Radiation‐induced morphoea treated with UVA‐1 phototherapy

Morphoea is a localized inflammatory disorder of the dermis and subcutaneous fat and radiotherapy is a rarely reported cause (estimated incidence of 2 per 1000). Morphoea is commonly mistaken for an inflammatory recurrence of breast cancer, resulting in unnecessary investigations and treatment. We report the case of a 40‐year‐old woman who developed radiation‐induced morphoea of the breast 7 months following adjuvant radiotherapy. She was treated with topical and systemic steroids as well as psoralen plus ultraviolet (UV)A before proceeding to UVA1 phototherapy. We also review the literature and discuss other management options.     

Postirradiation linear morphoea

Postirradiation morphoea is an uncommon side‐effect of radiotherapy. We report a 74‐year‐old woman who was treated with radiotherapy for endometrial carcinoma. About 3.5 years after the first dose of radiotherapy, the patient developed linear morphoea starting from the radiation port and affecting distant, nonirradiated skin. Lesions of radiation‐induced morphoea are generally described as well‐demarcated, indurated plaques with varying amounts of associated erythema; however, there is no previous publication of unilateral band‐like distribution of morphoea associated with radiotherapy, to our knowledge.     

Success treatment of post-irradiation morphoea with acitretin and narrowband UVB

Post-irradiation morphoea is a rare but under-recognised complication of radiotherapy treatment for breast cancer. Management of this condition is difficult, and many cases are recalcitrant to therapy. A 43-year-old woman with breast cancer received radiotherapy following a mastectomy and partial axillary lymph node dissection, shortly after which she developed a hot, tender, erythematous and indurated plaque at the mastectomy site. Subsequently the skin became retracted, depressed and hyperpigmented. The clinical features, along with histological findings, were consistent with post-irradiation morphoea. Treatment with narrowband ultraviolet B and acitretin 10 mg daily was commenced 5 years following radiotherapy. After 2 months of therapy the patient reported significant improvement in tenderness and range of left arm movement. Objectively the plaque was less indurated and softer to palpation. We propose that this treatment regimen is an option in the management of post-irradiation morphoea.     

Urticaria pigmentosa localized on radiation field

A woman previously treated by radiotherapy for a breast cancer developed urticaria pigmentosa mainly restricted to the irradiation field, without any systemic symptoms. Localized forms of urticaria pigmentosa are exceptional, and their triggering factors are poorly understood. Several hypotheses can be discussed in this peculiar observation, among which a direct role of radiotherapy in the occurrence of cutaneous lesions cannot be ruled out.     

Sustained improvement in urticaria pigmentosa and pruritus in a case of indolent systemic mastocytosis treated with cladribine

Systemic mastocytosis (SM) is a myeloproliferative disorder, characterized by a clonal proliferation of abnormal mast cells accumulating in internal organs and sometimes in the skin, leading to cutaneous and systemic symptoms. Mutations within the gene KIT, which encodes the receptor tyrosine kinase (KIT) on mast cells, is found in most patients with SM. We report a case of a 62‐year‐old woman presenting with a pruritic rash on her limbs and trunk. Several years later she developed gastrointestinal symptoms, associated with raised serum tryptase. Skin and bone marrow biopsies confirmed a diagnosis of SM, initially presenting with urticaria pigmentosa. Responses to multiple therapies, including potent topical steroids, oral antihistamines, phototherapy and the tyrosine kinase inhibitor, nilotinib, were inadequate. Treatment with cladribine (2‐chlorodeoxyadenosine) produced a marked and sustained reduction in her symptoms and serum tryptase level.     

Widespread morphoea following radiotherapy for carcinoma of the breast

We report a case of a 60-year-old lady who was treated with radiotherapy for breast cancer of both breasts 8 years apart. Thirteen years after the first dose of radiotherapy she developed localized morphoea in all the irradiated skin of the chest wall and also the gaiter regions of both lower legs. Radiation-induced localized morphoea has been previously reported; however, there is no previous publication of an occurrence at a distant site as in this case. This case demonstrates that morphoea can occur distant to the original breast carcinoma and site of radiotherapy. We postulate that radiotherapy can induce neoantigen formation, which initiates a T cell response and subsequent tissue growth factor alpha release. Tissue growth factor alpha induces fibroblast activation and collagen production may persist due to a positive feedback mechanism within the fibroblast. The reason why the disease did not generalize remains unclear.     

