Antiepiligrin cicatricial pemphigoid with autoantibodies to the beta subunit of laminin 5 and associated with severe laryngeal involvement necessitating tracheostomy

We report the case of a 67-year-old Korean woman with antiepiligrin cicatricial pemphigoid. The patient's serum immunoprecipitated polypeptides that comigrated with those identified in serum from a representative patient with antiepiligrin cicatricial pemphigoid, and was reactive with the laminin beta3-subunit on immunoblotting. She presented not only with cutaneous, oral and ocular, but also with laryngeal and esophageal involvement. Because the supraglottic stenosis was severe, she had to undergo tracheostomy to maintain airway patency.     

Intravenous immunoglobulin therapy for patients with bullous pemphigoid unresponsive to conventional immunosuppressive treatment

BACKGROUND: Up to 24% of patients with bullous pemphigoid (BP) do not respond to conventional therapy consisting of oral prednisone alone or combined with corticosteroid-sparing immunosuppressive agents (ISAs). They cannot sustain a prolonged clinical remission and continue to have relapses. OBJECTIVE: Fifteen patients with recurrent BP who had experienced several significant side effects resulting from conventional therapy were treated with intravenous immunoglobulin (IVIg) therapy. METHODS: A preliminary dose-finding study tested 7 additional patients to ascertain the optimal IVIg dose of 2 gm/kg per cycle. Objective parameters to determine clinical outcomes were recorded before and after IVIg therapy: doses of prednisone and ISAs, their duration, side effects, clinical course, frequency of relapses and recurrence, response to therapy, number of hospitalizations, total days hospitalized, and quality of life. RESULTS: While receiving IVIg as monotherapy, all study subjects achieved a sustained clinical remission. A statistically significant difference was noted in all the variables studied before and after IVIg therapy. IVIg had a corticosteroid-sparing effect and improved quality of life and did not produce any serious side effects. CONCLUSION: IVIg appears to be an effective alternative in treating patients with severe BP whose disease is nonresponsive to conventional therapy. IVIg may be particularly useful, if treatment is begun early, in patients who are at risk of experiencing serious or potentially fatal side effects from conventional immunosuppressive therapy. After clinical control is achieved, IVIg therapy should be gradually withdrawn and not abruptly discontinued.     

Intravenous immunoglobulin therapy for patients with pemphigus foliaceus unresponsive to conventional therapy.

BACKGROUND: Pemphigus foliaceus (PF) is a chronic autoimmune blistering skin disease that is commonly treated with oral corticosteroids and immunosuppressive therapy. In some patients, PF can be refractory to treatment and the resultant side effects of prolonged immune suppression can be potentially fatal. Alternative therapies are needed. OBJECTIVE: The purpose of this study is to report treatment outcomes with IVIg therapy in 11 patients with severe PF refractory to prednisone and other immunosuppressive therapy. METHODS: Selection criteria included documentation of a biopsy and immunopathology in 11 patients who were resistant to treatment or experienced side effects to conventional therapy. IVIg was administered according to a defined protocol. The parameters used to assess clinical response to IVIg included time observed for effective control of disease, duration of IVIg maintenance therapy, total duration of IVIg, number of IVIg cycles, systemic drug therapy, and the frequency of recurrences and relapses. The pre-IVIg and post-IVIg data were statistically analyzed by means of the SAS UNIVARIATE and 2-sided Wilcoxon sign rank and sign tests. RESULTS: All patients had an effective clinical response and remained in clinical remission for a mean period of 18.6 months after discontinuation of IVIg therapy. Serious side effects from IVIg use were not observed. CONCLUSION: IVIg therapy appears to have potential as a biologic alternative agent in inducing and maintaining clinical remissions in patients with PF who are resistant to more standard conventional treatment. IVIg is effective as monotherapy and may be needed for a period of several months to achieve a long-term clinical remission.     

Positive clinical outcome in a patient with recalcitrant bullous pemphigoid treated with rituximab and intravenous immunoglobulin

A 41‐year‐old white man was treated for bullous pemphigoid (BP) for 4 years, using high‐dose prednisone as well as ciclosporin and mycophenolate mofetil. Sustained clinical improvement was not observed. He suffered several serious side effects. Consequently, he was treated with a combination of rituximab (RTX) and intravenous immunoglobulin (IVIg). He received 12 infusions of RTX in 6 months and monthly IVIg until the end of the therapy. Within 5 weeks of this therapy, appearance of new lesions ceased. Within 8 weeks, all previous lesions resolved and previous medications were discontinued. No hospitalizations, relapses, infections or other serious adverse events occurred. The high levels of pathogenic autoantibody decreased and have remained undetectable. After three infusions of RTX, CD19+ B cells were undetectable and returned to normal levels within 18 months. The patient remains in complete clinical remission off all systemic therapy and free of disease for a 20‐month follow‐up.     

