shown in many clinical cases. According to the World Health Organization, the estimated risk for recurrence after polychemotherapy is 1.1% for paucibacillary, and 0.8% for multibacillary taking into account 9 years after treatment. The adverse effects of the drugs along with their administration were not considered at the moment of the polychemotherapy. Such fact contributed for the absence of the patients and the adoption of either monotherapeutical schemes or amount of drugs that are not sufficient for the cure. This procedure might contribute for recurrences. Dapsone is considered the leading cause of adverse effects. Case report: Male patient, 64 years old, 1 week ago had underlying bilateral erythematous patches in the jaws and also in the upper left eyelid and hypochromic stain with sensitivity changes in the left axillary line, and also paresthesia in both hands and feet. There was previous treatment with multibacillary polychemotherapy, but then the administration of dapsone was interrupted because of the adverse effects. The treatment continued solely with clofazimine, and the the patient stopped treatment after being cured. 40 mg/day of prednisone started. Positive bacilloscopy. Recurrence with lepromatous leprosy reactions. Alternative scheme was started for lepromatous leprosy without dapsone and gradual reduction of dosage of corticoids. Discussion: Health professionals must be well prepared for both the diagnosis and the management of adverse effects of the drugs in the multidrug therapy. Because of the scarcity of therapeutic measures, the training for early detection of recurrences is fundamental. Research must be encouraged to find new drugs with minor side effects and/or resistance in order to extinguish lepromatous leprosy. Commercial support: None identified.
APRIL 2013