Prurigo pigmentosa in a patient with primary biliary cirrhosis and Sjögren syndrome

A 44‐year‐old Japanese woman suddenly developed severely pruritic erythematous papules on her trunk in a symmetrical distribution. Biopsy specimens showed the typical histopathological findings of prurigo pigmentosa. She had had recurrent episodes of high fever spikes for several years, and lost 10 kg in the last year. She was diagnosed as primary biliary cirrhosis (PBC) associated with subclinical Sjögren syndrome (SjS). Predonisolone (60 mg/day) for two weeks was effective for the PBC and fever, but not for the prurigo pigmentosa. PBC may be involved in the pathogenesis of this rare skin disease.     

Radiation port xanthogranuloma: solitary xanthogranuloma occurring within the irradiated skin of a breast cancer patient‐report and review of cutaneous neoplasms developing at the site of radiotherapy

Xanthogranulomas are benign lesions composed of macrophages in which some of the cells have vacuolated cytoplasm. They commonly occur in children, referred to as juvenile xanthogranulomata, and are histopathologically characterized by the presence of Touton giant cells. Xanthogranuloma can also occur in adults. A woman who developed a solitary xanthogranuloma within the field of radiotherapy following treatment of her breast cancer is reported. In addition to xanthogranuloma, other benign lesions of keratinocytes, mast cells, endothelial cells and lymphatic cells have been observed at the site of radiation treatment. Also, several malignancies, including hematopoietic cancers, sarcomas, metastatic cancers and common skin cancers, have been observed within radiation ports. A causal relationship in the development of our patient's xanthogranuloma is suggested by the temporal association between the treatment with radiotherapy and the subsequent appearance of a xanthogranuloma directly within the radiation field. Cohen PR, Prieto VG. Radiation port xanthogranuloma and solitary xanthogranuloma occurring within the irradiated skin of a breast cancer patient: report and review of cutaneous neoplasms developing at the site of radiotherapy.     

Catamenial dermatoses: has anyone ever considered prostaglandins?

Catamenial dermatoses are unusual, cyclic, perimenstrual reactions to hormones produced during the menstrual cycle. They occur in a variety of clinical presentations, including urticaria, eczema, fixed drug eruptions, erythema multiforme and anaphylaxis. Autoimmune progesterone dermatitis is the most common, and is caused by an autoimmune response to endogenous progesterone in women of reproductive age. We report a case of catamenial dermatosis in a 42‐year‐old Jamaican woman with a 10‐year history of cyclic blistering and ulcerative eruptions of her mouth and limbs. Her symptoms were fully in keeping with a Stevens–Johnson‐type reaction, and were associated with production of prostaglandins occurring during her menstrual cycle.     

Cutaneous metastasis of inflammatory breast carcinoma mimicking an erythema annulare centrifugum: a sign of locally recurrent cancer

Erythema annulare centrifugum (EAC) is a clinical reaction pattern that includes lupus erythematosus, spongiotic dermatitis (particularly pityriasis rosea), pseudolymphoma and cutaneous B‐cell lymphoma. However, it can be the result of cutaneous metastasis by an internal carcinoma. We present the case of a 38‐year‐old woman with bilateral inflammatory breast cancer following multimodal therapy. After chemotherapy, the patient developed EAC on her back, clinically suspect of subacute cutaneous lupus erythematosus. A skin biopsy of annular lesion revealed dermal lymphatic infiltration by inflammatory breast carcinoma. Immunohistochemically, HER2 overexpression and negativity for hormone receptor are the hallmarks of this disease. Cutaneous metastasis by inflammatory breast carcinoma mimicking EAC is rare, and it has not been described in extramammary locations. Its recognition by the dermatologist is important because it can be a clinical manifestation of locally recurrent cancer.     

