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Acral persistent papular mucinosis is a rare subtype of localized lichen myxedematosus. We report a case of a 41-year-old woman with acral persistent papular mucinosis. She had a 5-year history of multiple, scattered, whitish papules, on the back of both hands, of 2-5 mm in diameter. Histopathological examination revealed a focal, fairly circumscribed area with sparse collagen fibers in the upper and middle dermis. The circumscribed area was positively stained with Alcian blue. The papules were clinically and histologically diagnosed as acral persistent papular mucinosis. We also summarized 24 Japanese acral persistent papular mucinosis cases, including ours. Although acral persistent papular mucinosis occurs predominantly in women according to overseas reports, the male : female ratio is 5:7 in Japan. It has been reported that tranilast (N-[3,4-dimethoxycinnamoyl]-anthranilic acid) was effective in two Japanese cases. In our case, the patient had been treated with tranilast for 2 months without improvement. Further studies are required to confirm the efficacy of tranilast for acral persistent papular mucinosis.

Background Cutaneous focal mucinosis (CFM) or focal dermal mucinosis is a benign reactive process categorized as a primary mucinosis. Skin biopsy is essential for diagnosis, as the clinical appearance is often non-specific. Follicular induction is a phenomenon whereby the epidermis is induced by an underlying process to form primitive or mature hair follicles, and is commonly seen overlying dermatofibromas. Follicular induction has been rarely described in CFM. Methods We performed a retrospective histological review of lesions of CFM confirmed by skin biopsy from 2010 to 2015 in our department. Results We found that 11% (11/98) of CFM lesions showed follicular induction. Cytokeratin 20 (CK20) immunostaining was performed on all 11 of these biopsies that showed follicular induction and highlighted an increased density of CK20+ Merkel cells within the basaloid epidermal proliferations. Conclusion As superficial basal cell carcinomas (BCC) often show a mucinous stroma around the basaloid islands, CFM with follicular induction may closely mimic a BCC histologically, particularly in superficial shave biopsies. Therefore, it is important that dermatopathologists be aware of this phenomenon. Furthermore, CK20+ staining within the basaloid epithelial proliferations may be helpful in differentiating CFM with follicular induction from a BCC.

Colony-stimulating factor 1 receptor (CSF1R) inhibitors represent a new class of immune-modulatory drugs, mostly investigated in clinical trials in different malignant neoplasms. Four patients, diagnosed with recurrent or advanced malignant neoplasm and treated with a combination of anti-programmed death ligand 1 and anti-CSF1R monoclonal antibodies, developed an asymptomatic cutaneous eruption characterized by an ill-defined pseudoedematous to waxy diffuse infiltration with a reticular cobblestone-like pattern. Histopathological examination revealed diffuse mucin deposition involving the superficial and mid-dermis with fragmented and scattered elastic fibers. The exact pathogenic mechanisms implicated in the development of mucin deposits in patients treated with CSF1R inhibitors remain to be elucidated. A reduced degradation and clearance of components of the extracellular matrix by macrophages secondary to CSF1 pathway inhibition may be hypothesized. Shredding and fragmentation of elastic fibers may be a result of the increased accumulation of mucopolysaccharides. This observation illustrates the new spectrum of skin-related toxicities secondary to new targeting therapies. This may contribute to a better understanding of the underlying pathogenic mechanisms in skin diseases characterized by a persistent dermal glycosaminoglycan deposition.

Nodular lichen myxoedematosus is a localised form of lichen myxoedematosus, a chronic idiopathic cutaneous mucinosis of known aetiology. Ustekinumab is a human interleukin-12/23 monoclonal antibody that could directly or indirectly increase mucin production. Herein, we report for the first time a case of nodular lichen myxoedematosus associated with ustekinumab.

