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Metabolic disorders and reticulohistiocytic proliferative disorders
Published in Rashmi Sarkar, Anupam Das, Sumit Sethi, Concise Dermatology, 2021
Cutaneous mucinoses are a class of disorders characterized by the deposition of mucin in the skin. They are primarily divided into two groups: (i) primary cutaneous mucinoses, in which mucin deposition is the main histopathological feature; and (ii) secondary mucinoses, in which mucin deposition is secondary only an additional finding. A classification of cutaneous mucinoses is given in Table 16.4. Lichen myxoedematosus/scleromyxedema is a prototype of idiopathic cutaneous mucinoses and is discussed next.
Other infiltrative conditions
Published in Aimilios Lallas, Enzo Errichetti, Dimitrios Ioannides, Dermoscopy in General Dermatology, 2018
Enzo Errichetti, Aimilios Lallas
With regard to dermal mucinoses, all the disorders included in the group of lichen myxedematosus (papular mucinosis) as well as connective tissue disease–associated mucinosis present with asymptomatic, dome-shaped, or flat-topped whitish/pinkish papules, nodules, and/or plaques, which may be diffuse (scleromyxedema) or more or less localized (connective tissue diseases-associated mucinosis, acral persistent papular mucinosis, discrete papular lichen myxedematosus, cutaneous papular mucinosis of infancy, and nodular lichen myxedematosus) (Figures 11.23A–11.25A).42 Of note, diffuse infiltration of the glabella (leonine face) and skin overlying the dorsal aspect of proximal interphalangeal joints (“doughnut” sign) (Figure 11.26A), linear arrangement of the papules (Figure 11.23A), and sclerodermoid changes are also commonly seen in scleromyxedema.42
Nail changes in systemic diseases and drug reactions
Published in Eckart Haneke, Histopathology of the NailOnychopathology, 2017
The histopathologic changes in scleromyxedema and lichen myxoedematosus are identical. The epidermis is usually normal or may become slightly flattened. There is a diffuse mucin deposition in the papillary and less pronounced in the reticular dermis with an increase in the number of fibroblasts and fibrosis. Inflammatory infiltrates are not common.207
Progressive mucinous histiocytosis treated successfully with thalidomide: a rare case report
Published in Journal of Dermatological Treatment, 2023
Reem Diab, Mohammad Shahidi Dadras, Azadeh Rakhshan, Ali Kaddah, Fahimeh Abdollahimajd
A 31-year-old female patient presented with multiple erythematous papular lesions on the face, appearing nine months beforehand (Figure 1). There were no associated symptoms except for very mild pruritus. The patient had no systemic manifestations or mucosal involvement. During the previous few months, she was unsuccessfully treated with topical and intra-lesional corticosteroids. Two biopsy samples were taken from her face and showed similar results, with differential diagnoses for histiocytosis, papular mucinosis, pseudolymphoma, and lichen myxedematosus. Histological examination revealed an infiltrate involving the full thickness of the dermis composed of epithelioid and spindle-shaped histiocytes arranged in a collagenous stroma with abundant intervening mucin deposition with a vague storiform pattern. There was no grenz zone between the infiltrate and follicular epithelium. Perivascular infiltration of lymphocytes, plasma cells, and mast cells was also noted (Figure 2(A)). The histiocytes were negative for S100 and CD1a. CD68 was positive in at least some of the histiocytes. CD31 stained the histiocytes as well as vascular endothelial cells, and CD34 was positive in the spindle-shaped histiocytes (Figure 2(B/C/D)). The findings were consistent with progressive mucinous histiocytosis, a non-Langerhans cell histiocytosis (Figure 3).
Advances in understanding and treatment of scleromyxedema
Published in Expert Opinion on Orphan Drugs, 2018
Laura Atzori, Caterina Ferreli, Franco Rongioletti
The characteristic pathological triad is composed of dermal mucin accumulation, increased collagen deposition, and fibroblast proliferation (Figure 6) [3]. Mucin in scleromyxedema is made mainly of hyaluronic acid and accumulates in the upper and mid-reticular dermis. The material is alcian blue-positive at pH 2.5 and digested by hyaluronidase, Congo red, and periodic-acid Schiff stains negative [23]. Dermal fibroblasts are numerous, stellate or spindle shaped, associated with thickened collagen bundles, irregularly arranged, while the elastic fibers are usually fragmented and decreased in number, explaining the loose redundant folds of the back skin referred as the Shar-pei sign [1] The above epidermis is normal or thinned by the underlying mucin and fibrosis, with atrophic hair follicles. Slight epidermal hyperkeratosis with acanthosis is present in a minority of cases, as well as other additional microscopic findings, including a mild superficial perivascular lymphoplasmacytic infiltrate, proliferation of eccrine sweat glands, and dermal eosinophilia [1]. Differential diagnosis (Table 2) includes the localized variants of lichen myxedematosus such as acral persistent popular mucinosis with a completely different prognosis and treatment approach [5]. The clinico-pathological correlation is the key to distinguish the other two major sclerodermoid disorders: scleredema adultorum of Buschke, typical of diabetic patients, whose interstitial mucin deposition is located between fenestrated collagen bundles in up to three times thickened reticular dermis; and the nephrogenic systemic fibrosis, which develops in a person with severe renal insufficiency exposed to gadolinium [2]. The latter also shows variable amounts of mucin, but differs for an extensive involvement of the subcutis, with thick elastic fibers oriented parallel to the collagen bundles, dystrophic calcification, and sclerotic bodies, also known as elasto-collagenous balls.