Extensive scleredema adultorum with loss of eccrine glands
Authors: I-Chun Lin, Hsien-Yi Chiu, Jung-Yi Chan, Sung-Jan Lin
Journal: Journal of the American Academy of Dermatology (2014)

To the Editor: Scleredema adultorum is a disease characterized by woody induration of the skin,1Boin F. Hummers L.K. Scleroderma-like fibrosing disorders.Rheum Dis Clin North Am. 2008; 34: 199-220Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar in which the skin appendages are usually preserved. Here, we report a case of scleredema adultorum with loss of eccrine glands leading to frequent heat strokes.A 50-year-old Taiwanese woman visited us for hardened and thickened skin extending from the nape to the lower aspect of her back over the past 5 years. Her back became anhidrotic and the front of her trunk seemed hyperhidrotic. In the meantime, she became progressively intolerant to heat and experienced several episodes of heat strokes. She refrained from outdoor activities to avoid heat strokes. She had a 20-year history of type 2 diabetes mellitus under insulin treatment over the past 10 years. She denied previous radiotherapy, topical medications, toxin exposure, or family history of similar skin changes. Neither photosensitivity nor arthritis was present.Examination revealed the skin from her nape to the lower aspect of her back was hardened and thickened, except the side aspects. The overlying skin was slightly erythematous with preservation of the hair follicles. Starch-iodine test showed the lesional skin was anhidrotic (Fig 1). Her blood tests were negative for antinuclear antibody, anti-Scl-70 antibody, and rheumatoid factor and pulmonary function test result was within normal limits. Fasting glucose level was 234 mg/dL and hemoglobin A1c (HbA1c) was 9.6%.The skin biopsy specimen showed markedly thickened dermis with increased collagen deposition, increased mucin, and decreased elastic fibers (Fig 2). Direct immunofluorescence was negative for IgG, IgA, IgM, complement component 3 (C3), fibrinogen, and complement component 1, q subcomponent (C1q). Although scleromyxedema cannot be ruled out by pathology findings, clinical presentations and history above favored scleredema adultorum associated with long-term diabetes. Eccrine glands were absent in serial sections (Fig 2), which explained her anhidrosis clinically. Neither squamatization nor perieccrine inflammation was noted. She was treated with oral allopurinol (100 mg/d).2Lee F.Y. Chiu H.Y. Chiu H.C. Treatment of acquired reactive perforating collagenosis with allopurinol incidentally improves scleredema diabeticorum.J Am Acad Dermatol. 2011; 65: e115-e117Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar After 8-month follow-up, the erythema and induration were slightly lessened, but the anhidrosis persisted.Fig 2Scleredema adultorum histopathology. A, Dermal sclerosis with loss of eccrine glands and slight focal lymphocytic infiltrate around hair follicles; B and C, increased mucin deposition and decreased elastic fibers (inset). (Hematoxylin-eosin stain; original magnification: ×25. Inset, orcein [B] and alcian blue [C] stains; original magnification: ×40.)View Large Image Figure ViewerDownload Hi-res image Download (PPT)Scleredema adultorum is divided into 3 distinct types that are associated with preceding infection, paraproteinemia, and diabetes mellitus, respectively.1Boin F. Hummers L.K. Scleroderma-like fibrosing disorders.Rheum Dis Clin North Am. 2008; 34: 199-220Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar In cases associated with infection, the disease onset is more abrupt and the skin hardening can usually regress. However, scleredema in diabetic patients, like our case, progresses slowly and the condition persists.The association with loss of eccrine glands is a distinct finding in our patient. Several diseases can lead to hardened skin with loss of skin appendages, including systemic sclerosis, lupus erythematosus, radiotherapy, and chronic graft-versus-host disease.3Akosa A.B. Lampert I.A. The sweat gland in graft versus host disease.J Pathol. 1990; 161: 261-266Crossref PubMed Scopus (31) Google Scholar, 4Nakamura M. Tokura Y. Expression of SNAI1 and TWIST1 in the eccrine glands of patients with systemic sclerosis: possible involvement of epithelial-mesenchymal transition in the pathogenesis.Br J Dermatol. 2011; 164: 204-205Crossref PubMed Scopus (21) Google Scholar, 5Akosa A.B. Lampert I.A. Sweat gland abnormalities in lichenoid dermatosis.Histopathology. 1991; 19: 345-349Crossref PubMed Scopus (19) Google Scholar One hypothesis is that cells in the eccrine glands are converted to myofibroblasts through epithelial-mesenchymal transition and promote fibrosis.4Nakamura M. Tokura Y. Expression of SNAI1 and TWIST1 in the eccrine glands of patients with systemic sclerosis: possible involvement of epithelial-mesenchymal transition in the pathogenesis.Br J Dermatol. 