Ann Dermatol.  2016 Jun;28(3):375-380. 10.5021/ad.2016.28.3.375.

Tumoral Calcinosis as an Initial Complaint of Juvenile-Onset Amyopathic Dermatomyositis

Affiliations
  • 1Department of Dermatology, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, Korea. sycho@snu.ac.kr
  • 2Department of Laboratory Medicine, Seoul Metropolitan Government Seoul National University Boramae Medical Center, Seoul, Korea.

Abstract

Calcinosis is rarely observed in juvenile-onset amyopathic dermatomyositis in contrast to juvenile-onset dermatomyositis. A 6-year-old female presented with several 0.5 to 2 cm-sized painless grouped masses on both knees for 3 years. The patient also presented with multiple erythematous scaly patches and plaques on both elbows, knuckles, buttock, ankles and cheeks. Her mother had similar skin lesions which were erythematous scaly patches on the knuckles and elbows, since her childhood. When skin biopsy was performed from a left knee nodule, liquid chalky discharge was observed. The biopsy results were consistent with calcinosis cutis. Other biopsies from erythematous patch of the patient and erythematous patch of her mother showed vacuolization of basal cell layer with inflammatory cell infiltrations. Laboratory findings showed normal range of serum phosphorus (4.5 mg/dl), calcium (9.3 mg/dl), 1,25-dihydroxy-vitamin D (10.8 ng/ml) and parathyroid hormone levels (11 pg/ml). Both patient and her mother had no history of muscle weakness and showed normal levels of muscle-specific enzyme. Both patients were diagnosed with juvenile-onset amyopathic dermatomyositis based on histopathology and cutaneous manifestations with no evidence of muscle weakness and no serum muscle enzyme abnormalities. Tumoral calcium deposits observed in daughter was diagnosed as dystrophic calcinosis which can be rarely seen in juvenile-onset amyopathic dermatomyositis. The patient is being treated with oral acetazolamide (40 mg/kg/d) for calcinosis.

Keyword

Adolescent; Amyopathic dermatomyositis; Calcinosis

MeSH Terms

Acetazolamide
Adolescent
Ankle
Biopsy
Buttocks
Calcinosis*
Calcium
Cheek
Child
Dermatomyositis*
Elbow
Female
Humans
Knee
Mothers
Muscle Weakness
Nuclear Family
Parathyroid Hormone
Phosphorus
Reference Values
Skin
Acetazolamide
Calcium
Parathyroid Hormone
Phosphorus

Figure

  • Fig. 1 (A) Skin manifestations of the patient. Subcutaneous nodules on both knees (black arrows, upper panels) and multiple erythematous scaly patches and plaques on bilateral knuckles, elbows, ankles and cheeks (middle and lower panels). (B) Skin manifestations of the mother. Erythematous scaly patches on the knuckles (left panel) and elbows (right panel).

  • Fig. 2 (A) A skin biopsy obtained from a left knee nodule of patient. Mainly calcified material which is consistent with calcinosis cutis (left panel, ×40) and bursa-like structure lined by calcifying front is observed (right upper panel, ×100). Sand-like and plate-like calcifications in and bordering around disintegrating lining cells (right lower panel, ×400). (B) Skin biopsy from right elbow of the patient. Parakeratosis and hyperkeratosis with intracorneal microabscess (left panel, ×200). Focal hydropic degeneration of basal keratinocytes and lichenoid inflammatory cell infiltration (right panel, ×200). (C) Skin biopsy from erythematous patch on left knee of the mother. Mild basal vacuolar change and papillomatosis (left panel, ×100). Perivascular lymphohistiocytic cell infiltration (right panel, ×100). A~C: H&E.

  • Fig. 3 (A) Multlobulated and multinodular 'popcorn-like' structures (white arrows) which are extensive soft tissue calcification are shown in plain radiography of both knee joints. (B) Axial T2-weighted magnetic resonance imaging of left leg image shows fluid collections with hypointense signal at the mainly anterior aspect of knee joint (white arrows). Diffuse edematous soft tissue swelling at anterolateral prepatellar fat pad is shown due to cellulitis.


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