A case of senear-usher syndrome.

Sir,

A 32-year-old female, presented with hyperpigmented papules of three months duration that commenced as erythematous papules over cheeks, ears, scalp, upper back, “V” of the chest and extensors of forearms and dorsae of hands. Lesions were associated with burning on sun exposure. Painful oral erosions interfered with food intake. Arthralgia of interphalangeal joints, knees and ankles with morning stiffness accompanied the skin changes.

Cutaneous examination revealed crusted scaly plaques with surrounding erythema over malar area [Figure 1a], ears, chest, back and sun exposed areas of forearms and arms. Oral cavity showed erosions over upper gingiva [Figure 1b]. Hands showed proximal nail fold erythema [Figure 2a].

Figure 1a

Crusted scaly plaques in a butterfly distribution over cheeks, ears and upper chest

Figure 1b

Erosion over gingival mucosa

Figure 2a

Proximal nail fold erythema

Value of serum ANA was 2.87 IU, ESR was 40 mm, Hb was 9.3 gm% and RA factor was positive. Chest X-ray, ECG, urinalysis and anti-ds DNA were normal. Skin biopsy of lesional skin from right forearm showed hyperkeratosis, subcorneal acantholysis [Figure 3a], basal cell vacuolation, dermoepidermal separation and superficial perivascular lymphocytic infiltrate [Figure 3b]. Direct IF of perilesional skin from right forearm revealed focal deposits of IgG [Figure 4a] in the intercellular space of the epidermis and granular deposits of IgG and IgM at dermoepidermal junction [Figure 4b].

Figure 3a

Photomicrograph showing subcorneal bulla with acantholysis (H and E, ×100)

Figure 3b

Basal cell vacuolation, dermoepidermal separation (arrow), perivascular lymphocytic infiltrate and melanin incontinence (arrowhead) (H and E, ×400)

Figure 4a

Focal IgG deposits in the intercellular space of superficial epidermis (arrow) and granular IgG at dermoepidermal junction (arrowhead) (IF ×400)

Figure 4b

Granular IgM deposits at DEJ (IF ×400)

Dexamethasone-cyclophosphamide pulse therapy was administered and since five pulses, the patient has been in remission [Figure 2b].

Figure 2b

Patient remission following DCP

Senear-Usher syndrome is a disorder which results from combination of lupus erythematosus and pemphigus foliaceus.[1] Mucous membrane involvement is uncommon.[2]

The relationship of this syndrome with lupus erythematosus and its place in pemphigus spectrum has long been debated. Some authors think it to be an abortive or localized form, or an early stage of pemphigus foliaceus, others argue that it may progress to pemphigus vulgaris and pemphigus foliaceus.[1] Jablonskaetal.[3] in their direct immunofluorescence (IF) study of fifty four patients found staining of the intercellular epidermal spaces. DEJ band was seen in exposed and unexposed skin in 81% and 23% of their patients respectively. ANA were found in 31% of patients. Detection of IF band in skin specimens from a significant majority of patients with pemphigus erythematosus, presence of ANA in some, and occasional coexistence of SLE suggest some relation of the disease with lupus erythematosus. Bean and Lynch[3] reported a case with widespread lesions which showed direct and indirect IF findings of superficial pemphigus along with IgG and complement at DEJ. Our case not only had features suggestive of pemphigus foliaceus (subcorneal acantholysis and focal deposits of IgG in the intercellular space of superficial epidermis) but also of lupus erythematosus (vacuolar degeneration of basal keratinocytes and granular deposits of IgG and IgM at DEJ).