2011; 164: 204-205Crossref PubMed Scopus (21) Google Scholar There were no signs of autoimmune diseases in this patient, such as Raynaud phenomenon, dysphagia, myopathy, or difficulty breathing, nor did the clinical presentation support such diagnoses.In reviewing 22 cases of scleredema adultorum at our hospital from 2000 to 2012, we found no similar changes of eccrine loss pathologically. How the eccrine glands were lost in this case is unknown. We noted a slight mononuclear infiltrate around the preserved hair follicles histologically. The complete loss of eccrine glands hinders further determination of whether eccrine glands were destroyed by preceding inflammatory infiltrate. To the Editor: Scleredema adultorum is a disease characterized by woody induration of the skin,1Boin F. Hummers L.K. Scleroderma-like fibrosing disorders.Rheum Dis Clin North Am. 2008; 34: 199-220Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar in which the skin appendages are usually preserved. Here, we report a case of scleredema adultorum with loss of eccrine glands leading to frequent heat strokes. A 50-year-old Taiwanese woman visited us for hardened and thickened skin extending from the nape to the lower aspect of her back over the past 5 years. Her back became anhidrotic and the front of her trunk seemed hyperhidrotic. In the meantime, she became progressively intolerant to heat and experienced several episodes of heat strokes. She refrained from outdoor activities to avoid heat strokes. She had a 20-year history of type 2 diabetes mellitus under insulin treatment over the past 10 years. She denied previous radiotherapy, topical medications, toxin exposure, or family history of similar skin changes. Neither photosensitivity nor arthritis was present. Examination revealed the skin from her nape to the lower aspect of her back was hardened and thickened, except the side aspects. The overlying skin was slightly erythematous with preservation of the hair follicles. Starch-iodine test showed the lesional skin was anhidrotic (Fig 1). Her blood tests were negative for antinuclear antibody, anti-Scl-70 antibody, and rheumatoid factor and pulmonary function test result was within normal limits. Fasting glucose level was 234 mg/dL and hemoglobin A1c (HbA1c) was 9.6%. The skin biopsy specimen showed markedly thickened dermis with increased collagen deposition, increased mucin, and decreased elastic fibers (Fig 2). Direct immunofluorescence was negative for IgG, IgA, IgM, complement component 3 (C3), fibrinogen, and complement component 1, q subcomponent (C1q). Although scleromyxedema cannot be ruled out by pathology findings, clinical presentations and history above favored scleredema adultorum associated with long-term diabetes. Eccrine glands were absent in serial sections (Fig 2), which explained her anhidrosis clinically. Neither squamatization nor perieccrine inflammation was noted. She was treated with oral allopurinol (100 mg/d).2Lee F.Y. Chiu H.Y. Chiu H.C. Treatment of acquired reactive perforating collagenosis with allopurinol incidentally improves scleredema diabeticorum.J Am Acad Dermatol. 2011; 65: e115-e117Abstract Full Text Full Text PDF PubMed Scopus (15) Google Scholar After 8-month follow-up, the erythema and induration were slightly lessened, but the anhidrosis persisted. Scleredema adultorum is divided into 3 distinct types that are associated with preceding infection, paraproteinemia, and diabetes mellitus, respectively.1Boin F. Hummers L.K. Scleroderma-like fibrosing disorders.Rheum Dis Clin North Am. 2008; 34: 199-220Abstract Full Text Full Text PDF PubMed Scopus (96) Google Scholar In cases associated with infection, the disease onset is more abrupt and the skin hardening can usually regress. However, scleredema in diabetic patients, like our case, progresses slowly and the condition persists. The association with loss of eccrine glands is a distinct finding in our patient. Several diseases can lead to hardened skin with loss of skin appendages, including systemic sclerosis, lupus erythematosus, radiotherapy, and chronic graft-versus-host disease.3Akosa A.B. Lampert I.A. The sweat gland in graft versus host disease.J Pathol. 1990; 161: 261-266Crossref PubMed Scopus (31) Google Scholar, 4Nakamura M. Tokura Y. Expression of SNAI1 and TWIST1 in the eccrine glands of patients with systemic sclerosis: possible involvement of epithelial-mesenchymal transition in the pathogenesis.Br J Dermatol. 2011; 164: 204-205Crossref PubMed Scopus (21) Google Scholar, 5Akosa A.B. Lampert I.A. Sweat gland abnormalities in lichenoid dermatosis.Histopathology. 1991; 19: 345-349Crossref PubMed Scopus (19) Google Scholar One hypothesis is that cells in the eccrine glands are converted to myofibroblasts through epithelial-mesenchymal transition and promote fibrosis.4Nakamura M. Tokura Y. Expression of SNAI1 and TWIST1 in the eccrine glands of patients with systemic sclerosis: possible involvement of epithelial-mesenchymal transition in the pathogenesis.Br J Dermatol. 2011; 164: 204-205Crossref PubMed Scopus (21) Google Scholar There were no signs of autoimmune diseases in this patient, such as Raynaud phenomenon, dysphagia, myopathy, or difficulty breathing, nor did the clinical presentation support such diagnoses. In reviewing 22 cases of scleredema adultorum at our hospital from 2000 to 2012, we found no similar changes of eccrine loss pathologically. How the eccrine glands were lost in this case is unknown. We noted a slight mononuclear infiltrate around the preserved hair follicles histologically. The complete loss of eccrine glands hinders further determination of whether eccrine glands were destroyed by preceding inflammatory infiltrate.