Dermal mucinoses are a heterogeneous group of disorders characterized by abnormal deposition of dermal mucin, an amorphous substance composed of hyaluronic acid and sulfated glycosaminoglycans. We describe two cases of dermal mucinosis in the setting of chronic venous insufficiency. Both patients presented with painful, edematous lower extremity plaques. Biopsies of all lesions showed striking dermal mucin deposition, a slight increase in small blood vessel density, slightly thickened vessel walls and no inflammation. Neither patient showed laboratory or clinical findings consistent with a secondary mucinosis such as thyroid dysfunction, lupus erythematosus, dermatomyositis, scleroderma, granuloma annulare, graft-vs.-host disease or mucin deposition post-ultraviolet or photochemotherapy treatment. Both patients were diagnosed with localized cutaneous mucinosis secondary to venous insufficiency. The clinicopathological features of this entity are described, and a pathogenic mechanism is proposed.

Reticular erythematous mucinosis (REM) is a rare chronic mucinosis. Histologically, the presence of mucin in the upper dermis is the most specific feature. A 73-year-old woman presented to our outpatient clinic with a 4-year history of netlike macular erythema with slight edema on her left arm. She was diagnosed as having REM on the basis of the clinical picture and histological findings. She was treated with ultraviolet B irradiation using the VTRAC Excimer Lamp system with favorable response. This is the first reported case that was treated with a 308-nm excimer lamp.

Nodular mucinosis is an extraordinary stromal lesion of the breast. The usual clinical presentation is that of an oozing, slow-growing, soft, non-tender, lobulated mass in the subareolar region. Histologically, it is a non-encapsulated myxoid/mucinous lesion with a sparse infiltrate of spindle cells within a collagenized stroma. The histogenesis of nodular mucinosis is undetermined but the pattern of staining of the spindle cells suggests it might be of myofibroblastic origin. We herein report for the first time in the English literature a case of nodular mucinosis occurring in a supernumerary nipple. We also discuss the main differential diagnoses and review the literature of previously published cases of this entity.

Cutaneous mucinosis affects primarily shar-pei dogs. Hyaluronic acid (HA) is considered to be the main component of mucin and CD44 is the major cell surface receptor of HA, necessary for its uptake and catabolism. The aims of this study were to identify the composition of the mucin in cutaneous mucinosis of shar-pei dogs, investigate the correlation between the deposition of HA and the expression of CD44, and determine whether shar-pei dogs with cutaneous mucinosis presented with elevated levels of serum HA. In skin biopsies, the mucinous material was stained intensely with Alcian blue and bound strongly by the hyaluronan-binding protein. No correlation was found between the degree of HA deposition in the dermis and the expression of CD44 in the skin of shar-pei dogs affected or unaffected by cutaneous mucinosis. A clear positive correlation was found between the existence of clinical mucinosis and the serum HA concentration. In control dogs, serum HA ranged from 155.53 to 301.62 microg L(-1) in shar-pei dogs; without mucinosis it ranged from 106.72 to 1251.76 microg L(-1) and in shar-pei dogs with severe mucinosis it ranged between 843.51 to 2330.03 microg L(-1). Altogether, the results reported here suggest that mucinosis of shar-pei dogs is probably the consequence of a genetic defect in the metabolism of HA.

Progressive mucinous histiocytosis is a rare, benign, non-Langerhans' cell histiocytosis limited to the skin. Ten cases – all women – in four families and one sporadic case have been described in the literature. The disorder usually begins in childhood and progresses slowly. We report two sporadic cases of adult-onset progressive mucinous histiocytosis in unrelated African-American women, aged 48 and 55 years, respectively, who developed red-brown and flesh-coloured, asymptomatic papules on the face, the arms and the legs without truncal, mucosal or visceral involvement. The lesions showed no spontaneous regression. Both patients lacked associated systemic symptoms, including polyuria, polydipsia or seizures. There was no underlying hyperlipidaemia, paraproteinaemia or lymphoproliferative disease. No family history of similar lesions could be identified. Light microscopy revealed dermal proliferation of spindle-shaped histiocytes with abundant mucin deposition. Electron microscopy demonstrated a high number of myelin figures or zebra bodies in the cytoplasm of histiocytes. On immunohistochemistry, positive staining with macrophage markers – CD68, HAM56 and lysozyme – and factor XIIIa, a transglutaminase present in dermal dendrocytes, and negative staining with Langerhans' cell markers – CD1a and S100 – and CD34, a marker present in dermal dendritic cells derived from uncommitted mesenchymal cells, were observed.