Intravenous immunoglobulin therapy in the treatment of patients with pemphigus vulgaris unresponsive to conventional immunosuppressive treatment

BACKGROUND: Severe pemphigus vulgaris (PV) is conventionally treated with high-dose oral prednisone, usually in combination with an immunosuppressive agent (ISA). Some patients experience significant side effects, which are sometimes fatal, from prolonged immunosuppression. OBJECTIVE: Intravenous immunoglobulin (IVIg) was administered to 21 patients with severe cutaneous and mucosal PV who had not responded to the prolonged use of oral prednisone and multiple ISAs. METHODS: A preliminary dose-determination study tested 7 additional volunteers to ascertain the optimal IVIg dose of 2 g/kg per cycle. Parameters to assess clinical outcome were recorded before and after IVIg therapy. Variables tested were highest dose, total dose, and duration of prednisone and ISAs, their side effects, frequency of recurrence and relapse, duration of IVIg therapy, clinical response, induction and duration of remission, number of hospitalizations, total days of hospital stay, and quality of life. RESULTS: Use of IVIg monotherapy resulted in effective control of disease and produced a sustained remission in the 21 patients. The patients became free of lesions and remained so after finishing IVIg therapy. IVIg had a steroid-sparing effect and produced a high quality of life. Serious side effects from the use of IVIg were not observed. IVIg needs to be gradually withdrawn after achievement of clinical control. CONCLUSION: In patients with PV who do not respond to conventional immunosuppressants, IVIg appears to be an effective treatment alternative. Its early use is of significant benefit in patients who may experience life-threatening complications from immunosuppression. IVIg is effective as monotherapy.     

Successful immunoapheresis of bullous autoimmune diseases: pemphigus vulgaris and pemphigoid gestationis

Background: Immunoapheresis/immunoadsorption is a specific tool to remove immunoglobulins and immune complexes from the circulation. Immunoapheresis is successfully used in various autoantibody‐mediated diseases (such as autoimmune renal disease and others). In dermatology immunoapheresis is increasingly applied as an adjuvant treatment for severe autoimmune bullous diseases. Case report: We successfully employed adjuvant immunoapheresis to treat a 57‐year‐old man with life‐threatening pemphigus vulgaris and a 30‐year‐old pregnant woman with severe pemphigoid gestationis. Immunoapheresis induced a rapid improvement and almost complete clearance of clinical symptoms without notable side effects. The clinical improvement was paralleled by a decline of the pathologic circulating autoantibodies. Following stabilization of his disease with immunoapheresis, the pemphigus vulgaris patient was continued on rituximab and remained almost free of symptoms for the next 12 months. The patient with pemphigoid gestationis was subsequently treated with systemic corticosteroids until the symptoms of her self‐limited disease ceased. Conclusion: Immunoapheresis might represent an excellent therapy for certain patients with severe pemphigus vulgaris or pemphigoid gestationis, unresponsive to conventional treatment regimens. We observed rapid improvement of clinical symptoms and no notable side effects.     

Treatment of severe pemphigus with protein A immunoadsorption, rituximab and intravenous immunoglobulins

BACKGROUND: Pemphigus is a life-threatening autoimmune blistering disease usually treated with high-dose corticosteroids and other immunosuppressants. However, this regimen may prove inadequate in severe cases and cause dangerous side-effects. While protein A immunoadsorption (PAIA) induces a rapid remission in severe pemphigus, the disease usually recurs once the treatment is stopped. In contrast, anti-CD20 antibody rituximab has a delayed onset of action but may lead to a long-term remission of pemphigus. OBJECTIVE: To develop a treatment protocol combining the rapid remission induced by PAIA with the positive long-term effects of rituximab. PATIENTS AND METHODS: Five patients with pemphigus vulgaris and two patients with pemphigus foliaceus were treated with a combination of PAIA, rituximab and conventional immunosuppressants. Patients who failed to respond to this therapy subsequently received intravenous immunoglobulins (IVIg). RESULTS: All seven patients showed a sharp decline of circulating autoantibody levels and rapid improvement of cutaneous and mucosal lesions within 4 weeks of therapy. Long-term remission was induced in three patients and one further patient showed a partial improvement of his disease. The three remaining patients who could not be weaned off PAIA and remained resistant to rituximab treatment showed a good response to IVIg therapy. CONCLUSION: The combination of PAIA and rituximab induces a rapid and durable remission in a subset of patients with severe pemphigus. IVIg therapy appears to be a good treatment option for rituximab nonresponders.     