Lower limb linear morphoea in a pregnant woman with known Graves' disease and cytomegalovirus immunoglobulin M positivity

We report a case of linear morphoea in a 21‐year‐old woman with known Graves' disease who was also cytomegalovirus (CMV) IgM‐positive and in her early first trimester of pregnancy. The histopathology showed hyalinisation of the dermis with perivascular superficial and deep lymphocytic infiltrates extending into the septae of the subcutis and impinging on adjacent lobules; there was also fibrosis of the subcutis. Magnetic resonance imaging showed T2 high intensity of the subcutaneous tissue and intermuscular planes of the thigh and leg. It is well known that morphoea can follow pregnancy and thyroid disease and that CMV is postulated to contribute in some patients. This case highlights a combination of risk factors for this disease that have not been reported collectively in the literature to our knowledge.     

Eruptive squamous cell carcinoma in a patient treated with concomitant pembrolizumab and imiquimod

Pembrolizumab, a humanized monoclonal antibody against programmed cell death 1, is used for various malignant neoplasms. Toll‐like receptor (TLR) agonists, specifically targeting the TLR9 subfamily (TLR7–9), are treatment options for solid tumors and hematological malignancies. We experienced a case of eruptive squamous cell carcinoma (SCC) in a patient treated concomitantly with pembrolizumab and imiquimod, a TLR7 agonist. A 75‐year‐old woman who was given a diagnosis of bladder cancer with lung metastasis received pembrolizumab for 3 months when she was referred to our department for the evaluation of skin rashes on her hands. Her skin lesions were diagnosed as well‐differentiated SCC and treated with topical imiquimod. Two months after the start of imiquimod, more than 10 reddish papules appeared on her hands. The histological diagnosis of a new plaque was the same as an earlier biopsy. We herein describe this case in detail and provide a published work review.     

Postirradiation pseudosclerodermatous panniculitis with involvement of breast parenchyma: a dramatic example of a rare entity and a pitfall in diagnosis

Postirradiation pseudosclerodermatous panniculitis is a rare complication of external beam radiotherapy. This inflammatory process typically presents as an erythematous indurated plaque in a previously irradiated region of skin. To date, 13 cases have been reported worldwide. We present a case of a 70‐year‐old female who received breast irradiation following conservation surgery for invasive breast carcinoma. In her third year of follow‐up, she developed an enlarging mass, involving the subcutis and underlying breast tissue, associated with mammographically detected coarse calcifications and density, at the surgical site. This was deemed highly suspicious of recurrent malignancy. Following several benign needle core biopsies, she had an excision of the mass. This revealed a lobular panniculitis and irradiation‐induced vascular changes affecting subcutaneous fat and underlying breast tissue. This is the 14th reported case of this rare entity. It is unique in the degree of involvement, affecting breast parenchyma as well as subcutaneous fat, and in its corresponding dramatic clinical and radiographic manifestations.     

Chronic urticaria due to autoreactivity to progesterone

A 23‐year‐old, regularly menstruating woman presented with recurrent urticarial eruptions, which occurred premenstrually. A skin prick test was positive for progesterone, but the urticaria was unresponsive to standard treatments. The patient was treated with goserelin (Zoladex), which suppressed her menstrual cycle, leading to the resolution of her symptoms. Subsequent flares were controlled by further goserelin injections, and the urticaria is currently in remission. However, the risks of inducing menopause artificially are significant, and alternative long‐term solutions may need to be considered in the event of a relapse.     

Lesions of pemphigus vulgaris on irradiated skin

Pemphigus vulgaris (PV) is an autoimmune blistering disease produced by IgG autoantibodies against desmoglein (Dsg)3. Lesions on the skin and mucosa can, in rare cases, be induced by radiotherapy. We report a patient with a history of microprolactinoma and PV, who had only oral lesions from the beginning of her illness but 2 months after treatment with radiotherapy for a breast neoplasia, developed skin lesions limited to the irradiated area. Over the following few months, she also developed autoantibodies against Dsg1.     

Lupus vulgaris–a case diagnosed more than 20 years after onset

A 47‐year‐old Turkish woman developed an erythematous scaly plaque on her thigh over 20 years. Cutaneous sarcoidosis had been diagnosed eight years previously on a skin biopsy. We confirmed Mycobacterium tuberculosis infection by polymerase chain reaction and cultivation of the causative organism from lesional skin. After several months of antituberculous treatment with isoniazid, rifampicin, ethambutol and pyrazinamide, the lesion resolved.     