According to a recent classification, self-healing papular mucinosis (SHPM) is a subtype of papular mucinosis (also known as lichen myxedematosus), which is in turn a type of idiopathic localized cutaneous mucinosis. SHPM tends to occur in children, but there have been a few reports of an adult type. We report a 70-year-old Japanese woman who presented with reddish, rice-kernel-sized papules of a few days' duration on her right arm. She had a 25-year history of rheumatoid arthritis, which had been well treated with a low dose of corticosteroid as well as some other medications. No paraproteinemia or thyroid dysfunction were observed. The eruptions spontaneously resolved within 2.5 months of onset. Histological findings showed a well-circumscribed mucinous stroma surrounded by dermal mesenchymal cells, such as fibroblast-like cells in the middle of the dermis. Immunohisto-chemically, these cells were positive only for vimentin on the mucinous lesion. On the circumference of the mucinous lesion, these cells expressed either CD34 or factor XIIIa (FXIIIa). Because vimentin was common to dermal mesenchymal cells, we defined the cells expressing CD34 or FXIIIa, except for vimentin+ cells lacking CD34 or FXIIIa, as dermal dendritic cells (DDC). The findings of the present case suggest that CD34+ or FXIIIa+ DDC and tryptase-positive mast cells on the perilesional area in combination with vimentin+ cells on the mucinous lesion might have given rise to the dermal deposition of mucin in our case. These cells, which are possibly activated in an autoimmune manner associated with rheumatoid arthritis, might play important roles in the development of dermal deposition of mucin in SHPM.

A 15-year-old boy first noticed multiple firm papules on his right upper chest two years before presenting to our clinic. These papules were densely distributed and showed epidermal nevus-like linear arrangement at some sites. The number, size, and distribution of these papules remained unchanged for one year of our observation. The papules flattened leaving scars and did not recur within a year after one shaving abrasion using a scalpel. Histopathologically, epidermis of the papule displayed acanthosis with elongated rete ridges. Accumulation of mucin was apparent in the papillary and subpapillary dermis. In mucinous nevus, the origin of cells with nevoid proliferation is obscure. In contrast with common collagenous connective tissue nevus, it is hard to define the localized persistent mucin accumulation as a nevoid manifestation. The present case of mucinous nevus might be caused by significantly stimulated glycosaminoglycan synthesis in a kind of epidermal nevus without extreme hyperkeratosis.

Background: Reticular erythematous mucinosis (REM) syndrome is a rare disorder. Its clinical course is cyclic with remissions and exacerbations. In this disease, photosensitivity has previously been noticed but rarely demonstrated. We report three new cases with positive photobiological investigation. Case reports: Three patients (two males, one female) with a mean age 47 years were seen with reticular erythematous papules on the upper chest and or back. After sun exposure, the lesions were exacerbated. Skin biopsies showed dermal lymphocytic perivascular infiltration with mucin deposition between collagen bundles. Direct immunofluorescence was negative. Antinuclear antibodies were absent. In cabin, ultraviolet (UV)A exposure reproduced clinically and histologically REM lesions in our cases. UVA and UVB provocating phototests were negative. In all patients treatment with oral antimalarials and external photoprotection was effective. Conclusions: In our patients, we confirm the photosensitive feature of REM syndrome by provocative irradiation in UVA cabin. The mechanism of triggering is actually unclear. It is supposed that UV radiation, heat, and perspiration are necessary to reveal this affection.