Corticosteroid/cyclophosphamide pulse treatment in South African patients with pemphigus

Background. Treatment of pemphigus remains a challenge. Corticosteroid/cyclo‐phosphamide pulse treatment has been used to reduce the morbidity associated with long‐term treatment with high‐dose corticosteroids. We describe our experience with pulse treatment in South African patients with pemphigus. Objectives. To assess, in patients who achieved remission of pemphigus, the number of pulses, time and total amounts of steroid and cyclophosphamide required to achieve remission of pemphigus, any side‐effects and the long‐term outcome. Methods. Patient charts were reviewed retrospectively. In those who had remission of disease (resolution of cutaneous lesions and no new mucosal lesions), details of medication were analysed. The relationships between medication, disease severity, type of disease and patient demographics were investigated. Results. Of the 70 patients, 35 achieved remission as defined in the study criteria; 43% of them with < 6 treatment pulses. Neither the type nor severity of pemphigus correlated with the number of treatment pulses or time to remission. However, 73% of patients who presented with severe disease did receive additional oral corticosteroids between treatment pulses. Lymphopenia occurred in 80% of patients, who needed more treatment pulses (P = 0.002) and thus larger total amounts of oral (P = 0.006) and intravenous (P = 0.02) cyclophosphamide to eventually achieve remission. Of the 35 patients who achieved remission, 94% remained in remission, although seven patients required retreatment to achieve this. Conclusions. Pulse treatment in our setting was associated with minimal morbidity. In the nine patients who relapsed, the regimen was not strictly followed, emphasizing the importance of compliance. Our use of low‐dose oral corticosteroids between treatment pulses, early in the management of poorly controlled patients, supports the current modified recommendation of Pasricha.     

Steroid sparing effect of intravenous immunoglobulin therapy in patients with pemphigus foliaceus

Pemphigus foliaceus (PF) is a rare autoimmune cutaneous blistering disease, with only skin involvement. Systemic corticosteroids and immunosuppressive agents are the mainstay of therapy. However, some patients develop multiple side effects to systemic corticosteroids, when they are used in high doses over prolonged periods. In some patients, immunosuppressive agents are not effective or contraindicated. In such patients, alternative treatment modalities are needed. The purpose of this study is to demonstrate the use of intravenous immunoglobulin (IVIg) therapy in seven patients with severe PF, who were steroid-dependent. The following information was recorded in each patient, before and after IVIg therapy initiation: total dose and total duration of prednisone therapy, and number of relapses. In addition, the highest dose and side effects of prednisone therapy, and duration of observation were documented. After the initiation of IVIg treatment, doses of systemic corticosteroids were gradually reduced and eventually discontinued over a mean period of 2.8 months. Thereafter, IVIg was used as monotherapy. A statistically significant difference was noted between pre-IVIg therapy and after the initiation of IVIg therapy in the total dose (p = 0.005), and total duration of prednisone treatment (p = 0.02), and the number of relapses (p = 0.002). In all seven patients, IVIg produced an effective clinical response and demonstrated a steroid-sparing effect. In patients with PF, who are steroid-dependent and in whom use of conventional immunosuppressive agents is contraindicated, IVIg appears a safe and effective agent to induce and maintain a prolonged clinical remission.     