The cosmetic treatment of urticaria pigmentosa with Nd:YAG laser at 532 nanometers

Background Urticaria pigmentosa is a cutaneous disorder involving infiltration of the skin with mast cells. Histologically the papillary dermis has an increased number of mast cells with an increase in basal layer pigmentation. In addition to possible systemic symptoms, patients with urticaria pigmentosa can suffer emotionally from the cosmetic nature of this skin disease. Objective The purpose was to investigate the use of a diode‐pumped Nd:YAG laser at 532 nm for the treatment of the cosmetic comorbidity of urticaria pigmentosa lesions. Methods A 19‐year‐old white male with urticaria pigmentosa had multiple lesions on the dorsum of the hands and forearms. A test site on the right inner arm was treated with a DioLite? 532 nanometer laser. Because of satisfaction with the treatment of the test site lesions, multiple lesions on the dorsal hands and forearms were also treated with the DioLite? 532 nanometer laser. Results There was a dramatic clinical reduction in the amount of lesions on the dorsum of the hands and forearms. The test site lesions on the right inner arm had not recurred. Conclusion The diode‐pumped Nd:YAG laser at 532 nanometers should be considered part of a dermatologist's armamentarium for the treatment of a patient's cosmetic concerns with lesions of cutaneous mastocytosis.     

Narrowband UVB phototherapy in children: A New Zealand experience

Background: Phototherapy is effective for many dermatoses in adults, but there is a paucity of data for its use in children. Objectives: To review the efficacy and tolerability of narrowband UVB phototherapy in children at a tertiary centre in New Zealand, and determine if there were any factors that differentiated responders from non‐responders. Methods: A prospective analysis of children (<16 years old) who had undergone phototherapy over a 15‐year period. Results: 116 children received phototherapy with a total of 144 courses. Mean age was 11.0 years with the majority being European and having skin phototype II. Atopic dermatitis was the most common indication for treatment followed by psoriasis, pityriasis lichenoides, nodular prurigo, morphea, vitiligo, urticaria pigmentosa and erythropoietic porphyria. Treatment was effective in the majority of children (72%). Most received only one course. For responders, the mean number of treatments was 32.4. The mean dose per treatment to achieve clearance was 886 mJ/cm² and the mean maximum treatment dose per treatment was 1328 mJ/cm². All children tolerated treatment well with 36% developing brief, minimally symptomatic, erythema. Only two children experienced exacerbations of their underlying dermatoses. Conclusions: This study shows that phototherapy is an effective and well‐tolerated treatment modality in children.     

Localized morphoea preceded by a pigmented purpuric dermatosis

Localized morphoea uncommonly occurs in a linear distribution and may present following trauma, although most cases are idiopathic. Pigmented purpuric dermatoses such as lichen aureus may also rarely occur in a linear distribution and have been associated with trauma. A middle-aged man is described who initially presented with lesions typical of lichen aureus in a linear distribution at a site exposed to chronic low-grade trauma. This eruption was transient and clinically underwent spontaneous complete resolution. Several months later he developed localized morphoea in an identical distribution. To our knowledge this is the first reported case of a pigmented purpuric dermatosis, presumably precipitated by trauma, evolving into linear morphoea.     

Bilateral breast keloids in an elderly woman associated with bilateral breast cancers and high concentration of serum tumor growth factor‐β

We report a case of bilateral annular breast keloids in a 72‐year‐old woman who had been suffering from bilateral breast cancers. Histopathologically, the keloids showed unique distribution of α‐SMA+, CD34? myofibroblasts and α‐SMA?, CD34+ fibroblasts depending on the region. High serum levels of tumor growth factor‐β were detected at 6 months after the development of the breast keloids, but not at 10 months. CD163‐positive cells were abundantly detected in the skin of the elevated portion of the keloids. In contrast, these cells were considerably less numerous in the skin of the central healing portion compared with the skin of the elevated expanding portion. One interesting idea based on these results is that high levels of tumor growth factor‐β released from CD163‐positive cells played a crucial role in the formation of breast keloids through active induction of fibroblast differentiation into myofibroblasts. The present case strongly supports the previously proposed idea that keloids can form as a paraneoplastic phenomenon in breast cancer patients with keloid constitution.