A rare case of mucinous nodules on the skin overlying rheumatoid synovitis in a 79-year-old man is described. The patient had a five year history of RA. The patient had complained of arthralgia in the left elbow joint, and X-ray demonstrated narrowing of the joint space along with bone destruction. He underwent an intraarticular injection of dexamethazone and lidocaine. Three weeks later, he noticed two dome-shaped nodules about 5 mm in size developing on the elbow. Histopathological examination demonstrated poorly defined mucinous nodules in the upper dermis. The mucinous material positively stained with alcian blue and colloid iron, and was metachromatic with toluidine blue. These positive stainings disappeared after hyaluronidase digestion. Five to six weeks after being resected, both nodules recurred. Lesional injections of triamcinolone were effective. The intraarticular injections preceding the appearance of the nodules might have created channels from the joint space to the skin. Leakage of activated synovial cells, which produced hyaluronic acid, through the channels might have caused the mucinous stroma of the nodules.

A 23-year-old woman presented with a 2-week history of an extensive facial eruption consisting of markedly crusted arciform plaques covered with a haemorrhagic eschar that commenced as pruritic follicular pustules. These were associated with facial oedema but no systemic symptoms or fever. Two skin biopsies revealed prominent tissue eosinophilia with eosinophilic folliculitis and areas of follicular mucinosis, as well as haemorrhagic scale crust over the surface. The clinical and histological findings may represent an example of a necrotizing variant of eosinophilic folliculitis that has recently been reported in atopic individuals. This patient, however, did not have evidence of atopy and appeared to have a florid form of Ofuji's disease that responded rapidly to indomethacin treatment.

The proband was evaluated for abdominal pain at age 61 years and found to have bilateral solid renal masses. He underwent a partial left nephrectomy. Two benign renal cysts were present, and two renal cell adenocarcinomas measuring 5.3 cm and 0.7 cm in greatest diameters were found. Histologically, both tumors contained both clear and granular cell types. He had a total right nephrectomy 2 months later. Fourteen or 15 lesions were scattered over the surface of this kidney, so an adrenal-sparing radical right nephrectomy was performed. On sectioning, the diameter of the largest tumor was 4 cm, and histologically this was described as renal cell carcinoma, clear cell type, Fuhrman grade II. One year later, the patient shows no evidence of recurrent disease. The proband's past medical history was notable for gastroesophageal reflux, degenerative disk disease, hyperlipidemia, and mild hyperglycemia. A genetics consultation was requested because of a family history of renal cancers in three maternal relatives (Fig. 1). On examination, the proband was a nondysmorphic man of normal stature with no unusual findings, except for widespread skin findings (Fig. 2). On his face were innumerable, small, slightly yellowish or flesh-colored papules on the forehead and especially over the cheeks. No intraoral lesions were seen. Around the neck were innumerable, dome-shaped, firm, ivory-colored papules ranging in size from 1 to 10 mm. Very tiny, similar papules were present over the trunk and in the antecubital area. Numerous acrochordons and some cherry angiomas were present. Several biopsies from the neck and upper back were obtained and were thought suggestive of papular mucinosis. The patient's mother had had a skin biopsy of a similar lesion from her face in 1947, interpreted as an adenoma sebaceum. No angiofibromas were evident on our patient's slides nor on physical examination. Figure 1. Pedigree of family with Birt-Hogg–Dube Syndrome Download figure to PowerPoint Figure 2. Photograph of the back of the neck of a 61–year-old man with Birt-Hogg–Dube Syndrome Download figure to PowerPoint At this point, diagnoses under consideration included von Hippel-Lindau disease (VHL), a familial chromosome no. 3 translocation, hereditary renal cell cancer (gene unknown), tuberous sclerosis (TS), or Birt-Hogg–Dube syndrome (BHDS). The patient underwent a variety of tests to evaluate these possibilities. He had no evidence of hemangioblastomas of the central nervous system, no liver or pancreatic cysts, no ocular findings for VHL or TS, and no hypomelanotic macules on skin exam. Chromosome analysis from peripheral blood showed a normal 46,XY karyotype. Direct DNA mutational analysis of the VHL gene detected no mutations. Additional biopsies from the patient's skin lesions were obtained, and tissue blocks from 1947 from his mother were resectioned (Figs 3 and 4). Upon further review, multiple fibrofolliculomas were found in both mother and son in skin biopsies. In aggregate, these data established a diagnosis of BHDS. Figure 3. (A) Skin biopsy from the upper back of the proband showing a dome shaped papule with abundant mucin in the papillary dermis. Follicular changes are suggestive of fibrofolliculoma. (H&E, 12.5X). (B) Mucinosis in the papillary dermis is highlighted with alcian blue stain (ABPAS, 25X) Figure 4. (A) Fibrofolliculoma seen on skin biopsy of a facial papule from the mother of the proband (H&E, 25X). (B) Fibrofolliculoma from the mother of the proband with sebaceous gland enlargement and mucin deposition highlighted by alcian blue stain (ABPAS, 25X) In addition to the renal cancers, the patient's and/or cousin's medical record reported that others in the family had the same skin manifestations, including his mother, maternal grandmother, maternal uncle, and two maternal first cousins by report. Cousin III.6′s medical record showed a clinical diagnosis initially of ‘‘sebaceous hyperplasia’' and upon skin biopsy was said to have ‘‘innumerable trichoepitheliomas over his face’'. He was treated with liquid nitrogen with good results. Unfortunately, we were not able to obtain tissue for review on the other renal cancers in relatives. Additional findings of note are a spontaneous pneumothorax in our patient's daughter and melanoma in our patient's brother and son. This family has been invited to participate in genetic studies underway at the National Cancer Institute.