Influence of intravenous immunoglobulin therapy on autoantibody titres to BP Ag1 and BP Ag2 in patients with bullous pemphigoid

BACKGROUND: Bullous pemphigoid (BP) is a subepidermal autoimmune blistering disease, which is characterized by blisters on the skin. Autoantibodies to components of the basement membrane zone are usually observed in the sera of patients with BP. Autoantibodies to the bullous pemphigoid antigens (BP Ag1, 230-kDa desmoplakin protein, and BP Ag2, 180-kDa hemidesmosomal protein) are present in the sera of BP patients. OBJECTIVE: The objective of this study was to report the influence of intravenous immunoglobulin (IVIg) therapy on autoantibody titres to BP Ag1 and BP Ag2. METHODS: In this prospective study, we measured autoantibody titres to both BP Ag1 and BP Ag2, in 10 patients with severe BP, over a period of 18 consecutive months on each patient, using an immunoblot assay. RESULTS: Prior to the initiation of IVIg therapy, the sera of nine patients demonstrated the presence of high autoantibody titres to both BP Ag1 and BP Ag2. One patient had autoantibodies to BP Ag1 only. A statistically significant decline in the autoantibody titres to both BP Ag1 and BP Ag2 was observed after 3 months of receiving the first cycle of IVIg therapy. This gradual decline in autoantibody titres continued until patients were observed to have non-detectable titres to BP Ag1 after 11 months and to BP Ag2 after 10 months of receiving IVIg therapy. Once patients achieved non-detectable titres, these patients were considered to be in a serological remission. This serological remission was sustained for an additional 7 months of observation. CONCLUSION: Autoantibody titres to BP Ag1 and BP Ag2 can be used to monitor the serological response to treatment in patients with BP. Patients with severe BP who are treated with IVIg therapy, as described in our protocol, achieve a long-term serological remission.     

Mycophenolatmofetil als effektive Therapieoption bei vernarbendem Pemphigoid

A 70-year-old woman with cicatricial pemphigoid of the skin and oral mucosa was treated with a combination of mycophenolate mofetil and prednisolone. This treatment led to complete remission of all lesions, and no side effects were observed.     

Role of intravenous immunoglobulin in the treatment of bullous pemphigoid: analysis of current data

BACKGROUND: Conventional therapy of severe bullous pemphigoid (BP) relies on the use of high-dose systemic corticosteroids with or without adjuvants, notably immunosuppressive agents. This approach can result in debilitating and potentially fatal side effects, hence the need to explore alternative therapies. Intravenous immunoglobulin (IVIg) therapy is emerging as one possibility. OBJECTIVE: Our purpose was to determine whether any preliminary conclusion can be drawn about the potential for the use of IVIg to treat patients with severe BP. METHODS: A literature search was done to identify reports, in English-language peer-reviewed journals, on the use of IVIg to treat patients with BP. Reports were examined for information on disease duration, severity, therapies used before and after IVIg, dose and frequency of IVIg administration, and its immediate and long-term effects. RESULTS: Data on treatment of 17 patients indicated that 12 patients (70%) experienced a beneficial clinical response to IVIg. In 5 patients (30%) no clinical benefit was observed. A minimum dose of 2 g/kg per cycle at monthly intervals for 3 months has been the most common approach. However, this should not be perceived as a "standard dose" at the present time. In some patients the use of IVIg appears to permit a systemic corticosteroid-sparing effect. Longer use has achieved sustained clinical remission in some patients. Lack of response was observed in patients who received low-dose IVIg or who received a single infusion only. Minimal side effects in the form of headaches and nausea and vomiting were observed in some patients. CONCLUSION: The present experience consists only of open uncontrolled trials in a few patients and does not allow for definitive conclusions. However, at this time IVIg appears to be a promising agent for the treatment of BP, especially for patients who do not respond to conventional therapy. Adequate doses for longer periods may be required to induce and maintain sustained clinical remissions. Large-scale controlled studies with defined entry criteria, objectives, end points, and long-term follow-up are necessary to determine the specific role of IVIg in the overall management of BP.     

Use of intravenous immunoglobulin therapy during pregnancy in patients with pemphigus vulgaris

Background Pemphigus vulgaris (PV) is a potentially fatal autoimmune disease characterized by the presence of in vivo deposition of antibodies against cell surface antigens desmoglein 1 and desmoglein 2 in the epidermis. Objectives To report the treatment outcomes in pregnant PV patients treated with intravenous immunoglobulin (IVIg) therapy. Methods Eight patients with active disease during pregnancy were treated. Patients were treated with a dose of 2 g/kg/cycle. Seven patients were treated for 2 months on post‐partum basis. Main Outcome Measures were as follows: (i) pregnancy outcome; (ii) presence of neonatal pemphigus; (iii) post‐partum flare; (iv) effect of IVIg on present and future pregnancies; (v) immediate and long‐term side‐effects in the mother and child. Results Patients ages ranged from 20 to 43 years (mean 29.6). All patients had severe and widespread disease involving the skin and multiple mucous membranes. Patients one to seven responded to IVIg therapy and did not have a post‐partum flare. Patient eight could not tolerate IVIg because of intense headaches and significant post‐partum flare. None of the neonates had pemphigus. Three patients who completed the IVIg protocol had normal second pregnancies. One patient who did not complete the protocol had a miscarriage during the second pregnancy. Since last observation, none of the patients have had a recurrence of the disease or another pregnancy. Conclusions The data suggests that IVIg can be useful and safe in treating pregnant patients with PV. No long‐term adverse effects of IVIg in the mother or in the child were observed based on a long‐term follow‐up.     

