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Wong‐type dermatomyositis with pulmonary involvement in a teenager: case report

A healthy 16-year-old female presented and complained about eight months of progressive thickening of her palms and soles as well as scaly patches on her hands, elbows and knees. Physical examination revealed palmoplantar keratoderma (Figure 1a), Gottron-like papules on the dorsum of both hands (Figure 1b), and discrete erythematous papules with rough hyperkeratotic surfaces on the patient’s elbows (Figure 1c) and knees (Figure 1d). Skin biopsies taken from the palms and buttocks revealed an altered epidermis, with orthokeratosis alternating with parakeratosis in horizontal and vertical directions in a checkerboard pattern (Figure 2a) and compatible with pityriasis rubra pilaris (PRP). A few necrotic keratinocytes were also identified (Figure 2b). There was a superficial perivascular inflammatory infiltrate in the dermis, with vacuolar changes in the basal epidermis. No dermal mucin was observed. We diagnosed PRP and requested laboratory investigations in order to start systemic retinoids. However, the patient returned a few days later (before the programmed check-up) due to extreme fatigue and dyspnea. During the physical examination, the patient was acutely distressed and unable to stand without support. M4 tetraparesis and evident respiratory difficulty were noted. We therefore decided to hospitalize her. Laboratory evaluation revealed a normal complete blood count with differential (but elevated) total CK (1333 IU/L, normal: 0–167), LDH (800 IU/L, normal: 135–214) and antinuclear antibodies (ANA, 1 : 1280, in nucleolar pattern). Anti-dsDNA, anti-Ro, anti-La, anti-Jo-1, anti-RNP, anti-Scl-70 and anti-Sm antibodies were negative. The CT chest scan showed subpleural reticulation associated with patchy ground-glass opacities in both lung bases, suggesting a diffuse interstitial lung disease (ILD). Based on these findings, dermatomyositis (DM) with associated ILD was suspected, so we started prednisone (1 mg/kg/day) and requested supplementary examinations. DOI: 10.1111/ddg.14068 Wong-type dermatomyositis with pulmonary involvement in a teenager: case report Clinical Letter