Summary We present female twins with reticular erythematous mucinosis (REM syndrome). Remarkably, the lesions developed in both sisters almost at the same time in the same locations after UV exposure. Reports of familial manifestations of REM syndrome are very rare and an association to a distinct HLA constellation has not been proven. Our report clearly suggests a genetic predisposition.

A 70-year-old woman presented with a 10-year history of cutaneous lesions on her calves that worsened with solar exposure. Her past medical history was irrelevant. Physical examination revealed 10–15 erythematous, firm nodules with a diameter of 2–5 mm, which were slightly painful on palpation. The lesions were located on both calves, although some elements extended to the posterior side of the thighs ( Fig. 1). General physical examination did not show any abnormalities. A biopsy specimen from one of the elements showed a normal epidermis and, in the upper dermis, a slight inflammatory infiltrate of mononuclear cells, with the presence of splits between collagen fibers ( Fig. 2). Alcian blue stain at pH 2.5 revealed the existence of mucinous material in these spaces ( Fig. 3). An electron microscopic study did not show any abnormalities. Figure 1. Clinical appearance of the lesions Figure 2. Histopathologic picture (hematoxylin and eosin, ×40) Figure 3. Alcian blue stain showing the presence of mucin in the dermis (Alcian blue, ×100) The patient was diagnosed as having papular mucinosis. The following studies were normal or negative: complete cell blood count, serum biochemistry, serum protein electrophoresis, serum immunoelectrophoresis, serologic test for human immunodeficiency virus (HIV), hepatitis B and C viruses, and syphilis, antinuclear antibodies, and thyroid hormone levels. No treatment was carried out.

A 19-year-old Hispanic woman presented to our clinic with a chief complaint of multiple pink papules on her face (Figure 1). The lesions were asymptomatic, had appeared several years before her consultation, and had remained stable in size. Similar lesions on her trunk and extremities had spontaneously resolved before presentation. She had no significant medical history and was not on any medications. Cutaneous examination was remarkable for a few scattered, well-demarcated, slightly raised pink papules and plaques with no scale, ranging from 0.5 to 1 cm in diameter. The lesions were distributed on the right cheek, right temple, left lower eyelid, and neck area. The rest of the cutaneous examination was unremarkable. A punch biopsy was performed from a lesion near the left premandibular area. Hematoxylin and eosin staining of the biopsy specimen revealed superficial perivascular dermatitis with a slight increase in the number of fibroblasts (Figure 2). Colloidal iron staining was diffusely positive with replacement of collagen with mucinous material in the dermal layer (Figure 3). Laboratory values for a complete blood cell count, metabolic panel, serum protein electrophoresis, urine protein electrophoresis, thyroid-stimulating hormone, and antinuclear antibody were within normal limits. The patient was diagnosed with cutaneous focal mucinosis. Because of cosmetic concerns, the patient declined surgical excision and intralesional corticosteroid injections. A 4-month trial of topical tacrolimus 0.1% ointment was ineffective.