Treatment of epidermolysis bullosa acquisita with intravenous immunoglobulin in patients non‐responsive to conventional therapy: clinical outcome and post‐treatment long‐term follow‐up

Background Epidermolysis bullosa acquisita (EBA) is a chronic subepidermal blistering disease that is caused by antibodies binding to type VII collagen within anchoring fibrils. It is rare disease with an incidence of 0.25 cases per 1 000 000 population. Objective The objective of this study is to report the treatment outcomes with intravenous immunoglobulin (IVIg) therapy in 10 patients with severe and widespread EBA non‐responsive to conventional therapy. Methods Patients were treated according to a protocol published in a Consensus Statement to treat autoimmune mucocutaneous blistering diseases, including EBA with IVIg. A dose of 2 g/kg/cycle was used. Results Ten patients: four males and six females, all were North American Caucasian. The age at onset varied from 37 to 75 years (mean 57.4). A satisfactory clinical response was observed in all 10 patients. The patients received 16–31 cycles (mean 23.1) of IVIg over a period of 30–52 months (mean 38.8). Once IVIg was initiated, earlier drugs (prednisone, dapsone and others) were gradually withdrawn over a 5–9 month period (mean 7.2). Thereafter, IVIg was used as monotherapy. No serious side‐effects were observed. The follow‐up period since discontinuation of IVIg varied from 29 to 123 months (mean 53.9). During this follow‐up period, recurrence of disease was not observed. Conclusion The data suggest that IVIg can produce a long‐term sustained clinical remission in patients with EBA. In the patients, of this study concomitant therapy could be discontinued and IVIg was used as monotherapy.     

Cicatricial pemphigoid with severe laryngeal involvement necessitating tracheotomy (laryngeal cicatricial pemphigoid)

Cicatricial pemphigoid is a rare autoimmune blistering disease with laryngeal involvement in only 8% of cases. We report the case of a 68-year-old woman with vitiligo, primary hypothyroidism and cicatricial pemphigoid with severe laryngeal involvement necessitating tracheotomy.     

IgA-epidermolysis bullosa acquisita in a child resulting in blindness

Epidermolysis bullosa acquisita (EBA) is an acquired subepidermal bullous disease characterized by IgG autoantibodies directed against type VII collagen, the major component of anchoring fibrils. The classical phenotype of EBA is a non-inflammatory, mechanobullous disease resembling the dystrophic forms of inherited epidermolysis bullosa. Mucous membrane involvement is frequent but usually mild. We report a 1-year-old girl suffering from IgA-EBA, who presented with an initial eruption of disseminated urticarial lesions and tense blisters of the skin but subsequently developed severe oral and ocular lesions reminiscent of cicatricial pemphigoid. Direct immunofluorescence of the skin and buccal mucosa revealed linear IgA and C3 at the basement membrane zone (BMZ). IgA anti-BMZ autoantibodies stained the dermal side of salt-split skin by indirect immunofluorescence and recognized a dermal protein of 290 kDa co-migrating with type VII collagen by immunoblotting. Direct and indirect immunoelectron microscopy revealed IgA deposits overlying the anchoring fibrils. The ocular involvement led to total blindness in spite of intense treatment. This case of childhood IgA-EBA is particularly striking because of the cicatricial pemphigoid phenotype with severe ocular involvement which resulted in blindness. It reinforces the necessity to use modern immunological methods to classify autoimmune bullous diseases in order to allow early and appropriate treatment.     

