Scleredema Clinical Presentation

Updated: Mar 15, 2019
  • Author: Lisa K Pappas-Taffer, MD; Chief Editor: Dirk M Elston, MD  more...
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Presentation

History

Patients report stiff or hard skin. The rapidity of onset and locations of involvement differ based on the clinical subgroup. A thorough history regarding preceding illnesses, history of diabetes, and a review of systems should be performed to help identify less commonly associated extracutaneous manifestations (lungs, heart, trouble eating or talking, or muscle weakness) or, rarely, reported associations with malignancies.

Scleredema can be categorized into three clinical subgroups. Each has a different history, course, and prognosis. Note the following:

Type 1, postinfectious

This subgroup was historically referred to as scleredema adultorum. However, this is considered by some to be a misnomer because most pediatric patients fall into this group. Patients report a hardening of the skin a few weeks after a febrile illness, most commonly an upper or lower respiratory tract streptococcal infection. [25] The skin hardening progresses rapidly, first involving the face and neck, then spreading distally to involve the trunk and proximal upper limbs in a symmetric manner. Hands and feet are typically spared. Complications may include difficulty in smiling, opening the mouth, limited range of motion, and, in severe cases, involvement of the pharynx or tongue can lead to dysphagia or dysphonia. The condition usually clears spontaneously in 6 months to 2 years. The duration is not affected by the use of antibiotics.

Type 2, associated plasma dyscrasia

This subgroup includes patients whose disease tends to occur insidiously, progressing slowly over many years, with no history of preceding illness. Rimon et al identified 52 cases of type 2 scleredema in the world literature from 1963 to 1986, of which 25% had plasma cell dyscrasias, including 3 with multiple myeloma and 10 with monoclonal gammopathy of unknown significance. They also noted that the gammopathies were diagnosed on average 10 years after the onset of scleredema skin changes. [13] IgG monoclonal gammopathy is most common, followed by an IgA type. Spontaneous remission is much less likely to occur than in the type 1 subgroup.

Type 3, associated diabetes mellitus (scleredema diabeticorum)

This subtype of scleredema tends to occur more often in middle-aged males (at a reported 10:1 ratio), often obese, with longstanding, often uncontrolled, diabetes mellitus. In a 2015 multicenter study, 30 (68%) of 44 patients with scleredema had poorly controlled diabetes, with a type 2‒to‒type 1 ratio of 6.5:1. [26] Subtle skin hardening of the upper back begins in an insidious manner, progressing slowly over many years, to involve the upper back, neck, and shoulders with associated erythema; often, a pebbled appearance may evolve. Patients typically experience a more protracted course that is refractory to therapy. Control of the hyperglycemia does not improve the scleredema. [27]

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Physical Examination

Scleredema presents as ill-defined, woody, nonpitting, indurated plaques. Generalized cases may clinically mimic edema. Erythema, hyperpigmentation, and/or a peau-d'-orange appearance of the affected areas may be present. The taught skin may appear shiny, and, when pinched, firmness is appreciated with noted wrinkling of overlying epidermis.

Scleredema is usually most evident in the upper part of the body, specifically the face, the neck, the trunk, and the extremities in type 1 and 2 subgroups. However, a case of scleredema confined to the thighs [28] and a case of scleredema confined to the periocular region [29] have been reported. Hands and feet are typically spared in scleredema, in contrast to systemic sclerosis, which has sclerodactyly. Scleredema patients with extensive facial involvement may appear expressionless and may have difficulty in opening their mouths. They may have difficulty with tongue protrusion.

Physical examination should include evaluation of range of motion of the neck, upper extremities, and tongue. Auscultation of the heart and lungs should also be performed.

Note the images below:

Middle-aged man who has diabetes with scleredema o Middle-aged man who has diabetes with scleredema on the upper part of the back.
Middle-aged man who has diabetes with scleredema o Middle-aged man who has diabetes with scleredema on the upper part of the back.
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Complications

Complications include the following:

  • Limited range of motion
  • Poor wound healing
  • Recurrent skin infections
  • Restrictive lung disease
  • Dysarthria
  • Dysphagia
  • Difficulty in closing the eyes
  • Death (rare)
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