Cutaneous lupus mucinosis (CLM) is a rare variant of lupus erythematosus eruptions. Our 5 cases with CLM were reviewed. All were men with systemic lupus erythematosus (SLE). CLM occurred as asymptomatic cutaneous papules, nodules, or plaques on the trunk, upper and lower extremities, and face. Histopathology of CLM mainly revealed abundant mucin deposits among splayed collagen bundles throughout the dermis. However, some CLM lesions showed discoid lupus erythematosus-like epidermal and dermal changes and/or lupus profundus. Vasculitis was also revealed in the CLM lesions of 2 cases. The pathogenesis of CLM may be closely related to its two important features, the male preponderance and the association with SLE. Vasculopathy may also be involved in the development of CLM.

We report a patient with the typical lesions of diabetic scleredema. Histological findings of the involved skin were thickening of the dermis, depositions of mucins, and fibrosis. Biochemical analysis revealed an increase in glycosaminoglycans in the involved skin as well as in the cutaneous lupus mucinosis. Mucinous materials were composed of hyaluronic acid.

A rare case of papular mucinosis on the back of a 78-year old man is described. The patient developed approximately 20 discrete papules on the upper back. His serum glucose concentration was borderline elevated, but no other systemic symptoms or signs were apparent. A biopsy revealed mucin deposits only in the upper reticular dermis. The papules were distinctly different from lichen myxedematosus in clinical and histochemical appearance. The present case may represent very early-stage lichen myxedematosus complicated by borderline diabetes mellitus.

We report a case of reticular erythematous mucinosis syndrome associated with psoriasis-like eruptions. Although the two skin diseases are clinically distinct, they may be related in some ways.

The patient was a 44-year-old female. She was diagnosed as having systemic lupus erythematosus at the age of 21 years and had been receiving systemic steroid treatment (5 mg prednisolone/day). Nodules began to appear on her neck, chest and back from June in 2000, and she was referred to our clinic for examination and treatment. Examination of a biopsy sample of an eruption on the neck revealed mucin deposition in the dermis. Based on the clinical and histopathological findings, she was diagnosed as having typical nodular cutaneous lupus mucinosis. The dose of steroid was increased, and the eruptions gradually disappeared.

Cutaneous mucinosis of infancy has been reported only rarely in the literature. We describe a case occurring in a black infant giri. Although no associated abnormalities have been described previously, our patient had a history of developmental delay, congenital cataracts, bilateral inguinal hernias, and an accessory tragus. The significance of these features is unclear.

Dermal mucinosis occurred in a 3-month-old and persisted for six years. The features suggest is represents a novel type of childhood mucinosis.

Cutaneous or dermal mucinoses are a heterogeneous group of connective tissue disorders in which there is an accumulation of mucin in the skin or within the hair follicle. They have a number of different morphologic presentations and can often be confused with other diseases. We report a 12-year-old Chinese girl with a 1-year history of a well-defined, hypopigmented plaque on her chin, consistent with cutaneous focal mucinosis, a rare condition in children.

Cutaneous muclnosis Is rarely associated with systemic lu-pus erythematosus. A 13-year-old girl had a two-month history of numer-ous cutaneous mucinous papules and the new onset of systemic lupus erythematosus. The differential diagnosis and pathogenesis of this vari-ant of cutaneous muclnosis are discussed. This Is the first report of a child with this unusual entity.

A case of childhood cutaneous mucinosis is described. This is a clinical condition that is rarely seen and has only recently been included in the group of primary mucinosis.