Solar urticaria successfully treated with intravenous immunoglobulin

Idiopathic solar urticaria (SU) is a rare, debilitating photodermatosis, which may be difficult to treat. First‐line treatment with antihistamines is effective in mild cases, but remission after phototherapeutic induction of tolerance is often short‐lived. Other treatment options include plasma exchange, photopheresis and ciclosporin. We present two cases of severe, idiopathic SU, which were resistant to conventional treatment. Both patients achieved remission after administration of intravenous immunoglobulin (IVIg) and have remained in remission at 13 months and 4 years, respectively. There are only two case reports of successful treatment of solar urticaria with IVIg. In our experience IVIg given at a total dose of 2 g/kg over several 5‐day courses about a month apart is an effective treatment option for severe idiopathic SU. It is also generally safe, even if certainly subject to significant theoretical risks, such as induction of viral infection or anaphylaxis.     

Comparison between intravenous immunoglobulin and conventional immunosuppressive therapy regimens in patients with severe oral pemphigoid: effects on disease progression in patients nonresponsive to dapsone therapy

CONTEXT: Mucous membrane pemphigoid has a wide clinical spectrum. The clinical context was to determine whether pemphigoid disease that initiates in the oral cavity progresses to involve other mucosae and to determine the influence of systemic therapy on such progression. OBJECTIVE: To determine the clinical outcomes and disease progression in patients with oral pemphigoid for whom dapsone therapy was impossible. DESIGN: Retrospective analysis of a cohort of 20 patients with immunopathologic-proven oral pemphigoid studied between September 1, 1994, and October 31, 2000. Twelve patients received conventional therapy that consisted of a combination of oral prednisone with an immunosuppressive agent. Eight patients in whom such therapy was contraindicated received intravenous immunoglobulin therapy. Patients were followed up for 33 to 62 months (mean follow-up, 47.5 months). SETTING: Patients were treated in an ambulatory tertiary medical care facility of a university-affiliated hospital. PATIENTS: The 20 patients had pemphigoid disease limited to the oral cavity only at the initial clinical presentation and when enrolled in the study. MAIN OUTCOME MEASURES: The following variables were compared between the 2 groups of patients: (1) duration of treatment, (2) frequency of relapses, (3) induction of remission, (4) adverse effects of therapy, (5) extra oral involvement, and (6) quality of life. RESULTS: Using the aforementioned factors, the group treated with intravenous immunoglobulin had statistically significant shorter treatment duration, fewer relapses, higher remission rate, fewer adverse effects, no extraoral involvement, and a better quality of life compared with the group who received conventional therapy. CONCLUSIONS: Intravenous immunoglobulin is a safe and effective modality to treat mucous membrane pemphigoid. It seems to be a good option for patients who cannot be treated with dapsone and in whom conventional therapy is contraindicated or results in the development of serious adverse effects. In patients with progressive mucous membrane pemphigoid, intravenous immunoglobulin therapy may arrest disease progression.     

Successful treatment of recalcitrant cicatricial pemphigoid with a combination of plasma exchange and cyclophosphamide

We describe two patients with severe oral cicatricial pemphigoid, one of whom also had severe pharyngeal involvement. Both patients were resistant to treatment with corticosteroids and other standard immunosuppressive therapies. Plasma exchange alone proved to be only temporarily effective, but the combination of plasma exchange with subsequent cyclophosphamide resulted in a remission in both patients. Both patients experienced mild side-effects during the plasma exchange treatment (urticaria and mild hypotension). At present, at follow-up of 6 and 9 years, respectively, the patients have no symptoms of active disease and have not required any further immunosuppressive treatment.     

Coexistence of mucous membrane pemphigoid and connective‐tissue disease

Mucous membrane pemphigoid (MMP) is an autoimmune blistering disease that predominantly affects the mucosa. We report eight patients with MMP who also had systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), or both. Eight women (six white, two Hispanic; mean age of onset 53.5 years [range 44–68]) were studied. In four patients, both diseases were detected simultaneously, and in the other four patients, MMP preceded SLE or MCTD. MMP was widespread and resistant to conventional immunosuppressive therapy, but responded to intravenous immunoglobulin (IVIg). During a mean follow‐up of 10.25 years (range 6–18), three patients had stable SLE/MCTD, whereas in the other five patients the SLE/MCTD required systemic corticosteroids either with or without immunosuppressive agents. Renal, serosal, pulmonary and neurological features were not observed in any patient. At the time of reporting, the MMP was in a prolonged sustained remission in all eight patients. The SLE/MCTD remained mild, did not involve vital organs and had continued with low‐grade activity. In summary, we report the simultaneous occurrence of two rare diseases in a group of patients.